title: Treatment of severe myasthenia gravis with protein A immunoadsorption and cyclophosphamide.
abstract: Myasthenia gravis (MG) is an autoimmune disease of the neuromuscular junction caused by antibodies against the nicotinic acetylcholine receptor (AChR-Ab). We report a 16-year-old girl with severe MG who showed a poor response to plasma exchange and tryptophan-linked polyvinylalcohol gel immunoadsorption. Further improvement of muscle strength and decline of AChR-Ab could be achieved after initiation of protein-A immunoadsorption (PA-IA). Maintenance therapy with PA-IA and intravenous pulses of cyclophosphamide resulted in a stabilisation of the disease, with a complete remission during the follow-up period of six months. We suggest that PA-IA may be valuable and safe in the management of patients with severe MG, and maintenance therapy with PA-IA and cyclophosphamide may prevent serious and potentially life-threatening relapses of the disease.
authors.affiliation: Department of Neurology, Julius-Maximilians-Universitaet Wuerzburg, Germany.
authors.forename: P
authors.initials: P
authors.lastname: Flachenecker
authors.forename: B M
authors.initials: BM
authors.lastname: Taleghani
authors.forename: R
authors.initials: R
authors.lastname: Gold
authors.forename: R
authors.initials: R
authors.lastname: Grossmann
authors.forename: D
authors.initials: D
authors.lastname: Wiebecke
authors.forename: K V
authors.initials: KV
authors.lastname: Toyka
mesh_terms: D000293:Adolescent; D003520:Cyclophosphamide / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D007163:Immunosorbent Techniques; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D009157:Myasthenia Gravis / Q000628:therapy*; D013205:Staphylococcal Protein A