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X连锁无丙种球蛋白血症与类风湿关节炎。

X linked agammaglobulinaemia and rheumatoid arthritis.

作者信息

Verbruggen G, De Backer S, Deforce D, Demetter P, Cuvelier C, Veys E, Elewaut D

机构信息

Department of Rheumatology, University Hospital Gent, De Pintelaan 185, 9000 Gent, Belgium.

出版信息

Ann Rheum Dis. 2005 Jul;64(7):1075-8. doi: 10.1136/ard.2004.030049. Epub 2004 Nov 25.

DOI:10.1136/ard.2004.030049
PMID:15564308
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC1755569/
Abstract

BACKGROUND

Much interest has been shown recently in the pathogenic role of B cells in rheumatoid arthritis (RA) owing to the marked clinical responses to anti-CD20 treatment in RA.

CASE REPORT

A patient with X linked agammaglobulinaemia (XLA) presented with an erosive symmetric polyarthritis with histological features of RA, including formation of a destructive pannus. Furthermore, the patient developed subcutaneous nodules that were histologically indistinguishable from rheumatoid nodules. Surprisingly, lymphocytic infiltrates in both the synovium and nodule consisted almost exclusively of CD8+ T cells.

DISCUSSION

Although some peculiar B cell subsets have been described in patients with XLA, no B cell subsets could be demonstrated in synovial tissue or the subcutaneous nodule. This case illustrates that classical RA can develop in the absence of mature B cells.

摘要

背景

由于类风湿关节炎(RA)患者对抗CD20治疗有显著临床反应,近来人们对B细胞在RA中的致病作用产生了浓厚兴趣。

病例报告

一名患有X连锁无丙种球蛋白血症(XLA)的患者出现了具有RA组织学特征的侵蚀性对称性多关节炎,包括破坏性血管翳的形成。此外,该患者还出现了组织学上与类风湿结节无法区分的皮下结节。令人惊讶的是,滑膜和结节中的淋巴细胞浸润几乎完全由CD8 + T细胞组成。

讨论

尽管在XLA患者中已描述了一些特殊的B细胞亚群,但在滑膜组织或皮下结节中未发现B细胞亚群。该病例表明,在没有成熟B细胞的情况下也可发生典型的RA。

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