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    Assessment of clinical phenotypic characteristics of patients with angioedema without wheals in a turkish population
    (Ocean Side Publications Inc, 2024) Cakmak, Mehmet Erdem; Bostan, Ozge Can; Kaya, Saltuk Bugra; Karakaya, Gul; Kalyoncu, Ali Fuat; Damadoglu, Ebru
    Background: Angioedema (AE) is defined fi ned as localized, self-limited swelling of subcutaneous tissues and mucosa. Objective: The aim of this study was to compare the phenotypic characteristics of patients with AE without wheals. Methods: This prospective study included adult patients with recurrent AE without wheals. Demographic and laboratory data of the patients were recorded in the patient fi le when they presented to the outpatient clinic between August 2018 and August 2020. The patients were contacted by phone to evaluate whether their AE had gone into remission between October 2023 and January 2024. The phenotypic characteristics of AE subtypes were compared. Results: The study included a total of 143 patients. The average age, age of onset of AE, rates of diabetes mellitus, hypertension and coronary artery disease were higher in the patients with angiotensin-converting enzyme inhibitor (ACEI) use related acquired AE (AAE) (AAE-ACEI). The rates of allergic rhinitis, drug allergy, atopy, and aeroallergen sensitivity, and the median total immunoglobulin E level were higher in patients with idiopathic histaminergic AAE (AAE-IH). The rate of face and/or perioral AE attacks was higher in the patients with AAE-ACEI, AAE-IH, and idiopathic non-histaminergic AAE. The rate of AE attacks in limbs, abdominal, genital and other parts of the body was higher in patients with hereditary AE (HAE). The baseline AE activity score was lower in the patients with AAE-IH and higher in the patients with HAE. In longterm follow-up, the remission rate of AE attacks was significant fi cant higher in patients with AAE-ACEI and AAE-IH. Conclusion: The phenotypic characteristic features of Turkish patients with AE without wheals may vary, depending on the underlying AE pathogenesis. C1 inhibitor level and function, complement C4 and C1q, and genetic tests contributed to the diagnosis; other laboratory tests did not contribute to the diagnosis.
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    Respiratory system evaluation of adult primary immunodeficiency patients: a tertiary care center experience
    (Polish Acad Sciences, Inst Immunol & Exp Therapy, 2025) Kaya, Saltuk Bugra; Cakmak, Mehmet Erdem; Bostan, Ozge Can; Damadoglu, Ebru; Karakaya, Gul; Kalyoncu, Ali Fuat; Esenboga, Saliha
    Introduction Primary immunodeficiencies (PIDs) are rare diseases in which chronic pulmonary diseases are common. Chronic pulmonary complications affect the long-term survival of these patients. The aim of this study was to evaluate the accompanying lung diseases and respiratory functions in adult PID patients in the Turkish population.Materials and Methods Patients' files who applied to the immunology clinic between 2015 and 2020 were evaluated retrospectively. The respiratory system was evaluated by physical examination, and if necessary, computed tomography, chest radiography, and pulmonary function test (PFT) were performed. The diagnosis of PIDs was based on the European Society of Immunodeficiency's (ESID) criteria.Results A total of 186 patients were included in the study. The median age of the patients was 38 years. The distribution of the diseases included in the study in order of frequency is: Common Variable Immunodeficiency (CVID) (47.8%), Severe Combined Immunodeficiency (SCID) (22.6%), Selective IgA deficiency (SIgAD) (10.8%), X-Linked Agammaglobulinemia (XLA) (10.2%), Chronic Granulomatous Disease (CGD) (8.6%). The most common findings on chest radiology were bronchiectasis (37.1%), parenchymal nodule (32.8%), ground glass opacity (31.2%), lymphadenopathy (24.7%), fibrotic changes (24.8%), reticular opacities (23.7%) and bronchial wall thickening (23.1%). PFT's results were lower in patients with CGD. Bronchiectasis (37.1%), asthma (22%), and tuberculosis (9.7%) were the most common lung complications.Conclusion We think that the frequency of other lung complications, especially asthma and bronchiectasis, is higher in adult patients with PIDs, and patient management is poor as there are no guidelines for the follow-up, diagnosis, and treatment of pulmonary complications.