Lee A H, Levinson A I, Schumacher H R
Division of Rheumatology, University of Pennsylvania School of Medicine, Philadelphia.
Semin Arthritis Rheum. 1993 Feb;22(4):252-64. doi: 10.1016/0049-0172(93)80073-o.
Primary hypogammaglobulinemia describes a heterogeneous group of immunoglobulin disorders mainly composed of X-linked agammaglobulinemia, common variable immunodeficiency, and selective immunoglobulin (Ig) A deficiency. The most serious problems are related to recurrent infections with high-grade encapsulated bacteria. However, a wide variety of rheumatologic disorders also occur in association with hypogammaglobulinemic states. Septic arthritis with usual bacterial pathogens such as Staphylococcus aureus, and unusual bacteria such as Mycoplasma and Ureaplasma species, have been described in these patients. An aseptic nonerosive polyarticular arthritis that resembles rheumatoid arthritis is seen in 10% to 30% of hypogammaglobulinemic patients. Autoimmune disorders such as immune thrombocytopenic purpura, immune hemolytic anemia, juvenile rheumatoid arthritis, systemic lupus erythematosus, dermatomyositis, Sjögren's syndrome, essential mixed cryoglobulinemia, chronic active hepatitis, and sarcoidosis have been reported in hypogammaglobulinemic patients. Finally, to complicate matters, many disease-modifying antirheumatic drugs, including gold, D-penicillamine, sulfasalazine, azathioprine, and cyclophosphamide, cause symptomatic hypogammaglobulinemia in some patients.
原发性低丙种球蛋白血症描述了一组异质性免疫球蛋白疾病，主要包括X连锁无丙种球蛋白血症、常见变异型免疫缺陷和选择性免疫球蛋白A缺乏症。最严重的问题与由高毒力包膜细菌引起的反复感染有关。然而，多种风湿性疾病也与低丙种球蛋白血症状态相关。在这些患者中已描述了由常见细菌病原体如金黄色葡萄球菌以及不常见细菌如支原体和脲原体引起的化脓性关节炎。10%至30%的低丙种球蛋白血症患者会出现一种类似于类风湿关节炎的无菌性非侵蚀性多关节炎。低丙种球蛋白血症患者中曾报告过自身免疫性疾病，如免疫性血小板减少性紫癜、免疫性溶血性贫血、青少年类风湿关节炎、系统性红斑狼疮、皮肌炎、干燥综合征、原发性混合性冷球蛋白血症、慢性活动性肝炎和结节病。最后，复杂的是，许多改善病情的抗风湿药物，包括金制剂、D-青霉胺、柳氮磺胺吡啶、硫唑嘌呤和环磷酰胺，在一些患者中会导致症状性低丙种球蛋白血症。
