Comparison of clinical features in FMF patients according to severity scores: An analysis with the ISSF scoring system.

OBJECTIVE Familial Mediterranean fever (FMF), is an auto-inflammatory disease characterized by attacks of fever and serositis. Some scoring systems have been developed to evaluate the severity of the disease, however, predicting the severity of FMF is not possible with current knowledge. Our aim in this study was to evaluate the factors affecting disease severity in FMF. METHODS This study included 150 FMF patients. The medical data of the patients were collected retrospectively and the International severity scoring system for Familial Mediterranean fever (ISSF) was used to evaluate disease severity. RESULTS Patients were sorted into 3 groups based on the ISSF scores; ≤2=mild (Group 1; n: 61), 3-5=moderate (Group 2; n: 70), and ≥6=severe (Group 3; n: 19). Age at the onset of disease and age at diagnosis was younger in patients with severe disease (p: 0.009 and p: 0.031, respectively). Fever, peritonitis, and vasculitis were similar in all 3 groups. Pleuritis, erysipelas-like erythema (ELE), arthritis, myalgia, amyloidosis, and chronic kidney disease (CKD) were more common in Group 3. CONCLUSION FMF patients with early onset and early diagnosis, having more frequent pleuritis, ELE, arthritis, and myalgia tended to manifest a more severe form of the disease. Close monitoring of such patients may prevent the development of amyloidosis and CKD and improve the long-term prognosis of the disease.