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Review
. 2021 Feb 13;14(2):154.
doi: 10.3390/ph14020154.

The Treatment of Lung Involvement in Systemic Sclerosis

Barbara Ruaro  1 , Marco Confalonieri  1 , Marco Matucci-Cerinic  2 , Francesco Salton  1 , Paola Confalonieri  1 , Mario Santagiuliana  1 , Gloria Maria Citton  1 , Elisa Baratella  3 , Cosimo Bruni  2
Affiliations
Review

The Treatment of Lung Involvement in Systemic Sclerosis

Barbara Ruaro et al. Pharmaceuticals (Basel). .

Abstract

Systemic sclerosis (SSc) patients are often affected by interstitial lung disease (ILD) and, although there have been recent treatment advances, it remains the leading cause of death among SSc, with a 10-year mortality up to 40%. African Americans and subjects with diffuse cutaneous SSc or anti-topoisomerase 1 antibodies are most commonly affected. Currently, early ILD diagnosis can be made, and it is pivotal to improve the prognosis. The diagnostic mainstay test for SSc-ILD is high-resolution computed tomography for the morphology and pulmonary function tests for the functional aspects. Treatment planning and intensity are guided by the disease severity and risk of progression. Traditionally, therapy has depended on combinations of immunosuppressants, particularly cyclophosphamide and mycophenolate mofetil, which can be supplemented by targeted biological and antifibrotic therapies. Benefits have been observed in trials on hematopoietic autologous stem cell transplantation for patients with progressive SSc, whilst lung transplantation is reserved for refractory SSc-ILD cases. Herein, recent advances in SSc-ILD treatment will be explored.

Keywords: high-resolution computed tomography; interstitial lung disease; pulmonary function tests; scleroderma; systemic sclerosis.

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Conflict of interest statement

The authors declare that there is no conflict of interest.

References

    1. Varga J., Trojanowska M., Kuwana M. Pathogenesis of systemic sclerosis: Recent insights of molecular and cellular mechanisms and therapeutic opportunities. J. Scleroderma Relat. Disord. 2017;2:137–152. doi: 10.5301/jsrd.5000249. - DOI
    1. Ruaro B., Nallino M.G., Casabella A., Salton F., Confalonieri P., de Tanti A., Bruni C. Monitoring the microcirculation in the diagnosis and follow-up of systemic sclerosis patients: Focus on pulmonary and peripheral vascular manifestations. Microcirculation. 2020;27:e12647. doi: 10.1111/micc.12647. - DOI - PubMed
    1. Nihtyanova S.I., Denton C.P. Pathogenesis of systemic sclerosis associated interstitial lung disease. J. Scleroderma Relat. Disord. 2020;5:6–16. doi: 10.1177/2397198320903867. - DOI - PMC - PubMed
    1. Bruni C., de Luca G., Lazzaroni M.G., Zanatta E., Lepri G., Airo P., Dagna L., Doria A., Matucci-Cerinic M. Screening for pulmonary arterial hypertension in systemic sclerosis: A systematic literature review. Eur. J. Intern. Med. 2020;78:17–25. doi: 10.1016/j.ejim.2020.05.042. - DOI - PubMed
    1. Occhipinti M., Bruni C., Camiciottoli G., Bartolucci M., Bellando-Randone S., Bassetto A., Cuomo G., Giuggioli D., Ciardi G., Fabbrizzi A., et al. Quantitative analysis of pulmonary vasculature in systemic sclerosis at spirometry-gated chest CT. Ann. Rheum. Dis. 2020;79:1210–1217. doi: 10.1136/annrheumdis-2020-217359. - DOI - PubMed