In medicine, vasculitis (plural: vasculitides) is a group of diseases featuring inflammation of the wall of blood vessels. Its main causes are autoimmune disorders and (occasionally) infections. Treatment depends on the cause. While most vasculitides are rare diseases, they generally affect several organ systems and can cause severe disability.

Diagnosis

The types of vasculitis are distinguished by the type of blood vessel affected (aorta, large arteries, arterioles, capillaries and veins), the appearance of biopsy tissue of affected organs on light microscopy, and if necessary, with the help of immunohistochemistry (use of monoclonal antibodies against specific inflammatory protein markers).

Other diagnostic tools are the detection of circulating antibodies that are associated with forms of vasculitis. While these measurements have a low positive and negative predictive value (due to the high rates of both false positives and false negatives), they can direct the clinician to specific causes for vasculitis.

Treatment

Infectious vasculitis is generally treated with directed antibiotics, while autoimmune forms often require treatment with immune suppression: steroids, DMARDs ("steroid-sparing agents") or cyclophosphamide (a mild form of chemotherapy). For very severe forms, bone marrow transplantation is presently being investigated as the ultimate silencing of the immune system.

Causes and types

• Large vessel vasculitis
  • Giant cell arteritis (also temporal arteritis)
  • Takayasu's arteritis

• Medium-sized vessel vasculitis
  • Polyarteritis nodosa
  • Kawasaki's disease
  • Cerebral vasculitis (primary granulomatous)

• Small-vessel vasculitis
  • Associated with ANCAs (anti-neutrophil cytoplasmatic antibody):
    • Microscopic polyangiitis
    • Wegener's granulomatosis
    • Churg-Strauss syndrome
    • Drug-induced
  • Associated with deposition of immune complexes :
    • Henoch-Schönlein purpura (HSP)
    • Cryoglobinemic vasculitis
    • Lupus erythematosus vasculitis
    • Rheumatoid vasculitis
    • Sjögren's syndrome vasculitis
    • Urticarial vasculitis associated with decreased complement
    • Behçet's disease
    • Goodpasture's syndrome
    • Serum sickness-vasculitis
    • Drug-induced
    • Infection-induced (not infectious)
  • Paraneoplastic
    • Lympho- and myeloproliferative neoplasm associated
    • Carcinoma-associated
  • Inflammatory bowel disease vasculitis

Source

• Jenette JC, Falk RJ. Small-vessel vasculitis. N Engl J Med 1997; 337(21):1512-23. (Abstract).