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.
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.
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。
Zotero 插件