新发现的老年类风湿关节炎患者的共同可变免疫缺陷

Oksana V. Moskaletc, Yuliya Ju. Tchuksina
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摘要

常见变异性免疫缺陷是一组异质性疾病,是主要缺乏抗体合成的原发性免疫缺陷。最常见的表现是呼吸道的慢性感染性病理,较少出现在胃肠道,以及脓毒性关节炎。非感染性表现包括自身免疫性疾病、非感染性腹泻、癌症、良性淋巴样增生。在一名患者中,几种综合征的组合是可能的。与大多数在儿童早期出现的原发性免疫缺陷不同,这种病理通常在生命的第三或第四个十年出现,有时在表面健康的人身上更晚。虽然常规的实验室方法用于确定主要种类免疫球蛋白的浓度,可以很容易地确定常见的变异性免疫缺陷患者,但诊断往往是在首次临床症状出现多年后做出的,此时严重的并发症已经出现。该病的特点是发病缓慢,病程缓慢,标准治疗无效。主要的治疗方法是静脉注射免疫球蛋白终身替代治疗。本文考虑了一个临床病例,当一个老年患者血清阴性类风湿关节炎与少年发病诊断多年后。免疫学研究的结果,包括外周血和骨髓淋巴细胞的免疫表型,提出。继发性低γ球蛋白血症和淋巴细胞增生性疾病的鉴别诊断问题被强调。据指出,到目前为止,各个专业的医生对原发性免疫缺陷的问题还不够熟悉,特别是当涉及到成人时,非感染性表现在临床图片中占主导地位。
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Newly identified common variable immunodeficiency in an elderly patient with rheumatoid arthritis
Common variable immunodeficiency is a group of heterogeneous diseases that are primary immunodeficiencies with a predominant lack of antibody synthesis. The most common manifestation is chronic infectious pathology of the respiratory tract, less often of the gastrointestinal tract, as well as septic arthritis. Non-infectious manifestations include autoimmune diseases, non-infectious diarrhea, cancer, benign lymphoid hyperplasia. In one patient, a combination of several syndromes is possible. Unlike most other primary immunodeficiencies that debut in early childhood, this pathology often manifests in the third or fourth decade of life, and sometimes later in apparently healthy people. Although the routine laboratory method for determining the concentration of the main classes of immunoglobulins makes it quite easy to identify patients with common variable immunodeficiency, often the diagnosis is made many years after the onset of the first clinical symptoms, when serious complications have already developed. The disease is characterized by a gradual onset, chronic progressive course and ineffectiveness of standard therapy. The main method of treatment is lifelong replacement therapy with intravenous immunoglobulins. The article considers a clinical case when an elderly patient with seronegative rheumatoid arthritis with a juvenile onset was diagnosed many years later. The results of immunological studies, including immunophenotyping of peripheral blood and bone marrow lymphocytes, are presented. Issues of differential diagnosis with secondary hypogammaglobulinemia and lymphoproliferative diseases are highlighted. It has been stated that until now, doctors of various specialties are not sufficiently familiar with the problem of primary immunodeficiencies, especially when it comes to adults, and non-infectious manifestations dominate in the clinical picture.
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