HAE diagnosis and course

PubMed, the Internet portal of biomedical and life sciences literature, indexed an interesting article, entitled “Hereditary angioedema: new findings concerning symptoms, affected organs and course” (Am J Med. 2006 Mar;119(3):267-74). Authors are Bork K, Meng G, Staubach P et al., from the Department of Dermatology, Johannes Gutenberg University, Mainz, Germany. Hereditary angioedema (HAE) due to C1 inhibitor deficiency is clinically characterized by relapsing skin swellings, abdominal pain attacks, and life-threatening upper airway obstruction. The aim of the authors was to examine a temporal and spatial pattern of the edema episodes by evaluating the long-term course of hereditary angioedema in order to establish a specific swelling pattern. Data were generated from 221 patients with C1 inhibitor deficiency. A total of 131 110 edema episodes were observed. Clinical symptoms started at a mean age of 11.2 years. On average, women had a more severe course of the disease than men. Patients with early onset of clinical symptoms were affected more severely than those with late onset. The conclusion is that the described swelling pattern is specific for HAE and allows a tentative diagnosis based on clinical symptoms and the course of the disease. To access the full abstract of the article, click here.