Hereditary angioedema - retrospective of the last 20 years

Abstract Rationale We characterized a group of patients with Hereditary Angioedema (HA) and evaluated different options for prevention, prophylaxis and therapy of crises. Methods Clinical review of patients with HA followed at Immunoallergology Unit (HSM) or in CAIC since 1980. Results Among 28 patients (18F/10M, mean age 39,5 ± 16,9 years), belonging to 12 families, only 2 had no family history. Mean age at first symptoms was 11,7 ± 7,4 years and mean age at diagnosis 14,5 ± 15,5 years. Clinical manifestations at presentation were cutaneous-50%, gastrointestinal (GI)-25%, cutaneous and GI-11%, respiratory-7%, cutaneous and respiratory-4%. Almost all patients, 92%, had already had cutaneous symptoms, 71% GI and 68% respiratory symptoms. During an attack, 3 patients had been submitted to exploratory laparotomy and 4 patients to endotracheal entubation. Most frequent triggering factors were stress and trauma. HA was classified as type I or type II in 18 and 10 patients, respectively. Only 7 patients were not on preventive therapy, the others being medicated with androgens (AD)-13, antifibrinolitics (AF)-3 or with both-5. Therapeutic of acute episodes had been AD+AF-8 patients, AF-6, AD-4, AD+AF+plasma-4, AF+plasma-3 or other approaches-2. Prophylactic terapy was prescribed to 11 patients AD-8, AD+AF-2, plasma or C1 inhibitor-1 with good efficacy. Conclusion Clinical features of this group were similar to those described in the literature concerning prevalence, sex distribution, age at first symptoms and type of clinical manifestations. Mean time between age at first symptoms and diagnosis was quite variable and on average higher than in other studies. Many different therapeutic options have been adopted in severe attacks.