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Ann Clin Transl Neurol. 2018 Apr 14;5(6):710-716. doi: 10.1002/acn3.564. eCollection 2018 Jun.

The impact of rituximab infusion protocol on the long-term outcome in anti-MuSK myasthenia gravis.

Author information

1
Neuromuscular Diseases Unit Department of Neurology Hospital de la Santa Creu i Sant Pau Universitat Autònoma de Barcelona Barcelona Spain.
2
Centre for Biomedical Network Research on Rare Diseases (CIBERER) Madrid Spain.
3
Neuromuscular Diseases Unit Department of Neurology Hospital Universitari de Bellvitge - IDIBELL L'Hospitalet de Llobregat Barcelona Spain.
4
Neuromuscular Diseases Unit Department of Neurology Hospital Universitario Clínico San Carlos Madrid Spain.
5
ALS-Neuromuscular Unit Department of Neurology Hospital General Universitario Gregorio Marañón IISGM Madrid Spain.
6
Department of Neurology Hospital Universitario Cruces Baracaldo Spain.
7
Neuromuscular Diseases Unit Department of Neurology and Neurophysiology Hospital Universitario Virgen del Rocío Sevilla Spain.
8
Department of Neurology Hospital Clínico Santiago de Compostela Spain.
9
Department of Neurology Hospital Juan Ramón Jiménez Huelva Spain.
10
Department of Neurology Hospital Son Llàtzer Palma de Mallorca Spain.
11
Department of Neurology Hospital Universitario Central de Asturias Oviedo Spain.
12
Neuromuscular Unit Department of Neurology Hospital Universitari i Politècnic La Fe Valencia Spain.
13
Department of Medicine University of Valencia Valencia Spain.

Abstract

Objective:

To evaluate whether the clinical benefit and relapse rates in anti-muscle-specific kinase (MuSK) myasthenia gravis (MG) differ depending on the protocol of rituximab followed.

Methods:

This retrospective multicentre study in patients with MuSK MG compared three rituximab protocols in terms of clinical status, relapse, changes in treatment, and adverse side effects. The primary effectiveness endpoint was clinical relapse requiring a further infusion of rituximab. Survival curves were estimated using Kaplan-Meier methods and survival analyses were undertaken using Cox proportional-hazards models.

Results:

Twenty-five patients were included: 11 treated with protocol 4 + 2 (375 mg/m2/4 weeks, then monthly for 2 months), five treated with protocol 1 + 1 (two 1 g doses 2 weeks apart), and nine treated with protocol 4 (375 mg/m2/4 weeks). Mean follow-up was 5.0 years (SD 3.3). Relapse occurred in 18.2%, 80%, and 33.3%, and mean time to relapse was 3.5 (SD 1.5), 1.1 (SD 0.4), and 2.5 (SD 1.4) years, respectively. Based on Kaplan-Meier estimates, patients treated with protocol 4 + 2 had fewer and later relapses than patients treated with the other two protocols (log-rank test P = 0.0001). Patients treated with protocol 1 + 1 had a higher risk of relapse than patients treated with protocol 4 + 2 (HR 112.8, 95% CI, 5.7-2250.4, P = 0.002). Patients treated with protocol 4 showed a trend to a higher risk of relapse than those treated with protocol 4 + 2 (HR 9.2, 95% CI 0.9-91.8, P = 0.059).

Interpretation:

This study provides class IV evidence that the 4 + 2 rituximab protocol has a lower clinical relapse rate and produces a more durable response than the 1 + 1 and 4 protocols in patients with MuSK MG.

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