Summary
- Primarily a genetic disease due to Mendelian-recessive inheritance of MEFV gene mutations.
- Occurs mainly in people of Mediterranean ancestry, especially from Arabian countries, Turkey, and Armenia.
- Characterised by recurrent attacks of fever and systemic inflammation, typically lasting 24 to 72 hours and presenting in childhood.
- Rare manifestations include chronic arthritis, spondyloarthropathy, myopathies, and fibromyalgia.
- Clinical judgement is important in establishing the diagnosis, which is often made retrospectively.
- Majority respond to colchicine, which is almost always diagnostic and distinguishes from other inherited periodic fever syndromes.
- Colchicine is the only proven treatment and prevents the most serious and life-threatening complication, renal amyloidosis.
Other related conditions
- Osteoarthritis
- Juvenile idiopathic arthritis
- Rheumatoid arthritis
- Reactive arthritis
- Psoriatic arthritis
- Septic arthritis
- Assessment of seronegative arthritis
- Assessment of fever of unknown origin in children
- Assessment of fever of unknown origin
- Aphthous ulcers
- Acute pharyngitis
- Juvenile idiopathic arthritis
- Crohn's disease
- Gangrene
- Acne vulgaris
- Transient synovitis of the hip
- Tenosynovitis of the hand and wrist
- Assessment of pustular rash
- Osteomyelitis
- Behcet's syndrome
- Henoch-Schonlein purpura
- Polyarteritis nodosa
- Amyloidosis
- Assessment of pleuritis
- Spontaneous bacterial peritonitis
- Pericarditis
- Constipation in children
- Constipation
- Overview of meningitis
- Assessment of splenomegaly
- Assessment of lymphadenopathy
Last updated: Jul 17, 2012
