[Respiratory manifestations in hereditary angioneurotic edema]. / Manifestations respiratoires des oedèmes angioneurotiques héréditaires.

ABSTRACT

Hereditary angioneurotic oedema is an autosomal dominant state associated with a quantitative, and sometimes purely functional, deficiency of C1 esterase inhibitor (C1 INH). The clinical manifestations may begin during adulthood or childhood; they are periodical and of varying severity. Beside oedema of the skin and digestive disorders, respiratory disorders are bound to attract attention. They consist of laryngeal oedema, which may end in lethal asphyxia if tracheotomy is not performed, or, exceptionally, of pulmonary oedema requiring assisted ventilation, as in the case reported here. The diagnosis, suspected in the presence of a decrease in CH50 and C4, is confirmed by a quantitative assay of C1 INH, which is low, and/or by the Fong and Good's functional tests. The physiopathological mechanisms are complex. They involve complement activation through the classical route, and activation of the coagulation system contact phase. Patients with severe attacks now benefit from treatment with preparations of C1 INH in high concentrations. The best treatment, however, is prophylactic, using testosterone derivatives, danazol and stanozolol, which can be prescribed for long periods taking into account their usually moderate side-effects.