PMID- 29928654
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20220410
IS  - 2328-9503 (Print)
IS  - 2328-9503 (Electronic)
IS  - 2328-9503 (Linking)
VI  - 5
IP  - 6
DP  - 2018 Jun
TI  - The impact of rituximab infusion protocol on the long-term outcome in anti-MuSK 
      myasthenia gravis.
PG  - 710-716
LID - 10.1002/acn3.564 [doi]
AB  - 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/m(2)/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/m(2)/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.
FAU - Cortes-Vicente, Elena
AU  - Cortes-Vicente E
AD  - Neuromuscular Diseases Unit Department of Neurology Hospital de la Santa Creu i 
      Sant Pau Universitat Autonoma de Barcelona Barcelona Spain.
AD  - Centre for Biomedical Network Research on Rare Diseases (CIBERER) Madrid Spain.
FAU - Rojas-Garcia, Ricard
AU  - Rojas-Garcia R
AD  - Neuromuscular Diseases Unit Department of Neurology Hospital de la Santa Creu i 
      Sant Pau Universitat Autonoma de Barcelona Barcelona Spain.
AD  - Centre for Biomedical Network Research on Rare Diseases (CIBERER) Madrid Spain.
FAU - Diaz-Manera, Jordi
AU  - Diaz-Manera J
AD  - Neuromuscular Diseases Unit Department of Neurology Hospital de la Santa Creu i 
      Sant Pau Universitat Autonoma de Barcelona Barcelona Spain.
AD  - Centre for Biomedical Network Research on Rare Diseases (CIBERER) Madrid Spain.
FAU - Querol, Luis
AU  - Querol L
AD  - Neuromuscular Diseases Unit Department of Neurology Hospital de la Santa Creu i 
      Sant Pau Universitat Autonoma de Barcelona Barcelona Spain.
AD  - Centre for Biomedical Network Research on Rare Diseases (CIBERER) Madrid Spain.
FAU - Casasnovas, Carlos
AU  - Casasnovas C
AD  - Neuromuscular Diseases Unit Department of Neurology Hospital Universitari de 
      Bellvitge - IDIBELL L'Hospitalet de Llobregat Barcelona Spain.
FAU - Guerrero-Sola, Antonio
AU  - Guerrero-Sola A
AD  - Neuromuscular Diseases Unit Department of Neurology Hospital Universitario 
      Clinico San Carlos Madrid Spain.
FAU - Munoz-Blanco, Jose Luis
AU  - Munoz-Blanco JL
AD  - ALS-Neuromuscular Unit Department of Neurology Hospital General Universitario 
      Gregorio Maranon IISGM Madrid Spain.
FAU - Barcena-Llona, Jose Eulalio
AU  - Barcena-Llona JE
AD  - Department of Neurology Hospital Universitario Cruces Baracaldo Spain.
FAU - Marquez-Infante, Celedonio
AU  - Marquez-Infante C
AD  - Neuromuscular Diseases Unit Department of Neurology and Neurophysiology Hospital 
      Universitario Virgen del Rocio Sevilla Spain.
FAU - Pardo, Julio
AU  - Pardo J
AD  - Department of Neurology Hospital Clinico Santiago de Compostela Spain.
FAU - Martinez-Fernandez, Eva Maria
AU  - Martinez-Fernandez EM
AD  - Department of Neurology Hospital Juan Ramon Jimenez Huelva Spain.
FAU - Uson, Mercedes
AU  - Uson M
AD  - Department of Neurology Hospital Son Llatzer Palma de Mallorca Spain.
FAU - Oliva-Nacarino, Pedro
AU  - Oliva-Nacarino P
AD  - Department of Neurology Hospital Universitario Central de Asturias Oviedo Spain.
FAU - Sevilla, Teresa
AU  - Sevilla T
AD  - Centre for Biomedical Network Research on Rare Diseases (CIBERER) Madrid Spain.
AD  - Neuromuscular Unit Department of Neurology Hospital Universitari i Politecnic La 
      Fe Valencia Spain.
AD  - Department of Medicine University of Valencia Valencia Spain.
FAU - Illa, Isabel
AU  - Illa I
AD  - Neuromuscular Diseases Unit Department of Neurology Hospital de la Santa Creu i 
      Sant Pau Universitat Autonoma de Barcelona Barcelona Spain.
AD  - Centre for Biomedical Network Research on Rare Diseases (CIBERER) Madrid Spain.
LA  - eng
PT  - Journal Article
DEP - 20180414
PL  - United States
TA  - Ann Clin Transl Neurol
JT  - Annals of clinical and translational neurology
JID - 101623278
PMC - PMC5989782
EDAT- 2018/06/22 06:00
MHDA- 2018/06/22 06:01
PMCR- 2018/04/14
CRDT- 2018/06/22 06:00
PHST- 2018/02/02 00:00 [received]
PHST- 2018/03/07 00:00 [revised]
PHST- 2018/03/20 00:00 [accepted]
PHST- 2018/06/22 06:00 [entrez]
PHST- 2018/06/22 06:00 [pubmed]
PHST- 2018/06/22 06:01 [medline]
PHST- 2018/04/14 00:00 [pmc-release]
AID - ACN3564 [pii]
AID - 10.1002/acn3.564 [doi]
PST - epublish
SO  - Ann Clin Transl Neurol. 2018 Apr 14;5(6):710-716. doi: 10.1002/acn3.564. 
      eCollection 2018 Jun.