Reviews
Pharmacological treatment of systemic sclerosis-associated interstitial lung disease: an updated review and current approach to patient care
F.A. Mendoza1, T. Allawh2, S.A. Jimenez3
- Rheumatology Division, Department of Medicine, and Jefferson Institute of Molecular Medicine and Scleroderma Center, Thomas Jefferson University, Philadelphia, PA, USA. fam002@jefferson.edu
- Rheumatology Division, Department of Medicine, Thomas Jefferson University, Philadelphia, PA, USA.
- Jefferson Institute of Molecular Medicine and Scleroderma Center, Thomas Jefferson University, Philadelphia, PA, USA.
CER16355
2023 Vol.41, N°8
PI 1704, PF 1712
Reviews
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PMID: 37382458 [PubMed]
Received: 10/11/2022
Accepted : 24/02/2023
In Press: 22/06/2023
Published: 03/08/2023
Abstract
Interstitial lung disease (ILD) has a high prevalence among patients with systemic sclerosis (SSc), carrying high mortality and morbidity. During the last decade, the emergence of new pharmacological therapies for SSc-associated ILD (SSc-ILD) and improved tools for its diagnosis and monitoring have changed the prevailing clinical approach, highlighting the need for early recognition and prompt treatment for SSc-ILD. Furthermore, the recent approval of multiple therapies for SSc-ILD poses challenges for the rheumatologist and pulmonologist in choosing the appropriate therapy for individual clinical scenarios. We review the pathophysiology of SSc-ILD, and the mechanisms of action and rationale behind current therapies. We also review the evidence of the efficacy and safety of immunosuppressive drugs, antifibrotic agents, and immunomodulators from cyclophosphamide and mycophenolate to novel agents such as nintedanib and tocilizumab. We also emphasise the importance of early diagnosis and monitoring and describe our approach to pharmacological therapy for SSc-ILD patients.
