Interstitial lung disease in systemic sclerosis: current and future treatment

Abstract

Systemic sclerosis (SSc) has the highest fatality rate among connective tissue diseases and is characterized by vascular damage, inflammation and fibrosis of the skin and various internal organs. Interstitial lung disease (ILD) frequently complicates SSc and can be a debilitating disorder with a poor prognosis. ILD is the most frequent cause of death in SSc, and the management of SSc–ILD patients is a great challenge. Early detection of pulmonary involvement based on a recent decline of lung function tests and on the extent of lung involvement at high-resolution computed tomography is critical for the best management of these patients. This article summarizes classification, pathogenesis, diagnosis, prognosis, survival and finally current and future treatment options in SSc–ILD.

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Acknowledgements

The authors thank Mrs. Federica Sensini for her technical assistance.

Funding

This work was supported by FIRA (Fondazione Italiana Ricerca per l’Artrite) 2012 through the Young Investigator Award that Dr. Vasiliki Liakouli was a recipient (RN1366/2013).

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Correspondence to Roberto Giacomelli.

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RG, VL, OB, PR, PDB, FCa, GG, SDB, FCi, GT and PC declare that they have no conflict of interest for this work.

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This article does not contain any studies with animals or human participants performed directly by any of its authors. Authors of the included studies have declared in their published articles that their protocols were approved by institutional review boards or ethics committee at each participating site.

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The authors of this article did not directly involve any human subjects; however, the individual studies have declared obtaining informed consent from the patients.

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Giacomelli, R., Liakouli, V., Berardicurti, O. et al. Interstitial lung disease in systemic sclerosis: current and future treatment. Rheumatol Int 37, 853–863 (2017). https://doi.org/10.1007/s00296-016-3636-7

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Keywords

  • Systemic sclerosis
  • Scleroderma
  • Interstitial lung disease
  • Fibrosis
  • Treatment