This site is intended for healthcare professionals
Journals
  • Home
  • /
  • Journals
  • /
  • Myasthenia Gravis
  • /
  • Frequency and clinical features of treatment-refra...
Journal

Frequency and clinical features of treatment-refractory myasthenia gravis

Read time: 1 mins
Published:1st Apr 2020
Author: Rath J, Brunner I, Tomschik M, Zulehner G, Hilger E, Krenn M et al.
Availability: Free full text
Ref.:J Neurol. 2020 Apr;267(4):1004-1011.
DOI:10.1007/s00415-019-09667-5
Frequency and clinical features of treatment-refractory myasthenia gravis


Background:
To investigate the frequency and characterize the clinical features of treatment-refractory myasthenia gravis in an Austrian cohort.

Methods: Patient charts of 126 patients with generalized myasthenia gravis and onset between 2000 and 2016 were analyzed retrospectively. Patients were classified as treatment-refractory according to strict, predefined criteria. These mandated patients being at least moderately symptomatic (i.e., MGFA class III) or needing either maintenance immunoglobulins or plasma exchange therapy for at least 1 year in spite of two adequately dosed immunosuppressive drugs. Clinical features and outcome at last follow-up were compared to treatment-responsive patients.

Results: 14 out of 126 patients (11.1%) met these criteria of treatment-refractory myasthenia gravis. Treatment-refractory patients had more frequent clinical exacerbations and more often received rescue treatments or a further escalation of immunosuppressive therapies. They also remained more severely affected at last follow-up. An early onset of myasthenia gravis was associated with a higher risk for a refractory course.

Conclusion: A small subgroup of patients with generalized myasthenia gravis do not respond sufficiently to standard therapies. Refractory disease has considerable implications for both patients and health care providers and highlights an unmet need for new treatment options.


Read abstract on library site  Access full article