Successful Treatment of Neuropsychiatric Syndrome with Rituximab in a Patient with Systemic Lupus Erythematosus and Dermatomyositis Overlap Syndrome

Seung Geun Lee; Bin Yoo; Kyung Min Kim; Hyung Oh Choi; Seung-Su Nah; Yong Gil Kim; Chang-Keun Lee; Ji Seon Oh

doi:10.4078/jkra.2008.15.2.170

Journal List > J Korean Rheum Assoc > v.15(2) > 1003620

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Lee, Yoo, Kim, Choi, Nah, Kim, Lee, and Oh: Successful Treatment of Neuropsychiatric Syndrome with Rituximab in a Patient with Systemic Lupus Erythematosus and Dermatomyositis Overlap Syndrome

Original Article

J Korean Rheum Assoc 2008;15(2):170-174.

Published online: 20 June 2008

DOI: https://doi.org/10.4078/jkra.2008.15.2.170

Successful Treatment of Neuropsychiatric Syndrome with Rituximab in a Patient with Systemic Lupus Erythematosus and Dermatomyositis Overlap Syndrome

Seung Geun Lee, Bin Yoo, Kyung Min Kim, Hyung Oh Choi, Seung-Su Nah^∗, Yong Gil Kim, Chang-Keun Lee, Ji Seon Oh

Division of Rheumatology, Department of Internal Medicine, Asan Medical Center, College of Medicine, University of Ulsan, Seoul, Korea

^∗Division of Rheumatology, Department of Internal Medicine, College of Medicine, Soonchunhyang University, Cheonan, Korea

Received 30 March 2008 Accepted 2 May 2008

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

A 31-year-old woman was referred to our hospital and diagnosed as overlap syndrome with systemic lupus erythematosus and dermatomyositis. After completing the fourth cycle of intravenous immunoglobulin therapy, the patient developed acute confusional state with the Glasgow Coma Scale of 7. Considering the lack of response to high dose corticosteroid therapy (methylprednisolone 1 g per day for 3 days), rituximab (500 mg per week) was administered twice. The next day after the administration of the first dose of rituximab, the level of consciousness started to improve and 15 days after rituximab, mental status was fully recovered. The proportion of CD19+ B cells started to decrease within 1 week after the administration of rituximab and remained depleted for 14 weeks. There was also a gradual decrease in serum CD40 and CD80 concentration measured by ELISA up to 4 months. This case suggests the effect of rituximab for the treatment of neuropsychiatric lupus.

Keywords: Central nervous system lupus, Rituximab, CD40, CD80

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Fig. 1.

Serial changes in the proportion of CD19 (＋) lymphocytes, the concentration of C3 and C4, and the dosage of prednisolone. Transverse axis indicates days after the administration of rituximab. C: complement.

Fig. 2.

Serial change in the concentration of CD40 and CD80. Transverse axis indicates days after the administration of Rituximab. Vertical axis indicates the concentration of CD40 and CD80 measured by ELISA. (Reference value, CD40:7.8 ∼500 pg/mL, CD80:320∼20.000 pg/mL)

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