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Systemic lupus erythematosus-myasthenia gravis overlap syndrome: Presentation and treatment depend on prior thymectomy

机译: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

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摘要

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.
机译:在这项研究中,我们调查了四例符合重叠系统性红斑狼疮(SLE)和重症肌无力(MG)诊断标准的患者,但对治疗的反应不同。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SLE患者已通过MG胆碱酯酶抑制剂有效治疗。这些患者发生胸腺切除术后出现光敏性,皮疹和关节炎的SLE,分别在29年和40年之前进行。在新发作的SLE中,发现另外两名患者具有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。这些病例的进展与衰老过程中胸腺在狼疮发病中的作用以及SLE-MG重叠患者的治疗选择有关。

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