Decrease in the rate of secondary amyloidosis in Turkish children with FMF: are we doing better?

摘要: Familial Mediterranean fever (FMF) is the most common autoinflammatory disease in world. The serious complication of FMF development secondary amyloidosis. Besides genetic factors, environment has been implicated this complication. main objective study to analyze whether there a substantial decrease amyloidosis Turkey and possible effective factors. For purpose, clinical features patients diagnosed with between years 1978 1990 were compared those 2000 2009. Severity scores determined by use scoring system modified for children. Median ages group (n = 115; 12.1% among total 947 renal biopsies) after (n = 19; 2% 974 12 13 years, respectively. There no significant differences two patient groups according gender, age, age onset, duration, severity. was, however, clear percentage biopsies from (1978–1990) (after 2000; p < 0.001). Our results have shown that rate Turkey. reason better medical care increased awareness treatment disease. However, we suggest improvement infectious milieu may possibly had positive effect on course monogenic disease, since inflammatory pathways related innate immunity are deregulated.