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[Myositis disclosing systemic scleroderma].

D Leys, P Y Hatron, M Parent

    Revue Neurologique
    |January 1, 1984
    PubMed
    Summary
    This summary is machine-generated.

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    Progressive systemic sclerosis can manifest as muscular atrophy and weakness, mimicking polymyositis. Corticosteroid therapy may not ensure full recovery in these rare cases.

    Area of Science:

    • Rheumatology
    • Neuromuscular Diseases
    • Dermatology

    Background:

    • Progressive systemic sclerosis (PSS) is a multisystem autoimmune disease characterized by fibrosis, vasculopathy, and autoantibodies.
    • The CREST syndrome (calcinosis, Raynaud phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia) is a variant of PSS.
    • Musculoskeletal manifestations, including myositis, are recognized complications of PSS, though often infrequent.

    Observation:

    • A 61-year-old patient presented with shoulder girdle muscular atrophy and weakness as the initial symptom.
    • Clinical and histological findings were consistent with polymyositis.
    • The patient exhibited other signs of the CREST syndrome, indicating underlying PSS.

    Findings:

    • Histological examination confirmed polymyositis in a patient with PSS and CREST syndrome.

    Related Experiment Videos

  • Corticosteroid therapy was initiated but did not result in complete recovery.
  • The case highlights the infrequent occurrence of myositis in PSS.

    • Implications:

    • This case underscores the importance of considering neuromuscular involvement in PSS, even when it presents as the primary symptom.
    • The nosological relationship between PSS, CREST syndrome, polymyositis, and Mixed Connective Tissue Disease warrants further investigation.
    • Treatment strategies for myositis in PSS may require optimization beyond standard corticosteroid therapy.