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Objective: Recurrent Angioedema (RAE) is characterized by sudden swelling of mucosal surfaces or deep dermis and is either mast cell-(MMAE) or bradykinin-mediated (BMAE). How patients with BMAE and MMAE differ in terms of disease activity and impact remains largely unknown. Here, we determined validity, reliability, and sensitivity to change of Turkish versions of angioedema activity score (AAS) and quality of life questionnaire (AE-QoL) and used both instruments to investigate and compare patients with BMAE and MMAE.Methods: Turkish versions of AAS28 and AE-QoL were applied to 94 patients with RAE (18-72 years). Patients' global self-assessment of QoL (PGA-QoL), disease activity (PGA-DA-VRS, PatGA-DA-VAS), and 12-Item-Short Form Survey were used at week 4 (visit 2), and week 8 (visit 3). Demographic characteristics, clinical features, and AAS28 and AE-QoL values were compared between 31 patients with BMAE and 63 patients with MMAE.Results: Turkish AAS28 and AE-QoL showed excellent internal consistency, high reproducibility and known-groups validity. Compared to patients with MMAE, BMAE patients were younger (34.6 AE 10.7 vs. 40.7 AE 13.3 years), had longer disease duration (236 AE 178 vs. 51 AE 78 months), high prevalence of family history (63% vs 14%), longer duration of attacks (65 AE 20 vs. 40 AE 25 h), and they were more commonly affected by upper airway angioedema (70% vs 23%). Disease activity (AAS28) was lower (29.3 AE 24.6 vs 55.2 AE 52.9), but AE-QoL was higher (44.2 AE 16.1 vs 34.5 AE 22.5) in BMAE patients as compared to MMAE patients.Conclusions: Patients with BMAE and MMAE have distinct disease characteristics. Recurrent bradykinin-mediated angioedema impacts quality of life more than mast cell-mediated angioedema. The discriminating characteristics of patients with BMAE and MMAE may help to improve the diagnosis and management of patients with RAE.

KOCATÜRK eT Al. | INTRODUC TI ONChronic urticaria (CU) is an inflammatory disease characterized by the appearance of wheals, angioedema, or both for longer than 6 weeks. CU is classified into chronic spontaneous urticaria (CSU) and chronic inducible urticaria (CIndU), where signs and symptoms occur in response to definite triggering factors such as cold in cold urticaria and perspiration-inducing activities in cholinergic urticaria. 1 CSU is more common in females than males as confirmed by a recent systematic review and meta-analysis of the available epidemiological data, which showed that the point prevalence of CSU is 1.3% in women versus 0.8% in men. 2 CIndUs are also more common in females, with a female:male ratio of 2:1 to 3:1. 3 In a study from China, women, across all CindUs, accounted for 58% of patients, with higher rates in some CIndUs, cold urticaria for example, where 66% of patients were female. 4The reasons for this female preponderance in CU are currently unknown and subject of ongoing investigations, but several points are important in this context. First, it is well known that autoimmune diseases are more common in women, with female:male ratios of 3:1 for multiple sclerosis and 15:1 for autoimmune thyroiditis, for example. 5 It is now widely accepted that autoantibodies and their activating effects on skin mast cells play a role in the pathogenesis of CSU in a proportion of patients. In CSU, two types of underlying autoimmunity are postulated; type I autoimmunity (also called autoallergy) with IgE autoantibodies against autoallergens and type IIb autoimmunity with stimulatory IgG and IgM autoantibodies to receptors on mast cells; even some authors proposed two types of autoimmunity co-existing in some patients. 6,7 This is supported by the common association of CSU with autoimmune diseases, which has been reported in many studies. 8 Second, mast cells, the key drivers of CU pathogenesis, express hormone receptors, and sex hormones can influence mast cell functions including their activation and release of proinflammatory mediators. Estrogens increase histamine release in rat mast cells and sensitized human basophils upon stimulation

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Background Although chronic urticaria (CU) is a common and primarily affects females, there is little data on how pregnancy interacts with the disease. Objective To analyse the treatment use by CU patients before, during and after pregnancy as well as outcomes of pregnancy. Methods PREG‐CU is an international, multicentre study of the Urticaria Centers of Reference and Excellence network. Data were collected via a 47‐item‐questionnaire completed by CU patients who became pregnant during their disease course. Results Questionnaires from 288 CU patients from 13 countries were analysed. During pregnancy, most patients (60%) used urticaria medication including standard‐dose second generation H1‐antihistamines (35.1%), first generation H1‐antihistamines (7.6%), high‐dose second‐generation H1‐antihistamines (5.6%) and omalizumab (5.6%). The preterm birth rate was 10.2%; rates were similar between patients who did and did not receive treatment during pregnancy (11.6% vs. 8.7%, respectively). Emergency referrals for CU and twin birth were risk factors for preterm birth. The caesarean delivery rate was 51.3%. More than 90% of new‐borns were healthy at birth. There was no link between any patient or disease characteristics or treatments and medical problems at birth. Conclusion Most CU patients used treatment during pregnancy especially second‐generation antihistamines which seem to be safe during pregnancy regardless of the trimester. The rates of preterm births and medical problems of new‐borns in CU patients were similar to population norms and not linked to treatment used during pregnancy. Emergency referrals for CU increased the risk of preterm birth and emphasize the importance of sufficient treatment to keep urticaria under control during pregnancy.