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Systemic lupus erythematosus-myasthenia gravis overlap syndrome: Presentation and treatment depend on prior thymectomy
Clinical Immunology ( IF 8.6 ) Pub Date : 2018-07-17 , DOI: 10.1016/j.clim.2018.07.007
Scott Brian Minchenberg 1 , Geeta Chaparala 1 , Zachary Oaks 1 , Katalin Banki 2 , Andras Perl 1
Affiliation  

In this study, we investigated four patients who met the diagnostic criteria for overlapping systemic lupus erythematosus (SLE) and myasthenia gravis (MG) but responded differently to treatment. All patients were acetylcholine receptor (AChR) and antinuclear antibody positive at the time of SLE diagnosis. Two patients presented with SLE who have been effectively treated with cholinesterase inhibitors for MG. These patients developed SLE with photosensitivity, rash, and arthritis post thymectomy, which had been performed 29 years and 40 years earlier, respectively. Two other patients were found to have AChR antibodies and MG in the context on new-onset SLE. These subjects were responsive to hydroxychloroquine and immunosuppression but failed cholinesterase inhibitors. The evolution of these cases is relevant for the role of thymus in lupus pathogenesis during aging and for treatment selection in SLE-MG overlap patients.



中文翻译:

系统性红斑狼疮-重症肌无力重叠综合征:表现和治疗取决于先前的胸腺切除术

在这项研究中,我们调查了四名符合重叠系统性红斑狼疮 (SLE) 和重症肌无力 (MG) 诊断标准但对治疗反应不同的患者。所有患者在 SLE 诊断时均为乙酰胆碱受体 (AChR) 和抗核抗体阳性。两名患有 SLE 的患者已接受胆碱酯酶抑制剂有效治疗 MG。这些患者在分别于 29 年前和 40 年前进行胸腺切除术后,出现了伴有光敏反应、皮疹和关节炎的 SLE。另外两名患者在新发 SLE 的情况下被发现有 AChR 抗体和 MG。这些受试者对羟氯喹和免疫抑制有反应,但胆碱酯酶抑制剂无效。这些病例的演变与胸腺在衰老过程中狼疮发病机制中的作用以及 SLE-MG 重叠患者的治疗选择有关。

更新日期:2018-07-17
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