Follow-up and treatment of patients with Common Variable Immune Deficiency: A single-center experience

Zeynep Yüksel, A. Çalışkaner
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Abstract

Objectives: Common Variable Immunodeficiency (CVID) is a primary immunodeficiency characterized by immunoglobulin production defect. Our study aimed to create awareness of primary immunodeficiency in adult patients, establish standard approaches for clinical follow-up of CVID patients, and reveal the clinical characteristics of CVID patients in our region. Method: The study was conducted in patients with diagnosed and newly diagnosed CVID. The demographic and clinical characteristics of the patients and their treatment data were analyzed retrospectively and prospectively. Results: Thirteen of our patients were female and 12 were male. The mean age at diagnosis of the patients was 30.32 (2-57) and the mean delay in diagnosis was 9.32 months (0-30). The most common clinical finding of our patients at the time of admission was an infection. Among the infections identified, 3 patients had URTI, 19 had LRTI, and 2 had gastroenteritis. In 16 of our patients, bronchiectasis was detected at the time of diagnosis, and in 1 during the follow-up period. In the examinations performed in terms of organomegaly, splenomegaly was found in 11 patients and hepatomegaly was found in 8 patients. When patients were screened for autoimmune disease, ITP and celiac were found in 2 patients at the beginning, while autoimmune thyroiditis was developed in 1 patient and SLE in 1 patient during follow-up. Our patients were given IVIG treatment at regular intervals. The number of reactions seen in a total of 421 IVIG infusions was two. Conclusion: Primary immunodeficiencies should definitely be considered in patients with recurrent infections and resistance to antibiotic therapy. Patients should be followed according to established follow-up and treatment protocols in order to reduce and diagnose complications.
常见可变免疫缺陷患者的随访和治疗:单中心经验
目的:共同可变免疫缺陷(CVID)是一种以免疫球蛋白产生缺陷为特征的原发性免疫缺陷。本研究旨在提高成人原发性免疫缺陷患者的认识,建立CVID患者临床随访的标准方法,揭示本地区CVID患者的临床特点。方法:对已确诊和新确诊的CVID患者进行研究。回顾性和前瞻性分析患者的人口学、临床特征及治疗资料。结果:女性13例,男性12例。患者平均诊断年龄30.32岁(2 ~ 57岁),平均诊断延迟9.32个月(0 ~ 30岁)。我们的患者在入院时最常见的临床发现是感染。其中尿路感染3例,下呼吸道感染19例,胃肠炎2例。16例患者在诊断时发现支气管扩张,1例在随访期间发现支气管扩张。器官肿大检查中,脾肿大11例,肝肿大8例。在对患者进行自身免疫性疾病筛查时,最初有2例患者发现ITP和乳糜泻,随访中有1例患者出现自身免疫性甲状腺炎,1例患者出现SLE。我们的病人定期接受试管婴儿治疗。421例IVIG输注中出现2例反应。结论:复发性感染和抗生素耐药患者应考虑原发性免疫缺陷。患者应按照既定的随访和治疗方案进行随访,以减少和诊断并发症。
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