Behcet Tratamento

14/06/2023, 23:53 Treatment of Behçet syndrome - UpToDate
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Treatment of Behçet syndrome

AUTHORS: Ellison L Smith, MD, Yusuf Yazici, MD
SECTION EDITOR: Peter A Merkel, MD, MPH
DEPUTY EDITOR: Philip Seo, MD, MHS
All topics are updated as new evidence becomes available and our peer review process is complete.
Literature review current through: May 2023.

This topic last updated: Apr 25, 2023.
INTRODUCTION
Behçet syndrome, also known as Behçet disease, is an inflammatory disease characterized by recurrent oral aphthous
ulcers and numerous potential systemic manifestations. These include genital ulcers, ocular disease, skin lesions,
arthritis, and vascular, gastrointestinal, and neurologic disease.
Most clinical manifestations of Behçet syndrome are believed to be due to vasculitis. Among the systemic vasculitides,
Behçet syndrome is remarkable for its ability to involve blood vessels of all sizes – small, medium, and large – on both
the arterial and venous sides of the circulation, with a predilection for the venous side.
The treatment of Behçet syndrome will be reviewed here. The pathogenesis, clinical manifestations, and diagnosis are
discussed separately. (See "Pathogenesis of Behçet syndrome" and "Clinical manifestations and diagnosis of Behçet
syndrome".)
GENERAL PRINCIPLES OF MANAGEMENT
Our treatment guidelines are generally consistent with those developed by the European Alliance of Associations for
Rheumatology (EULAR; formerly known as European League Against Rheumatism), and this includes several
overarching principles listed below [1-5]:
● The syndrome typically runs a relapsing and remitting course, and the goal of treatment is to promptly suppress
inflammatory exacerbations and recurrences to prevent irreversible organ damage
● A coordinated multidisciplinary approach is necessary for optimal care
● Treatment choices should be individualized based on age, sex, type, and severity of organ involvement as well as
patient preferences
● Ocular, vascular, neurologic, and gastrointestinal involvement may be associated with a poor prognosis
● Disease manifestations may improve over time in many patients
Given the clinical heterogeneity of Behçet syndrome, the therapeutic approach is highly variable and is guided by the
predominant disease manifestation.
ORGAN-BASED TREATMENT
Overall approach — Due to the clinical heterogeneity of Behçet syndrome and the unpredictable disease course, the
management is tailored to the individual patient, taking into account the severity of symptoms and organ
involvement along with the sex and age of the patient, as young males tend to have the more severe and complicated
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course compared with females and older patients, in general. The choice of therapy in active disease is dictated by the
predominant disease manifestation.
The following discussion of treatment is organized by the disease manifestations, which include:
● Mucocutaneous manifestations
● Arthritis
● Ocular disease
● Gastrointestinal disease
● Renal disease
● Vascular disease
● Neurologic disease
Mucocutaneous manifestations
Oral aphthae and genital ulcers — The treatment of oral and genital ulcers is guided by the severity of symptoms
and the presence of other disease manifestations. The following is our general approach to drug therapy in patients
with oral and/or genital ulcers:
● For isolated oral and genital aphthae, we suggest initial treatment with topical corticosteroids ( table 1) such
as triamcinolone acetonide cream (0.1% in Orabase). This should be applied three to four times daily and be
used until pain from the ulcer ceases. Potent topical corticosteroids may also be used for genital ulcers. The
efficacy of topical corticosteroids for the treatment of aphthous ulcers in Behçet syndrome was evaluated in a
small study including 60 patients who were given either phenytoin syrup or triamcinolone acetonide ointment
[6]. Improvements in the triamcinolone acetonide and phenytoin groups were 87 percent and 53 percent,
respectively. Intralesional corticosteroid preparations for large ulcers can be employed in collaboration with a
dermatologist. A typical preparation is triamcinolone (5 to 10 mg/mL). Our approach to the use of topical
therapies is generally consistent with that used for recurrent aphthous stomatitis and is discussed in detail
separately. (See "Recurrent aphthous stomatitis", section on 'Topical corticosteroids'.)
Topical sucralfate can also be used in combination with or as an alternative to topical corticosteroids. Topical
sucralfate 1g/5mL four times daily as a mouthwash reduced pain, frequency, and healing time in a randomized
controlled trial for oral ulcers in Behçet syndrome [7]. In some countries, including the United States, the
commercial sucralfate suspension strength is 1 g/10 mL. Pimecrolimus improved healing time in a randomized
trial of 90 patients with genital aphthous ulcers [8]. Although generally less effective, topical anesthetics can also
provide temporary relief of discomfort if used prior to eating and performing dental hygiene. (See "Recurrent
aphthous stomatitis", section on 'General measures'.)
● For prevention of recurrent oral and genital ulcers, we suggest colchicine 1 to 2 mg/day in divided doses (titrated
to a dose without gastrointestinal side effects) rather than oral glucocorticoids. In places where 0.5 mg tablets
are not available (eg, United States), doses in the range of 1.2 to 1.8 mg can be used. Apremilast, an orally
administered phosphodiesterase-4 inhibitor, has also been shown to be effective for prevention of recurrent oral
ulcers, and is a reasonable alternative to colchicine as a glucocorticoid-sparing agent for patients with recurrent
oral ulcers. Apremilast is typically up-titrated at a rate of 10 mg daily over six days to achieve a maintenance
dose of 30 mg twice daily. We wait approximately 12 weeks to confirm an adequate response to either
medication. There are no trials that have directly compared colchicine with apremilast, and both have been
shown to help improve oral ulcers. However, the authors generally prefer a trial of colchicine first given the rapid
onset of action, lower cost, and better tolerability.
Data on the effects of colchicine for patients with minor oral ulcers or genital lesions are mixed, and generally
more favorable for genital ulcers [9-13]. In a randomized trial of 116 patients comparing colchicine with placebo,
colchicine therapy was associated with a reduction in the number of genital ulcers and erythema nodosum in
women [9]. A randomized crossover trial in 169 patients showed improvement in overall disease activity as well
as oral aphthosis, genital aphthosis, pseudofolliculitis and erythema nodosum in patients treated with colchicine
versus placebo [13]. Patients may develop significant gastrointestinal intolerance of colchicine if the medication
is taken at total doses higher than 1.5 mg/day. Colchicine has a narrow therapeutic window and doses should be
adjusted if adverse symptoms develop, as cytopenias and other complications may develop. Dose adjustment of
colchicine is necessary in patients with renal impairment. Dose adjustments are found in the Lexicomp drug
patient information topic within UpToDate. Colchicine is also used for patients with arthritis associated with
Behçet syndrome. (See 'Arthritis' below.)
Data from randomized trials suggest that apremilast is beneficial in treating oral ulcers, and it is approved in the
United States, Canada, and Japan for the treatment of oral ulcers in Behçet syndrome [14-17]. A randomized
phase II trial including 111 patients with Behçet syndrome found that patients who received apremilast had a
lower mean number of oral ulcers at 12 weeks, as compared with those in the placebo group (0.5 versus 2.1)
[14]. A phase III trial with 207 patients also reported an improvement in the mean number of oral ulcers after 12
weeks, with continuation of this benefit out to 64 weeks in 143 patients that completed week 64 [15,16]. Other
outcomes that favored the apremilast group included a decrease in pain of oral ulcers as well as the proportion
of complete responders for oral ulcer resolution (52.9 versus 22.3 percent), and improvement in quality of life. In
both trials, some patients had genital ulcers, and there was a trend towards improvement, but the studies were
not designed to assess this outcome. More data are needed to determine whether apremilast is effective for
genital ulcers as well as other manifestations of Behçet syndrome. Apremilast has been associated with more
adverse events than placebo, including diarrhea, nausea, and headache.
● When isolated oral aphthae or genital ulcers are refractory to topical corticosteroids, colchicine, or apremilast or
when multiple lesions are present, systemic glucocorticoids should be employed. An appropriate starting dose
for mucocutaneous disease in the absence of other disease manifestations is prednisone 15 mg/day (or the
equivalent with other glucocorticoids), with tapering of the dose to 10 mg/day after one week and
discontinuation of prednisone entirely over a two- to three-week period, assuming that the aphthae are no
longer symptomatic. Some patients may require higher initial doses. Patients with recurrent oral aphthae may
require longer periods of maintenance treatment with low-dose prednisone (eg, 5 mg/day).
General clinical experience with Behçet syndrome and extrapolation from the treatment of recurrent aphthous
stomatitis suggest that systemic glucocorticoids are effective for oral aphthae and genital ulcers resistant to
topical therapy [18,19].
● Escalation of treatment with other medications for the management of oral and genital ulcers should be
determined on a case-by-case basis. We generally reserve these options for patients in whom the combination of
daily oral colchicine or apremilast and a daily prednisone dose of more than 10 mg is required to control oral
aphthae and genital lesions or if the adverse effects of prednisone and/or colchicine are intolerable even at
lower doses. The choice of therapeutic options listed below and can also depend on the presence of other organ
involvement. Adverse effects of individual therapeutic agents and patient preferences must also be taken into
consideration. Options in order of preference include:
• Azathioprine – Azathioprine has been shown to improve oral and genital ulceration. In a randomized trial, 73
patients with Behçet syndrome were treated with either azathioprine (2.5 mg/kg per day) or placebo, with
glucocorticoids available to both groups [20]. After two years, patients taking azathioprine had less frequent
oral and genital ulcers. We generally start with azathioprine 50 mg daily, and then usually increase the daily
dose by 50 mg every 4 weeks as tolerated up to the target dose of 2.5 mg/kg/day, while monitoring a
complete blood count (CBC) every 2 weeks until therapy has stabilized, and every 6 to 12 weeks thereafter. A
lower dose is indicated in patients with renal insufficiency. Before the initiation of azathioprine, we perform
genetic testing for mutations in the gene for thiopurine methyltransferase. (See "Pharmacology and side
effects of azathioprine when used in rheumatic diseases", section on 'Pharmacogenetics and azathioprine
toxicity'.)
• TNF-alpha inhibitors – Tumor necrosis factor (TNF)-alpha inhibitors (infliximab, adalimumab, or etanercept)
have been beneficial for mucocutaneous disease in several case reports/series and prospective studies
[21,22], as well as one randomized trial with etanercept [23]. They should be used in combination with an oral
disease-modifying antirheumatic drug (DMARD) such as azathioprine to help prevent development of
potentially neutralizing antibodies.
The efficacy of TNF-alpha inhibitors for mucocutaneous disease was demonstrated in a retrospective
multicenter study of 124 patients with severe or refractory Behçet syndrome treated with infliximab (in 62
percent) or adalimumab (in 30 percent) [22]. In addition to an overall clinical response in 90 percent of
patients, a clinical improvement was seen in 88 percent of patients with mucocutaneous disease. A
randomized trial in which 40 patients with mucocutaneous disease and/or arthritis were assigned to
etanercept (25 mg subcutaneously twice a week) or placebo injections for four weeks found that more
patients on etanercept remained free of oral ulcers (45 percent versus 5 percent) and nodular skin lesions (85
versus 25 percent) [23]. However, there was no difference between the two groups in terms of genital ulcers,
likely due to the small size and relative infrequency of genital ulcers during the short study period.
• Cyclosporine – Although typically used for ocular manifestations, there is some evidence supporting the
efficacy of cyclosporine for mucocutaneous disease. In a randomized trial with 96 patients with
mucocutaneous manifestations, cyclosporine (10 mg/kg per day in divided doses) was superior to colchicine
in the management of oral and genital ulcers as well as other skin lesions [24]. However, adverse effects due
to cyclosporine were common and were reversible when the cyclosporine dose was reduced [25].
Hypertension is common with cyclosporine treatment. Neurotoxicity is also frequently seen and may be
difficult to differentiate from disease-related neurologic disease; thus, this agent is less preferred in patients
with neurologic disease [26]. (See "Pharmacology of cyclosporine and tacrolimus" and 'Neurologic disease'
below.)
• Interferon alfa – Interferon alfa-2a and interferon alfa-2b (generally given three to six million units three
times weekly) have been beneficial for mucocutaneous disease. This was shown in a randomized trial of 50
patients in which interferon alfa-2a significantly decreased the duration and pain of oral ulcers as well as the
frequency of genital ulcers and papulopustular lesions [27]. The mean frequency and duration of erythema
nodosum-like lesions, thrombophlebitis, and articular symptoms also decreased, but not in a statistically
significant fashion. In a systematic review of 32 original articles and four abstracts including 338 patients
treated with interferon alfa-2a or alfa-2b, partial or complete responses were seen in 86 percent of the
patients with mucocutaneous symptoms [28]. However, flu-like symptoms and other toxicities, including
depression, are not uncommon.
• Thalidomide – Given the generally greater efficacy of other agents for mucocutaneous Behçet syndrome and
the significant risk of neuropathy and teratogenicity associated with thalidomide, thalidomide is now seldom
used for this indication in practice. The use of thalidomide should be restricted to clinicians experienced in
the management of Behçet syndrome and the use of this medication [29]. The efficacy of thalidomide
monotherapy for mucocutaneous manifestations of Behçet syndrome was demonstrated in a trial with 96
patients who were randomly assigned to one of three treatment groups: thalidomide 300 mg/day,
thalidomide 100 mg/day, or placebo [30]. At 24 weeks, a complete response occurred in two of the 32
patients receiving 100 mg/day of thalidomide; in five of the 31 patients receiving 300 mg/day; and in none of
the 32 patients receiving placebo. Only a minority of thalidomide-treated patients responded to treatment,
and some manifestations of disease (erythema nodosum) actually worsened. In addition, 7 percent of the
thalidomide-treated patients developed peripheral neuropathy, a condition that is often irreversible.
Mycophenolate mofetil has not been found to be effective in the treatment of mucocutaneous manifestations. A trial
of mycophenolate mofetil for the mucocutaneous manifestations of Behçet syndrome was terminated due to lack of
efficacy in the first six patients [31]. However, our authors may use mycophenolate mofetil in combination with TNF-
alpha inhibitors for patients who do not tolerate azathioprine.
Cutaneous lesions — With the exception of erythema nodosum and pyoderma gangrenosum, most other
cutaneous manifestations of Behçet syndrome (acneiform and papulopustular lesions, nodules, superficial
thrombophlebitis, and palpable purpura) respond well to the more moderate measures discussed above, particularly
colchicine and low to moderate doses of glucocorticoids.
The usual approach to cutaneous disease in Behçet syndrome is to begin with colchicine for mild manifestations (1 to
2 mg daily in divided doses) and to institute prednisone (up to 40 mg/day initially) for lesions that are refractory to
colchicine. Prednisone doses maintained between 5 and 10 mg/day are usually sufficient to control most skin
manifestations of Behçet syndrome.
Erythema nodosum and pyoderma gangrenosum require special consideration.
● Erythema nodosum – Because of the possibility of a dermal vasculitis underlying the clinical presentation of
erythema nodosum, clinicians should consider moving quickly to systemic glucocorticoids and other
immunosuppressive medications if colchicine is ineffective. Infectious etiologies for erythema nodosum should
also be ruled out before adding immunosuppressive therapy. (See "Erythema nodosum".)
The presence of a medium-vessel vasculitis through biopsy or by the clinical appearance of ulcerations of the
erythema nodosum lesions is an indication for systemic glucocorticoid treatment combined with another
immunosuppressive agent. In this setting, we suggest as initial therapy the combination of oral prednisone (40
to 60 mg daily) and azathioprine. Azathioprine is generally started at 50 mg daily, and the daily dose is increased
by 50 mg every 4 weeks as tolerated up to the target dose of 2.5 mg/kg/day, while monitoring a CBC every 2
weeks until therapy has stabilized, and every 6 to 12 weeks thereafter. A lower dose is indicated in patients with
renal insufficiency. Before the initiation of azathioprine, we perform genetic testing for mutations in the gene for
thiopurine methyltransferase. The prednisone dose is maintained at its initial level for one month and then
tapered off over three to four months. Treatment, particularly the rapidity of the prednisone taper, should be
dictated by the clinical response (see "Pharmacology and side effects of azathioprine when used in rheumatic
diseases", section on 'Pharmacogenetics and azathioprine toxicity'). If azathioprine is ineffective, other agents
should be considered. (See 'Posterior uveitis' below.)
● Pyoderma gangrenosum – Pyoderma gangrenosum associated with Behçet syndrome is often complicated by
the pathergy phenomenon. Thus, extensive debridement of pyoderma in Behçet syndrome is discouraged. The
treatment of pyoderma gangrenosum is reviewed in detail elsewhere. (See "Pyoderma gangrenosum: Treatment
and prognosis".)
Our treatment recommendations are largely in keeping with dermatology guidelines from a consensus conference on
treatment of skin and mucosal lesions in Behçet syndrome [32].
Arthritis — The nondeforming arthritis characteristic of Behçet syndrome is seldom the determinant of the level of
therapy. When present, joint symptoms are usually accompanied by other manifestations of disease that dictate the
intensity of treatment. The following is our general approach to drug therapy in patients with arthritis associated with
Behçet syndrome:
● For patients with arthritis with or without other non-organ-threatening disease (eg, mucocutaneous
involvement), we suggest colchicine 1 to 2 mg daily in divided doses. We may also use nonsteroidal
antiinflammatory drugs (NSAIDs) for symptomatic relief of arthritic pain if not otherwise contraindicated. There
is no consensus among experts about a preferred NSAID for this indication. (See "Initial treatment of
rheumatoid arthritis in adults", section on 'NSAIDs'.)
The efficacy of colchicine for the treatment of arthritis associated with Behçet syndrome was evaluated in a trial
comparing colchicine with placebo in 116 patients with Behçet syndrome; colchicine therapy was associated with
a decrease in the number of arthritic joints after two years of follow-up [9].
● In patients whose arthritis is not controlled by colchicine, low-dose systemic glucocorticoids may be added, with
continuous efforts to maintain the minimally effective dose. Prednisone (10 mg/day) is an appropriate starting
dose for the arthritis of Behçet syndrome, but if continuous therapy is required, 5 mg/day or even lower doses
should be the target dose, as these doses balance the efficacy and long-term side effects of glucocorticoids well.
(See "Initial treatment of rheumatoid arthritis in adults", section on 'Oral glucocorticoids'.)
● For refractory or persistent arthritis, we use azathioprine and/or TNF-alpha inhibitors instead of colchicine.
Although these agents are typically reserved for patients with more severe disease manifestations (eg, ocular
disease), they have been shown to be effective for arthritis associated with Behçet syndrome. In a trial of 73
patients with Behçet syndrome, patients were randomized to receive either azathioprine (2.5 mg/kg per day) or
placebo and followed for two years [20]. Patients on azathioprine had fewer episodes of new or recurrent eye
disease, oral and genital ulcers, and arthritis. The efficacy of TNF-alpha inhibitors for arthritis was demonstrated
in a multicenter study of 124 patients with severe or refractory Behçet syndrome treated with TNF-alpha
inhibitor therapy (infliximab in 62 percent and adalimumab in 30 percent) [22]. The overall response (ie,
complete and partial) rate was 90.4 percent, and the efficacy was similar between the different TNF-alpha
inhibitors used. Clinical responses were observed in 70 percent of patients with joint disease.
● For patients with arthritis refractory to the aforementioned treatments, other immunosuppressive agents that
may be used include interferon alfa or methotrexate. Most of the data for the efficacy of interferon alfa-2a or
alfa-2b are based on indications for refractory eye disease [27,28,33-47], but it has also been shown to be
effective for arthritis. A systematic review including 338 patients treated with interferon alfa-2a or alfa-2b
reported a partial or complete response in 96 percent of patients with arthritis [28]. The use of methotrexate is
based on the experience of clinicians specialized in the care of patients with Behçet syndrome and extrapolated
from the benefit of methotrexate in inflammatory arthritis in other conditions. The dose, side effects,
monitoring, and other considerations associated with methotrexate use are discussed elsewhere. (See "Initial
treatment of rheumatoid arthritis in adults", section on 'Initial therapy with methotrexate'.)
Apremilast may also have some benefit for arthritis in patients with arthritis that has not responded to colchicine
or other DMARDs. An observational study of 50 patients with articular or mucocutaneous Behçet syndrome
resistant to colchicine or other DMARDs reported complete response of articular symptoms after six months of
apremilast in 65 percent and a partial response in 17 percent [48].
Ocular disease — Ocular disease should be managed in collaboration with an ophthalmologist with experience in the
evaluation and treatment of uveitis, with the goal of inducing and maintaining remission. The two most common
ocular manifestations, anterior and posterior uveitis, have significantly different therapeutic implications. However,
isolated anterior uveitis is rare in Behçet syndrome, and patients more commonly present with a panuveitis. (See
"Clinical manifestations and diagnosis of Behçet syndrome", section on 'Ocular disease'.)
Anterior uveitis — The anterior uveitis of Behçet syndrome is generally treated with topical corticosteroids and a
dilating drop such as scopolamine (0.25%) or cyclopentolate (1%). The dilating drop relieves pain due to spasm of the
muscles controlling the pupil and also helps prevent the formation of posterior synechiae that may interfere with the
function of the pupil. Failure to use a mydriatic (dilating) agent promptly may lead to the development of pupillary
distortion, caused by the formation of scar tissue between the iris and lens. (See "Uveitis: Treatment", section on
'Initial treatment'.)
Patients whose anterior uveitis is not controlled with topical corticosteroids may require a short-term period of
therapy with systemic glucocorticoids. Assuming no other organ system involvement that requires higher doses, we
recommend oral prednisone at an initial dose of 40 mg daily. The prednisone may be tapered to discontinuation over
one month, assuming prompt disease control.
Posterior uveitis — Posterior uveitis (inflammation in the uveal tract that is posterior to the lens) in Behçet
syndrome constitutes a threat to vision and requires intensive immunosuppression. The combination of high-dose
glucocorticoids and another immunosuppressive agent is required. Data to support the use of any one of these
additional immunosuppressive agents over the others are limited, but preferred agents include either azathioprine,
cyclosporine, interferon alfa, or a monoclonal TNF-alpha antagonist in combination with azathioprine.
Initial therapy — We and others prefer to use azathioprine together with glucocorticoids for initial therapy for
most cases of posterior uveitis, based upon the limited available data, its adverse event profile, and experience with
its use in Behçet syndrome [3,20,49]. However, for patients who present with an initial or recurrent episode of sight-
threatening uveitis, we suggest initial treatment with TNF-alpha inhibitors in combination with azathioprine. It should
be borne in mind that it takes approximately three months for azathioprine to be effective. (See 'Severe or refractory
disease' below.)
Depending upon the severity of disease, we generally start prednisone (1 mg/kg per day for one month, with tapering
thereafter as tolerated). Initial pulse therapy with intravenous methylprednisolone (1 g/day for three days) is
sometimes used empirically for sight-threatening disease [50-53]. Intraocular triamcinolone may be beneficial for
panuveitis for two to six months [54]. Systemic glucocorticoids help reduce the acute inflammatory features of serious
end-organ disease, but disease activity tends to recur or progress if glucocorticoids are used without other
immunosuppressive therapy and thus should not be used as monotherapy.
Azathioprine treatment is usually started at 50 mg/day, and thereafter we usually increase the daily dose by 50 mg
every 4 weeks as tolerated up to the target dose of 2.5 mg/kg/day, while monitoring a CBC every 2 weeks until
therapy has stabilized, and every 6 to 12 weeks thereafter. A lower dose is indicated in patients with renal
insufficiency. Before the initiation of azathioprine, we perform genetic testing for the gene for thiopurine
methyltransferase if testing is available. If testing is not available, clinicians should start at a low dose and titrate up
slowly as tolerated. (See "Pharmacology and side effects of azathioprine when used in rheumatic diseases", section on
'Pharmacogenetics and azathioprine toxicity' and "Pharmacology and side effects of azathioprine when used in
rheumatic diseases", section on 'Dose titration and monitoring'.)
In a randomized trial of 73 men with Behçet syndrome both with and without eye involvement, patients were treated
with either azathioprine (2.5 mg/kg per day) or placebo and followed for two years [20]. Among the 25 patients
without eye disease at the outset, there were fewer new cases of ocular involvement with azathioprine (one versus
eight). Among the 48 patients with eye disease, there were fewer cases of hypopyon uveitis (1 versus 15). Azathioprine
therapy was also associated with less frequent oral ulcers, genital ulcers, and arthritis. In another study, which
included 57 patients from the aforementioned trial who were followed for eight years, a reduction in visual acuity and
vision loss were significantly less frequent in those assigned to azathioprine, despite similar treatment for patients in
both groups after the two-year trial ended [49]. In an observational study with 157 consecutive patients treated with
azathioprine for active posterior uveitis or panuveitis due to Behçet syndrome, 52 percent were complete responders
and 41 percent were partial responders [55].
An expert panel from the American Uveitis Society recommends initial treatment with TNF-alpha inhibitors due to the
observed improvement in ocular manifestations compared with other treatments [56-58]. The data also suggest that
infliximab and adalimumab may be more effective than etanercept [21,50,59]. However, as described in the next
section, we reserve the use of TNF-alpha inhibitors if there is an inadequate or no response to azathioprine or in those
with severe disease. (See 'Severe or refractory disease' below.)
Severe or refractory disease — In patients without an adequate response to initial therapy or with severe eye
disease at presentation, we use a monoclonal TNF-alpha inhibitor (with high-dose glucocorticoids), most commonly
infliximab or adalimumab, in addition to an oral DMARD, most commonly azathioprine. Severe eye disease can be
defined as greater than two lines of drop in visual acuity on a 10/10 scale and/or retinal disease, such as retinal
vasculitis or macular involvement [3]. Infliximab is generally used together with another immunosuppressive agent
(usually azathioprine in Behçet syndrome, where the latter drug has been used extensively). The choice between
infliximab and adalimumab largely depends upon patient factors such as comorbidities and patient preferences (eg,
for route of administration and frequency of treatment), regulatory or insurance restrictions on drug choice, and
safety issues, as the two are both effective [60,61]. There are less data available on the other TNF-alpha agents
including golimumab and certolizumab [62-64].
Infliximab is typically given at an initial dose of 5 mg/kg at 0, 4, 8, 16, and 24 weeks or 0, 2, 6, and every 8 weeks
[56,65]. The US Food and Drug Administration (FDA)-approved doses of adalimumab for the treatment of
intermediate, posterior, and panuveitis is to start with a loading dose of 80 mg given once, followed by 40 mg one
week later, and 40 mg every two weeks thereafter. These regimens are similar to those used for patients with
inflammatory bowel disease or rheumatoid arthritis. Adverse effects of TNF inhibitors are discussed in more detail in
the context of these diseases. (See "Tumor necrosis factor-alpha inhibitors: An overview of adverse effects" and
"Treatment of Crohn disease in adults: Dosing and monitoring of tumor necrosis factor-alpha inhibitors", section on
'Dosing and administration' and "Overview of dosing and monitoring of biologic agents and small molecules for
treating ulcerative colitis in adults", section on 'Dosing and administration'.)
A number of observational studies have suggested efficacy of infliximab for the treatment of inflammatory eye
disease, major organ involvement, and other symptoms in Behçet syndrome [56,57,65-84]. As an example, a
multicenter observational study including 164 patients with Behçet syndrome with uveitis who were treated with
infliximab (5 mg/kg/infusion) for more than one year found that infliximab reduced the frequency of ocular attacks
per year and improved visual acuity [83]. Uveitis relapsed in approximately 60 percent of all patients after initiating
treatment with infliximab, with 80 percent of relapses occurring within the first year. However, 90 percent of relapses
were controlled by increasing doses of topical corticosteroids and shortening the interval of infliximab infusion. In
another study of 50 patients with refractory uveitis (n = 36) or idiopathic posterior segment uveitis (n = 14), treatment
with infliximab (5 mg/kg) was associated with complete and partial responses in 68 and 22 percent of patients,
respectively, with a significant reduction in ocular attacks and cystoid macular edema, compared with baseline, and
with stable or improved visual acuity [81]. The mean follow-up period was 37 months. In an open-label prospective
cohort study, 555 patients starting infliximab for uveoretinitis in Behçet syndrome were followed for 24 months, and
response rate by the physician global assessment was 80.7 percent [85]. In addition, the median frequency of ocular
attacks was lower compared with the patient's baseline at the start of treatment, visual acuity was maintained, and
adverse events were comparable to those observed in patients treated with infliximab for other diseases.
A number of studies have also shown benefit with adalimumab treatment [86-90]. A retrospective review of 40
patients (66 eyes) with Behçet-associated uveitis treated with adalimumab found a reduction in disease flares,
improvement in best corrected visual acuity, reduction in mean central macular thickness on optical coherence
tomography (OCT), and a reduction in retinal vasculitis (as assessed by fluorescein angiography) at baseline, 3, and 12
months [90]. In another study with 177 patients with refractory uveitis due to Behçet syndrome, patients were treated
with adalimumab or infliximab with glucocorticoids but without other immunosuppressives, and were evaluated at
one year [91]. Both groups of patients improved; however, patients treated with adalimumab had better outcomes
compared with those on infliximab, including anterior chamber inflammation, vitritis, and visual acuity. Continuation
of use of the drug was also higher with adalimumab. A follow-up study of the infliximab patients in this study
reported 75 percent reached remission at a mean of 31.5 months of therapy. Once patients reached remission,
infliximab doses were reduced or infusion frequency was reduced if possible in collaboration with the patient. Overall,
these reductions resulted in similar ocular outcomes but with lower costs and adverse events in those treated with
lower doses [92]. In 1 case series, 11 male patients with ocular Behçet syndrome all showed at least some
improvement with adalimumab treatment [93]. Seventeen of 69 patients who failed infliximab were treated with
adalimumab, and 12 of 17 had a good response [94]. A retrospective study of 45 patients with Behçet syndrome
retinal vasculitis compared patients treated initially with conventional treatment (with an immunosuppressive agent
and glucocorticoids) with those treated with conventional treatment plus adalimumab, and found that patients
treated with adalimumab had better outcomes as measured by fluorescein angiography, risk of relapse, duration of
remission, visual acuity, and glucocorticoid requirements, with similar rates of adverse events [95]. A retrospective
cohort study of 42 patients (72 eyes) compared outcomes in patients with sight-threatening refractory Behçet
syndrome uveitis with vasculitis treated with immunosuppressives and glucocorticoids with or without adalimumab,
and found that those patients treated with adalimumab had greater improvement in anterior chamber inflammation,
fluorescein angiography scores, and glucocorticoid use with lower risk of relapse [96]. Other studies have also shown
effectiveness with other TNF-alpha inhibitors [86,97-99].
Based on limited data from observational studies and case reports, along with our own experience, we generally use
the alternative agents listed below in order of preference if there is no response to the treatment approach described
above. The choice of therapy also varies with patient and disease characteristics and comorbidities.
● Cyclosporine – Cyclosporine (2 to 5 mg/kg/day) can be efficacious in patients with Behçet syndrome with
posterior uveitis and may be used together with glucocorticoids and azathioprine as an alternative to infliximab
[25,100-104]. In a randomized trial of 23 patients with active uveitis, cyclosporine (5 mg/kg per day) was
compared with monthly intravenous pulse cyclophosphamide (1000 mg), and more improvement in visual acuity
was seen in the cyclosporine group at six months [100]. However, similar improvement was noted in the two
groups at two years. In another randomized trial of 40 patients with ocular Behçet syndrome, cyclosporine was
compared with treatment with glucocorticoids or chlorambucil and found that ocular disease was controlled
more effectively by cyclosporine, but articular, cutaneous, and mucocutaneous disease manifestations were not
[103].
Adverse effects due to cyclosporine are common. Approximately one-half of patients with Behçet syndrome
treated with this agent had an elevation in the serum creatinine concentration that was reversible when the
cyclosporine dose was reduced [25]. Hypertension is common with cyclosporine treatment. Neurotoxicity is also
frequently seen and may be difficult to differentiate from disease-related neurologic disease; thus, cyclosporine
is less preferred in patients with neurologic disease [26]. (See "Pharmacology of cyclosporine and tacrolimus".)
● Interferon alfa – Patients with retinal vasculitis or ocular disease refractory to the combination of
glucocorticoids, cyclosporine, and azathioprine may respond to treatment with interferon alfa-2a alone
(generally given three to six million units three times weekly) [28,39,46,47,105,106]. Flu-like symptoms and other
toxicities, including depression, are not uncommon and limit the use of this medication. Leukopenia is a
significant risk when interferon alfa is used with azathioprine [105].
A 2004 systematic review of 32 original articles and four abstracts included 338 patients treated with interferon
alfa-2a or alfa-2b and found partial or complete responses in 94 percent of those with uveitis, in addition to
improvement in mucocutaneous symptoms and arthritis [28]. Higher doses of interferon were more effective
and produced longer remissions. Many patients with improvement in their uveitis had not achieved benefit from
a number of other immunosuppressive agents, and interferon therapy led to long-term remissions in some
patients. Combination therapy with glucocorticoids and other agents was common. In such studies, it is difficult
to impute efficacy for the interferon agent. Relapse often followed cessation of therapy. Subsequent
observational studies have also support benefit from interferon alfa-2a in patients with ocular involvement
[38,45-47,84,107].
A randomized trial compared treatment of refractory Behçet uveitis in 26 patients and 44 eyes with interferon
alfa-2a plus tapering glucocorticoids versus cyclosporine plus tapering glucocorticoids [108]. Patients on
interferon had a better treatment response compared with those on cyclosporine, achieving response in 85.7
versus 66.7 percent, complete response in 50 versus 25 percent, and complete remission in 3.3 versus 7 months.
In addition, visual acuity and Behçet syndrome ocular attack scores at study conclusion were better in the
interferon group. Tolerability of the treatments was similar between the two groups.
● Cyclophosphamide – Cyclophosphamide should be reserved for refractory cases in which overall disease activity
warrants the risk of treatment. We sometimes use it before the above options in cases of severe ocular disease.
We use dosing regimens that are similar to those typically used for systemic vasculitis (see "General principles of
the use of cyclophosphamide in rheumatic diseases"). We generally administer 500 mg/m2 to 1 g/m2 of body
surface area (BSA) monthly for six months. Daily oral cyclophosphamide dosing at 2 to 3 mg/kg per day is an
alternative option in some patients. Combination therapy with cyclophosphamide (1 g monthly for six months
and every two to three months thereafter as needed), prednisolone (0.5 mg/kg daily), and azathioprine (2 to 3
mg/kg daily) was evaluated in 295 patients followed for up to 10 years [109]. Patients on combination therapy
demonstrated significant improvements in visual acuity scores as well as improvements across several measures
of ocular disease activity.
However, the data regarding the efficacy of cyclophosphamide for the treatment of ocular manifestations are
generally mixed. A systematic review from the Cochrane database concluded that there was insufficient evidence
to support the use of cyclophosphamide in the treatment of Behçet syndrome, particularly the ocular
manifestations [104]. In a single masked trial comparing cyclophosphamide (1 g intravenous bolus monthly) to
cyclosporine (5 mg/kg per day) in 23 patients with active and potentially reversible uveitis, visual acuity at six
months improved with cyclosporine but not cyclophosphamide [100]. Lack of benefit on ocular disease was
noted in another report [11]. On the other hand, an open trial comparing intravenous pulse cyclophosphamide
to pulse methylprednisolone for uveitis found benefit only with cyclophosphamide [110], and some
observational studies have suggested benefit for ocular and central nervous system disease [111].
● Methotrexate – Methotrexate may be considered a less toxic alternative, although data supporting its use for
treating ocular manifestations of Behçet syndrome are limited. We generally reserve this therapeutic option for
less severe ocular disease. Methotrexate can be administered with the same dosing regimen as that used for
rheumatoid arthritis, generally starting at a dose of 15 mg per week and increasing as tolerated up to 25 mg per
week. All patients receiving methotrexate should be treated with folic acid 1 mg daily or folinic acid weekly to
prevent hematologic and other side effects (see "Use of methotrexate in the treatment of rheumatoid arthritis").
The treatment of posterior uveitis with methotrexate (7.5 to 15 mg/week) and prednisolone (0.5 mg/kg daily)
was evaluated in 682 patients followed for up to 15 years [112]. Visual acuity scores, posterior uveitis, retinal
vasculitis, and the total adjusted disease activity index improved in 47, 75, 54, and 69 percent, respectively.
● Mycophenolate – Mycophenolate can be used despite the paucity of published trials in the absence of other
treatment options [113]. We typically start with a lower dose of 500 mg twice daily, and after several days, we
increase to the target dose of 1000 mg to 1500 mg twice daily. (See "Mycophenolate: Overview of use and
adverse effects in the treatment of rheumatic diseases", section on 'Mycophenolate dose and administration'.)
● Rituximab – Rituximab has also shown benefit in a small trial in patients with severe ocular manifestations
resistant to cytotoxic agents [114]. Rituximab was administered intravenously at a dose of 1000 mg on days 1
and 15 and repeated every six months or as warranted, which is the same dosing regimen as that used to treat
rheumatoid arthritis. In a randomized pilot trial of 20 patients with Behçet syndrome, retinal vasculitis, and
edema resistant to cytotoxic treatment, treatment with rituximab plus methotrexate and prednisolone was more
effective than treatment with cyclophosphamide plus azathioprine and prednisolone [114]. (See "Treatment of
rheumatoid arthritis in adults resistant to initial biologic DMARD therapy" and "Treatment of rheumatoid
arthritis in adults resistant to initial conventional synthetic (nonbiologic) DMARD therapy".)
● Intravitreal glucocorticoid implants – Several small series have reported success with intravitreal dexamethasone
implants for patients with severe or refractory Behçet syndrome uveitis or macular edema [115-117].
Gastrointestinal disease — The presence of significant gastrointestinal involvement in Behçet syndrome signals the
need for glucocorticoid therapy plus an additional agent designed to limit the long-term need for prednisone [118].
Treatment with prednisone should be instituted in consultation with a gastroenterologist, who can assess the extent
and severity of gastrointestinal disease.
For gastrointestinal ulceration (typically involving the terminal ileum, cecum, and ascending colon) in Behçet
syndrome, our approach is as follows:
● Glucocorticoids plus azathioprine – Typical starting doses for prednisone for gastrointestinal Behçet syndrome
are 0.5 to 1 mg/kg daily [119]. The initial dose of glucocorticoids is usually maintained for at least one month or
until symptoms improve before the institution of a taper, which is designed to decrease the daily dose to 10
mg/day within two to three months. Subsequent tapering to discontinuation over an additional two months may
proceed if disease control is maintained.
Azathioprine should be instituted at essentially the same time as prednisone. Treatment is begun at 50 mg/day,
after testing for mutations in the gene for thiopurine methyltransferase, and the daily dose is increased by 50
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mg every 4 weeks as tolerated up to the target dose of 2.5 mg/kg/day, while monitoring a CBC every 2 weeks
until therapy has stabilized, and every 6 to 12 weeks thereafter. A lower dose is indicated in patients with renal
insufficiency. Azathioprine should be maintained for at least six months, with periodic reassessments of its need
and serial endoscopies conducted by the gastroenterologist as appropriate. In an observational study including
37 patients with moderate to severe gastrointestinal manifestations of Behçet syndrome who were prescribed
azathioprine initially, remission was observed in 65 percent of patients during a mean follow-up of 69 months
[120]. (See "Pharmacology and side effects of azathioprine when used in rheumatic diseases", section on
'Pharmacogenetics and azathioprine toxicity'.)
● TNF-alpha inhibitors – We generally use TNF-alpha inhibitors in patients for whom treatment with
glucocorticoids plus azathioprine has failed to control the disease. TNF-alpha inhibitors are generally used in
combination with other therapies, preferably azathioprine. Several reports indicate successful treatment of
intestinal Behçet syndrome with infliximab and adalimumab using regimens approximating those approved for
the treatment of inflammatory bowel disease [82,118,120-126].
The typical schedule of infliximab administration is 5 mg/kg at zero, two, and six weeks, followed by 5 mg/kg
every eight weeks. In a retrospective study of 28 patients with moderate to severe intestinal Behçet syndrome,
54 percent of patients who had received infliximab demonstrated lasting control of disease activity and
symptom improvement over the follow-up period (median duration of 30 months) [82]. Factors predictive of
sustained response included older age at diagnosis, female sex, longer disease duration, concomitant
immunomodulator use, and achievement of remission at four weeks.
● Sulfasalazine – Though not well studied, sulfasalazine and other aminosalicylate agents are often used to treat
gastrointestinal disease in the same fashion as they are used in the treatment of inflammatory bowel disease.
Treatment with oral 5-aminosalicyclic acid was studied in 41 patients with intestinal manifestations of Behçet
syndrome [127]. At eight weeks in the 28 patients who remained in the study, clinical response occurred in 61
percent and remission in 57 percent. Endoscopic evaluation of 17 patients at 52 weeks demonstrated endoscopic
response in 71 percent and remission in 35 percent. In all 41 patients, rescue therapy-free survival occurred in 73
percent and surgery-free survival in 100 percent. As described above, we generally use sulfasalazine in
combination with TNF-alpha inhibitors. Pretreatment testing prior to the use of sulfasalazine, including
screening patients at high risk for glucose-6-phosphate dehydrogenase (G6PD) deficiency, is discussed in detail
separately. (See "Sulfasalazine and 5-aminosalicylates in the treatment of inflammatory bowel disease".)
● Other – Alternative options include mycophenolate and methotrexate. Total parenteral nutrition, enteral
nutrition, and surgery may be used when clinically indicated [124]. Recurrence is common after surgery, and
medical treatment is indicated to reduce this risk [118].
Renal disease — Treatment of Behçet renal disease varies depending on the type of lesion and may already be part
of therapy for other manifestations:
● Patients with minimal or mild nephritis (eg, hematuria, protein excretion <500 to 1000 mg/day, and normal
creatinine) may require no specific therapy, although monitoring is warranted for possible disease progression.
Evaluation for other causes of proteinuria is strongly advised.
● Patients with AA (secondary) amyloidosis (rather rare in Behçet syndrome [128]), which results from chronic
inflammation, require therapy for Behçet syndrome. The preferred therapy is colchicine (1 to 1.2 mg daily), which
treats the Behçet syndrome and thus helps prevent further accumulation of amyloid deposition and
accumulated damage. (See "Treatment of AA (secondary) amyloidosis", section on 'Colchicine'.)
● The treatment of renal artery aneurysms is discussed below. (See 'Large artery disease' below.)
Clinically important glomerulonephritis is rarely seen in Behçet syndrome; thus, consideration of an alternative
diagnosis is warranted. There are only small case series and retrospective reviews to guide therapy. Given the
vasculitic nature of Behçet syndrome, a reasonable approach is to extrapolate from the treatment efficacy of
medications used in granulomatosis with polyangiitis, particularly in patients with focal necrotizing lesions or
crescentic glomerulonephritis. (See "Granulomatosis with polyangiitis and microscopic polyangiitis: Induction and
maintenance therapy", section on 'Initial treatment approach'.)
In addition to these specific therapies, all patients with persistent proteinuric renal disease should be treated with
general therapies to slow disease progression, such as angiotensin-converting enzyme inhibitors and/or angiotensin
II receptor blockers. (See "Overview of the management of chronic kidney disease in adults", section on 'Slowing the
rate of progression' and "Antihypertensive therapy and progression of nondiabetic chronic kidney disease in adults".)
Vascular disease
Large artery disease — Arterial involvement in Behçet syndrome is uncommon but can lead to dilatations and
aneurysms of medium- and large-sized arteries [129,130]. These complications may require combined medical and
surgical or interventional radiology treatments.
● Immunosuppression – The medical approach involves high-dose glucocorticoids and another

immunosuppressive agent, typically cyclophosphamide [51]. The approach with both of these medications for
this indication is as described above in the treatment of posterior uveitis (see 'Posterior uveitis' above). Several
small studies also report improvement with baricitinib [131] and TNF-alpha inhibitors, including infliximab and
adalimumab, and tocilizumab [132-136].
● Surgical intervention – When surgery and/or interventional radiology procedures are indicated, it is ideal to
perform these interventions when the patient's disease is quiescent. However, it is necessary to intervene
urgently for enlarging or ruptured aneurysms or organ-threatening ischemia [137].
The outcomes of interventions for patients with large-vessel arterial disease are mixed [138-141]. A retrospective
review of 176 patients with vascular Behçet syndrome evaluated the results of 20 open and 31 endovascular
interventions in 36 patients and 51 arterial aneurysms [141]. The risk of recurrent aneurysmal formation at the
operative site was lower when patients were treated with both intervention and immunosuppressants than with
intervention alone. For extremity arteries, there were more complications with endovascular than open
procedures. Perioperative high-dose glucocorticoids may be administered on a case-by-case basis given the
potential for increased risk of infection and impaired wound healing associated with glucocorticoid use. Another
retrospective study evaluated 47 surgical procedures for Behçet arterial disease in 23 patients with a mean
follow-up of 8.4 years [142]. Arterial lesions included aneurysms (85 percent) and thrombosis (15 percent), of
which 48 percent were aortic and 52 percent were peripheral. The recurrence rate was 51 percent and consisted
of false aneurysms, thrombosis, and true aneurysms. The recurrence rate was 28 percent in patients receiving
preoperative medical treatments including glucocorticoids, colchicine, and immunosuppressants and 75 percent
in those not treated preoperatively. The recurrence rate was 42.5 percent in patients treated medically
postoperatively and 80 percent in patients not treated postoperatively. The recurrence rate was three times
lower with the use of a prosthetic sleeving technique.
● Interventional radiology – Interventional radiologic procedures provide an alternative to surgery for patients
with aneurysmal dilation of the aorta or major arteries [138,143,144]. Percutaneous placement of stent-grafts
was successful in six of seven patients with a variety of arterial aneurysms (aortic, subclavian, common carotid,
brachiocephalic, and iliac) [143]. Arterial occlusion and a recurrent aneurysm beyond the stent-graft occurred in
one patient each. In a another study of 22 patients treated with a primary stent graft for Behçet peripheral
artery aneurysm, 21 of the 22 had technical success post procedure, but at a mean follow-up of 23 months, 27
percent were occluded by computed tomography angiography [145]. Transcatheter embolotherapy of
pulmonary artery aneurysms was beneficial in a study of 17 patients presenting with hemoptysis [146].
● Complications of intervention – Techniques or therapies that may decrease the risk of recurrence after surgical
intervention for arterial involvement remain uncertain. In a study of 47 surgical procedures in 23 patients with
arterial Behçet syndrome, the recurrence rate was 28 percent in patients treated with immune-modulating
treatment including colchicine, glucocorticoids, and immunosuppressants, and 75 percent in untreated patients
[142]. The recurrence rate was lower with the use of a prosthetic sleeving technique for anastomoses but did not
vary otherwise by intervention utilized (vein, prosthetic graft, allograft, stent graft, or direct anastomosis).
Vascular complications following arterial bypass surgery are common in patients with Behçet syndrome. The
most frequent are graft occlusion and aneurysm formation at the anastomotic site. A retrospective study of 10
patients who underwent one or more vascular surgical interventions noted a 24 percent rate of graft occlusion
and a 13 percent rate of anastomotic pseudoaneurysm formation [147].
A retrospective study reviewed the outcomes of 20 patients with severe aortic regurgitation caused by Behçet
syndrome treated with perioperative biologic therapy, immunosuppressants, and glucocorticoids. Paravalvular
leakage developed in a smaller-than-expected number of patients, disease activity was reduced, and
glucocorticoids and immunosuppressants were successfully reduced, with no serious adverse events [148].
Venous thrombosis — Venous disease in Behçet syndrome is believed to result from endothelial inflammation
leading to thrombosis [51,149]. The approach to preventing venous thrombotic events in Behçet syndrome is control
of systemic inflammation rather than the institution of primary anticoagulation. Treatment should include
glucocorticoids in combination with another immunosuppressive agent used in the same manner as for posterior
uveitis (see 'Posterior uveitis' above). However, if venous thrombotic events occur, they should be treated with
anticoagulation using standard approaches (see "Overview of the treatment of proximal and distal lower extremity
deep vein thrombosis (DVT)"). Intracranial hypertension and, rarely, seizures may result from cerebral vein
thrombosis, requiring other interventions. (See "Cerebral venous thrombosis: Treatment and prognosis".)
A retrospective study of 296 patients with Behçet syndrome with venous thrombosis found that 34 percent of patients
suffered at least one relapse of venous thrombosis. Use of immunosuppressants and glucocorticoids reduced risk of
relapse [150]. Mortality was 6.4 percent at 4.75 years.
A retrospective study of 70 patients with Behçet-associated venous thrombosis demonstrated more effective and
rapid resolution of venous thrombosis with adalimumab-based treatment (35 patients) than with patients treated with
agents other than adalimumab, including azathioprine (18 patients), cyclosporine (9 patients), cyclophosphamide (5
patients), and methotrexate (3 patients) [134]. Furthermore, patients on adalimumab were able to taper
glucocorticoids more quickly; anticoagulation did appear to demonstrate benefit.
Complicated venous thrombotic events such as cerebral sinus thrombosis and pulmonary vein thrombosis can occur
in Behçet syndrome. The management of these conditions must be handled on a case-by-case basis, through
consultation with appropriate subspecialists [151]. Treatment with systemic glucocorticoids with or without another
immune-modulating treatment is indicated to reduce the inflammation that drives Behçet-associated thrombosis.
There is no consensus whether anticoagulation is beneficial in patients with Behçet-associated dural sinus thrombi,
and, if patients have Behçet-associated aneurysms, anticoagulation increases the risks of hemorrhage [152,153].
There are case reports of successful use of catheter-directed endovascular thrombolysis for refractory thrombosis
[154,155]. (See "Cerebral venous thrombosis: Treatment and prognosis".).
Neurologic disease — The choice of disease-modifying treatment in patients with a significant parenchymal disease
depends on severity, responsiveness to glucocorticoids, prior neurologic disease, disease course, and other features
of Behçet syndrome [152]. Azathioprine is commonly used as the first-line agent, with alternatives including
mycophenolate, methotrexate, and cyclophosphamide. Focal parenchymal lesions, encephalitis, and medium-vessel
vasculitis are all potentially life-threatening disease manifestations that should be treated in the same manner as
posterior uveitis with high doses of glucocorticoids in combination with an immunosuppressive agent (see 'Posterior
uveitis' above). A TNF-alpha inhibitor, such as infliximab, may be used if first-line agents are insufficient or for
aggressive clinical features. In a retrospective study of 19 patients treated with infliximab for parenchymal neurologic
Behçet syndrome for a mean of 32.3 months, 58 percent achieved disease remission, 37 percent achieved disease
stability with no new neurologic findings, and glucocorticoids could be discontinued in 18 patients [156]. Treatment
with cyclosporine should be avoided as it has been associated with more episodes of neurologic involvement in
patients with Behçet syndrome [3,152,157].
The management of neurologic manifestations of Behçet syndrome is based on limited data from uncontrolled trials
and expert opinion. The Japanese national research committee has released recommendations to reflect their
approach to the management of neurologic disease, which differs from the approach described above [158]. There
are also case reports of benefit with mycophenolate mofetil in some patients with predominantly neurologic
manifestations of Behçet syndrome [159]. A case series of 11 patients with refractory parenchymal neurologic Behçet
syndrome suggested that treatment with tocilizumab in combination with glucocorticoids was beneficial, and had a
glucocorticoid-sparing effect [160].
The treatment of cerebral sinus thrombosis is discussed above. (See 'Venous thrombosis' above.)
DURATION OF THERAPY
It is not clear how long to continue immunosuppressive treatment in patients in whom Behçet syndrome
manifestations are inactive. As noted, disease manifestations may improve over time in many patients, and treatment
choices should be individualized based on age, sex, type, and severity of organ involvement as well as patient
preferences. In patients with severe manifestations, we usually continue some form of immunosuppressive therapy
for at least 18 to 24 months. During this time, glucocorticoid therapy is tapered as tolerated, and immunosuppressive
agents with the greater potential for toxicity (eg, cyclosporine) are discontinued in favor of medications that are likely
to be safer (eg, azathioprine). Some patients require one or more immunosuppressive agents indefinitely to sustain
remission. In our experience, up to approximately 50 percent of patients with serious eye disease may be able to
completely discontinue immunosuppressive therapy without recurrence. In one study of 87 patients with severe
Behçet manifestations refractory to conventional immunosuppression and who responded to TNF-alpha treatment
for a median of two years, 41 percent were able to maintain complete remission for three years off TNF-alpha
treatment, though some remained on azathioprine treatment [161]. In another study, adalimumab dosing frequency
was tapered (from every two weeks to every three, four, six, and eight weeks and then potentially discontinued, as
determined by their treating physician) in 23 of 65 patients with Behçet syndrome that required and achieved
remission on adalimumab therapy for refractory uveitis [162]. Ocular outcomes were similar in the two groups, and
those patients treated with a reduced frequency of adalimumab had fewer associated adverse events and costs.
INVESTIGATIONAL THERAPIES
A number of other therapeutic approaches have been tried or are under investigation for the various manifestations
of Behçet syndrome. These include anakinra, canakinumab, gevokizumab, tocilizumab, pentoxifylline, intravenous
immune globulin, plasmapheresis, antibiotics, alemtuzumab (Campath 1-H), dapsone, antimalarials, rebamipide,
ustekinumab, secukinumab, rituximab, hematopoietic stem cell transplantation (autologous, or derived from
allogeneic bone marrow or umbilical cord blood), and granulocytapheresis [7,35,114,160,163-189]. Limited data also
suggest that laser therapy may be beneficial for Behçet-associated oral ulcers [190,191].
PROGNOSIS
Behçet syndrome typically has a waxing and waning course characterized by exacerbations and remissions. The
disease appears to be more severe in young, male, and Middle-Eastern or Far-Eastern patients [192,193]. However, for
some patients, particularly those with predominantly mucocutaneous and articular manifestations, the disease
burden dissipates with time and many patients can become asymptomatic [192]. In a series of 2200 patients followed
at a specialty clinic in Korea, only seven died from Behçet syndrome over a nine-year period [194]. The mortality in a
cohort of 817 patients in France was 5 percent at a median follow-up of 7.7 years [195].
The greatest morbidity and mortality comes from neurologic, ocular, and large-vessel arterial or venous disease
(causing pulmonary disease, gastrointestinal bleeding, bowel perforation, superior and inferior vena cava syndrome,
and cerebrovascular disease). In a 20-year follow-up of 387 Turkish patients with Behçet syndrome, most were doing
better at 20 years, with decreased activity of most manifestations other than central nervous system involvement and
major-vessel disease; the latter manifestations may have their onset after 5 to 10 years of disease [192].
Mucocutaneous, articular, and ocular disease are often at their worst in the early years of disease, but central nervous
system and large-vessel disease, if they develop, typically do so later in the disease course. Though disease activity
may decline with passing years, disease burden may rise due to cumulative ocular, neurologic, or vascular damage.
Pulmonary artery aneurysms and associated hemoptysis were previously associated with a very poor prognosis, but
survival appears to have improved, possibly as the result of earlier recognition and treatment using glucocorticoids
and other immunosuppressive agents. This was illustrated in a retrospective study of 26 patients followed at one
center in Turkey [130]. The five-year survival for patients with Behçet syndrome and pulmonary artery aneurysms who
were diagnosed and treated in 1992 or later was 80 versus 40 percent for 24 patients in whom the diagnosis was
made prior to that year.
Ocular and neurologic lesions may improve with immunosuppressive therapy but are often not fully reversible.
Without aggressive therapy, they generally progress. A significant proportion of patients with ocular disease,
particularly those of Turkish and Japanese descent, suffer progressive vision loss. Up to one-third of patients with
neurologic Behçet syndrome experience relapse, and the risk of relapse may be higher in those with a positive human
leukocyte antigen (HLA)-B51 [196].
The prognosis also varies with the type of neurologic process. Those with dural venous thrombosis or other
nonparenchymal processes are less likely to have recurrent disease, disability, or premature death. By comparison,
patients with parenchymal disease have a worse outcome and may follow a relapsing-remitting or progressive
disease course [152,197,198]. As an example, in an observational study including 58 patients with neurologic Behçet
syndrome in Iran, 84 percent had parenchymal and 16 percent had nonparenchymal disease [199]. Among those with
parenchymal involvement, 31 percent had a monophasic, 27 percent had a polyphasic, and 20 percent had a
progressive disease course. Those with a progressive disease course had a significantly higher rate of brainstem
atrophy. In another study of 125 patients with neurologic Behçet syndrome, ocular disease was more common in
patients with parenchymal disease, and vascular disease was more common in patients with nonparenchymal
disease; 33.6 percent experienced at least one relapse, and relapse was more common in younger patients and those
with cranial nerve dysfunction [200]. The prognosis was poorest for patients with progressive neurologic disease and
those with an initially higher modified Rankin scale.
Cerebrospinal fluid (CSF) analysis may help determine prognosis. In one study, roughly 90 percent of those with
elevated CSF protein levels or CSF pleocytosis had additional neurologic events, progressive disability, or death during
at least three years of follow-up. Only 25 to 30 percent of those with normal CSF protein levels suffered one such
event [197].
Quality of life is impaired by Behçet syndrome, and this varies with disease severity and degree of organ damage
[201].
SOCIETY GUIDELINE LINKS
Links to society and government-sponsored guidelines from selected countries and regions around the world are
provided separately. (See "Society guideline links: Behçet syndrome" and "Society guideline links: Vasculitis".)
INFORMATION FOR PATIENTS
UpToDate offers two types of patient education materials, "The Basics" and "Beyond the Basics." The Basics patient
education pieces are written in plain language, at the 5th to 6th grade reading level, and they answer the four or five
key questions a patient might have about a given condition. These articles are best for patients who want a general
overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more
sophisticated, and more detailed. These articles are written at the 10th to 12th grade reading level and are best for
patients who want in-depth information and are comfortable with some medical jargon.
Here are the patient education articles that are relevant to this topic. We encourage you to print or e-mail these topics
to your patients. (You can also locate patient education articles on a variety of subjects by searching on "patient info"
and the keyword(s) of interest.)
● Basics topic (see "Patient education: Behçet syndrome (The Basics)")
SUMMARY AND RECOMMENDATIONS
● General management principles – The goal of treatment of Behçet syndrome is to promptly suppress
inflammatory exacerbations and recurrences to prevent irreversible organ damage. A coordinated
multidisciplinary approach is necessary for optimal care. (See 'General principles of management' above.)
● Organ-based treatment approach – Treatment is dictated both by the type of organ system involved and by
the severity of disease within that organ system. Because many patients have more than one organ system
involved, treatment is often guided by the degree of disease severity in the most critical organ. (See 'Overall
approach' above.)
● Oral aphthae and genital ulcers – For oral aphthae and genital ulcers, we suggest initial treatment with topical
corticosteroids (Grade 2C). Topical anesthetics can also be added to provide temporary relief of discomfort prior
to eating and performing dental hygiene. (See 'Oral aphthae and genital ulcers' above.)
For prevention of recurrent oral and genital ulcers, we suggest colchicine 1 to 2 mg/day in two to three divided
doses rather than oral glucocorticoids (Grade 2B). In places where 0.5 mg tablets are not available (eg, United
States), doses in the range of 1.2 to 1.8 mg can be used. Apremilast is a reasonable alternative to colchicine as a
glucocorticoid-sparing agent for patients with recurrent oral ulcers. Apremilast is typically up-titrated at a rate of
10 mg daily over six days to achieve a maintenance dose of 30 mg twice daily. We wait approximately 12 weeks
to confirm an adequate response to either medication. There are no trials that have directly compared colchicine
with apremilast, and both have been shown to improve oral ulcers. However, the authors generally prefer a trial
of colchicine first given the rapid onset of action, lower cost, and better tolerability. (See 'Oral aphthae and
genital ulcers' above.)
When isolated oral aphthae or genital ulcers are refractory to topical corticosteroids, colchicine, or apremilast or
when multiple lesions are present, we add systemic glucocorticoids typically at a starting dose of prednisone 15
mg/day. Escalation of treatment with other medications should be determined on a case-by-case basis. (See
'Oral aphthae and genital ulcers' above.)
● Arthritis – For patients with arthritis with or without other non-organ-threatening disease (eg, mucocutaneous
involvement), we suggest colchicine 1 to 2 mg daily in divided doses (titrated to a dose without gastrointestinal
side effects) (Grade 2C). We may also use nonsteroidal antiinflammatory drugs (NSAIDs) for symptomatic relief
of arthritic pain if not otherwise contraindicated. In patients whose arthritis is not controlled by colchicine, low-
dose systemic glucocorticoids can be added. (See 'Arthritis' above.)
For refractory or persistent arthritis, we suggest azathioprine and/or tumor necrosis factor (TNF)-alpha inhibitors
instead of colchicine (Grade 2C). For patients with arthritis refractory to colchicine or azathioprine and/or TNF-
alpha inhibitors, other immunosuppressive agents such as interferon alfa or methotrexate may be used. (See
'Arthritis' above.)
● Ocular disease – Ocular disease should be managed in collaboration with an ophthalmologist with experience
in the evaluation and treatment of uveitis.
• Anterior uveitis – The anterior uveitis of Behçet syndrome is generally treated with topical corticosteroids
and a dilating drop such as scopolamine (0.25%) or cyclopentolate (1%). Patients whose anterior uveitis is not
controlled with topical corticosteroids may require a short-term period of systemic glucocorticoid therapy.
(See 'Anterior uveitis' above and "Uveitis: Treatment".)
• Posterior uveitis – For patients with posterior uveitis, initial treatment consists of high-dose glucocorticoids
in combination with a second immunosuppressive agent. (See 'Posterior uveitis' above.)
- Systemic glucocorticoids – We typically employ an initial prednisone dose of 1 mg/kg per day, not to
exceed 80 mg/day. Pulse therapy with intravenous methylprednisolone (1 g/day for three days) may be
used empirically for sight-threatening disease. (See 'Initial therapy' above.)
- Second immunosuppressive agent – We prefer azathioprine over other immunosuppressive agents as

initial therapy for posterior uveitis. For patients with sight-threatening disease at presentation or
refractory disease, we use a monoclonal TNF-alpha inhibitor in combination with azathioprine.
Alternative options for a second immunosuppressive agent include cyclosporine, interferon alfa,
cyclophosphamide, methotrexate, mycophenolate mofetil, or rituximab. (See 'Severe or refractory
disease' above.)
● Gastrointestinal disease – For patients with gastrointestinal ulcerations, we suggest initial treatment with
systemic glucocorticoids plus azathioprine (Grade 2C). Typical starting doses for prednisone are 0.5 to 1 mg/kg
daily. We generally use TNF-alpha inhibitors in patients who have failed treatment with glucocorticoids plus
azathioprine. (See 'Gastrointestinal disease' above.)
● Vascular disease – Complications from arterial involvement in Behçet syndrome, which include dilatations and
aneurysms of medium- and large-sized arteries, may require combined medical and surgical or interventional
radiology treatments. The medical approach involves high-dose glucocorticoids and another
immunosuppressive agent, typically cyclophosphamide. (See 'Large artery disease' above.)
Venous thrombotic events in Behçet syndrome are believed to be related to vascular inflammation rather than
inherent problems with coagulation. Thus, immunosuppressive agents are often used in combination with
anticoagulation, which is not always indicated. (See 'Vascular disease' above.)
● Neurologic disease – The choice of disease-modifying treatment in patients with a significant neurologic
parenchymal disease depends on severity, glucocorticoid responsiveness, prior neurologic disease, disease
course, and other Behçet syndrome features. Azathioprine is commonly used as the first-line agent, with
alternatives including mycophenolate, methotrexate, and cyclophosphamide. Focal parenchymal lesions,
encephalitis, and medium-vessel vasculitis are all potentially life-threatening disease manifestations that should
be treated in the same manner as posterior uveitis with high doses of glucocorticoids in combination with an
immunosuppressive agent. (See 'Neurologic disease' above.)
● Prognosis – Behçet syndrome typically has a waxing and waning course characterized by exacerbations and
remissions. The disease appears to be more severe in young, male, and Middle-Eastern or Far-Eastern patients.
The greatest morbidity and mortality comes from neurologic, ocular, and large-vessel arterial or venous disease.
Mucocutaneous, articular, and ocular diseases are often at their worst in the early years of disease, but central
nervous system and large-vessel diseases, if they develop, may do so later in the disease course. (See 'Prognosis'
above.)
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Topic 8239 Version 41.0
GRAPHICS
Comparison of representative topical corticosteroid preparations (classified according to the

United States system)
Available
Brand names strength(s),
Potency group* Corticosteroid Vehicle type/form
(United States) percent
(except as noted)
Super-high potency Betamethasone Ointment (optimized) Diprolene 0.05

(group 1) dipropionate,
augmented Gel, lotion [Generic only] 0.05
Clobetasol propionate Cream, ointment Temovate 0.05
Gel, solution (scalp) [Generic only] 0.05
Cream Tasoprol 0.05
Cream (emollient base) Temovate E ¶ 0.05
Lotion, shampoo, Clobex 0.05

spray aerosol
Foam aerosol Olux, Olux-E, Tovet 0.05
Lotion Impeklo 0.05
Ointment Clobetavix 0.05
Shampoo Clodan 0.05
Solution (scalp) Cormax ¶ 0.05
Diflucortolone valerate Ointment, oily cream Nerisone Forte (United 0.3

(not available in United Kingdom, others)
States)
Fluocinonide Cream Vanos 0.1
Flurandrenolide Tape (roll) Cordran 4 mcg/cm 2
Halobetasol Lotion Ultravate 0.05

propionate
Cream, ointment [Generic only] 0.05
Foam Lexette 0.05
High potency Amcinonide Ointment Cyclocort ¶ , Amcort ¶ 0.1

(group 2)
Betamethasone Ointment Diprosone ¶ 0.05
dipropionate
Cream, augmented Diprolene AF 0.05
formulation (AF)
Clobetasol propionate Cream Impoyz 0.025
Desoximetasone Cream, ointment, Topicort 0.25

spray
Gel Topicort 0.05
Diflorasone diacetate Ointment ApexiCon ¶ , Florone ¶ 0.05
Cream (emollient) ApexiCon E 0.05
Fluocinonide Cream, gel, ointment, Lidex ¶ 0.05

solution
Halcinonide Cream, ointment, Halog 0.1

solution
Halobetasol Lotion Bryhali 0.01

propionate
High potency Amcinonide Cream Cyclocort ¶ , Amcort ¶ 0.1

(group 3)
Lotion Amcort ¶ 0.1
Betamethasone Cream (hydrophilic Diprosone ¶ 0.05

dipropionate emollient)
Betamethasone Ointment Valisone ¶ 0.1

valerate
Foam Luxiq 0.12
Desoximetasone Cream, ointment Topicort, Topicort LP ¶ 0.05
Diflorasone diacetate Cream Florone ¶ , Psorcon 0.05
Diflucortolone valerate Cream, oily cream, Nerisone (United 0.1

(not available in United ointment Kingdom, others)
States)
Fluocinonide Cream (aqueous Lidex-E ¶ 0.05

emollient)
Fluticasone propionate Ointment Cutivate ¶ 0.005
Mometasone furoate Ointment Elocon ¶ 0.1
Triamcinolone Cream, ointment Aristocort HP ¶ , 0.5

acetonide Kenalog ¶ , Triderm
Medium potency Betamethasone Spray Sernivo 0.05

(group 4) dipropionate
Clocortolone pivalate Cream Cloderm 0.1
Fluocinolone acetonide Ointment Synalar 0.025
Flurandrenolide Ointment Cordran 0.05
Fluticasone propionate Cream Cutivate ¶ 0.05
Hydrocortisone Ointment Westcort ¶ 0.2

valerate
Mometasone furoate Cream, lotion, solution Elocon ¶ 0.1
Triamcinolone Cream Kenalog ¶ , Triderm 0.1

acetonide
Ointment Kenalog ¶ 0.1
Ointment Trianex, Tritocin 0.05
Aerosol spray Kenalog 0.2 mg per 2 second

spray
Dental paste Oralone 0.1

¶
Lower-mid potency Betamethasone Lotion Diprosone 0.05
Betamethasone Cream Beta-Val ¶ , Valisone ¶ 0.1

valerate
Desonide Ointment DesOwen ¶ , 0.05

Tridesilon ¶
Gel Desonate, DesRx 0.05
Fluocinolone acetonide Cream Synalar 0.025
Flurandrenolide Cream, lotion Cordran, Nolix 0.05
Fluticasone propionate Lotion Beser ¶ , Cutivate ¶ 0.05
Hydrocortisone Cream, lotion Locoid, Locoid 0.1

butyrate Lipocream
Ointment, solution [Generic only] 0.1
Hydrocortisone Cream Pandel 0.1

probutate
Hydrocortisone Cream Westcort ¶ 0.2

valerate
Prednicarbate Cream (emollient), Dermatop ¶ 0.1

ointment
Triamcinolone Lotion Kenalog ¶ 0.1

acetonide
Ointment Kenalog ¶ 0.025
Low potency Alclometasone Cream, ointment Aclovate ¶ 0.05

Betamethasone Lotion Beta-Val ¶ , Valisone ¶ 0.1

valerate
Desonide Cream DesOwen, Tridesilon 0.05
Lotion DesOwen ¶ , LoKara ¶ 0.05
Foam Verdeso 0.05
Fluocinolone acetonide Cream, solution Synalar 0.01
Shampoo Capex 0.01
Oil Δ Derma-Smoothe/FS 0.01

Body, Derma-
Smoothe/FS Scalp
Triamcinolone Cream, lotion Kenalog ¶ , Aristocort ¶ 0.025

acetonide
Least potent Hydrocortisone (base, Cream Ala-Cort, Hytone ¶ , 2.5

¶
(group 7) ≥2%) Nutracort
Ointment Hytone ¶ 2.5
Lotion Hytone ¶ , Ala Scalp, 2

Scalacort DK
Solution Texacort 2.5
Hydrocortisone (base, Ointment Cortaid ¶ , Cortizone 10, 1

<2%) Hytone ¶ , Nutracort ¶
Cream Ala-Cort, Cortaid ¶ , 1

Cortizone 10, Hytone ¶ ,
KeriCort, Synacort ¶
Gel Cortizone 10 1
Lotion Aquanil HC, Cortizone 1

10, Sarnol-HC
Spray Cortaid ¶ 1
Solution Cortaid ¶ , Noble ¶ , 1

Scalp Relief, Scalpicin
Cream, ointment Cortaid ¶ 0.5
Cream Instacort 0.5
Hydrocortisone Cream MiCort-HC ¶ 2.5

acetate
Cream Vanicream HC 1
Lotion Nucort 2
* Listed by potency according to the United States classification system: group 1 is the most potent, group 7 is the least
potent. Other countries use a different classification system with only 4 or 5 groups.
¶ Inactive United States brand name for specific product; brand may be available outside United States. This product may be
available generically in the United States.
Δ 48% refined peanut oil.
Data from:
1. Lexicomp Online. Copyright © 1978-2023 Lexicomp, Inc. All Rights Reserved.
2. Tadicherla S, Ross K, Shenefelt D. Topical corticosteroids in dermatology. Journal of Drugs in Dermatology 2009; 12:1093.
3. U.S. Food & Drug Administration Approved Drug Products with Therapeutic Equivalence (Orange Book). Available at:
https://www.accessdata.fda.gov/scripts/cder/ob/default.cfm (Accessed on June 18, 2017).
4. The British Association of Dermatologists' information on topical corticosteroids – established and alternative proprietary names, potency, and
discontinuation. British Association of Dermatologists. Available at: https://www.bad.org.uk/shared/get-file.ashx?id=3427&itemtype=document
(Accessed on April 26, 2021).
Graphic 62402 Version 65.0
Contributor Disclosures
Ellison L Smith, MD No relevant financial relationship(s) with ineligible companies to disclose. Yusuf Yazici,
MD Grant/Research/Clinical Trial Support: Amgen [Rheumatoid arthritis, Behçet syndrome]; Bristol-Myers Squibb [Rheumatoid
arthritis, Behçet syndrome]; Genentech [Rheumatoid arthritis, Behçet syndrome]. All of the relevant financial relationships listed
have been mitigated. Peter A Merkel, MD, MPH Equity Ownership/Stock Options: Kyverna [Systemic lupus erythematosus].
Grant/Research/Clinical Trial Support: AbbVie [Vasculitis]; AstraZeneca [Vasculitis]; Boehringer Ingelheim [Scleroderma]; Bristol-
Myers Squibb [Vasculitis]; ChemoCentryx [Vasculitis]; Electra [Vasculitis]; Genentech/Roche [Vasculitis]; GlaxoSmithKline [Vasculitis];
InflaRx [Vasculitis]; Sanofi [Vasculitis]; Takeda [Vasculitis]. Consultant/Advisory Boards: AbbVie [Vasculitis]; AstraZeneca [Vasculitis];
Boehringer Ingelheim [Scleroderma]; Bristol-Myers Squibb [Vasculitis]; ChemoCentryx [Vasculitis]; CSL Behring [Scleroderma,
vasculitis]; Dynacure [Vasculitis]; EMDSerono [Vasculitis]; GlaxoSmithKline [Vasculitis]; InflaRx [Vasculitis]; Janssen [Vasculitis];
Kyverna [Scleroderma, vasculitis]; MiroBio [Vasculitis]; Neutrolis [Vasculitis]; Novartis [Vasculitis]; NS Pharma [Vasculitis]; Otsuka
[Vasculitis]; Q32 [Vasculitis]; Regeneron [Vasculitis]; Sparrow [Vasculitis]; Takeda [Vasculitis]. All of the relevant financial
relationships listed have been mitigated. Philip Seo, MD, MHS No relevant financial relationship(s) with ineligible companies to
disclose.
Contributor disclosures are reviewed for conflicts of interest by the editorial group. When found, these are addressed by vetting
through a multi-level review process, and through requirements for references to be provided to support the content.
Appropriately referenced content is required of all authors and must conform to UpToDate standards of evidence.
Conflict of interest policy

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14/06/2023, 23:53 Treatment of Behçet syndrome - UpToDate

Official reprint from UpToDate®

Treatment of Behçet syndrome

Literature review current through: May 2023.

GENERAL PRINCIPLES OF MANAGEMENT

● A coordinated multidisciplinary approach is necessary for optimal care

● Disease manifestations may improve over time in many patients

Erythema nodosum and pyoderma gangrenosum require special consideration.

● Immunosuppression – The medical approach involves high-dose glucocorticoids and another

SOCIETY GUIDELINE LINKS

INFORMATION FOR PATIENTS

● Basics topic (see "Patient education: Behçet syndrome (The Basics)")

SUMMARY AND RECOMMENDATIONS

- Second immunosuppressive agent – We prefer azathioprine over other immunosuppressive agents as

Use of UpToDate is subject to the Terms of Use.

Comparison of representative topical corticosteroid preparations (classified according to the

Super-high potency Betamethasone Ointment (optimized) Diprolene 0.05

Clobetasol propionate Cream, ointment Temovate 0.05

Gel, solution (scalp) [Generic only] 0.05

Cream Tasoprol 0.05

Cream (emollient base) Temovate E ¶ 0.05

Lotion, shampoo, Clobex 0.05

Foam aerosol Olux, Olux-E, Tovet 0.05

Lotion Impeklo 0.05

Ointment Clobetavix 0.05

Shampoo Clodan 0.05

Solution (scalp) Cormax ¶ 0.05

Diflucortolone valerate Ointment, oily cream Nerisone Forte (United 0.3

Fluocinonide Cream Vanos 0.1

Flurandrenolide Tape (roll) Cordran 4 mcg/cm 2

Halobetasol Lotion Ultravate 0.05

Foam Lexette 0.05

High potency Amcinonide Ointment Cyclocort ¶ , Amcort ¶ 0.1

Clobetasol propionate Cream Impoyz 0.025

Desoximetasone Cream, ointment, Topicort 0.25

Gel Topicort 0.05

Diflorasone diacetate Ointment ApexiCon ¶ , Florone ¶ 0.05

Cream (emollient) ApexiCon E 0.05

Fluocinonide Cream, gel, ointment, Lidex ¶ 0.05

Halcinonide Cream, ointment, Halog 0.1

Halobetasol Lotion Bryhali 0.01

High potency Amcinonide Cream Cyclocort ¶ , Amcort ¶ 0.1

Betamethasone Cream (hydrophilic Diprosone ¶ 0.05

Betamethasone Ointment Valisone ¶ 0.1

Desoximetasone Cream, ointment Topicort, Topicort LP ¶ 0.05

Diflorasone diacetate Cream Florone ¶ , Psorcon 0.05

Diflucortolone valerate Cream, oily cream, Nerisone (United 0.1

Fluocinonide Cream (aqueous Lidex-E ¶ 0.05

Fluticasone propionate Ointment Cutivate ¶ 0.005

Mometasone furoate Ointment Elocon ¶ 0.1

Triamcinolone Cream, ointment Aristocort HP ¶ , 0.5

Medium potency Betamethasone Spray Sernivo 0.05

Clocortolone pivalate Cream Cloderm 0.1

Fluocinolone acetonide Ointment Synalar 0.025

Flurandrenolide Ointment Cordran 0.05

Fluticasone propionate Cream Cutivate ¶ 0.05

Hydrocortisone Ointment Westcort ¶ 0.2

Mometasone furoate Cream, lotion, solution Elocon ¶ 0.1

Triamcinolone Cream Kenalog ¶ , Triderm 0.1