Tess Litchman1, Bhaskar Roy1, Aditya Kumar1, Aditi Sharma1, Valentine Njike2, Richard J Nowak3. 1. Yale School of Medicine, Department of Neurology, New Haven, CT, USA. 2. Griffin Hospital-Derby, Yale University Prevention Research Center, Derby, CT, USA. 3. Yale School of Medicine, Department of Neurology, New Haven, CT, USA. Electronic address: richard.nowak@yale.edu.
Abstract
BACKGROUND: B-cell targeted therapy with rituximab has shown durable response in treating refractory myasthenia gravis (MG). This study compares the response to rituximab between patients with acetylcholine receptor autoantibody positive (AChR+) and muscle-specific kinase autoantibody positive (MuSK+) MG. METHODS: This retrospective study included 33 patients with either AChR+ or MuSK+ MG who were treated with rituximab from 05/31/2003 to 05/31/2017. Pretreatment and post-treatment immunotherapy regimens, clinical symptoms, and examination findings were evaluated. RESULTS: Median MGFA Class of II at baseline improved to an asymptomatic median classification at 12-months and last follow-up (p-values <.001) post-rituximab. Improvement in MGFA class was not significantly different between the groups. Twenty-one patients achieved clinical remission (12/17 AChR+, 9/16 MuSK+) with time to remission of 441.4 ± 336.6 days for AChR+ versus 230 ± 180.8 days for MuSK+ patients (p-value 0.049). The mean prednisone dosage requirement decreased significantly in both groups post-rituximab. AChR+ patients required more hospitalizations for exacerbation post-rituximab (p-value 0.046). CONCLUSION: Rituximab treatment response is observed in both AChR+ and MuSK+ patients supporting the role of B cell depletion in the management of MG. While there was no significant difference between these groups in terms of clinical improvement, symptom-free state, and prednisone burden, MuSK+ MG patients may experience greater benefits, including earlier time to remission, fewer exacerbations and hospitalizations post-treatment.
BACKGROUND: B-cell targeted therapy with rituximab has shown durable response in treating refractory myasthenia gravis (MG). This study compares the response to rituximab between patients with acetylcholine receptor autoantibody positive (AChR+) and muscle-specific kinase autoantibody positive (MuSK+) MG. METHODS: This retrospective study included 33 patients with either AChR+ or MuSK+ MG who were treated with rituximab from 05/31/2003 to 05/31/2017. Pretreatment and post-treatment immunotherapy regimens, clinical symptoms, and examination findings were evaluated. RESULTS: Median MGFA Class of II at baseline improved to an asymptomatic median classification at 12-months and last follow-up (p-values <.001) post-rituximab. Improvement in MGFA class was not significantly different between the groups. Twenty-one patients achieved clinical remission (12/17 AChR+, 9/16 MuSK+) with time to remission of 441.4 ± 336.6 days for AChR+ versus 230 ± 180.8 days for MuSK+ patients (p-value 0.049). The mean prednisone dosage requirement decreased significantly in both groups post-rituximab. AChR+ patients required more hospitalizations for exacerbation post-rituximab (p-value 0.046). CONCLUSION: Rituximab treatment response is observed in both AChR+ and MuSK+ patients supporting the role of B cell depletion in the management of MG. While there was no significant difference between these groups in terms of clinical improvement, symptom-free state, and prednisone burden, MuSK+ MGpatients may experience greater benefits, including earlier time to remission, fewer exacerbations and hospitalizations post-treatment.
Authors: Juan Carlos López-Hernández; Javier A Galnares-Olalde; Enrique Gómez-Figueroa; Adib Jorge de Sarachaga; Edwin Steven Vargas-Cañas Journal: Cureus Date: 2021-02-08