• 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., NSAID) 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) and Thickening of the Aortic arch Keeps All Young Asian Sisters Unhappy.

References:[1][2][3][4]

Medium-sized vessel vasculitis

Kawasaki disease (mucocutaneous lymph node syndrome)

See Kawasaki disease for more information.

Polyarteritis nodosa (PAN) [2][5]

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 (Wegener granulomatosis)

See granulomatosis with polyangiitis for more information.

Wegener granulomatosis 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 Wegener granulomatosis, but it does not affect vessels in the upper and lower respiratory tract (no sinusitis or rhinitis), does not involve granuloma formation, and is associated with pANCA, not cANCA.

References:[1][6][7][8]

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.

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.

Cutaneous leukocytoclastic vasculitis (hypersensitivity vasculitis)

  • Definition: necrotizing vasculitis of cutaneous small vessels
  • Epidemiology: most common vasculitis seen in clinical practice
  • Etiology: drug-induced (e.g., PTU, hydralazine, allopurinol, penicillins, sulfasalazine); infections
  • Clinical features:
  • Diagnostics:
    • Skin biopsy
    • Investigation of underlying cause
    • Systemic vasculitis should be ruled out.
  • Treatment: symptomatic; discontinue drug intake; immunosuppression may be required

References:[1][9][10]

Behcet disease

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

Differential diagnosis of vasculitis

Clinical presentation Diagnostic clues Treatment

Giant cell arteritis

Takayasu arteritis
  • ESR
  • Angiography shows stenosis of aortic arch and proximal great vessels (gold standard).
  • Biopsy shows granulomatous inflammation of aorta and its major branches.
Kawasaki syndrome
  • High-dose ASA and IVIG
Polyarteritis nodosa
  • Peak incidence: ∼ 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)
  • PR3-ANCA/cANCA-associated
  • Biopsy shows granulomatous, necrotizing inflammation of vessels, kidneys, and the lungs.
  • Chest x-ray/CT: multiple bilateral cavitating nodular lesions
Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)
  • Severe allergic asthma, sinusitis
  • Skin manifestations (e.g., tender nodules)
  • Peripheral neuropathy
  • Gastrointestinal, cardiac, renal involvement possible
Microscopic polyangiitis
lmmunoglobulin A
vasculitis
(Henoch-Schönlein purpura)
  • Often secondary to upper respiratory tract infections
  • IgA in serum
  • Biopsy: leukocytoclastic vasculitis with IgA and C3 immune complex deposition
Cryoglobulinemic vasculitis
Cutaneous leukocytoclastic vasculitis
  • Drug-induced, infections
  • Discontinue drug intake
Behcet disease

References:[15][16][17][8]