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Organ-specific and nonorgan-specific autoimmune diseases commonly coexist with aquaporin4 antibody (AQP4-IgG)–positive neuromyelitis optica spectrum disorder (NMOSD). We report an unusual case in which a young woman with known systemic lupus erythematosus (SLE) was discovered to be AQP4-IgG seropositive when she developed simultaneous severe longitudinally extensive transverse myelitis, reversible intracranial vasculopathy, cutaneous vasculitis, and myocarditis. We discuss the pathogenic mechanisms that may underlie systemic and CNS disease activity in these coexisting diseases.

Reversible Cerebral Vasculopathy, Transverse Myelitis, and Active Systemic Lupus Erythematosus in an Aquaporin-4 Antibody–Positive Patient