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

• Published in: Clinical immunology (Orlando, Fla.) Vol. 194; pp. 100 - 104
• Main Authors: Minchenberg, Scott Brian, Chaparala, Geeta, Oaks, Zachary, Banki, Katalin, Perl, Andras
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01.09.2018
• Subjects: Acetylcholine receptor antibody; Antinuclear antibody; Antiphospholipid antibody; Female; Humans; Lupus Erythematosus, Systemic - diagnosis; Male; Middle Aged; Myasthenia gravis; Myasthenia Gravis - diagnosis; Systemic lupus erythematosus; Thymectomy; Thymectomy - methods; Undifferentiated Connective Tissue Diseases - diagnosis; CRP; anti-Sm; dRVVT; APS; MuSK; PT; CBC; Antiphospholipid antibody; SLE-MG; INR; SLE; Cr; AChR; Myasthenia gravis; Systemic lupus erythematosus; anti-AChR antibody; PNP; WBC; BUN; Hb; MG; aPL; Acetylcholine receptor antibody; ESR; HPPNA; anti-RNP; CK; NMJ; anti-dsDNA; Thymectomy; ANA; Antinuclear antibody
• ISSN: 1521-6616; 1521-7035; 1521-7035
• DOI: 10.1016/j.clim.2018.07.007

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.