Episodic Fever and Lymphadenopathy
Majority of patients with familial Mediterranean fever (FMF) become symptomatic before 10 years of age and up to 90% experience attacks before the age of 20
Incomplete FMF attacks are common, when recurrent painful attacks are associated with fever below 38 °C, resolution of attacks in less than 12 hours or more than 4 days or when fewer than 3 episodes have occurred.
Hyper-IgD syndrome (HIDS) can also closely mimic symptoms of familial relapsing fevers, with febrile episodes, abdominal pain and mucocutaneous manifestations in form of papules, urticaria, erythema, and petechiae
Recurrent episodes of systemic inflammation in FMF are associated with increased SAA, which is also elevated during symptom-free episodes.
The most serious complication of FMF is renal amyloidosis however, which gives rise to nephrotic syndrome
KeywordsHyper-IgD syndrome Familial Mediterranean fever Serum amyloid A
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