Journal of Internal Medicine 1996 ; 239 : 119–130 Hereditary angio-oedema : new clinical observations and autoimmune screening, complement and kallikrein-kinin analyses E. W. NIELSEN, J. T. GRAN,* B. STRAUME,† O. J. MELLBYE,‡ H. T. JOHANSEN § & T. E. MOLLNES ¶ From the Department of Anaesthesiology, Nordland Central Hospital, Bodø, and University of Tromsø, Tromsø,* Department of Rheumatology, Aust Agder Central Hospital, † Institute of Community Medicine, University of Tromsø, Tromsø, ‡ Department of Immunology and Rheumatology, National Hospital, Oslo, § Institute of Pharmacy, University of Oslo, Oslo, and ¶ Department of Immunology and Transfusion Medicine, Nordland Central Hospital, Bodø and University of Tromsø, Tromsø, Norway Abstract. Nielsen EW, Gran JT, Straume B, Mellbye OJ, Johansen HT, Mollnes TE. (Department of Anaes- thesiology, Nordland Central Hospital, Bodø, and University of Tromsø, Tromsø, Norway). Hereditary angio-oedema : new clinical observations and auto- immune screening, complement and kallikrein-kinin analyses. J. Intern Med 1996 ; 239: 119–130. Objectives. To study clinical and laboratory mani- festations of hereditary angio-oedema (HAE). Subjects. Thirty-three affected members of a kindred of 63. Results. Oedematous attacks in the skin, mucous membranes and gastrointestinal tract with fluid displacement were elicited by mental and physical stress, minor traumas, dental and surgical pro- cedures, eruption of teeth, tonsillitis, pregnancies, and use of oestrogen-containing pills including meno- pausal substitution. Every adult woman with symp- tomatic HAE (n  11) showed symptoms of urinary tract infections in conjunction with the attacks (P  0010), and also experienced more spontaneous Introduction Persons suffering from hereditary angio-oedema (HAE) experience episodic attacks of swellings of varying severity and location (Fig. 1). The skin of the extremities, groin and face is often involved, as is the mucosa of the oro-pharynx where life-threatening laryngeal oedema may evolve [1]. Oedema of the gastro-intestinal tract causes nausea, vomiting, ab- dominal pain and sometimes diarrhoea. Ultrasound abortions or premature labours (P  0037) than healthy relatives. Patients with HAE of both sexes more frequently reported heartburn or peptic ulcers (P  0002). Rheumatic complaints were reported by 53 % of HAE patients and 12 % of their unaffected relatives (P  0013), but biochemical screening for 18 autoantibodies and quantitation of immuno- globulins did not reveal statistically significant differences between the two groups. C3, prekallikrein, total kininogen, high molecular weight kininogen (HK), alpha-2-macroglobulin and factor XII were not significantly different in HAE patients. In contrast, levels of C1-INH and C4 were depressed and cleaved HK increased in patients compared to unaffected relatives. Conclusions. HAE manifests in a variety of ways, and may influence risk of spontaneous abortions and premature labour. Keywords : C1-inhibitor, hereditary angio-oedema, premature labour. examinations during attacks have shown a 1-cm thickening of the gut wall and ascites [2], which have also been found when these patients have erroneously undergone laparotomy [3]. Transient aphasia and haemiplegia are serious but less frequent manifestations [4, 5]. Hereditary angio-oedema is caused by an alter- ation in the gene on either of the chromosomes 11 coding for C1-inhibitor (C1-INH), an important inhibitor of C1, factor XII and plasma kallikrein. The  1996 Blackwell Science Ltd 119