• Clinical science

Vasculitides

Summary

Vasculitides are a heterogeneous group of autoimmune diseases, all characterized by inflammation of blood vessels (vasculitis) and subsequent ischemia and damage to the organs supplied by these vessels. Vasculitis may occur as a primary disease (idiopathic) or as a secondary response to an underlying disease (e.g., hepatitis B infection). Based on the size of the vessel affected, it can be classified into small-vessel, medium-vessel, or large-vessel vasculitis. While the inflammatory process may be confined to one organ, it may also involve several organ systems. Vasculitis should be considered in patients presenting with palpable purpura, pulmonary infiltrates, unexplained ischemic events, and multisystem disease. The detection of antineutrophil cytoplasmic antibodies (ANCA) in the blood is an important diagnostic marker; however, there are also ANCA-negative vasculitis syndromes. Immunosuppressive treatment is administered to stop vascular inflammation. Specific (e.g., antiviral drugs) or symptomatic (e.g., NSAIDs) management may be necessary. If the vasculitis is secondary to an underlying disease, treatment of the underlying disease should be initiated.

Overview

Large vessel vasculitis

Giant cell arteritis (temporal arteritis)

See “Giant cell arteritis” for more information.

Takayasu arteritis (aortic arch syndrome)

In Takayasu arteritis, I can't TAKA YA pulse (pulseless disease).

Medium-sized vessel vasculitis

Kawasaki disease (mucocutaneous lymph node syndrome)

See “Kawasaki disease” for more information.

Polyarteritis nodosa (PAN)

PAN should be considered in young adults presenting with stroke or myocardial infarction. The diagnosis may be confirmed with a biopsy of involved tissue.

In PAN, the Pulmonary Artery is Not involved, PANmural inflammation of the arterial wall is present, and PAN is often associated with hePANitis B.

Thromboangiitis obliterans

See “Thromboangiitis obliterans” for more information.

ANCA-associated small vessel vasculitis

Granulomatosis with polyangiitis

See “Granulomatosis with polyangiitis” for more information.

Granulomatosis with polyangiitis is the 'C' disease: Curvy nose (saddle nose deformity), Chronic sinusitis, Cough, Conjunctivitis and Corneal ulceration, Cardiac arrhythmias, nonCaseating granulomas on biopsy, cANCA, Corticosteroids and Cyclophosphamide as treatment.

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)

Churg-Strauss syndrome: pANCA and polyneuropathy (foot or wrist drop), allergic rhinitis/sinusitis/asthma, vasculitis, eosinophilia, and skin nodules

Microscopic polyangiitis

The presentation of microscopic polyangiitis is similar to that of granulomatosis with polyangiitis, but it does not affect vessels in the upper respiratory tract (no sinusitis or rhinitis), does not involve granuloma formation, and is associated with pANCA (not cANCA).

Microscopic Polyangiitis has MyeloPeroxidase antibodies (i.e., pANCA).

Non-ANCA-associated (leukocytoclastic) vasculitis of small vessels

On histopathology, the following leukocytoclastic vasculitis syndromes appear as nuclear debris from neutrophil leukocytes that have infiltrated blood vessel walls. [1]

Henoch-Schonlein purpura (immunoglobulin A vasculitis)

See “Henoch-Schonlein purpura” for more information.

Cryoglobulinemic vasculitis

The triad of arthralgia, palpable purpura, and fatigue is seen in ∼ 30% of patients with mixed cryoglobulinemia.

Cryoglobulinemia is caused by Cold-precipitable immunoglobulins and associated with hepatitis C.

Cutaneous leukocytoclastic vasculitis (hypersensitivity vasculitis) [19]

Behcet disease

Definition

A systemic vasculitis that is characterized by the deposition of immune complexes in arteries and veins of all sizes

Epidemiology [21]

  • Most common from the Mediterranean region to eastern Asia, with the highest prevalence observed in Turkey and Japan
  • Peak incidence: 20–40 years

Etiology

  • Autoimmune and infectious triggers (e.g., precipitating HSV or parvovirus infection) have been suggested. [22]
  • Strong HLA-B51 association

Clinical features [23][24]

Diagnostics [26][27]

PATHERGY: Positive pathergy test, Aphthous mouth ulcers, Thrombosis (arterial and venous), Hemoptysis (pulmonary artery aneurysm), Eye lesions (uveitis, retinal vasculitis), Recurrent Genital ulcers, Young at presentation (3rd decade)

Diagnostic criteria (International Study Group criteria)

  • Recurrent oral ulceration at least three times within a 12-month period
    AND
  • ≥ 2 of the following

Differential diagnosis

Treatment [28][27]

Differential diagnosis of vasculitis

Clinical presentation Diagnostic clues Treatment

Giant cell arteritis

Takayasu arteritis
Kawasaki syndrome
  • High-dose ASA and IVIG
Polyarteritis nodosa
  • 45–65 years, >
  • Fever, malaise
  • Abdominal, muscle, and joint pain
  • Renal impairment
  • Neurologic dysfunction (e.g., polyneuropathy, stroke)
  • Rash, ulcerations, nodules
Thromboangiitis obliterans (Buerger disease)
  • Smoking cessation
Granulomatosis with polyangiitis (Wegener)
Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)
Microscopic polyangiitis
lmmunoglobulin A
vasculitis
(Henoch-Schönlein purpura)
Cryoglobulinemic vasculitis
  • Fatigue
  • Arthralgia
  • Palpable purpura
  • Glomerulonephritis
Cutaneous leukocytoclastic vasculitis
  • Drug-induced, infections
  • Discontinue drug intake
Behcet disease

References:[11]

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last updated 11/26/2020
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