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Biomedicines
Volume 10
Issue 2
10.3390/biomedicines10020504
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Review

Emerging Evidence and Treatment Perspectives from Randomized Clinical Trials in Systemic Sclerosis: Focus on Interstitial Lung Disease

by
Caterina Oriana Aragona
1,
Antonio Giovanni Versace
1,
Carmelo Ioppolo
1,
Daniela La Rosa
1,
Rita Lauro
1,
Maria Concetta Tringali
1,
Simona Tomeo
1,
Guido Ferlazzo
2,
William Neal Roberts
3,
Alessandra Bitto
1,
Natasha Irrera
1,† and
Gianluca Bagnato
1,*,†
1
Department of Clinical and Experimental Medicine, University of Messina, 98125 Messina, Italy
2
Department of Human Pathology “G. Barresi”, University of Messina, 98125 Messina, Italy
3
Department of Medicine, University of Kentucky, Lexington, KY 40506, USA
*
Author to whom correspondence should be addressed.
†
These authors contributed equally to this work.
Biomedicines 2022, 10(2), 504; https://doi.org/10.3390/biomedicines10020504
Submission received: 26 January 2022 / Revised: 16 February 2022 / Accepted: 17 February 2022 / Published: 21 February 2022
(This article belongs to the Special Issue Frontiers of Targeted Therapy and Predictors of Treatment Response in Systemic Sclerosis)
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Abstract

Systemic sclerosis (SSc) is a complex rare autoimmune disease with heterogeneous clinical manifestations. Currently, interstitial lung disease (ILD) and cardiac involvement (including pulmonary arterial hypertension) are recognized as the leading causes of SSc-associated mortality. New molecular targets have been discovered and phase II and phase III clinical trials published in the last 5 years on SSc-ILD will be discussed in this review. Details on the study design; the drug tested and its dose; the inclusion and exclusion criteria of the study; the concomitant immunosuppression; the outcomes and the duration of the study were reviewed. The two most common drugs used for the treatment of SSc-ILD are cyclophosphamide and mycophenolate mofetil, both supported by randomized controlled trials. Additional drugs, such as nintedanib and tocilizumab, have been approved to slow pulmonary function decline in SSc-ILD. In this review, we discuss the therapeutic alternatives for SSc management, offering the option to customize the design of future studies to stratify SSc patients and provide a patient-specific treatment according to the new emerging pathogenic features of SSc-ILD.
Keywords: systemic sclerosis; interstitial lung disease; clinical trial systemic sclerosis; interstitial lung disease; clinical trial

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MDPI and ACS Style

Aragona, C.O.; Versace, A.G.; Ioppolo, C.; La Rosa, D.; Lauro, R.; Tringali, M.C.; Tomeo, S.; Ferlazzo, G.; Roberts, W.N.; Bitto, A.; et al. Emerging Evidence and Treatment Perspectives from Randomized Clinical Trials in Systemic Sclerosis: Focus on Interstitial Lung Disease. Biomedicines 2022, 10, 504. https://doi.org/10.3390/biomedicines10020504

AMA Style

Aragona CO, Versace AG, Ioppolo C, La Rosa D, Lauro R, Tringali MC, Tomeo S, Ferlazzo G, Roberts WN, Bitto A, et al. Emerging Evidence and Treatment Perspectives from Randomized Clinical Trials in Systemic Sclerosis: Focus on Interstitial Lung Disease. Biomedicines. 2022; 10(2):504. https://doi.org/10.3390/biomedicines10020504

Chicago/Turabian Style

Aragona, Caterina Oriana, Antonio Giovanni Versace, Carmelo Ioppolo, Daniela La Rosa, Rita Lauro, Maria Concetta Tringali, Simona Tomeo, Guido Ferlazzo, William Neal Roberts, Alessandra Bitto, and et al. 2022. "Emerging Evidence and Treatment Perspectives from Randomized Clinical Trials in Systemic Sclerosis: Focus on Interstitial Lung Disease" Biomedicines 10, no. 2: 504. https://doi.org/10.3390/biomedicines10020504

APA Style

Aragona, C. O., Versace, A. G., Ioppolo, C., La Rosa, D., Lauro, R., Tringali, M. C., Tomeo, S., Ferlazzo, G., Roberts, W. N., Bitto, A., Irrera, N., & Bagnato, G. (2022). Emerging Evidence and Treatment Perspectives from Randomized Clinical Trials in Systemic Sclerosis: Focus on Interstitial Lung Disease. Biomedicines, 10(2), 504. https://doi.org/10.3390/biomedicines10020504

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