Five Years of Experience in a Single Center: Retrospective Analysis of Adult Patients with Common Variable Immunodeficiency

IF 0.3 Q4 ALLERGY
G. Aytekin, Eray Yıldız, Fatih Çölkesen, Ş. Arslan, A. Çalışkaner
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Abstract

Objective: Common Variable Immune Deficiency (CVID) is a heterogeneous immune disorder characterized by impaired and/or inadequate B cell differentiation with hypogammaglobulinemia. It is characterized by frequent and recurrent respiratory infections, autoimmune disorders, chronic lung diseases, granulomatous diseases, and increased risk for lymphoid malignancies. Materials and Methods: The medical records of 47 patients (22 females, 25 males) who had been followed up at our clinic and had sufficient data in their files were retrospectively reviewed. Patients were diagnosed with CVID according to the ESID (European Society for Immunodeficiency) criteria. Results: The median age of the patients was 32 (19-65) years. The most frequent clinical presentation of the patients was with recurrent upper respiratory infections (46%), pneumonia (29.8%), otitis media (23.4%) and chronic sinusitis (17%). During the follow-up period, 17 patients (36.8%) developed autoimmune complications, 14 (29.8%) of whom had autoimmune cytopenia. A total of 26 patients (55.3%) had bronchiectasis confirmed with computed tomography of the thorax. Lymphopenia was detected in 13 patients (27.7%). The median immunoglobulin level at the time of diagnosis was IgG 2.77 (0.33-6.90) g/L, IgM 0.31 (0.06-5.99) g/L, and IgA 0.25 (0.01- 5.02) g/L. Conclusion: CVID is very heterogeneous in terms of both clinical and laboratory features. Moreover, it is more common than expected, particularly in adulthood. The centers dealing with CVID should share their experiences in order to increase awareness among physicians.
在单一中心的五年经验:对成人常见变异性免疫缺陷患者的回顾性分析
目的:常见可变免疫缺陷(CVID)是一种异质性免疫疾病,其特征是B细胞分化受损和/或不足,伴有低丙种球蛋白血症。其特征是频繁和复发的呼吸道感染、自身免疫性疾病、慢性肺部疾病、肉芽肿性疾病以及淋巴恶性肿瘤风险增加。材料与方法:回顾性分析47例(女性22例,男性25例)患者的病历资料。根据ESID(欧洲免疫缺陷学会)标准,患者被诊断为CVID。结果:患者的中位年龄为32岁(19-65岁)。患者最常见的临床表现是复发性上呼吸道感染(46%)、肺炎(29.8%)、中耳炎(23.4%)和慢性鼻窦炎(17%)。在随访期间,17名患者(36.8%)出现自身免疫性并发症,其中14名患者(29.8%)患有自身免疫性细胞减少症。共有26名患者(55.3%)经胸部计算机断层扫描证实患有支气管扩张症。13例(27.7%)患者检测到淋巴管开放,诊断时免疫球蛋白的中位水平为IgG 2.77(0.33-6.90)g/L,IgM 0.31(0.06-5.99)g/L和IgA 0.25(0.01-5.02)g/L。结论:CVID在临床和实验室特征上具有很大的异质性。此外,它比预期的更常见,尤其是在成年期。处理CVID的中心应该分享他们的经验,以提高医生的意识。
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来源期刊
CiteScore
0.80
自引率
0.00%
发文量
10
期刊介绍: Asthma Allergy Immunology has been published three times a year in April, August and December as the official and periodical journal of the Turkish National Society of Allergy and Clinical Immunology since 2003. All articles published in the journal have been available online since 2003. A peer reviewed system is used in evaluation of the manuscripts submitted to Asthma Allergy Immunology. The official language of the journal is English. The aim of the journal is to present advances in the field of allergic diseases and clinical immunology to the readers. In accordance with this goal, manuscripts in the format of original research, review, case report, articles about clinical and practical applications and editorials, short report and letters to the editor about allergic diseases and clinical immunology are published in the journal. The target reader population of the Asthma Allergy Immunology includes specialists and residents of allergy and clinical immunology, pulmonology, internal medicine, pediatrics, dermatology and otolaryngology as well as physicians working in other fields of medicine interested in allergy and immunological diseases.
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