Prompt Response to Prednisone Predicts Benign Course in MuSK-MG

• Published in: European neurology Vol. 78; no. 3-4; p. 137
• Main Authors: Gungor-Tuncer, Ozlem, Yilmaz, Vuslat, Toker, Alper, Saruhan-Direskeneli, Guher, Gulsen-Parman, Yesim, Oflazer-Serdaroglu, Piraye, Deymeer, Feza
• Format: Journal Article
• Language: English
• Published: Switzerland 01.01.2017
• Subjects: Adult; Anti-Inflammatory Agents - therapeutic use; Autoantibodies - immunology; Female; Humans; Male; Middle Aged; Myasthenia Gravis - drug therapy; Myasthenia Gravis - immunology; Prednisone - therapeutic use; Receptor Protein-Tyrosine Kinases - immunology; Receptors, Cholinergic - immunology; Retrospective Studies; Treatment Outcome; Anti-muscle-specific tyrosine kinase antibody positive MG; Corticosteroids; Myasthenia gravis; Outcome; Muscle-specific kinase antibody
• ISSN: 1421-9913
• DOI: 10.1159/000479228

The difficult course of patients with myasthenia gravis (MG) with anti-muscle-specific tyrosine kinase antibodies (MuSK) has been emphasized. However, no clear information is available on patients who have a benign course. This study was aimed at comparing patients with favorable (minimal manifestations [MM] or better) and unfavorable outcomes to determine whether excellent response to corticosteroid (CS) treatment within 3 months (good response-3 months) has any predictive effect on the prognosis. Forty-six percent of 46 patients had a favorable outcome at year 3 and 54% at final follow-up. The major finding of this study was its high predictive value with good response-3 months. Those with good response-3 months had significantly more favorable outcome as compared to those without at year 3. The positive predictive value of good response-3 months was high (89% at year 3 and 84% at final follow-up). The negative predictive value diminished from 85% at year 3 to 67% at final follow-up due to increasing number of patients improving in the long run. Overall, 33% of the patients had a benign course with good response-3 months and no major exacerbations until the end of follow-up. Excellent response to CSs within 3 months appears to predict a favorable outcome in MuSK-MG.