Symptoms of Familial Mediterranean fever

Symptoms of Familial Mediterranean fever

  • • Majority (90%) of patients have onset within the first or second decade of life.
  • • Acute and self-limited flares result in:
    • • Fever.
      • Serositis: generalized peritonitis (80%–90%), pleuritis (25%–50%). Abdominal pain is often severe, presenting as an acute abdominal pain.
      • Arthritis: large joint monoarticular arthritis (50%–75%) in the hip, knee, ankle, and wrist most commonly. Pain out of proportion of swelling. Poor response to prednisone.
      • Rash: erysipelas-like rash (30%).
    • • Less common manifestations include polyarthritis, prolonged arthritis, aseptic meningitis, pericarditis, scrotal swelling, and lymphadenopathy.
    • • Flares typically last 1 to 3 days; recurrence of attacks average every 2 to 4 weeks.
    • • During flares: elevation of ESR and other acute-phase reactants (i.e., CRP, amyloid A).
    • • Systemic amyloidosis (AA) can develop as a sequel of chronic inflammation and can lead to renal failure. A subset of patients present with systemic amyloidosis as their initial manifestation, without a history of inflammatory episodes.
      • Prevalence of AA is more common in certain ethnic populations, increases with age, and is not associated with the severity or frequency of acute attacks (but is associated with untreated disease).
      • End-stage renal disease as a result of AA is treated with renal transplantation and continuation of colchicine therapy.
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