Clinical profile of idiopathic angioedema based on severity and treatment response is independent of the presence of concomitant wheals

Background Idiopathic angioedema varies in disease severity and treatment response, possibly due to different pathophysiological mechanisms. The presence of wheals is an indicator for histamine-mediated angioedema. patients are treated accordance with chronic spontaneous urticaria guidelines. Little known about effectiveness idiopathic without comparison concomitant wheals. Objective To describe the profile unknown cause relation prophylactic or absence Methods In this retrospective cohort study, all records visiting outpatient clinic UMC Utrecht between January 2015 March 2020 were screened. Patients angioedema, including those wheals, included. Attack frequency, patient-reported control attack as analysed at follow-up outcomes compared subordinary Results Two hundred thirty-six included: 95% (139/236) only 41% (97/236) No was prescribed 27% (64/236), well-controlled 86% (25/29) patients. Antihistamine monotherapy used 59% resulted 68% (62/92). Add-on 14% (33/236) patients, omalizumab 9% (22/236) specifically, complete response 38% (6/16) low frequency another 18% (3/16). Difficult-to-treat seen 8% (18/236), no a fourfold dose antihistamines omalizumab. All findings independent from Conclusion Angioedema well manageable majority therapy antihistamine monotherapy, but substantial proportion does not respond and/or Treatment