Pulmonary Hypertension in the CREST Syndrome: Variant of Systemic Sclerosis (Scleroderma) — A Case Report

DOI Web Site 2 Citations
  • J.T. Lie
    Department of Pathology, Mayo Clinic and Mayo Medical School, Rochester, Minnesota

Abstract

<jats:p> The CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia) is a variant but not a benign form of scleroderma. Pulmonary hypertension without pulmonary fibrosis appears to be more prevalent in the CREST than in scleroderma patients. Moreover, pulmonary hypertension in CREST may be rapidly progressive and a cause of sudden death from severe cor pulmonale. </jats:p>

Journal

  • Angiology

    Angiology 40 (8), 764-767, 1989-08

    SAGE Publications

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