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Interstitial Lung Disease in Systemic Sclerosis

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Pulmonary Manifestations of Rheumatic Disease

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Abstract

Interstitial lung disease is one of the most prevalent complications in systemic sclerosis and is now the leading cause of death. Risk factors include early disease, scleroderma auto-antibodies particularly the anti-topoisomerase (anti-Scl 70) antibody. Pulmonary function tests and high resolution computed tomography should be performed annually to identify patients with potentially reversible disease. Treatment with cyclophosphamide has shown stabilization of disease and newer agents in ongoing large clinical trials will better define optimal management.

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Authors and Affiliations

  1. Department of Medicine, NIH-Inova Advanced Lung Disease Program, Falls Church, VA, USA

    Nargues Weir M.D.

  2. Department of Medicine, Georgetown University Medical Center, Washington, DC, USA

    Virginia Steen M.D.

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  1. Nargues Weir M.D.
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  2. Virginia Steen M.D.
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Correspondence to Virginia Steen M.D. .

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Editors and Affiliations

  1. Harvard Medical School, Department of Medicine, Division of Rheumatology Brigham and Women’s Hospital, Boston, Massachusetts, USA

    Paul F. Dellaripa

  2. Division of Rheumatology, Autoimmune and Interstitial Lung Disease Program, Department of Medicine, National Jewish Health, Denver, Colorado, USA

    Aryeh Fischer

  3. Department of Internal Medicine, Division of Pulmonary/Critical Care Medicine, University of Michigan Medical School, Ann Arbor, Michigan, USA

    Kevin R. Flaherty

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Weir, N., Steen, V. (2014). Interstitial Lung Disease in Systemic Sclerosis. In: Dellaripa, P., Fischer, A., Flaherty, K. (eds) Pulmonary Manifestations of Rheumatic Disease. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-0770-0_4

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