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.
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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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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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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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DOI: https://doi.org/10.1007/s00296-016-3636-7