Mild Encephalitis/Encephalopathy with Reversible Splenial Lesions in SLE.

A 25-year-old woman presented with fever, butterfly erythema, oral ulcers, and arthritis. Anti-nuclear and anti-Sm antibodies were positive, and she had hypocomplementemia, indicating a diagnosis of systemic lupus erythematosus (SLE). Twelve months later she developed hyperthermia, headache, and thrombocytopenia. Magnetic resonance imaging showed a lesion in the splenium of the corpus callosum (Picture 1), with an elevated cerebrospinal interluekin-6 level (18.8 pg/mL), which disappeared after 4 weeks of high-dose glucocorticoid therapy (Picture 2). Mild encephalitis/encephalopathy with reversible splenic lesions (MERS) associated with neuropsychiatric-SLE (NPSLE) was thus diagnosed. Glucocorticoids were tapered off without recurrence after 5 years. This is the first reported adult case of MERS with active NPSLE. Although NPSLE is difficult to treat, MERS with SLE may show a benign clinical course.

Picture 1.

Picture 2.

Author's disclosure of potential Conflicts of Interest (COI).

Kazuhisa Nakano: Honoraria, Bristol-Myers, Sanofi, AbbVie, Eisai, Eli Lilly, Chugai, Pfizer, Takeda and Mitsubishi-Tanabe; Research funding, Mitsubishi-Tanabe and Eisai. Yoshiya Tanaka: Honoraria, Daiichi-Sankyo, Astellas, Chugai, Eli Lilly, Pfizer, Abbvie, YL Biologics, Bristol-Myers, Takeda, Mitsubishi-Tanabe, Novartis, Eisai, Janssen and Teijin; Research funding, Asahi-kasei, Mitsubishi-Tanabe, Chugai, Takeda, Sanofi, Bristol-Myers, UCB, Daiichi-Sankyo, Eisai and Ono.