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Polyarteritis nodosa

Up Antiphospholipid syn Polyarteritis nodosa SLE Wegener's


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

 

©Charles Gomersall, April, 2014 unless otherwise stated. The author, editor and The Chinese University of Hong Kong take no responsibility for any adverse event resulting from the use of this webpage.
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