Vasculitis

In medicine, vasculitis (plural: vasculitides) is a group of diseases featuring inflammationof the wall of blood vessels. Its main causes are autoimmune disordersand (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, capillariesand veins), the appearance of biopsytissue of affected organs on light microscopy, and if necessary, with the help of immunohistochemistry(use of monoclonal antibodiesagainst specific inflammatoryprotein markers).

Other diagnostic tools are the detection of circulating antibodiesthat are associated with forms of vasculitis. While these measurements have a low positiveand negative predictive value(due to the high rates of both false positivesand false negatives), they can direct the clinicianto specific causes for vasculitis.

Treatment

Infectious vasculitis is generally treated with directed antibiotics, while autoimmuneforms often require treatment with immune suppression: steroids, DMARDs("steroid-sparing agents") or cyclophosphamide(a mild form of chemotherapy). For very severe forms, bone marrow transplantationis 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-neutrophilcytoplasmaticantibody):
    • Microscopic polyangiitis
    • Wegener's granulomatosis
    • Churg-Strauss syndrome
    • Drug-induced
  • Associated with deposition of immune complexes:
    • Henoch-Schönlein purpura(HSP)
    • Cryoglobinemicvasculitis
    • Lupus erythematosusvasculitis
    • Rheumatoidvasculitis
    • Sjögren's syndromevasculitis
    • Urticarialvasculitis 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 diseasevasculitis

Source

• Jenette JC, Falk RJ. Small-vessel vasculitis. N Engl J Med1997; 337(21):1512-23. PMID 9366584.

External links

• European Vasculitis Study Group
• Johns Hopkins Vasculitis Centerde:Vaskulitis

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This article is licensed under the GNU Free Documentation License.
It uses material from the http://en.wikipedia.org/wiki/Vasculitis Wikipedia article Vasculitis.