[DFPP in myasthenia gravis: case report]

G Ital Nefrol. Jan-Feb 2015;32(1):gin/32.1.5.
[Article in Italian]

Abstract

Introduction: Myasthenia Gravis (MG) is a neuromuscular disease due to a decrease in the number of acetylcholine receptors (AChR) present at the level of the neuromuscular junction. It is characterized by weakness and muscle fatigue. The pathogenesis of MG would seem to be autoimmune (autoantibodies against AChR, Musk, Titin). The treatment of MG includes acetylcholinesterase inhibitors, immunosuppressants, intravenous human immunoglobulin, thymectomy and therapeutic apheresis.

Materials and methods: We report a case of a 40-year-old woman, suffering from MG, subjected to thymectomy, in therapy with corticosteroids, azathioprine and antagonist of acetylcholinesterase. The patient came under our observation for the appearance of a severe acute worsening of neurological disease unresponsive to medical therapy. She underwent a series of four treatments, every other day, of double filtration plasmapheresis (DFPP). RESULTS AND DISCUSSION : The DFPP removed from the patient's blood high-molecular-weight substances. It showed a reduction of Immunoglobulins, Fibrinogen, C3 and C4 complement fractions and anti ACh-R Ab. The DFPP resulted in disappearance of symptoms with improvement in motor and sensory conduction parameters evaluated by electromyography.

Conclusion: The DFPP quickly reduces the anti ACh-R Ab and anti Titin Ab, as well as the risk of infections and allergies, compared to Plasma Exchange. It improves clinical symptoms, therefore it is proved to be an effective therapy for the acute exacerbation of MG.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Cholinesterase Inhibitors
  • Female
  • Humans
  • Immunoglobulins, Intravenous / administration & dosage
  • Myasthenia Gravis / blood
  • Myasthenia Gravis / therapy*
  • Plasmapheresis / methods*
  • Thymectomy

Substances

  • Cholinesterase Inhibitors
  • Immunoglobulins, Intravenous