Systemic Lupus Erythematosus

1
 Introduction
• Systemic lupus erythematosus (SLE)
– an autoimmune disease associated with autoantibody
production
– Characterized by disorders of the innate and adaptive immune
systems
• “lupus”………Latin for wolf
– Used to describe erosive lesions that looked like skin that had
been gnawed by a wolf
• Current understanding
– SLE is a multisystem disease
2
 Epidemiology
• Incidence: 0.3 to 23.7 per 100,000 person years
• Prevalence: 6.5 to 178 per 100,000 persons
• Most frequent in
– Women of reproductive age (15–50 years old) vs men
– People of color and low socioeconomic status
• More severe in men, children, and age > 50 years
• Standardized mortality ratio: 2.6 to 3 times higher than in the general
population
– cardiovascular, renal disease and infections
• Survival varies depending on the involved organ
– lupus nephritis

3
 Etiology
• Exact etiology…..unknown
• Multiple factors are identified
– Genetic influences
– Incidence higher in first-degree relatives of patients with SLE (20x)
– Medications: hydralazine and procainamide, vaccines & biologics
– Cigarette smoke…….. cutaneous lupus
– Viruses……..Epstein–Barr virus
– Other triggers include infections, psychological stress, silica dust,
hydrazines, petroleum, solvents (nail polish remover and metal
cleaners), dyes, and pesticides
– Hormones……estrogens and progesterones…….the X chromosome
– Men with Klinefelter (XXY) syndrome
– Low in women with Turner (XO) syndrome
4
 Pathophysiology
• A multisystem disease
– Altered T and B lymphocyte activation and signaling
– Abnormal clearance of apoptotic debris containing nuclear
material
• Stimulate immune responses
• Increased number of plasma cells
– Produce autoantibodies………..directed at dsDNA…….tissue
damage
• Neutrophil dysfunction can increase the risk for infection.
• Titers of anti-dsDNA………..predict disease flare
5
 Pathophysiology
• Some autoantibodies may play a role in the pathogenesis of clinical
features of SLE; these autoantibodies may target
– Ro/SSA (antigen Ro/Sjögren syndrome A, ribonucleoprotein
complex)
– La/SSB (antigen La/Sjögren syndrome antigen B, RNA-binding
protein)
– C1q (subunit of the C1 complement component)
– Sm (nuclear particles)
– N-methyl-D aspartate (NMDA) receptor (amino acid released by
neurons),
– Phospholipids
– Nucleosomes (from apoptotic cellular debris), and
– Histones (protein core of nucleosomes)
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7
 Pathophysiology
• Immune complexes

– Form when autoantibodies bind to nuclear material and deposit in

tissues

• Activate the complement cascade……….influx of inflammatory

cells and tissue injury

– Autoantibodies might also directly react with proteins in tissues

• Antibodies

– to blood cells………….cytopenias

– Against phospholipids…….…thrombosis and fetal loss

– T cell abnormalities………..immune disorders

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 Pathophysiology

• Cytokines play multiple roles in SLE and contribute to

inflammation and tissue damage

– Increase in the levels of Interleukin-6,10, 17, type 1

interferon, B-lymphocytestimulator(BLyS)

– decrease in interleukin-2

– TNF-α role in SLE is unclear

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 Clinical Presentation
• SLE can involve almost any organ
• Sign and symptom vary based on the affected body part
• Symptoms
– Fatigue, depression, anxiety, photosensitivity, joint pain,
headache, weight loss, nausea/abdominal pain
• Signs
– Rash, alopecia, fever, oral and nasal ulcers, arthritis, renal
dysfunction, seizure, psychosis, pleuritis, pleural effusion,
cardiovascular disease, pericarditis/myocarditis, heart
murmur, hypertension, anemia, leukopenia,
thrombocytopenia, lymphadenopathy, Raynaud’s
phenomenon, vasculitis
• Disease manifestations fluctuate
– Periods of remission, flares, and progression.
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 Diagnostic Tests
– Serology: autoantibodies, antiphospholipid antibodies,
complement
– Inflammatory markers: C-reactive protein, erythrocyte
sedimentation rate
– Blood chemistries
– Complete blood count
– Urinalysis
– lumbar puncture
– Renal biopsy

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Systemic Lupus International Collaborating Clinics
Classification Criteria (SLICC)

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 Diagnosis

• To satisfy the SLICC criteria, a patient must meet at least

four of the elements, including

– at least one clinical and one immunologic criterion or

– the patient must have biopsy-proven lupus nephritis

with positive ANA or anti-dsDNA antibodies.

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• SLE flare:
– A measurable increase in disease activity in one or more
organ systems involving new or worse clinical signs and
symptoms and/or laboratory measurements
• The increase in disease activity has to be clinically
significant
– To consider a change or an increase in treatment

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• Cutaneous lupus erythematosus………..50-70% of patients with
SLE
– Acute cutaneous lupus erythematosus
• Characterized by a photosensitive malar rash over the
cheeks and nose with sparing of the nasolabial folds.
• Arms and trunk may be involved
• Wax and wane without scarring

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• Cutaneous lupus erythematosus………..50-70% of patients with
SLE
– Subacute cutaneous lupus erythematosus
• Highly photosensitive
• Manifested by annular or papulosquamous lesions that
usually heal without scarring
• accompanied by musculoskeletal complaints
• 70% of patients have anti-Ro/SSA autoantibodies
• Common in drug-induced lupus

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• Chronic cutaneous lupus erythematosus
– The most common is discoid lupus
• Confined to the head and neck in about two-thirds of pts,
but it can be generalized.
• Common in smokers and African Americans
• May be associated with scarring, scarring alopecia, malar
rash, photosensitivity, oral ulcers, leukopenia, vasculitis,
and chronic seizures
• Associated with a lower incidence of arthritis, end-stage
renal disease, and immunologic markers such as ANA,
anti-dsDNA, and antiphospholipid antibodies

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• Lupus nephritis
– Present at the time of SLE diagnosis in about 35% of adult patients
and 50-60% of patients develop it by 10 years
– More common in African American and Hispanic patients
– More common in men than in women
• classification based on histologic findings:
– Class I: minimal mesangial
– Class II: mesangial proliferative
– Class III: focal (less than 50% of glomeruli involved)
– Class IV: diffuse (50% or more of glomeruli involved)
– Class V: membranous
– Class VI: advanced sclerosing (at least 90% globally sclerosed
glomeruli without residual activity).
• Patients may also have hypertension and atherosclerosis
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• Neuropsychiatric lupus
– Central and peripheral nervous system involvement
– Prevalence around 19-38%, can range from 6.4-93%
– About 50% of neuropsychiatric events appear within the
first 2 years after the diagnosis of SLE
• Findings more indicative of neuropsychiatric lupus include
– cerebrovascular disease (ischemic stroke and/or
transient ischemic attack), anxiety, and seizures in 5-10%
of patients;
– Severe cognitive dysfunction, acute confusional state,
peripheral neuropathy, and psychosis in 3-5%; and
– Chorea, movement disorders, cranial nerve
neuropathies, and aseptic meningitis in less than 1-2% of
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patients
• Cardiac manifestations of SLE
– Pericarditis and myocarditis
– Increased risk for accelerated atherosclerosis
– Related to the chronic inflammation associated with the
disease and adverse effects of the drugs used in the
treatment
– Antiphospholipid antibodies and type I interferons may
play a role in the pathogenesis
– Drugs such as hydroxychloroquine and mycophenolate
mofetil may have a cardioprotective effect

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• Treatment
– Treatment of SLE is determined by the patient’s
symptoms, organ involvement, comorbidities, and other
patient-specific factors.
• Desired Outcomes
– To prevent disease flares and involvement of other
organs
– To decrease disease activity and prevent damage
– To achieve and maintain remission
– To reduce use of corticosteroids
– To improve quality of life, while minimizing adverse
effects and costs

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 General Approach
• Lifestyle modifications
– Protection from the sun, smoking cessation, exercise,
and weight control
• Immunizations ………. appropriate timing with respect to
immunosuppressive drug use

• Disease activity and treatment on pregnancy outcomes

• Evaluation & treatment of comorbidities

– hypertension, hyperlipidemia, and depression

• Osteoporosis prevention………most patients on

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corticosteroids
 General Approach
• Importance of adherence to treatment

• Expected time for response to medications

• Prognosis better if lupus is limited to skin &musculoskeletal

findings

• worst prognosis is seen with renal or CNS involvement.

• Mortality affected by SLE disease activity, cardiovascular

risks, and infections

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 Non-pharmacologic Therapy
• Good social support
– For patients well being, improved quality of life and
outcomes
• Counseling and support groups
• Aerobic exercise (limited data support)
– To decrease patients’ risk for cardiovascular events and
osteoporosis
– Improve fatigue, depression, anxiety, and sleep
disturbances
– Also help with weight loss

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 Non-pharmacologic Therapy

• Photosensitivity………..common in SLE

– patients should

• wear protective clothing and hats

• use sunscreens…….. SPFs of 15 to 50+……use the one

with the highest SPF

–apply them every 2 hours while in the sun

• Avoid tanning salons

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 Non-pharmacologic Therapy

• Patients should be counseled to stop smoking

– Smoking

• Increases cardiovascular risk

• Exacerbate SLE and diminish the effectiveness of

antimalarials and belimumab

– Smokers also have a higher incidence of active rashes

with skin damage and scarring

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 Pharmacologic Therapy
• Treatment is personalized based on the manifestations of
SLE in the patient
• Consists of a combination of
– Immunosuppression and
– Symptomatic and supportive therapies
• The only drugs approved by the FDA for treatment of SLE
– aspirin, prednisone, hydroxychloroquine, and
belimumab
• Other drugs are used “off-label”

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Algorithm for the treatment
of SLE.

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• Lupus nephritis
– All patients should receive hydroxychloroquine
• reduce damage and flares
– ACE inhibitor or ARB
• In patients with proteinuria of ≥0.5 g/day
– Maintain BP to < 130/80 mmHg
– Statin therapy
• When LDL-cholesterol >100 mg/dL
– Specific treatment is based on the type of nephritis.
• Minimal mesangial and mesangial proliferative lupus
nephritis (Class I & II)
– Do not usually need immunosuppressive therapy
• Focal & diffuse lupus nephritis (Classes III and IV)
– aggressive use of glucocorticoids and immunosuppressive
29
Rx
American College of Rheumatology
guidelines for therapy for Class III/IV
lupus nephritis

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• Neuropsychiatric lupus

– Treatment depends on the manifestations

– Symptomatic therapy

• anticonvulsants and antidepressants as needed

– More specific treatment

• Problem related to inflammation or thrombosis or

both.

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• Neuropsychiatric lupus

– Inflammation or neurotoxic damage

• Glucocorticoids alone or in conjunction with

immunosuppressive drugs such as azathioprine or
cyclophosphamide(Strong evidence).

– If no response plasma exchange, IV immunoglobulin, or

rituximab

– Moderate-to-high titers of antiphospholipid antibodies and/or

thrombosis

• anticoagulants and/or inhibitors of platelet aggregation should

be used (Sufficient evidence). 32
• Intermittent joint pain associated with SLE
– NSAIDs are good initial therapy
– Severe or persistent pain
• Prednisone 10 mg/day or less + hydroxychloroquine
– Localized joint pain
• Intra-articular corticosteroid injections
– Inadequate treatment response
• Add methotrexate to hydroxychloroquine
– Intolerant patient
• Use mycophenolate mofetil or azathioprine
– Alternative treatment
• leflunomide, belimumab, rituximab, abatacept, or
TNF-α inhibitors 33
• Cutaneous lupus erythematosus

– First step: protection from ultraviolet light

– Drug Rx is based on extent & severity of involvement

– Topical corticosteroids

• To relieve symptoms such as itching or burning

• May not provide adequate clearing of lesions when

used alone

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• Cutaneous lupus erythematosus
– The choice of corticosteroid depends on the location of
application
• Low potency corticosteroids
– Fluocinolone acetonide 0.01% and hydrocortisone 1%
– On thin skin such as the face and groin
• Mid-potency corticosteroids
– triamcinolone acetonide for trunk and extremities
• High potency corticosteroids
– Clobetasol propionate
– For thick-skin areas such as scalp, soles, and palms
– More severe disease
• Creams or ointments on the body
• Foams or solutions on the scalp
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• Cutaneous lupus erythematosus
– Discoid lupus
• Intralesional corticosteroids
• Should not be repeated more often than every 4 to 6
weeks
– Adverse effects of topical corticosteroids
• Skin atrophy, telangiectasias, and steroid-induced
dermatitis
• Use the lowest effective potency and duration of therapy
– Alternative to topical corticosteroids
• Topical calcineurin inhibitors
– Pimecrolimus and tacrolimus
– Pimecrolimus more lipophilic and has greater affinity
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for the skin
• Cutaneous lupus erythematosus
• Systemic therapy
– First-line systemic therapy
• Antimalarials…….photoprotective effects
– hydroxychloroquine alone or + quinacrine
• Refractory disease
– Systemic immunosuppressive drugs
• Corticosteroids, methotrexate, mycophenolate mofetil, or
azathioprine
– Immunomodulatory drugs
• dapsone, thalidomide, or lenalidomide
– Biologics
• rituximab or belimumab
– Oral retinoids
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Dosing of Drugs Used to Treat SLE

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Dosing of Drugs Used to Treat SLE

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40
Non-steroidal Anti-inflammatory Drugs (NSAIDs)
• Used as first-line treatment for
– Arthritis, musculoskeletal complaints, fever, and serositis
• Low-dose aspirin
– In patients with antiphospholipid antibodies
• Concern with NSAIDs:
– Decrease renal function
• Can complicate evaluation of lupus nephritis
– Increase cardiac events
– Other adverse effects
• Hepatotoxicity, gastrointestinal (GI) bleeding, and
aseptic meningitis
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 Corticosteroids
• Corticosteroids
– Used as monotherapy or as adjuncts to other treatments
– Control flares and maintain low disease activity
– Rapid onset
– Used topically or systemically
– Pulse IV dose (methylprednisolone)
• To treat flares and quickly reduce inflammation
– Taper doses to the lowest effective dose
– high-dose
• Increased lupus organ damage accrual, infection, and
death
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 Corticosteroids
• Chronic use of any dose
– Cardiovascular complications, psychological
disturbances, glaucoma, cataracts, hyperglycemia,
weight gain, avascular necrosis of bone, and
osteoporosis
• Patients taking prednisone ≥2.5 mg daily for at least 3
months
– should optimize calcium & vitamin D intake

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 Antimalarials
• Chloroquine and hydroxychloroquine
• Hydroxychloroquine
– Anti-inflammatory, immunomodulatory, and
antithrombotic effects
– Reduces concentrations of inflammatory cytokines such
as interleukins 1, 2, 6, 17, and 22, interferon alpha and
gamma, and TNF-α
– Alters antigen presentation and T-cell proliferative
responses
– Decrease activation of toll-like receptors
– Reduces platelet aggregation and thrombosis
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 Antimalarials
• Hydroxychloroquine
– Fewer ADRs & is the preferred drug.
– Used in all patients with SLE, including pregnant women
– Prevent lupus flares and improve long-term survival
– Protects against bone mass loss
– Protective effects against thrombosis and irreversible organ
damage
– Beneficial effect on lipids & fasting blood glucose, decreases
infections
– Allow corticosteroid doses to be decreased
– Patients receiving hydroxychloroquine often have disease
flares when the drug is discontinued
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 Antimalarials
• Hydroxychloroquine
– Minimum therapeutic concentration 500 ng/mL
• Used as a measure of adherence to therapy
– Eliminated by the kidneys
• long tissue half-life of about 40 to 50 days
– Therapeutic effects
• 2-8 weeks
– Maximum clinical efficacy
• 3-6 months

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 Antimalarials
• Hydroxychloroquine
– Adverse effects
• Usually mild
• Most common are GI and skin reactions
– Improve with dose reduction
• Main concern is retinal toxicity……incidence is low
– Major risk factors
» Duration of use over 5 years
» Daily doses more than 5 mg/kg actual body weight
» concurrent use of tamoxifen, or
» patients with kidney dysfunction or preexisting macular
disease.
– Annual examinations…..after five years of drug use
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 Biologic Agents
• Target B cells

• Belimumab

– A fully human IgG1-λ monoclonal antibody

– Binds to soluble B-lymphocyte stimulator (BLyS), which

prevents BLyS from binding to receptors on B cells and
promotes apoptosis of B lymphocytes

– FDA-approved for treatment of auto-antibody positive

active SLE in addition to standard therapy
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 Biologic Agents
• Rituximab
– A chimeric monoclonal antibody directed at the CD20
antigen on B cells
– More effective
• In African descent and Hispanics with lupus nephritis
• In combination with cyclophosphamide instead of
mycophenolate mofetil
– It may serve as alternative therapy
• For refractory lupus nephritis, severe hematological
lupus, & some CNS manifestations of the disease.
– It may be useful for maintenance Rx, as steroid-sparing
agent, or when preservation of fertility is desired

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 Biologic Agents
• TNF-α inhibitors: infliximab and etanercept
– Short-term induction therapy with infliximab may confer
long-lasting benefits in patients with lupus nephritis.
– refractory lupus arthritis good response with etanercept
• Biologic drugs should not be combined
• Other drugs being investigated
– blisibimod, atacicept, sifalimumab and anifrolumab,
abatacept

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 Immunosuppressive Drugs
• Cyclophosphamide
– An alkylating agent that causes cross-linkage of DNA leading
to cell death
– Also suppress B cells and IgG production
– Decrease production of adhesion molecules and cytokines
– Used to treat severe organ involvement in SLE
• lupus nephritis, neuropsychiatric lupus, and severe
systemic vasculitis
– Oral bioavailability of 75-100%
– A prodrug………metabolized to active and inactive
metabolites via the cytochrome P450 enzyme system
– Primarily cleared by the liver, but its active metabolites may
persist in renal failure
51
 Immunosuppressive Drugs
• Cyclophosphamide
– ADR: hemorrhagic cystitis and bladder cancer
• Acrolein : a metabolite of the drug concentrates in the
bladder
• Higher risk
– oral administration, higher cumulative doses, and in
smokers.
– Hydration and frequent voiding may decrease the risk
• With oral administration
– Dose in the morning and drink fluids for several hours
• With IV administration
– IV fluids are begun before administration of the drug and
continued for several hours after
– Should maintain oral hydration for 72 hrs 52
 Immunosuppressive Drugs
• Cyclophosphamide
– Hemorrhagic cystitis
– Use mesna
• binds acrolein and prevents its harmful effects on the
bladder.
• IV doses: equivalent to 20% of the cyclophosphamide
dose
–15 to 30 minutes before the cyclophosphamide,
then 4 and 8 hours after
• Oral dose: equivalent to 40% of the administered
dose of cyclophosphamide 4 and 8 hours after
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 Immunosuppressive Drugs
• Mycophenolic acid (MPA)
– reversibly inhibits the enzyme inosine 5-monophosphate
dehydrogenase (IMPDH)
• which is important for synthesis of purine (guanosine)
nucleotides
– Inhibits lymphocyte proliferation, chemotaxis, and antibody
production
– Immunomodulating effects
• Induction of activated T-cell apoptosis
• inhibition of adhesion molecule expression, and
• antifibrotic and antiproliferative effects on cells such as
fibroblasts, dendritic cells, and vascular smooth muscle cells
– Mycophenolate mofetil hydrolyzed to MPA
– Bound to albumin, excreted renally 54
 Immunosuppressive Drugs
• Mycophenolate mofetil
– Used in nephritis, arthritis, cutaneous lupus, and
hematologic and cardiorespiratory involvement
– Can be steroid sparing
• Common ADE
– Infections and GI complaints including nausea, vomiting,
and diarrhea
• Might require discontinuation of therapy…..enteric
coated formulations
– Red cell aplasia ……. Diminish with dose reduction
• contraindicated in pregnancy
55
 Immunosuppressive Drugs
• Azathioprine
– A purine analog……metabolized to mercaptopurine
– Inhibits nucleic acid synthesis and affects cellular and
humoral immune functions
– Less effective than cyclophosphamide for induction
therapy in lupus nephritis
– Can be useful as an alternative to mycophenolate mofetil
for maintenance treatment.
– Used for SLE-related arthritis, serositis, and
mucocutaneous manifestations
– Steroid -sparing effects
– metabolism inhibited by allopurinol and febuxostat 56
 Immunosuppressive Drugs
Methotrexate

– Inhibitor of dihydrofolate reductase

• Needed for DNA synthesis and cell proliferation

– Toxicities……reduced by folic acid administration

– Dosed once weekly in the management of SLE

– Used for arthritis and skin disease and as a steroid

sparing drug

57
 Alternative Treatments
• To address unmet needs
– Psychosocial needs and may include anxiety or depression
• Assess the use of complementary and alternative medicine
• Supplementation of dehydroepiandrosterone (DHEA)…..weak
evidence
• Vitamin D level….decreased in SLE
– greater SLE disease activity, insulin resistance, osteoporosis and
fatigue
– vitamin D supplements
• At least 400 IU/day of vitamin D3
• For those on glucocorticoids for 3 months or more, 600 to 800
IU/day
• Goal 25(OH) vitamin D concentration……30 ng/mL…check after
58
three month
 Special Populations
Pregnancy and Contraception
• Pregnancy planning
• contraception counseling
– Timing of pregnancies with respect to disease activity and
use of teratogenic drugs
– Cyclophosphamide Rx ……ovarian failure and infertility
– Estrogen containing contraceptives…….thrombosis
– Use combined estrogen-progestin or progestin-only oral
contraceptives
• SLE is inactive or stable active and no antiphospholipid
antibodies
• As Hormone replacement therapy
– Intrauterine devices may be better choices for contraception
59
 Special Populations
Pregnancy
• Pregnancy during SLE is considered to be high risk
• High risk of
– maternal mortality, cesarean delivery, preterm labor, and
preeclampsia
– thrombotic, infectious, and hematologic complications
• The best pregnancy outcomes
– In patients who have had inactive disease for at least 6 months
prior to the pregnancy
• Hydroxychloroquine safe to be used
• Any potentially teratogenic drugs (eg, methotrexate, leflunomide,
mycophenolate, cyclophosphamide, and thalidomide)
– should be stopped at least 3 months before attempting
pregnancy. 60
 Special Populations
Pregnancy
• If a flare occurs and an immunosuppressive drug is required
during the pregnancy, azathioprine may be considered,
– Since the fetal liver is unable to metabolize it to its active
form.
– The dose should not exceed 2 mg/kg/day
• Calcineurin inhibitors (cyclosporine, tacrolimus) are
alternative choices.
• Supplements with calcium, vitamin D, and folic acid should
be offered.

61
 Special Populations
Pregnancy
• If corticosteroids are needed,
– Use the lowest possible dose
• To decrease the risk of gestational diabetes mellitus,
hypertension, infections, and premature rupture of
membranes
• Patients on long-term steroid therapy may need stress doses at
the time of delivery.
• Avoid fluorinated corticosteroids (dexamethasone or
betamethasone)
– Cross the placenta
• Cyclophosphamide should only be used during the 2nd or 3rd
trimester of pregnancy if alternatives failed and the mother’s
life is in danger.
• Avoid biologics…..limited data……..Rituximab??? 62
 SLE–Antiphospholipid Syndrome Overlap
• The antiphospholipid antibodies

– anticardiolipin, anti-β-2-glycoprotein I, and lupus

anticoagulant

– Promote clotting and pregnancy morbidity

– Diagnosis with one clinical and one laboratory measures

• Clinical aspects: vascular events such as venous or arterial

thrombi and/or obstetric complications

• Laboratory criteria: antiphospholipid antibodies on two

separate occasions, 12 weeks apart 63
 SLE–Antiphospholipid Syndrome Overlap
• The obstetric complications meeting the criteria are
– three or more unexplained consecutive miscarriages before
the 10th week of gestation,
– one or more unexplained deaths of fetuses at or beyond the
10th week of gestation, and
– one or more births of infants before the 34th week of
gestation associated with eclampsia or severe preeclampsia
or features of placental insufficiency
• In deciding choice, intensity, and duration of treatment
– balance benefits with the patient’s risk of bleeding.
• Identify transient precipitating factors

64
Recommendations for Thromboprophylaxis in
Patients with SLE and Antiphospholipid
Antibodies

65
66
 Drug-Induced Lupus
• About 10-15% of cases of SLE can be attributed to drugs
• Idiosyncratic reactions precipitated by interplay of
– Genetic predisposition, concurrent illnesses, environmental
factors, and other drugs or foods
• Diagnosis
– Lupus-like findings in a patient with no history of the disease &
– temporal relationship with the drug
• onset at least one month after initiation and
• Improvement in symptoms within days to months after the
drug is discontinued
67
 Drug-Induced Lupus
• Positive ANA or anti-histone antibodies
• Drugs implicated
– Procainamide (20%) and hydralazine (5%-8%), quinindine
(moderate risk)
– Hydralazine doses over 200 mg/day or a cumulative dose of more
than 100 g
– It is more common in patients who are slow acetylators.
• Positive ANAs with these drugs is 80-90% and 50% for procainamide
and hydralazine respectively
• Low risk: minocycline, isoniazid, methyldopa, carbamazepine, and
chlorpromazine 68
 Drug-Induced Lupus
• Primary treatment for drug-induced lupus is stopping the
implicated drug

• Use corticosteroids or other topical or systemic drugs based

on type and severity of the manifestations.

• If patients do not improve, a diagnosis of idiopathic SLE

should be considered.

69
 Immunizations
• Patients with SLE are at increased risk for infections

– Immune dysfunction caused by the disease itself and

– The immunosuppressive therapy the patients receive

• Vaccines should be administered when SLE is stable and prior to

initiating immunosuppressive medications

• Killed vaccines are considered safe in immunosuppressed patients.

70
 Immunizations
• SLE patient should receive
– Pneumococcal vaccine
– annual influenza vaccine
– Haemophilus influenzae and meningococcal vaccines
• Patients with splenectomy
– hepatitis B vaccine……. Those considered to be at risk
• Live-attenuated virus vaccines, such as measles–mumps–rubella,
varicella, zoster, intranasal influenza, and yellow fever, are
contraindicated in patients receiving biologic agents or high dose of
immunosuppressive therapy
• The recombinant zoster vaccine can be given during therapy with
immunosuppressive drugs but should not be given during pregnancy
• prednisone 20 mg/day or more given for at least 2
weeks……..considered immunosuppressive 71
 Evaluation of Therapeutic Outcomes
• The physician global assessment (PGA) scale
– assesses patients’ general health status
• Different tools
• HRQoL…………SF-36
• SLE manifestations, drug toxicity, and comorbidities
• Those with active disease should be assessed at least every 1 to 3
months with
– BP, urinalysis, renal function, anti-dsDNA antibodies, complement
concentrations, CRP , complete blood count, and liver function
tests with further testing as warranted.
• Clinical and laboratory assessments should be performed every 6 to
12 months in patients with inactive disease and no organ damage,
and more frequently if abnormalities are found
72
Monitoring of Drugs Used to Treat SLE

73
Monitoring of Drugs Used to Treat SLE

74
Monitoring of Drugs Used to Treat SLE

75