[Aortic insufficiency in certain so-called systemic diseases]

The authors present a retrospective study of all the patient followed up for systemic disease in the rheumatology Department of Bichat hospital between 1975 and 1984 in whom aortic regurgitation developed. Only rare or previously undescribed associations were retained: two MacDuffie syndromes, one adult form of Still's disease, one Takayashu's disease, one association of rheumatoid arthritis and Takayashu's disease, one rheumatoid arthritis, one Cogan's syndrome and two cases of disseminated lupus erythematosis. The authors use these cases and a review of the literature to discuss the possible physiopathological mechanisms of the aortic regurgitation. This study confirms the value of regular clinical cardiovascular examination with echocardiography in cases with progressive symptoms. The evolution of the vascular disease seems to be more or less parallel to that of the systemic disease and in a significant number of cases it becomes sufficiently severe to become the main clinical problem. In our series, there was one sudden death, one death due to cardiogenic pulmonary oedema and three patients required aortic valve replacement.