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Epidemiology
- uncommon
- male:female = 2:1
- more common between 40 and 60 years
Clinical features
Varies from mild limited disease to progressive fatal disease
- constitutional features of fever, malaise, weight loss
- skin: palpable purpura, infarctions, ulceration, livedo
reticularis, digital
ischaemia
- peripheral neuropathy - classically mononeuritis multiplex
- seizures & hemiparesis due to CNS involvement
- asymmetric polyarthritis
- renal involvement in about 70%. Proteinuria common as are changes suggestive
of glomerular lesion (red cells and red cell casts). ARF unusual. Hypertension
may develop as a result of renal artery or glomerular involvement
- gut involvement leading to abdominal pain which roughly correlates in location
to the organ involved. Diffuse abdominal pain may be due to mesenteric ischaemia
- high percentage of patients HBsAg positive
Investigations
Usually present:
- high ESR
- normochromic normocytic anaemia
- thrombocytosis
- decreased albumin
Others:
- decreased serum whole complement, C3 and C4 may be present in 25%
- rheumatoid factor may be positive in patients with hypocomplementaemia
- biopsy of accessible clinically involved tissue
- if biopsy not possible: visceral angiogram
Treatment
- based on severity of disease
- extensive visceral involvement and progressive disease despite
glucocorticoids: cyclophosphamide
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