Lu sur : https://www.ncbi.nlm.nih.gov/pubmed/28801338
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Rituximab as treatment for anti-MuSK myasthenia gravis: Multicenter blinded prospective review.
Hehir MK1, Hobson-Webb LD2, Benatar M2, Barnett C2, Silvestri NJ2, Howard JF Jr2, Howard D2, Visser A2, Crum BA2, Nowak R2, Beekman R2, Kumar A2, Ruzhansky K2, Chen IA2, Pulley MT2, LaBoy SM2, Fellman MA2, Greene SM2, Pasnoor M2, Burns TM2.
Neurology. 2017 Aug 11. pii: 10.1212/WNL.0000000000004341. doi: 10.1212/WNL.0000000000004341.
To evaluate the efficacy of rituximab in treatment of anti-muscle-specific kinase (MuSK) myasthenia gravis (MG).
This was a multicenter, blinded, prospective review, comparing anti-MuSK-positive patients with MG treated with rituximab to those not treated with rituximab. The primary clinical endpoint was the Myasthenia Gravis Status and Treatment Intensity (MGSTI), a novel outcome that combines the Myasthenia Gravis Foundation of America (MGFA) postintervention status (PIS) and the number and dosages of other immunosuppressant therapies used. A priori, an MGSTI of level ≤2 was used to define a favorable outcome. Secondary outcomes included modified MGFA PIS of minimal manifestations or better, mean/median prednisone dose, and mean/median doses of other immunosuppressant drugs.
Seventy-seven of 119 patients with anti-MuSK MG evaluated between January 1, 2005, and January 1, 2015, at 10 neuromuscular centers were selected for analysis after review of limited clinical data by a blinded expert panel. An additional 22 patients were excluded due to insufficient follow-up. Baseline characteristics were similar between the rituximab-treated patients (n = 24) and the controls (n = 31). Median follow-up duration was >3.5 years. At last visit, 58% (14/24) of rituximab-treated patients reached the primary outcome compared to 16% (5/31) of controls (p = 0.002). Number needed to treat for the primary outcome is 2.4. At last visit, 29% of rituximab-treated patients were taking prednisone (mean dose 4.5 mg/day) compared to 74% of controls (mean dose 13 mg/day) (p = 0.001 and p = 0.005).
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- CLASSIFICATION OF EVIDENCE:
This study provides Class IV evidence that for patients with anti-MuSK MG, rituximab increased the probability of a favorable outcome.