title: Myasthenia gravis and scleroderma: two cases and a review of the literature.
abstract: Myasthenia gravis is uncommon in patients with scleroderma, and when diagnosed is usually associated with previous use of d-penicillamine. Clinically, both myasthenia and scleroderma may present with fatigue, weakness and bulbar symptoms, so one of diagnoses may be delayed. We report two new cases and review clinical features of 12 other reported cases of co-existing scleroderma and myasthenia gravis, unrelated to previous d-penicillamine therapy. Co-occurrence of myasthenia and scleroderma was reported almost exclusively (13/14) in women with a mean latency of 7.03 years. Most patients (10/11) had seropositive generalized myasthenia, and there were no cases with exclusively ocular symptoms. Three patients with pre-existing myasthenia were safely treated with d-penicillamine. Myasthenia and scleroderma occur in the context of an underlying autoimmune diathesis, but their co-occurrence could be underreported as the recognition of either disorder may be delayed by overlapping clinical symptoms. Our findings also suggest that d-penicillamine may be cautiously used in selected patients with pre-existing scleroderma and myasthenia, when potential benefits outweigh the risk of possible myasthenia exacerbation.
authors.affiliation: VA Pittsburgh Healthcare System, Pittsburgh, PA 15240, United States. zivkovics@upmc.edu
authors.forename: Sasa A
authors.initials: SA
authors.lastname: Zivković
authors.forename: Thomas A
authors.initials: TA
authors.lastname: Medsger
mesh_terms: D000328:Adult; D000974:Antibodies, Antinuclear / Q000097:blood; D001323:Autoantibodies / Q000097:blood; D015897:Comorbidity; D005500:Follow-Up Studies; D050031:Hashimoto Disease / Q000175:diagnosis; D006801:Humans; D008875:Middle Aged; D009157:Myasthenia Gravis / Q000175:diagnosis* / Q000601:surgery; D009460:Neurologic Examination; D010398:Penicillic Acid / Q000008:administration & dosage / Q000031:analogs & derivatives; D011928:Raynaud Disease / Q000175:diagnosis; D012595:Scleroderma, Systemic / Q000175:diagnosis* / Q000188:drug therapy; D012859:Sjogren's Syndrome / Q000175:diagnosis; D013934:Thymectomy; D013952:Thymus Hyperplasia / Q000175:diagnosis / Q000601:surgery
chemical_list: D000974:Antibodies, Antinuclear; D001323:Autoantibodies; C028100:penicillamide; D010398:Penicillic Acid