Department of Rheumatology and Immunology, Hannover Medical School, Hannover, Germany.
Cluster of Excellence RESIST (EXC 2155), Hannover Medical School, Hannover, Germany.
RMD Open. 2022 Dec;8(2). doi: 10.1136/rmdopen-2022-002664.
The clinical spectrum of primary antibody deficiencies (PADs) and especially common variable immunodeficiency (CVID) includes various autoimmune disorders. We studied the prevalence and the features of articular rheumatic disease in a cohort of patient with PADs.
In this retrospective cohort study, complete clinical data of 268 patients with PADs, mainly consisting of patients with CVID, visiting the immunology outpatient clinic of a German tertiary hospital between 2018 and 2021 were collected. Those included case history, physical examination, laboratory as well as radiological findings.
Inflammatory arthritis was diagnosed in 16.4% of studied patients and was significantly more common among patients with PAD-associated enteropathy (OR 13.39, p=0.0001), splenomegaly (OR 6.09, p=0.0001) or atopic diseases (OR 3.31, p=0.021). Given HLA-B27 status, the involvement of the axial skeleton and the presence of features, such as anterior uveitis, inflammatory bowel disease, psoriasis and/or dactylitis, 75% of studied patients fulfilled the Assessment of Spondyloarthritis International Society classification criteria.
PAD-associated arthritis frequently shares features with spondyloarthritis (SpA) and enteropathic arthritis. The latter may suggest the interconnected pathomechanisms of inflammatory arthritis in SpA and PADs.
原发性抗体缺陷症（PAD），尤其是常见可变免疫缺陷症（CVID）的临床表现包括各种自身免疫性疾病。我们研究了一组 PAD 患者关节风湿性疾病的患病率和特征。
在这项回顾性队列研究中，我们收集了 2018 年至 2021 年间在德国一家三级医院免疫门诊就诊的 268 名 PAD 患者的完整临床资料，这些患者主要为 CVID 患者。这些资料包括病史、体格检查、实验室和影像学发现。
研究中 16.4%的患者被诊断为炎症性关节炎，且 PAD 相关肠病（OR 13.39，p=0.0001）、脾肿大（OR 6.09，p=0.0001）或特应性疾病（OR 3.31，p=0.021）患者中炎症性关节炎更为常见。鉴于 HLA-B27 状态、轴骨受累以及前葡萄膜炎、炎症性肠病、银屑病和/或指（趾）炎等特征的存在，75%的研究患者符合脊柱关节炎国际学会评估分类标准。
与 PAD 相关的关节炎常与脊柱关节炎（SpA）和肠病性关节炎具有共同特征。后者可能提示 SpA 和 PAD 中炎症性关节炎的相互关联的发病机制。
