How do the periodic fever syndromes differ in episode duration?

FMF: 12-72 hours; PFAPA: 3-7 days every 3-6 weeks; MKD/HIDS: 3-7 days every 4-8 weeks; TRAPS: 7-21 days; CAPS/FCAS: 12-24 hours triggered by cold (FCAS) or continuous (NOMID).

What is the role of colchicine in FMF?

Colchicine 1-2 mg/day prevents FMF attacks in >90% of patients and prevents amyloid A amyloidosis (the main cause of renal failure in FMF); lifelong treatment is required.

What interleukin drives CAPS (cryopyrinopathies)?

IL-1β overproduction from mutant NLRP3 (cryopyrin) drives FCAS, MWS, and NOMID/CINCA; IL-1 blockers (anakinra, canakinumab, rilonacept) are highly effective treatments.

What amyloidosis complication is preventable?

Serum amyloid A (SAA) amyloidosis develops in ~10-25% of untreated FMF and TRAPS; regular SAA monitoring (<10 mg/L target) and adequate anti-inflammatory treatment prevent amyloid nephropathy.

What distinguishes PFAPA from other periodic fever syndromes?

PFAPA (Periodic Fever, Aphthous stomatitis, Pharyngitis, Adenitis) is NOT genetic; regular clockwork 3-6 week periodicity, dramatic response to single prednisolone dose, and tonsillectomy is curative in most.

Autoinflammatory Periodic Fever Syndrome Classifier

Classifies autoinflammatory periodic fever syndromes (FMF, TRAPS, CAPS, MKD, PFAPA) using fever episode characteristics, duration, associated features, serum amyloid A, and genetic testing results.

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Frequently Asked Questions

How do the periodic fever syndromes differ in episode duration?

What is the role of colchicine in FMF?

What interleukin drives CAPS (cryopyrinopathies)?

What amyloidosis complication is preventable?

What distinguishes PFAPA from other periodic fever syndromes?