Clinical Features of Hereditary and Mast Cell-mediated Angioedema Focusing on the Differential Diagnosis in Japanese Patients

• Published in: Internal Medicine 2018/02/01, Vol.57(3), pp.319-324
• Main Authors: Ohsawa, Isao, Honda, Daisuke, Hisada, Atsuko, Inoshita, Hiroyuki, Onda-Tsueshita, Kisara, Mano, Satoshi, Sato, Nobuyuki, Nakamura, Yuya, Shimizu, Tatsuo, Gotoh, Hiromichi, Goto, Yoshikazu, Suzuki, Yusuke, Tomino, Yasuhiko
• Format: Article
• Language: English
• Subjects: Angioedema; C1-inhibitor; Consultation; Differential diagnosis; Edema; Extremities; Gastrointestinal tract; Genetics; hereditary angioedema; IgE; Internal medicine; Larynx; mast cell; Original; Statistical analysis
• ISSN: 0918-2918; 1349-7235
• DOI: 10.2169/internalmedicine.8624-16

Objective The present study was designed to identify the clinical characteristics that permit the differential diagnosis of hereditary angioedema (HAE) and mast cell-mediated angioedema (Mast-AE) during the first consultation. Methods The medical histories and laboratory data of 46 patients with HAE and 41 patients with Mast-AE were compared. Results The average age of onset in the HAE group (19.8±9.0 years) was significantly lower than that in the Mast-AE group (35.2±12.0 years). The incidence of familial angioedema (AE) in the HAE group (73.9%) was significantly higher than that in the Mast-AE group (9.7%). The frequency of history of AE in the extremities, larynx, or gastrointestinal tract was significantly higher in the HAE group. The frequency of AE episodes of the lips and eyelids was significantly lower in the HAE group. The serum C4 concentration and CH50 titer were lower than the normal limit in 91.3% and 45.6% of the patients in the HAE group, respectively; in Mast-AE group the serum C4 concentration and CH50 titer were significantly lower than the normal limit in 4.8% and 0% of the patients, the difference between the two groups was statistically significant. A C1-inhibitor (C1-INH) activity level of