Familial Mediterranean Fever (FMF) Familial Mediterranean Fever (FMF)

Familial Mediterranean Fever (FMF)

Familial Mediterranean Fever is the most common hereditary auto-inflammatory disease. It shows autosomal recessive inheritance. It is common in Eastern Mediterranean countries. Its prevalence in these populations is between 1/200 and 1/1000. Familial Mediterranean Fever is actually polyserositis with recurrent abdominal pain (peritonitis), joint pain (synovitis), chest pain (pleuritis) and fever attacks. Mutations in the MEFV (Mediterranean Fever) gene, which is located on the short arm of chromosome 16 and encodes pyrin or marenostrin protein, are responsible for the disease.
The first attack usually begins before the age of 20 in 80 percent of patients. The onset may rarely be delayed until the age of forties. Abdominal attack usually lasts 12-72 hours. Chest pain occurs due to pleural and sometimes pericardial effusion. There may also be attacks of arthritis lasting up to a week, usually in the form of acute monoarthritis of large joints. Sometimes chronic monoarthritis lasting longer than a month may occur. There may be erysipelas-like, painful erythema and edema on the ankles and dorsum of the feet, which usually lasts 24-48 hours. During febrile attacks, myalgia may occur in the hip and thigh muscles. 

Ref: Prevention and Control of Musculoskeletal System Diseases Program of Türkiye (2015-2020).