[Familial Mediterranean fever. Immunogenetic and rheumatologic aspects of the disease in a Turkish sibship].
A family study was performed in 24 members of a Turkish sibship with familial Mediterranean fever (FMF) with 5 patients affected in 3 generations. The well-known autosomal-recessive inheritance of the disease was masked by a pseudodominant appearance, reflecting the striking frequency of congenial marriages. The immunogenetic investigation excluded a linkage between the expression of the disease and the HLA system. The arthritis of FMF was characterized typically by monarticular attacks in large joints of the lower limb. Frequently this manifestation led to diagnostic problems, particularly at the onset of the disease. No patient presented the clinical or radiological signs of sacroileitis. An observation of the disease process up to 3 years showed a benign prognosis of FMF-arthritis in 3 of 4 patients. Neither long-lasting functional impairment nor radiological signs of erosion had to be recognized. One patient suffered from a necrosis of the femoral head, possibly caused by the recurrent inflammation of the hip joint. Laboratory findings reflected the clinical picture of relapsing acute inflammation in an uncharacteristic manner. Their diagnostic significance exists mainly for the exclusion of other diseases.
Langer
H E
HE
Robin-Winn
M
M
Stangel
W
W
Zeidler
H
H
D000328:Adult; D001172:Arthritis, Rheumatoid / Q000175:diagnosis / Q000235:genetics*; D002648:Child; D010505:Familial Mediterranean Fever / Q000175:diagnosis / Q000235:genetics*; D005260:Female; D005838:Genotype; D006680:HLA Antigens / Q000235:genetics*; D006801:Humans; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D014421:Turkey
D006680:HLA Antigens
Familiäres Mittelmeerfieber. Immungenetische und rheumatologische Aspekte der Erkrankung in einer türkischen Sippschaft.