HEREDITARY ANGIO-EDEMA - NEW CLINICAL OBSERVATIONS AND AUTOIMMUNE SCREENING, COMPLEMENT AND KALLIKREIN-KININ ANALYSES

Objectives. To study clinical and laboratory manifestations of heredit ary angio-oedema (HAE). Subjects. Thirty-three affected members of a k indred of 63. Results. Oedematous attacks in the skin, mucous membrane s and gastrointestinal tract with fluid displacement were elicited by mental and physical stress, minor traumas, dental and surgical procedu res, eruption of teeth, tonsillitis, pregnancies, and use of oestrogen -containing pills including menopausal substitution. Every adult woman with symptomatic HAE (n = 11) showed symptoms of urinary tract infect ions in conjunction with the attacks (P = 0.010), and also experienced more spontaneous abortions or premature labours (P = 0.037) than heal thy relatives. Patients with HAE of both sexes more frequently reporte d heartburn or peptic ulcers (P = 0.002). Rheumatic complaints were re ported by 53% of HAE patients and 12% of their unaffected relatives (P = 0.013), but biochemical screening for 18 autoantibodies and quantit ation of immunoglobulins did not reveal statistically significant diff erences between the two groups, C3, prekallikrein, total kininogen, hi gh molecular weight kininogen (HK), alpha-2-macroglobulin and factor X II were not significantly different in HAE patients. In contrast, leve ls of C1-INH and C4 were depressed and cleaved HK increased in patient s compared to unaffected relatives. Conclusions. HAE manifests in a va riety of ways, and may influence risk of spontaneous abortions and pre mature labour.