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Vasculitis

  • Clinicopathologic process characterized by the presence of inflammatory leukocytes in vessel walls with reactive damage to mural structures

1. Classification & Nomenclature

1. Definition of Vessel size

  • Large vessel : aorta and its major branches. Any size artery may be affected.
  • Medium-sized vessel : main visceral arteries and their branches. Inflammatory aneurysms are common.
  • Small vessel : small intraparenchymal arteries, arterioles, capillaries and venules. Medium sized arteries and veins may be affected.

2. Vessel size and classification

  • Large vessel : TA, GCA
  • Medium vessel : PAN, Kawasaki disease
  • Small vessel
  • ANCA-Associated Vasculitis : MPA, GPA, EGPA
  • Immune complex-mediated : Cryoblobulinemic vasculitis, IgA vasculitis, Anti-C1q vasculitis, Anti-GBM vasculitis

3. CHCC 2012 Nomenclature

CHCC2012 Name CHCC2012 Definition
Large vessel vasculitis (LVV) 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 (MVV) 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 (SVV) Vasculitis predominantly affecting small vessels, defined as small intraparenchymal arteries, arterioles, capillaries, and venules. Medium arteries and veins may be affected.
ANCA-associated vasculitis (AAV) Necrotizing vasculitis, with few or no immune deposits, predominantly affecting small vessels (i.e., 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, e.g., MPO-ANCA, PR3-ANCA, ANCAnegative.
Microscopic polyangiitis (MPA) Necrotizing vasculitis, with few or no immune deposits, predominantly affecting small vessels (i.e., 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 polyangiitis (Wegener’s) (GPA) Necrotizing granulomatous inflammation usually involving the upper and lower respiratory tract, and necrotizing vasculitis affecting predominantly small to medium vessels (e.g., capillaries, venules, arterioles, arteries and veins). Necrotizing glomerulonephritis is common.
Eosinophilic granulomatosis with polyangiitis (ChurgStrauss) (EGPA) Eosinophil-rich and necrotizing granulomatous inflammation often involving the respiratory tract, and necrotizing vasculitis predominantly affecting small to medium vessels, and associated with asthma and 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 (i.e., capillaries, venules, arterioles, and small arteries). Glomerulonephritis is frequent.
Anti–glomerular basement membrane (anti-GBM) disease Vasculitis affecting glomerular capillaries, pulmonary capillaries, or both, with GBM deposition of anti-GBM autoantibodies. Lung involvement causes pulmonary hemorrhage, and renal involvement causes glomerulonephritis with necrosis and crescents.
Cryoglobulinemic vasculitis (CV) Vasculitis with cryoglobulin immune deposits affecting small vessels (predominantly capillaries, venules, or arterioles) and associated with serum cryoglobulins. Skin, glomeruli, and peripheral nerves are often involved.
IgA vasculitis (Henoch-Scho¨nlein) (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 urticarial vasculitis (HUV) (anti-C1q vasculitis) Vasculitis accompanied by urticaria and hypocomplementemia affecting small vessels (i.e., capillaries, venules, or arterioles), and associated with anti-C1q antibodies. Glomerulonephritis, arthritis, obstructive pulmonary disease, and ocular inflammation are common.
Variable vessel vasculitis (VVV) 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).
Behcet’s disease (BD) Vasculitis occurring in patients with Behc ¸et’s disease that can affect arteries or veins. Behc ¸et’s disease 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 (CS) Vasculitis occurring in patients with Cogan’s syndrome. Cogan’s syndrome 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 (SOV) 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 (e.g., 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 SOV will develop additional disease manifestations that warrant redefining the case as one of the systemic vasculitides (e.g., cutaneous arteritis later becoming systemic polyarteritis nodosa, etc.).
Vasculitis associated with systemic disease Vasculitis that is associated with and may be secondary to (caused by) a systemic disease. The name (diagnosis) should have a prefix term specifying the systemic disease (e.g., 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 (e.g., hydralazine-associated microscopic polyangiitis, hepatitis B virus–associated vasculitis, hepatitis C virus–associated cryoglobulinemic vasculitis, etc.).

4. Vessel size and treatment response

Vessel size에 따라 증상도 다르고, 치료 방법도 다르다.**

Dominant vessel Corticosteroid alone CYC and corticosteroid Rituximab Other treatment
Large arteries +++ - - +
Medium arteries + ++ - ++*
Small vessel (ANCA) + +++ +++ +
Small vessel (IC) ++ +/- + ++*
  • Plasmaphresis, anti-viral therapy for Hepatitis B associated PAN and HCV-associated cryoglobulinemia, IVIG for Kawasaki disease
  • GCA - IL6R inhibitor
  • TA - ESR/CRP 상승 여부 or image or 변화 : f/u 했는데 image상 변화가 있는가?

2. Incidence and Epidemiology

3. Clinical manifestations

  1. Systemic Sx.
  2. (어떤 혈관을 침범했느냐에 따른) Localized Sx.
Organ Symptoms
Systemic Fever, weight loss
Musculoskeletal system Arthritis, myalgia
Skin Palpable purpura, nodule, ulcer, urticaria, livedo reticularis
Cardiovascular system Infarction, CHF
Renal system Glomerulonephritis, Hypertension
Nervous system Mononeuritis multiplex, CVA
Gastrointestinal system Ischemia, infarction

4. Diagnosis

* There are no current diagnostic (as opposed to classification) criteria

  • Behcet's ds.도 이름은 diagnostic criteria 이지만 실상은 classification criteria이다.

1. 1990 ACR classification criteria

-> ACR 기준으로 분류하면 오류가 많다. (Rheumatology, 2017;56:1154)

5. Essential steps for diagnosis of vasculitis

  1. Recognition that the patient might have vasculitis : 의심!
  2. Exclusion of mimics and secondary causes of vasculitis including drugs
  3. A compatible phenotype and serology (e.g. ANCA)
  4. Confirmation by radiology (e.g. angiography) or histology
  5. The increased certainty of diagnosis with time : 진단이 시간이 지나면서 바뀔 수도 있다.

1. Clinical features suggestive of vasculitis

일단 의심해야 한다

  1. Mononeuritis multiplex
  2. Palpable purpura
  3. Pulmonary renal involvement
  4. Unexplained ischemic events
  5. Absent pulse, bruit
  6. Fever of Unknown Origin*
  7. Etc.

2. Investigations of suspected vasculitis

Systemic + Localized Sx. 확인

  1. Detailed history taking and physical examination are essential
  2. General (Inflammation)
  3. CBC, eosinophil, ESR/CRP, LFT, ANCA (Elisa/IIF)
  4. Organ involvement
  5. U/A, RFT, LFT, CXR
  6. Where appropriate : NCV, MRI, CSF exam, EKG, 2DE, PET-CT
  7. Differential diagnosis
  8. Blood cultures, viral serology (HBV, HCV, HIV, CMV), Anti-cardiolipin antibody, cryoglobulin, complement, ANA, RF, echocardiography

3. Exclusion of vasculitis mimics

Infection을 놓치지 말자

Systemic multi-system disease DDx.
Infection Infective endocarditis* (esp. SBE;Subacute Bacterial Endocarditis), mycotic aneurysm
Malignancy Metastatic carcinoma
Systemic rheumatic diseases Systemic lupus erythematosus, systemic sclerosis
Drug reaction Prophylthiouracil, Hydralazine (MPA 유발 가능)
Local Inflammation Rhinosinusitis
Occlusive Vasculopathy
Embolic Cholesterol crystals, atrial myxoma, infection
Thrombotic Anti-phospholipid symdrome, thrombocytopenic purpura
Occlusion Fibromuscular dysplasia, coarctation, amyloidosis
Aneurysmal
Aneurysmal Atherosclerosis, Marfan's syndrome, Ehler-Danlos syndrome, segmental arterial mediolysis

4. Compatible phenotypes

Classification criteria만 따지지 말고, 질환의 정의를 잘 생각해 보자.

5. Confirmation by histology (or angiography)

  1. When to biopsy?
  2. Acute active stage
  3. Within 2 weeks after starting treatment
  4. Where to biopsy?
  5. Active sites : nasal cavity는 bx.를 했을 때 vessel에 infiltration이 안 될 수도 있다. (=yield가 낮은 편이다)
  6. Findings
  7. Necrotizing vasculitis
  8. Leukocytoclasis (IgA vasculitis, Hypersensitivity vasculitis)
  9. Granulomatous inflammation (GPA, EGPA, MPA, TA, GCA)

6. Monitoring

  1. Vasculitis can relapse with different features from previous presentation
  2. Vasculitis can relapse long after remission
  3. Fibrotic sequelae of acute injury
Last update: November 1, 2021
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