Interstitial Lung Disease in Systemic Sclerosis

  • Athol U. Wells
  • George A. Margaritopoulos
  • Katerina M. Antoniou
  • Andrew G. Nicholson


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.


Idiopathic Pulmonary Fibrosis Interstitial Lung Disease Force Vital Capacity Pulmonary Function Test High Resolution Compute Tomography 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


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Copyright information

© Springer-Verlag London 2015

Authors and Affiliations

  • Athol U. Wells
    • 1
  • George A. Margaritopoulos
    • 2
  • Katerina M. Antoniou
    • 3
  • Andrew G. Nicholson
    • 4
  1. 1.Interstitial Lung Disease UnitRoyal Brompton HospitalLondonUK
  2. 2.Department of Thoracic Medicine Medical SchoolUniversity of CreteHeraklionCrete Greece
  3. 3.Head of Molecular and Cellular Pneumonology Laboratory, Department of Thoracic Medicine Medical SchoolUniversity of CreteHeraklionCrete Greece
  4. 4.Department of PathologyRoyal Brompton HospitalLondonUK

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