Elsevier

The Lancet

Volume 338, Issue 8768, 14 September 1991, Pages 661-662
The Lancet

SHORT REPORTS
Immunogenetic prediction of pulmonary fibrosis in systemic sclerosis

https://doi.org/10.1016/0140-6736(91)91235-MGet rights and content

Abstract

75 systemic sclerosis patients were independently tested for pulmonary fibrosis, autoantibodies, and MHC class II genes. 24 of 42 (57%) patients with pulmonary fibrosis had either HLA DR3/DRw52a or anti-Scl-70 vs 2 of 33 (6%) patients without pulmonary fibrosis. The presence of DR3/DRw52a or anti-Scl-70 gives a relative risk of 16·7 for the development of pulmonary fibrosis in a patient with scleroderma—a risk substantial enough to require careful monitoring of these patients and treatment at an early stage of disease.

References (9)

  • Ec Leroy et al.

    Scleroderma (systemic sclerosis): classification, subsets, and pathogenesis

    J Rheumatol

    (1982)
  • Cj Lynch et al.

    Histocompatibility antigens in progressive systemic sclerosis (scleroderma)

    J Clin Immunol

    (1982)
  • Vd Steen et al.

    Clinical correlations and prognosis based on serum autoantibodies in patients with systemic sclerosis

    Arthritis Rheum

    (1988)
  • M. Turner-Warwick et al.

    Cryptogenic fibrosing alveolitis: clinical features and their influence on survival

    Thorax

    (1980)
There are more references available in the full text version of this article.

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