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Interstitial Lung Disease in Systemic Sclerosis

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Orphan Lung Diseases

Abstract

The last decade has seen major advances in the prognostic evaluation and treatment of systemic sclerosis-associated interstitial lung disease (SSc-ILD). Risk factors for the development of SSc-ILD include diffuse cutaneous disease and the presence of autoantibodies against topoisomerase I. Accumulated evidence indicative of a genetic predisposition to SSc has yet to provide added clinical value. High resolution computed tomography is highly sensitive in the identification of SSc-ILD but the identification of clinically significant requires the multidisciplinary integration of pulmonary function tests, CT data and the degree of exercise intolerance. Reversible inflammatory disease predominates in a minority of patients but in most cases, prevention of progression of pulmonary fibrosis is the most realistic therapeutic goal. Routine treatment largely consists of immunosuppressive therapy, with proof of concept provided by treatment effects in two controlled trials of cyclophosphamide. Based on these data, the current consensus is that cyclophosphamide therapy is appropriate when SSc-ILD is extensive or overtly progressive. However, historical immunosuppressive agents are only partially efficacious. Rituximab may have an important future role, based on promising pilot data, and there is now an opportunity to evaluate anti-fibrotic agents with treatment effects in other fibrotic interstitial lung diseases. However, when SSc-ILD is limited and non-progressive, an initial policy of non-intervention and careful observation is often warranted.

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Author information

Authors and Affiliations

  1. Interstitial Lung Disease Unit, Royal Brompton Hospital, Emmanuel Kaye Building, 1B Manresa Road, London, SW3 6LR, UK

    Athol U. Wells

  2. Department of Thoracic Medicine Medical School, University of Crete, 71110 Voutes, Heraklion, Crete Greece

    George A. Margaritopoulos

  3. Head of Molecular and Cellular Pneumonology Laboratory, Department of Thoracic Medicine Medical School, University of Crete, 71110 Voutes, Heraklion, Crete Greece

    Katerina M. Antoniou

  4. Department of Pathology, Royal Brompton Hospital, London, UK

    Andrew G. Nicholson

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  1. Athol U. Wells
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  2. George A. Margaritopoulos
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  3. Katerina M. Antoniou
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  4. Andrew G. Nicholson
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Correspondence to Athol U. Wells .

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Editors and Affiliations

  1. Rare Pulmonary Diseases, Hôpital Louis Pradel, Lyon, France

    Vincent Cottin

  2. Rare Pulmonary Diseases, Hôpital Louis Pradel, Lyon, France

    Jean-Francois Cordier

  3. Respiratory Medicine, University of Southampton, Southampton, United Kingdom

    Luca Richeldi

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Wells, A.U., Margaritopoulos, G.A., Antoniou, K.M., Nicholson, A.G. (2015). Interstitial Lung Disease in Systemic Sclerosis. In: Cottin, V., Cordier, JF., Richeldi, L. (eds) Orphan Lung Diseases. Springer, London. https://doi.org/10.1007/978-1-4471-2401-6_25

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