05/04/2022 06:12 Overview of and approach to the vasculitides in adults - UpToDate

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Overview of and approach to the vasculitides in adults

Author: Peter A Merkel, MD, MPH
Section Editor: Eric L Matteson, MD, MPH
Deputy Editor: Monica Ramirez Curtis, MD, MPH

All topics are updated as new evidence becomes available and our peer review process is complete.

Literature review current through: Mar 2022. | This topic last updated: Mar 30, 2021.

INTRODUCTION

The vasculitides are defined by the presence of inflammatory leukocytes in vessel walls with
reactive damage to mural structures. Both loss of vessel integrity leading to bleeding, and
compromise of the lumen may result in downstream tissue ischemia and necrosis. In
general, affected vessels vary in size, type, and location in association with the specific type
of vasculitis. Vasculitis may occur as a primary process or may be secondary to another
underlying disease. The exact pathogenetic mechanisms underlying these diseases are
unknown.

The vasculitides are often serious and sometimes fatal diseases that require prompt
recognition and therapy. Symptomatic involvement generally reflects and follows the pattern
of affected organs. The distribution of affected organs may suggest a particular type of
vasculitis.

This topic will review the nomenclature of the different vasculitides and provide an overview
of the approach to the patient with suspected vasculitis. An overview of the treatment of
these disorders and detailed discussions of the individual disorders are presented
separately. (See "Overview of the management of vasculitis in adults".)

NOMENCLATURE

The disease names and definitions of the vasculitides continue to evolve as our
understanding of the pathogenesis advances. The international Chapel Hill Consensus
Conference (CHCC) has developed one of the most widely used nomenclature systems which
specifies the names and definitions for most forms of vasculitis [1-3]. The CHCC
nomenclature system has changed over the past few decades, and definitions that were put
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forth by the CHCC in 1994 have since been revised in the 2012 CHCC ( table 1 and
table 2) with a specific addendum issued to cover the many forms of vasculitis of the skin
[3].

Among the notable changes in the 2012 CHCC was the preferential use and adoption of new
names for several diseases, consistent with the trend of replacing eponyms with disease
names that reflect an increased pathophysiologic understanding of these conditions. Among
the name changes are: eosinophilic granulomatosis with polyangiitis, abbreviated EGPA, in
place of Churg-Strauss syndrome; granulomatosis with polyangiitis, abbreviated GPA, in
place of Wegener's granulomatosis; immunoglobulin A (IgA) vasculitis (Henoch-Schönlein),
abbreviated as IgAV, in place of Henoch-Schönlein purpura (HSP); anti-C1q vasculitis as an
alternative name for hypocomplementemic urticarial vasculitis, abbreviated HUV; and use of
the term "cryoglobulinemic vasculitis" in place of "essential cryoglobulinemic vasculitis."
Furthermore, the 2012 CHCC formally adopted the term antineutrophil cytoplasmic antibody
(ANCA)-associated vasculitis (AAV) for the group of three disorders that include microscopic
polyangiitis (MPA), GPA, and EGPA, with additional categories also named to describe
variable-vessel vasculitis and secondary forms of vasculitis. This nomenclature system is not
meant to substitute for classification criteria, which include clinical observations that classify
a specific patient into a category for a research purposes. (See 'Classification criteria' below.)

MAJOR CATEGORIES OF VASCULITIS

Classification of the noninfectious vasculitides is primarily based upon the predominant size
of the vessels involved ( figure 1), although there may be some overlap in the size of
arteries involved with all these diseases. Thus, large-vessel vasculitis, as its name suggests,
mostly affects large arteries. The same principle is true for the medium- and small-vessel
vasculitides in which medium-vessel vasculitis predominantly affects medium arteries and
small-vessel vasculitis predominantly affects small arteries and capillaries. The Chapel Hill
Consensus Conference (CHCC) also recognizes that some forms of vasculitis do not involve a
single predominant size of vessel (variable-vessel vasculitis).

Large-vessel vasculitis

Takayasu arteritis — Takayasu arteritis primarily affects the aorta and its major branches.
The inflammation and damage is often localized to a portion of the affected vessels, but
extensive involvement such as nearly pan-aortitis can be seen. The onset of disease usually
occurs before the age of 30 years. (See "Clinical features and diagnosis of Takayasu
arteritis".)

Giant cell arteritis — Giant cell arteritis (GCA), also known as temporal arteritis,
predominantly affects the aorta and/or its major branches, with a predilection for the
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branches of the carotid including the superficial temporal artery. The onset of disease
usually occurs in patients older than 50, with markedly increased incidence in the eighth and
ninth decades of life. (See "Clinical manifestations of giant cell arteritis".)

There are other forms of large-vessel vasculitis that either do not have a specific name, such
as idiopathic isolated aortitis, or are part of another form of vasculitis or systemic
inflammatory condition, such as Cogan syndrome or relapsing polychondritis. (See "Clinical
manifestations of giant cell arteritis", section on 'Large vessel involvement' and "Cogan
syndrome", section on 'Systemic vasculitis' and "Clinical manifestations of relapsing
polychondritis", section on 'Systemic vasculitis'.)

Medium-vessel vasculitis

Polyarteritis nodosa — Polyarteritis nodosa is a systemic necrotizing vasculitis that

typically affects the medium- and small-sized arteries. (See "Clinical manifestations and
diagnosis of polyarteritis nodosa in adults".)

Kawasaki disease — Kawasaki disease is an arteritis that predominantly affects the

medium and small arteries, but the aorta and large arteries may also be involved. The
disease usually occurs in children and is often associated with a mucocutaneous lymph node
syndrome. The coronary arteries may also be involved. A small number of cases have also
been reported in adults [4]. (See "Kawasaki disease: Clinical features and diagnosis".)

Small-vessel vasculitis

ANCA-associated vasculitis — Antineutrophil cytoplasmic antibody (ANCA)-associated

vasculitis (AAV) is a necrotizing vasculitis that does not substantially involve the deposition of
immune complexes. AAV predominantly affects small vessels and is associated with ANCA
specific for myeloperoxidase (MPO-ANCA) or proteinase 3 (PR3-ANCA). Cases of ANCA-
negative AAV do occur, especially in eosinophilic granulomatosis with polyangiitis (EGPA) but
also to some extent in granulomatosis with polyangiitis (GPA). ANCA-negative AAV describes
cases in which the patient otherwise fulfills the definition for AAV but has negative results on
serologic testing for ANCA. (See "Clinical spectrum of antineutrophil cytoplasmic
autoantibodies".)

The major clinicopathologic variants of AAV include microscopic polyangiitis (MPA), GPA, and
EGPA; additionally, AAV can occur in only a single organ, especially a subset referred to as
renal-limited AAV.

Microscopic polyangiitis — MPA is a necrotizing vasculitis that primarily affects

capillaries, venules, or arterioles, most commonly manifesting as necrotizing
glomerulonephritis and/or pulmonary capillaritis. Involvement of medium- and small- sized
arteries may also be present. Granulomatous inflammation is usually absent. ANCA is
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present in >90 percent of patients with MPA. (See "Granulomatosis with polyangiitis and
microscopic polyangiitis: Clinical manifestations and diagnosis".)

Granulomatosis with polyangiitis — GPA is a necrotizing vasculitis predominantly

involving small- to medium-sized vessels (eg, capillaries, venules, arterioles, arteries, and
veins). It typically produces granulomatous inflammation of the upper and lower respiratory
tracts as well as necrotizing, pauci-immune glomerulonephritis. ANCA is present in >80
percent of patients with GPA. (See "Granulomatosis with polyangiitis and microscopic
polyangiitis: Clinical manifestations and diagnosis".)

While MPA and GPA continue be regarded as distinct entities within AAV, they have markedly
overlapping manifestations and it can be sometimes extremely difficult to differentiate
between these two diseases within a patient. Furthermore, there is a growing recognition
that ANCA type (anti-MPO or anti-PR3) has more prognostic and clinical meaning rather than
the disease type (MPA or GPA), leading some experts to refer to MPO-AAV or PR3-AAV, and
many clinical trials in AAV now stratify enrollment by ANCA type (MPO or PR3) and report
results for each subgroup.

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) — EGPA is an

eosinophilic-rich necrotizing vasculitis predominantly affecting small- to medium-sized
vessels. Patients often have chronic rhinosinusitis, asthma, and prominent peripheral blood
eosinophilia. ANCA is present in approximately 40 percent of patients with EGPA, usually
anti-MPO ANCA. The presence of ANCA is more frequent in patients with glomerulonephritis.
(See "Epidemiology, pathogenesis, and pathology of eosinophilic granulomatosis with
polyangiitis (Churg-Strauss)".)

Immune complex small-vessel vasculitis — Immune complex small-vessel vasculitis

refers to vasculitis with moderate to marked vessel wall deposits of immunoglobulin and/or
complement, predominantly affecting small vessels. Glomerulonephritis is often present.
Medium-sized arterial involvement is much less common in immune complex vasculitis
compared with AAV.

Anti-glomerular basement membrane disease — Anti-glomerular basement

membrane (GBM) disease is a vasculitis affecting glomerular capillaries, pulmonary
capillaries, or both, with basement membrane deposition of anti-basement membrane
autoantibodies. Lung involvement typically causes pulmonary hemorrhage, and renal
involvement causes glomerulonephritis with necrosis and crescents. (See "Anti-GBM
(Goodpasture) disease: Pathogenesis, clinical manifestations, and diagnosis".)

Cryoglobulinemic vasculitis — Cryoglobulinemic vasculitis, previously termed

essential cryoglobulinemic vasculitis, is characterized by the presence of cryoglobulins,
which are serum proteins that precipitate in the cold and dissolve upon rewarming. In this

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disorder, which is most often due to hepatitis C virus infection, cryoglobulin immune
complexes are deposited in the walls of capillaries, venules, or arterioles, thereby resulting in
inflammation in small vessels. Skin, glomeruli, and peripheral nerves are often involved. (See
"Overview of cryoglobulins and cryoglobulinemia" and "Mixed cryoglobulinemia syndrome:
Clinical manifestations and diagnosis".)

IgA vasculitis (Henoch-Schönlein purpura) — Immunoglobulin A (IgA) vasculitis is a

systemic vasculitis characterized by the tissue deposition of IgA1-dominant immune
complexes affecting mostly small vessels (predominantly capillaries, venules, or arterioles).
IgA vasculitis typically affects the skin and gastrointestinal tract, and often causes arthritis. A
glomerulonephritis indistinguishable from IgA nephropathy may be observed. (See "IgA
vasculitis (Henoch-Schönlein purpura): Clinical manifestations and diagnosis".)

Hypocomplementemic urticarial vasculitis (anti-C1q

vasculitis) — Hypocomplementemic urticarial vasculitis (HUV) is a vasculitis associated with
urticaria and hypocomplementemia, and predominantly affects small vessels.
Glomerulonephritis, arthritis, obstructive pulmonary disease, and ocular inflammation may
also be observed. The presence of anti-C1q antibodies is one of the most distinctive findings
in HUV, and consideration of the name anti-C1q vasculitis for this entity has been suggested.
(See "Urticarial vasculitis", section on 'Hypocomplementemic urticarial vasculitis'.)

Variable-vessel vasculitis

Behçet syndrome — The vasculitis occurring in patients with Behçet syndrome can affect
any size artery or vein. Behçet syndrome is characterized by recurrent oral and/or genital
aphthous ulcers as well as cutaneous, ocular, articular, gastrointestinal, and/or central
nervous system involvement. Thrombosis and arterial aneurysms can also occur. (See
"Clinical manifestations and diagnosis of Behçet syndrome", section on 'Vascular disease'.)

Cogan syndrome — Vasculitis occurring in patients with Cogan syndrome is characterized

by ocular inflammatory lesions, including interstitial keratitis, uveitis, and episcleritis as well
as inner ear disease (eg, sensorineural hearing loss and vestibular dysfunction). Vasculitis
manifestations may include arteritis of any size vessel, aortitis, and aortic aneurysms. (See
"Cogan syndrome".)

Single-organ vasculitis — Single-organ vasculitis refers to vasculitis in arteries or veins of

any size in a single organ, and has no features suggesting it is a limited expression of a
systemic vasculitis. The involved organ and vessel type should be included in the name (eg,
primary central nervous system vasculitis [CNSV], cutaneous small vessel vasculitis [5],
isolated aortitis). Some patients initially diagnosed with single-organ vasculitis may develop
other disease manifestations, warranting reevaluation for another systemic vasculitis (eg,
cutaneous arteritis later becoming polyarteritis nodosa).

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Primary central nervous system vasculitis — Primary CNSV, or primary angiitis of the

CNS (PACNS), refers to vasculitis affecting the medium and small blood vessels of the brain,
spinal cord, and the meninges, without systemic (non-brain) involvement. (See "Primary
angiitis of the central nervous system in adults".)

Vasculitis associated with systemic disease — Subsets of patients with systemic lupus

erythematosus, rheumatoid arthritis, relapsing polychondritis, and other systemic rheumatic
diseases may develop an associated vasculitis. The vasculitic process in this setting most
frequently involves small muscular arteries, arterioles, and venules.

Vasculitis associated with probable etiology — Some of the vasculitides are associated

with a specific etiology and the diagnosis should have a prefix specifying the underlying
cause. Examples include hepatitis C virus-associated cryoglobulinemic vasculitis, hepatitis B
virus-associated polyarteritis nodosa, and hydralazine-associated ANCA-associated vasculitis.
Hematologic and solid organ neoplasms as well as clonal B cell lymphoproliferative
disorders can also be associated with vasculitis.

CLASSIFICATION CRITERIA

The classification of the vasculitides has been a challenging problem for decades [6]. In
1990, the American College of Rheumatology (ACR) proposed criteria for several types of
vasculitis as a means of categorizing patients for clinical research [7-12]. While these criteria
can be used to help inform the diagnosis, they lack sufficient sensitivity and specificity to be
used as diagnostic criteria [13]. The 1990 classification criteria can be found on American
College of Rheumatology website.

The European Medicines Agency (EMA) later developed an algorithm that was designed to
help classify the antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides as well
as polyarteritis nodosa for epidemiological studies, but also has its limitations [14]. The EMA
algorithm is discussed in detail separately. (See "Granulomatosis with polyangiitis and
microscopic polyangiitis: Clinical manifestations and diagnosis", section on 'Classification
criteria'.)

Although the ACR criteria, the EMA algorithm, and the Chapel Hill Consensus Conference
(CHCC) nomenclature system have been widely used by clinical researchers and clinicians to
help diagnose patients, accurate diagnostic criteria have yet to be developed. With an
increased understanding of the pathophysiology of vasculitis and improved laboratory
testing, the ACR and the European Alliance of Associations for Rheumatology (EULAR;
formerly known as European League Against Rheumatism) are in the process of making an
international effort to develop revised classification criteria and diagnostic criteria [15,16].

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CLINICAL FEATURES SUGGESTIVE OF SYSTEMIC VASCULITIS

It is not possible to outline a single algorithm for evaluating patients suspected of having
any one of the vasculitides because of the clinical heterogeneity of these diseases.
Nevertheless, there are some elements of the medical history, physical examination, and
laboratory evaluation that may be helpful when trying to identify a patient suspected of
having a vasculitis. While each form of vasculitis is rare, the potential for severe organ
damage or death from these diseases means it is appropriate to consider the possibility
early in the evaluation of patients with any possible manifestations of vasculitis.

In general, the presence of vasculitis should be considered in patients who present with
systemic or constitutional symptoms in combination with evidence of single and/or
multiorgan dysfunction, and especially with some key manifestations as outlined below. The
diagnosis of vasculitis is often delayed because the clinical manifestations can be mimicked
by a number of other diseases.

Diagnostic approach — The diagnosis of the individual vasculitides is generally based on

patterns of organ injury, the size of the vessels affected, histopathological features, and
characteristic findings on diagnostic imaging.

History — Although neither sensitive nor specific for the diagnosis of vasculitis, systemic
symptoms such as fever, fatigue, weight loss, and arthralgias are often present in patients
with vasculitis. A history of eye inflammation, particularly scleritis, are features sometimes
observed in vasculitis. Persistent nasal crusting, epistaxis, or other upper airway disease is
suggestive of granulomatosis with polyangiitis (GPA). The presence of acute foot drop or
wrist drop may be due to a motor neuropathy from an ischemic process. Limb claudication,
especially in the upper extremities or in a person at low risk for atherosclerosis, is suggestive
of a large arterial occlusion from Takayasu arteritis or giant cell arteritis (GCA).

Unexplained hemoptysis should always raise concern for alveolar hemorrhage and
antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Similarly, any
patients with suspected glomerulonephritis must be evaluated for possible vasculitis,
especially AAV or anti-glomerular basement membrane (GBM) disease. The combination of
lung hemorrhage and renal insufficiency (often referred to as "pulmonary-renal syndrome")
should immediately raise concern for vasculitis [17].

A detailed history is also important to assess whether the patient has recently (up to at least
some time in the prior 6 to 12 months) been exposed to specific medications or cocaine
which may be associated with drug-induced vasculitis, has a history of hepatitis, or has been
diagnosed with any disorder known to be associated with a vasculitis (such as systemic lupus
erythematosus). (See "Clinical spectrum of antineutrophil cytoplasmic autoantibodies",

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section on 'Drug-induced ANCA-associated vasculitis' and "Clinical spectrum of

antineutrophil cytoplasmic autoantibodies", section on 'Cocaine and levamisole'.)

The propensity of certain disorders to occur among certain age groups and/or in women
may favor the diagnosis of a specific vasculitides ( table 3). In a review of 807 patients from
the American College of Rheumatology (ACR) cohort, the mean age at onset was between 45
and 50 for GPA and polyarteritis nodosa compared with 17 and 26 years of age for
immunoglobulin A (IgA) vasculitis and Takayasu arteritis and with 69 years for GCA [7]. GCA
and especially Takayasu arteritis are observed more frequently in women.

Physical examination — A careful physical examination helps identify potential sites of

vasculitis and determine the extent of vascular lesions, the distribution of affected organs,
and the presence of additional disease processes. Certain findings, such as diminished
sensation to touch and motor weakness of the extremities due to neuropathic changes
consistent with mononeuritis multiplex and palpable purpura ( picture 1), are highly
suggestive of an underlying vasculitic process.

● Findings of a sensory and/or motor neuropathy – Both subtle and extensive

neuropathies can occur in many forms of vasculitis. These include classical
mononeuritis multiplex and peripheral symmetric or asymmetric polyneuropathy. (See
"Clinical manifestations and diagnosis of vasculitic neuropathies".)

● Palpable purpura – Palpable purpura is a strong sign of cutaneous leukocytoclastic

vasculitis ( picture 1) and is a common finding in many small-vessel vasculitides as
well as polyarteritis nodosa (see "Approach to the patient with retiform (angulated)
purpura", section on 'Vessel wall pathology' and "Evaluation of adults with cutaneous
lesions of vasculitis", section on 'When to suspect cutaneous vasculitis'). However, it is
important to recognize that not all palpable purpura is vasculitis and not all vasculitis in
the skin is represented as purpura.

● Absent, diminished, or tender pulses, bruits, or blood pressure discrepancies – A

careful and full vascular examination is extremely helpful in identifying signs of large-
vessel vasculitis [18]. The vascular examination should include palpation for pulses in
multiple areas including, but not limited to, the radial, brachial, carotid, femoral,
popliteal, posterior tibial, and dorsalis pedis arteries, and auscultation for bruits in the
regions of the carotid, subclavian, renal, and femoral arteries and the thoracic and
abdominal aorta.

Laboratory tests — Some laboratory tests may help identify the type of vasculitis, the
degree of organ involvement, or identify another disease. The initial laboratory evaluation of
a patient suspected of having vasculitis should include a complete blood cell count (CBC),
serum creatinine, liver function studies, erythrocyte sedimentation rate (ESR) and/or C-

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reactive protein (CRP), serologies for viral hepatitis, serum cryoglobulins, and a urinalysis
with urinary sediment. Blood cultures should be drawn to help exclude infection (eg,
infective endocarditis).

Additional, more specific laboratory testing that may further aid in the diagnosis include:

● Antinuclear antibody (ANA) – A positive ANA test may support the presence of an
underlying systemic rheumatic disease such as systemic lupus erythematosus. (See
"Measurement and clinical significance of antinuclear antibodies".)

● Complement – Low serum complement levels, especially low C4, may be present in
mixed cryoglobulinemia and systemic lupus erythematosus but not in most other
forms of vasculitis. (See "Overview and clinical assessment of the complement system"
and "Overview of cryoglobulins and cryoglobulinemia".)

● ANCA – Although not fully diagnostic on its own, the presence of ANCA directed against
either protease 3 (PR3) or myeloperoxidase (MPO) is extremely specific (often >95
percent) for a diagnosis of AAV in patients with some reasonable pre-test suspicion.
(See "Clinical spectrum of antineutrophil cytoplasmic autoantibodies".)

Additional tests — Additional testing should be guided by the clinical presentation.

Examples of such studies include:

● A chest radiograph or high-resolution computed tomography (HRCT) of the chest is

indicated in patients with respiratory symptoms and/or hemoptysis.

● Electromyography is useful if symptoms of neuromuscular disease are present, such as

findings suggestive of a mononeuritis multiplex. (See "Overview of electromyography".)

● A lumbar puncture with cerebral spinal fluid analysis should be considered in patients
with symptoms suggestive of primary angiitis of the central nervous system (PACNS).
(See "Primary angiitis of the central nervous system in adults", section on 'Lumbar
puncture'.)

Biopsy — Biopsy examination of the involved tissue is essential for diagnosis of many

vasculitides, but is not possible in all cases. For example, a temporal artery biopsy should
always be performed in cases of suspected GCA and is a generally a straightforward
procedure (see "Temporal artery biopsy technique"). Similarly, skin biopsies of purpuric
lesions and renal biopsies in patients with suspected glomerulonephritis have high
diagnostic yields. However, for patients with suspected Takayasu arteritis, except in cases
where surgical repair was conducted, tissue from the aortic arch and its primary branches is
not feasible and the diagnosis is based on other clinical and radiographic findings.

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Vascular imaging — Magnetic resonance imaging (MRI), MR angiograms, CT angiograms,

vascular ultrasound, and positron emission tomography (PET) may be used to detect large
artery lesions and, especially CT and MRI, have become the standard approach to screening
for large-vessel vasculitis. There is increasing adoption of using ultrasound to detect possible
giant cell arteritis, especially in countries and centers with the experience and expertise to
conduct ultrasound of the temporal arteries. PET is sometimes useful in identifying potential
inflammatory disease in the aorta and its branches. However, while more invasive imaging
studies generally can be avoided, conventional catheter-based angiography remains an
important diagnostic test in some situations. It is important to realize that no angiographic
abnormalities are themselves pathognomonic for vasculitis, but they can be used to support
a diagnosis when combined with other clinical data. Angiograms of mesenteric or renal
arteries in polyarteritis nodosa may show aneurysms, occlusions, and vascular wall
irregularities ( image 1). By contrast, angiography is unlikely to be helpful in assessing a
small-vessel vasculitis because the affected vessels are below the resolution of usual
angiograms.

DIFFERENTIAL DIAGNOSIS

Patients with nonvasculitic disease processes may present with symptoms and findings that
closely mimic various vasculitides. Perhaps most common are systemic rheumatic diseases,
such as systemic lupus erythematosus, atherosclerotic disease, drug reactions, and vaso-
occlusive processes. Among the most important diseases to exclude are infections and
malignancies, since the immunosuppressive therapy could worsen these conditions and a
delay in diagnosis can be extremely dangerous. While it is beyond the scope of this review to
provide a comprehensive list of all possible alternative diagnoses, we present several
categories of mimics of vasculitis in a table ( table 4) [19-21].

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
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Here is the patient education article that is relevant to this topic. We encourage you to print
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● Basics topics (see "Patient education: Polyarteritis nodosa (The Basics)" and "Patient
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● Beyond the Basics topics (see "Patient education: Vasculitis (Beyond the Basics)")

SUMMARY AND RECOMMENDATIONS

● The vasculitides are defined by the presence of inflammatory leukocytes in vessel walls
with reactive damage to mural structures. Both loss of vessel integrity leading to
bleeding, and compromise of the lumen may result in downstream tissue ischemia and
necrosis. In general, affected vessels vary in size, type, and location in association with
the specific type of vasculitis. Vasculitis may occur as a primary process or may be
secondary to another underlying disease. (See 'Introduction' above.)

● The international Chapel Hill Consensus Conference (CHCC) has developed one of the
most widely used nomenclature systems, which specifies the names and definitions for
most forms of vasculitis ( table 2). (See 'Nomenclature' above.)

● Classification of the noninfectious vasculitides is primarily based upon the predominant

size of the vessels involved ( figure 1), although some overlap in the size of arteries
involved occurs among the major size-based categories. There are also some forms of
vasculitis that do not involve a single predominant size of vessel (variable-vessel
vasculitis) (see 'Major categories of vasculitis' above). The major categories of the
vasculitides include:

• Large-vessel vasculitis – Takayasu arteritis and giant cell arteritis (GCA) (see 'Large-
vessel vasculitis' above)

• Medium-vessel vasculitis – Polyarteritis nodosa and Kawasaki disease (see 'Medium-

vessel vasculitis' above)

• Small-vessel vasculitis – Microscopic polyangiitis, granulomatosis with polyangiitis

(GPA), eosinophilic granulomatosis with polyangiitis (EGPA; Churg-Strauss), anti-
glomerular basement membrane (GBM) disease, cryoglobulinemic vasculitis,
immunoglobulin A (IgA) vasculitis (Henoch-Schönlein purpura [HSP]), and
hypocomplementemic urticarial vasculitis (anti-C1q-vasculitis). (See 'Small-vessel
vasculitis' above.)

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• Variable-vessel vasculitis – Behçet syndrome and Cogan syndrome (see 'Variable-

vessel vasculitis' above)

• Single-organ vasculitis – Primary central nervous system vasculitis (CNSV) (see

'Single-organ vasculitis' above)

• Vasculitis associated with systemic disease such as systemic lupus erythematosus,

rheumatoid arthritis, and relapsing polychondritis (see 'Vasculitis associated with
systemic disease' above)

• Vasculitis associated with probable etiology such as hepatitis C virus-associated

cryoglobulinemic vasculitis, hepatitis B virus-associated polyarteritis nodosa, and
hydralazine-associated antineutrophil cytoplasmic antibody (ANCA)-associated
vasculitis (see 'Vasculitis associated with probable etiology' above)

● The American College of Rheumatology (ACR), the European Medical Agency (EMA), and
the CHCC nomenclature system have been widely used by clinical researchers and
clinicians to help diagnose patients, but accurate diagnostic criteria have yet to be
developed. While these classification criteria can be used to help inform the diagnosis,
they lack sufficient sensitivity and specificity to be used as diagnostic criteria. (See
'Classification criteria' above.)

● In general, the presence of vasculitis should be considered in patients who present

with systemic or constitutional symptoms in combination with evidence of single
and/or multiorgan dysfunction. The diagnosis of the individual vasculitides is generally
based on patterns of organ injury, the size of the vessels affected, histopathological
features, and characteristic findings on diagnostic imaging. (See 'Clinical features
suggestive of systemic vasculitis' above and 'Diagnostic approach' above.)

● Diagnostic evaluation for a case of possible vasculitis should include a detailed history,
including drug use, infectious disease exposure, and symptoms of manifestations that
may characterize or exclude a suspected diagnoses; a careful physical examination to
identify potential sites of involvement of vasculitis and determine the extent of vascular
lesions; general laboratory testing to help identify the degree of organ involvement
and exclude another disease; additional laboratory testing, depending on the
suspected diagnosis and findings, such as tests for antinuclear antibodies (ANA),
complement levels, ANCA; a chest radiograph or high-resolution computed
tomography (HRCT) of the chest; electromyography; a lumbar puncture; a biopsy of the
involved tissue if possible; and vascular imaging. (See 'Diagnostic approach' above.)

● Patients with nonvasculitic disease processes may present with symptoms and findings
that closely mimic various vasculitides. Perhaps most common are systemic rheumatic

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diseases, such as systemic lupus erythematosus, atherosclerotic disease, drug

reactions, and vaso-occlusive processes. Among the most important diseases to
exclude are infections and malignancies since immunosuppressive therapy could
worsen these conditions and a delay in diagnosis can be extremely dangerous. While it
is beyond the scope of this review to provide a comprehensive list of all possible
alternative diagnoses, we present several categories of mimics of vasculitis in a table (
table 4). (See 'Differential diagnosis' above.)

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REFERENCES

1. Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus
Conference Nomenclature of Vasculitides. Arthritis Rheum 2013; 65:1.

2. Jennette JC, Falk RJ, Andrassy K, et al. Nomenclature of systemic vasculitides. Proposal of
an international consensus conference. Arthritis Rheum 1994; 37:187.

3. Sunderkötter CH, Zelger B, Chen KR, et al. Nomenclature of Cutaneous Vasculitis:

Dermatologic Addendum to the 2012 Revised International Chapel Hill Consensus
Conference Nomenclature of Vasculitides. Arthritis Rheumatol 2018; 70:171.
4. Gomard-Mennesson E, Landron C, Dauphin C, et al. Kawasaki disease in adults: report
of 10 cases. Medicine (Baltimore) 2010; 89:149.

5. Loricera J, Blanco R, Ortiz-Sanjuán F, et al. Single-organ cutaneous small-vessel vasculitis

according to the 2012 revised International Chapel Hill Consensus Conference
Nomenclature of Vasculitides: a study of 60 patients from a series of 766 cutaneous
vasculitis cases. Rheumatology (Oxford) 2015; 54:77.

6. Watts RA, Suppiah R, Merkel PA, Luqmani R. Systemic vasculitis--is it time to reclassify?
Rheumatology (Oxford) 2011; 50:643.
7. Hunder GG, Arend WP, Bloch DA, et al. The American College of Rheumatology 1990
criteria for the classification of vasculitis. Introduction. Arthritis Rheum 1990; 33:1065.

8. Leavitt RY, Fauci AS, Bloch DA, et al. The American College of Rheumatology 1990
criteria for the classification of Wegener's granulomatosis. Arthritis Rheum 1990;
33:1101.

9. Masi AT, Hunder GG, Lie JT, et al. The American College of Rheumatology 1990 criteria
for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis).
Arthritis Rheum 1990; 33:1094.

10. Lightfoot RW Jr, Michel BA, Bloch DA, et al. The American College of Rheumatology 1990
criteria for the classification of polyarteritis nodosa. Arthritis Rheum 1990; 33:1088.

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11. Arend WP, Michel BA, Bloch DA, et al. The American College of Rheumatology 1990
criteria for the classification of Takayasu arteritis. Arthritis Rheum 1990; 33:1129.

12. Hunder GG, Bloch DA, Michel BA, et al. The American College of Rheumatology 1990
criteria for the classification of giant cell arteritis. Arthritis Rheum 1990; 33:1122.

13. Rao JK, Allen NB, Pincus T. Limitations of the 1990 American College of Rheumatology
classification criteria in the diagnosis of vasculitis. Ann Intern Med 1998; 129:345.
14. Watts R, Lane S, Hanslik T, et al. Development and validation of a consensus
methodology for the classification of the ANCA-associated vasculitides and polyarteritis
nodosa for epidemiological studies. Ann Rheum Dis 2007; 66:222.

15. Craven A, Robson J, Ponte C, et al. ACR/EULAR-endorsed study to develop Diagnostic

and Classification Criteria for Vasculitis (DCVAS). Clin Exp Nephrol 2013; 17:619.

16. Luqmani RA, Suppiah R, Grayson PC, et al. Nomenclature and classification of vasculitis -
update on the ACR/EULAR diagnosis and classification of vasculitis study (DCVAS). Clin
Exp Immunol 2011; 164 Suppl 1:11.
17. Niles JL, Böttinger EP, Saurina GR, et al. The syndrome of lung hemorrhage and nephritis
is usually an ANCA-associated condition. Arch Intern Med 1996; 156:440.

18. Grayson PC, Tomasson G, Cuthbertson D, et al. Association of vascular physical

examination findings and arteriographic lesions in large vessel vasculitis. J Rheumatol
2012; 39:303.

19. Bateman H, Rehman A, Valeriano-Marcet J. Vasculitis-like Syndromes. Curr Rheumatol

Rep 2009; 11:422.
20. Molloy ES, Langford CA. Vasculitis mimics. Curr Opin Rheumatol 2008; 20:29.

21. Zarka F, Veillette C, Makhzoum JP. A Review of Primary Vasculitis Mimickers Based on the
Chapel Hill Consensus Classification. Int J Rheumatol 2020; 2020:8392542.
Topic 8226 Version 31.0

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GRAPHICS

Names for vasculitides adopted by the 2012 International Chapel Hill

Consensus Conference on the Nomenclature of Vasculitides

Large-vessel vasculitis

Takayasu arteritis

Giant cell arteritis

Medium-vessel vasculitis

Polyarteritis nodosa

Kawasaki disease

Small-vessel vasculitis
ANCA-associated vasculitis

Microscopic polyangiitis

Granulomatosis with polyangiitis (Wegener's)

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)

Immune complex small-vessel vasculitis

Anti-glomerular basement membrane disease

Cryoglobulinemic vasculitis

IgA vasculitis (Henoch-Schönlein)

Hypocomplementemic urticarial vasculitis (anti-C1q vasculitis)

Variable-vessel vasculitis

Behçet's syndrome

Cogan's syndrome

Single-organ vasculitis
Cutaneous leukocytoclastic angiitis

Cutaneous arteritis

Primary central nervous system vasculitis

Isolated aortitis

Others

Vasculitis associated with systemic disease

Lupus vasculitis

Rheumatoid vasculitis

Sarcoid vasculitis

Others
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Vasculitis associated with probable etiology

Hepatitis C virus-associated cryoglobulinemic vasculitis

Hepatitis B virus-associated vasculitis

Syphilis-associated aortitis

Drug-associated immune complex vasculitis

Drug-associated ANCA-associated vasculitis

Cancer-associated vasculitis

Others

ANCA: antineutrophil cytoplasmic antibody; IgA: immunoglobulin A.

Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of
Vasculitides. Arthritis Rheum 2013; 65:1. Reproduced with permission from John Wiley & Sons, Inc. Copyright © 2013 by the
American College of Rheumatology. All rights reserved.

Graphic 90188 Version 4.0

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Definitions for vasculitides adopted by the 2012 International Chapel Hill
Consensus Conference on the Nomenclature of Vasculitides (CHCC2012)
CHCC2012 name
Large-vessel vasculitis
Takayasu arteritis (TAK)
Giant cell arteritis (GCA)
Medium-vessel vasculitis
Polyarteritis nodosa (PAN)
Kawasaki disease (KD)
Small-vessel vasculitis
ANCA-associated
vasculitis (AAV)
Microscopic polyangiitis
(MPA)
Granulomatosis with
polyangiitis (Wegener's)
(GPA)
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Overview of and approach to the vasculitides in adults - UpToDate
CHCC2012 definition
Vasculitis affecting large arteries more often than other vasculitides.
Large arteries are the aorta and its major branches. Any size artery
may be affected.
Arteritis, often granulomatous, predominantly affecting the aorta
and/or its major branches. Onset usually in patients younger than 50
years.
Arteritis, often granulomatous, usually affecting the aorta and/or its
major branches, with a predilection for the branches of the carotid and
vertebral arteries. Often involves the temporal artery. Onset usually in
patients older than 50 years and often associated with polymyalgia
rheumatica.
Vasculitis predominantly affecting medium arteries defined as the
main visceral arteries and their branches. Any size artery may be
affected. Inflammatory aneurysms and stenoses are common.
Necrotizing arteritis of medium or small arteries without
glomerulonephritis or vasculitis in arterioles, capillaries, or venules,
and not associated with antineutrophil cytoplasmic antibodies (ANCAs).
Arteritis associated with the mucocutaneous lymph node syndrome
and predominantly affecting medium and small arteries. Coronary
arteries are often involved. Aorta and large arteries may be involved.
Usually occurs in infants and young children.
Vasculitis predominantly affecting small vessels, defined as small
intraparenchymal arteries, arterioles, capillaries, and venules. Medium
arteries and veins may be affected.
Necrotizing vasculitis, with few or no immune deposits, predominantly
affecting small vessels (ie, capillaries, venules, arterioles, and small
arteries), associated with myeloperoxidase (MPO) ANCA or proteinase 3
(PR3) ANCA. Not all patients have ANCA. Add a prefix indicating ANCA
reactivity, eg, MPO-ANCA, PR3-ANCA, ANCA-negative.
Necrotizing vasculitis, with few or no immune deposits, predominantly
affecting small vessels (ie, capillaries, venules, or arterioles).
Necrotizing arteritis involving small and medium arteries may be
present. Necrotizing glomerulonephritis is very common. Pulmonary
capillaritis often occurs. Granulomatous inflammation is absent.
Necrotizing granulomatous inflammation usually involving the upper
and lower respiratory tract, and necrotizing vasculitis affecting
predominantly small to medium vessels (eg, capillaries, venules,
arterioles, arteries and veins). Necrotizing glomerulonephritis is
common.
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Eosinophilic Eosinophil-rich and necrotizing granulomatous inflammation often

granulomatosis with involving the respiratory tract, and necrotizing vasculitis predominantly
polyangiitis (Churg- affecting small to medium vessels, and associated with asthma and
Strauss) (EGPA) eosinophilia. ANCA is more frequent when glomerulonephritis is
present.

Immune complex Vasculitis with moderate to marked vessel-wall deposits of

vasculitis immunoglobulin and/or complement components predominantly
affecting small vessels (ie, capillaries, venules, arterioles, and small
arteries). Glomerulonephritis is frequent.

Anti-glomerular Vasculitis affecting glomerular capillaries, pulmonary capillaries, or

basement membrane both, with GBM deposition of anti-GBM autoantibodies. Lung
(anti-GBM) disease involvement causes pulmonary hemorrhage, and renal involvement
causes glomerulonephritis with necrosis and crescents.

Cryoglobulinemic Vasculitis with cryoglobulin immune deposits affecting small vessels

vasculitis (CV) (predominantly capillaries, venules, or arterioles) and associated with
serum cryoglobulins. Skin, glomeruli, and peripheral nerves are often
involved.

IgA vasculitis (Henoch-
Schönlein) (IgAV)
Vasculitis, with IgA1-dominant immune deposits, affecting small
vessels (predominantly capillaries, venules, or arterioles). Often
involves skin and gastrointestinal tract, and frequently causes arthritis.
Glomerulonephritis indistinguishable from IgA nephropathy may
occur.

Hypocomplementemic Vasculitis accompanied by urticaria and hypocomplementemia

urticarial vasculitis (HUV) affecting small vessels (ie, capillaries, venules, or arterioles), and
(anti-C1q vasculitis) associated with anti-C1q antibodies. Glomerulonephritis, arthritis,
obstructive pulmonary disease, and ocular inflammation are common.

Variable-vessel vasculitis Vasculitis with no predominant type of vessel involved that can affect
vessels of any size (small, medium, and large) and type (arteries, veins,
and capillaries).

Behçet's syndrome Vasculitis occurring in patients with Behçet's syndrome that can affect

arteries or veins. Behçet's syndrome is characterized by recurrent oral
and/or genital aphthous ulcers accompanied by cutaneous, ocular,
articular, gastrointestinal, and/or central nervous system inflammatory
lesions. Small-vessel vasculitis, thromboangiitis, thrombosis, arteritis,
and arterial aneurysms may occur.

Cogan's syndrome Vasculitis occurring in patients with Cogan's syndrome. Cogan's

syndrome is characterized by ocular inflammatory lesions, including
interstitial keratitis, uveitis, and episcleritis, and inner ear disease,
including sensorineural hearing loss and vestibular dysfunction.
Vasculitic manifestations may include arteritis (affecting small,
medium, or large arteries), aortitis, aortic aneurysms, and aortic and
mitral valvulitis.

Single-organ vasculitis Vasculitis in arteries or veins of any size in a single organ that has no
features that indicate that it is a limited expression of a systemic
vasculitis. The involved organ and vessel type should be included in the

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name (eg, cutaneous small-vessel vasculitis, testicular arteritis, central

nervous system vasculitis). Vasculitis distribution may be unifocal or
multifocal (diffuse) within an organ. Some patients originally diagnosed
as having single-organ vasculitis will develop additional disease
manifestations that warrant redefining the case as one of the systemic
vasculitides (eg, cutaneous arteritis later becoming systemic
polyarteritis nodosa, etc).

Vasculitis associated with Vasculitis that is associated with and may be secondary to (caused by) a
systemic disease systemic disease. The name (diagnosis) should have a prefix term
specifying the systemic disease (eg, rheumatoid vasculitis, lupus
vasculitis, etc).

Vasculitis associated with
probable etiology
Vasculitis that is associated with a probable specific etiology. The name
(diagnosis) should have a prefix term specifying the association (eg,
hydralazine-associated microscopic polyangiitis, hepatitis B virus-
associated vasculitis, hepatitis C virus-associated cryoglobulinemic
vasculitis, etc).

Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of
Vasculitides. Arthritis Rheum 2013; 65:1. Reproduced with permission from John Wiley & Sons, Inc. Copyright © 2013 by the
American College of Rheumatology. All rights reserved.

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Distribution of vessel involvement by large-, medium-, and small-vessel

vasculitis

Distribution of vessel involvement by large-vessel vasculitis, medium-vessel vasculitis, and

small-vessel vasculitis. Note that there is substantial overlap with respect to arterial
involvement, and an important concept is that all three major categories of vasculitis can affect
any size artery. Large-vessel vasculitis affects large arteries more often than other vasculitides.
Medium-vessel vasculitis predominantly affects medium arteries. Small-vessel vasculitis
predominantly affects small vessels, but medium arteries and veins may be affected, although
immune-complex small-vessel vasculitis rarely affects arteries. Not shown is variable-vessel
vasculitis, which can affect any type of vessel, from aorta to veins. The diagram depicts (from
left to right) aorta, large artery, medium artery, small artery/arteriole, capillary, venule, and
vein.

IgA: immunoglobulin A; Anti-GBM: anti-glomerular basement membrane; ANCA: antineutrophil

cytoplasmic antibody.

Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of
Vasculitides. Arthritis Rheum 2013; 65:1. Reproduced with permission from John Wiley & Sons, Inc. Copyright © 2013
by the American College of Rheumatology. All rights reserved.

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Demographic characteristics of 807 patients with vasculitis

Percent with Mean age at Percent

Disease category
disorder disease onset female

Polyarteritis nodosa 15 48 38

Eosinophilic granulomatosis with 3 50 37

polyangiitis (Churg-Strauss)

Granulomatosis with polyangiitis 10 45 37

(Wegener's)

Hypersensitivity vasculitis 12 47 54

Henoch-Schonlein purpura (IgA 10 17 46

vasculitis)

Giant cell arteritis 26 69 75

Takayasu arteritis 8 26 86

Other vasculitis, type unspecified 16 44 55

Data from: Hunder GG, Arend WP, Bloch DA, et al. Arthritis Rheum 1990; 33:1065.

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Palpable purpura

Non-blanching, palpable purpuric skin lesions found over the lower

extremity of a patient with a small-vessel vasculitis affecting the skin.

Courtesy of Joseph F Merola, MD.

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Kidney arteriogram in polyarteritis nodosa

Kidney arteriogram in large-vessel polyarteritis nodosa showing

characteristic microaneurysms (small arrows) and abrupt cutoffs of
small arteries (large arrows).

From: Rose BD. Pathophysiology of Renal Disease, 2d ed, McGraw-Hill, New York,
1987.

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Major categories of mimics of vasculitis

Infectious causes (eg, endocarditis, HBV, HCV, HIV)

Atherosclerosis

Thromboembolic disease

Congenital causes (eg, aortic coarctation, middle aortic syndrome)

Hereditary disorders (eg, Marfan syndrome, Ehlers-Danlos syndrome)

Fibromuscular dysplasia

Hypercoagulable states (eg, APS, TTP)

Vasospastic disorders (eg, RCVS, drug exposures)

Other multisystem inflammatory disorders (eg, sarcoidosis, Susac syndrome)

Malignancy (eg, lymphoma, leukemia)

Iatrogenic (eg, postradiation therapy)

IgG4-related disease

HBV: hepatitis B virus; HCV: hepatitis C virus; HIV: human immunodeficiency virus; APS:
antiphospholipid syndrome; TTP: thrombotic thrombocytopenic purpura; RCVS: reversible cerebral
vasoconstriction syndrome; IgG4: immunoglobulin G4.

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Contributor Disclosures
Peter A Merkel, MD, MPH Grant/Research/Clinical Trial Support: AbbVie [Vasculitis]; AstraZeneca
[Vasculitis]; Boehringer Ingelheim [Scleroderma]; Bristol-Myers Squibb [Vasculitis]; ChemoCentryx
[Vasculitis]; Genentech/Roche [Vasculitis]; GlaxoSmithKline [Vasculitis]; InflaRx [Vasculitis]; Kypha
[Vasculitis]; MedImmune [Vasculitis]; Sanofi [Vasculitis]. Consultant/Advisory Boards: AbbVie
[Vasculitis]; AstraZeneca [Vasculitis]; Bristol-Myers Squibb [Vasculitis]; Boehringer Ingelheim
[Scleroderma]; ChemoCentryx [Vasculitis]; CSL Behring [Scleroderma, vasculitis]; Dynacure [Vasculitis];
EMDSerono [Vasculitis]; Forbius [Scleroderma]; Genentech/Roche [Vasculitis]; Genzyme/Sanofi
[Vasculitis]; GlaxoSmithKline [Vasculitis]; InflaRx [Vasculitis]; Janssen [Vasculitis]; Kiniksa [Vasculitis];
Kyverna [Scleroderma, vasculitis]; MiroBio [Vasculitis]; Neutrolis [Vasculitis]; Novartis [Vasculitis]; Pfizer
[Vasculitis]; Sparrow [Vasculitis]; Takeda [Vasculitis]; Talaris [Vasculitis]. All of the relevant financial
relationships listed have been mitigated. Eric L Matteson, MD, MPH Consultant/Advisory Boards:
Boehringer-Ingelheim [Interstitial lung disease]. Speaker's Bureau: Practice Point Communications
[Rheumatoid arthritis]. Other Financial Interest: Horizon Therapeutics [DSMB – Scleroderma]. All of the
relevant financial relationships listed have been mitigated. Monica Ramirez Curtis, MD, MPH 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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