The Efficacy and Safety of Rituximab for the Treatment of Pediatric Autoimmune Neuroinflammatory Disorders at a Single Center
고영준, 심영규, 김우중, 김수연, 김헌민, 황희, 채종희, 최지은, 김기중, 임병찬,
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고영준 ( Ko Young-Jun )
Seoul National University College of Medicine Seoul National University Children’s Hospital Department of Pediatrics
심영규 ( Shim Young-Kyu )
Seoul National University College of Medicine Seoul National University Children’s Hospital Department of Pediatrics
김우중 ( Kim Woo-Joong )
Seoul National University College of Medicine Seoul National University Children’s Hospital Department of Pediatrics
김수연 ( Kim Soo-Yeon )
Seoul National University College of Medicine Seoul National University Children’s Hospital Department of Pediatrics
김헌민 ( Kim Hun-Min )
Seoul National University College of Medicine Seoul National University Children’s Hospital Department of Pediatrics
황희 ( Hwang Hee )
Seoul National University College of Medicine Seoul National University Children’s Hospital Department of Pediatrics
채종희 ( Chae Jong-Hee )
Seoul National University College of Medicine Seoul National University Children’s Hospital Department of Pediatrics
최지은 ( Choi Ji-Eun )
Seoul National University College of Medicine Seoul National University Children’s Hospital Department of Pediatrics
김기중 ( Kim Ki-Joong )
Seoul National University College of Medicine Seoul National University Children’s Hospital Department of Pediatrics
임병찬 ( Lim Byung-Chan )
Seoul National University College of Medicine Seoul National University Children’s Hospital Department of Pediatrics
Abstract
Purpose: Rituximab is increasingly used as a second-line treatment of neuroinflammatory disorders to improve clinical outcomes in cases refractory to conventional immunotherapy and to reduce relapses. This study aimed to demonstrate the efficacy and safety of rituximab used for pediatric autoimmune neuroinflammatory disorders.
Methods: We retrospectively reviewed the medical records of 32 patients (median age, 8.5 years; range, 1.1 to 17.1; 23 girls) who received rituximab treatment at Seoul National University Children’s Hospital. The disease subgroups were anti-N-methyl-D-aspartate (NMDA) receptor (anti-NMDAR) encephalitis (n=11), opsoclonus-myoclonus ataxia syndrome (OMAS) (n=10), other suspected autoimmune encephalitis (n=5), neuromyelitis optica spectrum disorder (n=4), and chronic inflammatory demyelinating polyneuropathy (n=2). Efficacy was measured by modified Rankin Scale (mRS) scores at the initiation of rituximab administration, at 2 months after initiation, and at the last follow-up. A favorable clinical outcome was defined as an improvement of ≥2 in the mRS score or achievement of an mRS score ≤2. Safety was assessed by reviewing infusion-related adverse events and infectious complications, including progressive multifocal leukoencephalopathy.
Results: Two months after the initiation of rituximab therapy, 21patients (65.6%) had a favorable response, while 26 (82.1%) had a favorable response at the last follow-up. Among the disease subgroups, anti-NMDAR encephalitis and OMAS showed especially good responses. Rituximab infusion-related adverse events were identified in nine patients (28.1%). All complications recovered spontaneously or with only symptomatic treatment.
Conclusion: Rituximab can be used safely for various pediatric autoimmune neuroinflammatory diseases. Rituximab is expected to improve clinical outcomes in pediatric patients with anti-NMDAR encephalitis and OMAS.
키워드
Rituximab; Anti-N-methyl-D-aspartate receptor encephalitis; Opsoclonus-myoclonus syndrome; Neuromyelitis optica
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