[HL-A and periodic disease]

In the department of Professor Dausset 41 cases of periodic disease referred by 7 clinics in Paris were examined. Two studies were carried out. A series of 31 not related patients was testes with 30 locus A and B antigen and the frequencies observed were compared with the frequencies in a French and Yemenite Jew population. No statistically valid increase of an HLA gene indicative of a relationship between periodic disease and HLA was found. In 5 cases of amyloidosis HLA A 28 was present and it is possible that this gene is related to the amyloid complication. A family study on 12 families, 7 of which included at least 2 affected children, confirmed the recessive hereditary character of the disease, but not in relation to the HLA system. A parallel clinical study was made, which corroborated the previous studies as regards the frequency of clinical signs. The therapeutic study proved the value of long-term colchicin treatment with or without antihistaminics. The frequency of pain and abdominal crises was reduced, but there was little effect on the articular manifestations.