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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
- Medium-sized vessel vasculitis
- Polyarteritis
nodosa
- Kawasaki's
disease
- Cerebral vasculitis (primary granulomatous)
Source
- Jenette JC, Falk RJ. Small-vessel vasculitis. N Engl J Med
1997; 337(21):1512-23. (Abstract ).
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