Overview of and Approach To The Vasculitides in Adults

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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: Dec 2021. | 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
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the past few decades, and definitions that were put 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 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 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 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).
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].
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", 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-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.
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
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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].
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subjects by searching on "patient info" and the keyword(s) of interest.)
● Basics topics (see "Patient education: Polyarteritis nodosa (The Basics)" and "Patient
education: Vasculitis (The Basics)")
● 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.)
• 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
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.
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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.
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
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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
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
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
Definitions for vasculitides adopted by the 2012 International Chapel Hill

Consensus Conference on the Nomenclature of Vasculitides (CHCC2012)
CHCC2012 name CHCC2012 definition

Large-vessel vasculitis Vasculitis affecting large arteries more often than other vasculitides.
Large arteries are the aorta and its major branches. Any size artery may
be affected.
Takayasu arteritis (TAK) Arteritis, often granulomatous, predominantly affecting the aorta and/or
its major branches. Onset usually in patients younger than 50 years.
Giant cell arteritis (GCA) 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.
Medium-vessel vasculitis 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.
Polyarteritis nodosa (PAN) Necrotizing arteritis of medium or small arteries without

glomerulonephritis or vasculitis in arterioles, capillaries, or venules, and
not associated with antineutrophil cytoplasmic antibodies (ANCAs).
Kawasaki disease (KD) 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.
Small-vessel vasculitis Vasculitis predominantly affecting small vessels, defined as small

intraparenchymal arteries, arterioles, capillaries, and venules. Medium
arteries and veins may be affected.
ANCA-associated vasculitis Necrotizing vasculitis, with few or no immune deposits, predominantly

(AAV) 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.
Microscopic polyangiitis Necrotizing vasculitis, with few or no immune deposits, predominantly

(MPA) 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.
Granulomatosis with Necrotizing granulomatous inflammation usually involving the upper and
polyangiitis (Wegener's) lower respiratory tract, and necrotizing vasculitis affecting predominantly
(GPA)
small to medium vessels (eg, capillaries, venules, arterioles, arteries and

veins). Necrotizing glomerulonephritis is common.
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 Vasculitis with moderate to marked vessel-wall deposits of

immunoglobulin and/or complement components predominantly
affecting small vessels (ie, capillaries, venules, arterioles, and small
arteries). Glomerulonephritis is frequent.
Anti-glomerular basement Vasculitis affecting glomerular capillaries, pulmonary capillaries, or both,

membrane (anti-GBM) with GBM deposition of anti-GBM autoantibodies. Lung involvement
disease 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- Vasculitis, with IgA1-dominant immune deposits, affecting small vessels
Schönlein) (IgAV) (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 affecting

urticarial vasculitis (HUV) small vessels (ie, capillaries, venules, or arterioles), and associated with
(anti-C1q vasculitis) 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
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 Vasculitis that is associated with a probable specific etiology. The name
probable etiology (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.
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.
Demographic characteristics of 807 patients with vasculitis
Percent with Mean age at

Disease category Percent female
disorder disease onset
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.
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.
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.
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.
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.
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MAJOR CATEGORIES OF VASCULITIS

Polyarteritis nodosa — Polyarteritis nodosa is a systemic necrotizing vasculitis that typically

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

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

Microscopic polyangiitis — MPA is a necrotizing vasculitis that primarily affects

Granulomatosis with polyangiitis — GPA is a necrotizing vasculitis predominantly

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

Immune complex small-vessel vasculitis — Immune complex small-vessel vasculitis refers to

Anti-glomerular basement membrane disease — Anti-glomerular basement membrane

glomerulonephritis with necrosis and crescents. (See "Anti-GBM (Goodpasture) disease:

Cryoglobulinemic vasculitis — Cryoglobulinemic vasculitis, previously termed essential

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

Hypocomplementemic urticarial vasculitis (anti-C1q

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

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

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

microscopic polyangiitis: Clinical manifestations and diagnosis", section on 'Classification

CLINICAL FEATURES SUGGESTIVE OF SYSTEMIC VASCULITIS

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

extremities or in a person at low risk for atherosclerosis, is suggestive of a large arterial

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

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

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

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

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

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

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

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

INFORMATION FOR PATIENTS

SUMMARY AND RECOMMENDATIONS

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

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

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

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

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

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

Use of UpToDate is subject to the Subscription and License Agreement.

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

Names for vasculitides adopted by the 2012 International Chapel Hill

Giant cell arteritis

Granulomatosis with polyangiitis (Wegener's)

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)

Immune complex small-vessel vasculitis

Anti-glomerular basement membrane disease

IgA vasculitis (Henoch-Schönlein)

Hypocomplementemic urticarial vasculitis (anti-C1q vasculitis)

Primary central nervous system vasculitis

Vasculitis associated with systemic disease

Vasculitis associated with probable etiology

Hepatitis B virus-associated vasculitis

Drug-associated immune complex vasculitis

Drug-associated ANCA-associated vasculitis

ANCA: antineutrophil cytoplasmic antibody; IgA: immunoglobulin A.

Graphic 90188 Version 4.0

Definitions for vasculitides adopted by the 2012 International Chapel Hill

CHCC2012 name CHCC2012 definition

Polyarteritis nodosa (PAN) Necrotizing arteritis of medium or small arteries without

Small-vessel vasculitis Vasculitis predominantly affecting small vessels, defined as small

ANCA-associated vasculitis Necrotizing vasculitis, with few or no immune deposits, predominantly

Microscopic polyangiitis Necrotizing vasculitis, with few or no immune deposits, predominantly