J Korean Rheum Assoc.  2010 Dec;17(4):437-441. 10.4078/jkra.2010.17.4.437.

Co-occurrence of Myasthenia Gravis in a Patient with Systemic Sclerosis-Sjogren's Syndrome without D-penicillamine Therapy

Affiliations
  • 1Department of Internal Medicine, National Health Insurance Corporation Ilsan Hospital, Goyang, Korea. chanheell@paran.com
  • 2Department of Neurology, National Health Insurance Corporation Ilsan Hospital, Goyang, Korea.
  • 3Department of Nuclear Medicine, National Health Insurance Corporation Ilsan Hospital, Goyang, Korea.

Abstract

Systemic sclerosis is an autoimmune disease characterized by progressive fibrosis of the skin and visceral organs. Myasthenia gravis is also an autoimmune disease characterized by weakness and fatigue of skeletal muscles. The symptoms of systemic sclerosis and myasthenia gravis overlap clinically, so the recognition of disease co-occurrence may be delayed. Co-occurrence of myasthenia gravis and systemic sclerosis is very uncommon and usually diagnosed after use of D-penicillamine for treating the systemic sclerosis. We report a case of a 49-year-old female patient who complained of general weakness and was diagnosed with myasthenia gravis. Four months earlier she was diagnosed with systemic sclerosis with Sjogren's syndrome and her medications did not include D-penicillamine.

Keyword

Scleroderma; Myasthenia gravis; Sjogren's syndrome

MeSH Terms

Autoimmune Diseases
Fatigue
Female
Fibrosis
Humans
Middle Aged
Muscle, Skeletal
Myasthenia Gravis
Penicillamine
Scleroderma, Systemic
Sjogren's Syndrome
Skin
Penicillamine

Figure

  • Fig. 1. Salivary gland scan showed diffusely decreased uptake of radiotracer in the bilateral parotid (arrows) and submandibular glands (arrow heads).

  • Fig. 2. Repetitive nerve stimulation test revealed a decremental response in the right flexor carpi ulnaris muscle of more than 10% at a low stimulation rate (5 Hz), (A) but no decremental response in the right orbicularis oculi muscle (B).


Reference

1). Kucharz EJ. Thyroid disorders in patients with progressive systemic sclerosis: a review. Clin Rheumatol. 1993. 12:159–61.
Article
2). Lawrence RC., Helmick CG., Arnett FC., Deyo RA., Felson DT., Giannini EH, et al. Estimates of the prevalence of arthritis and selected musculoskeletal disorders in the United States. Arthritis Rheum. 1998. 41:778–99.
Article
3). Steen VD., Blair S., Medsger TA Jr. The toxicity of D-penicillamine in systemic sclerosis. Ann Intern Med. 1986. 104:699–705.
Article
4). Park DH., Lee EY., Ahn MS., Kim I., Chung SM., Shin JH, et al. A case of myasthenia gravis in a patient with diffuse systemic sclerosis developed during D-penicillamine treatment. J Korean Rheum Assoc. 2002. 9:131–6.
5). Jaretzki A., Barohn RJ., Ernstoff RM. Myasthenia gravis: recommendations for clinical research standards. Task force of the medical scientific advisory board of the myasthenia gravis foundation of America. Neurology. 2000. 55:16–23.
6). Kang SW., Lee YJ., Cha HS., Kim HA., Park MH., Oh MD, et al. Study on the clinical characteristics of systemic sclerosis. Korean J Med. 1999. 57:979–87.
7). Bhalla R., Swedler WI., Lazarevic MB., Ajmani HS., Skosey JL. Myasthenia gravis and scleroderma. J Rheumatol. 1993. 20:1409–10.
8). Thorlacius S., Aarli JA., Riise T., Matre R., Johnsen HJ. Associated disorders in myasthenia gravis: autoimmune diseases and their relation to thymectomy. Acta Neurol Scand. 1989. 80:290–5.
Article
9). Mitchell GW., Lichtenfeld PJ., McDonald CJ. Myasthenia gravis and scleroderma. An unusual combination of diseases. JAMA. 1975. 233:531.
Article
10). Averbuch-Heller L., Steiner I., Abramsky O. Neurologic manifestations of progressive systemic sclerosis. Arch Neurol. 1992. 49:1292–5.
Article
11). Keesey JC. Clinical evaluation and management of myasthenia gravis. Muscle Nerve. 2004. 29:484–505.
Article
12). Lee DW., Lee SM., Lee JH., Sin HJ., Chung JS., Cho GJ, et al. A case of severe aplastic anemia induced by d-penicillamine in systemic scleroderma. Korean J Med. 2005. 69:S970–3.
13). Drosos AA., Christou L., Galanopoulou V., Tzioufas AG., Tsiakou EK. D-Penicillamine induced myasthenia gravis: clinical, serological and genetic findings. Clin Exp Rheumatol. 1993. 11:387–91.
14). Hill M., Moss P., Wordsworth P., Newsom-Davis J., Wilcox N. T-cell responses to D-penicillamine in drug induced Myasthenia gravis: recognition of modified DR1: peptide complex. J Neuroimmunol. 1999. 97:146–53.
15). Zivkovic SA., Medsger TA Jr. Myasthenia gravis and scleroderma: two cases and a review of the literature. Clin Neurol Neurosurg. 2007. 109:388–91.
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