Clinical, endoscopic, immunological and therapeutic findings in Egyptian cases of familial Mediterranean fever

ABSTRACT

Ninety seven Egyptian individuals were studied. They were divided to 4 groups Group I [Healthy controls] included 20 healthy individuals. Group II [Diseased controls] comprised 24 patients complaining of abdominal symptoms with negative symptoms and signs of F.M.F. Group III [Definite F.M.F] included 40 selected uses of F.M.F. Group IV [Probable F.M.F.] included 13 cases. careful history, thorough clinical examination, routine investigations s exclude other diseases, upper gastrointestinal endoscopy, ismoidoscopy, screening for amyloidosis, immunological tests [C3, C4, circulating immune complexes, alpha-1-acid glycoprotein and haptoglobin], metaraminol provocative test and colchicine therapeutic trial were performed for every case and controls. In the 40 definite F.M.F cases, the male female ratio was 1.91 family history was elicited in 24 [60%] cases. Prodromal symptoms we found in 34 [85%] cases and precipitating factors were elicited In 32 [80%] cases. The attacks were febrile in all [100%] the cases and were manifested as peritonitis in 37 [92%] caes, pleuritis in 22 [55%], synovitis in 11 [28%] and skin rash in 4 [10%] cases. ftmteen [43%] cases were paucisymptormatic. Thirty seven surgical Wtocedures were performed in 25 [63%] cases. Upper gastrointestinal aioscopy revealed abnormal findings in 6 [27%] out of 22 F.M.F. m. No evidence of amyloidosis was elicited in any case. The mean value of C3 [1.05 +/- 0.20 gmIL] and C4 [0.46 +/- 0.17 gm/L] showed no significant difference when compared to controls. Circulating immune complexes were increased in all cases. The positivity of serum alpha-1-acid glycoprotein was nearly equal during the attacks [35%] and in between [33%] them while the positivity of serum haptoglobin was 29% during the attacks and 16% inbetween the attacks. Metaraminol provocative test had a 68% sensitivity and 100% specificity. Long term colchicine therapy was recommended for all the cases. Twenty four [71%] out of 34 F.M.F cases reported good response, 3 [9%] reported failure after initial successful trial and 1 [20%] reported no response. It is concluded from this study that there is no specific diagnostic test in F.M.F and that the diagnosis of the disease is still by exclusion