Treatment for Systemic Sclerosis-associated Interstitial Lung Disease

Overview

Journal Curr Opin Rheumatol

Specialty Rheumatology

Date 2021 Mar 20

PMID 33741803

Citations 22

Authors

David Roofeh

Alain Lescoat

Dinesh Khanna

Affiliations

Soon will be listed here.

Abstract

Purpose Of Review: This review provides an overview of the current treatments for systemic sclerosis-interstitial lung disease (SSc-ILD) and proposes a conceptual framework for disease management with case scenarios.

Recent Findings: Broad treatment categories include traditional cytotoxic therapies, biologic disease-modifying rheumatic drugs, antifibrotic agents, autologous hematopoietic stem cell transplant, and lung transplantation. The optimal use of each option varies depending on SSc-ILD severity, progression, and comorbidities of individual patients. A high-quality randomized controlled trial demonstrated nintedanib's ability to retard decline of lung function in patients with limited and diffuse cutaneous disease, with established ILD. Tocilizumab, recently approved by the FDA, provides a unique intervention in those with early SSc associated with ILD with elevated acute-phase reactants: two well designed trials showed lung function preservation in phase 2 and phase 3 trials.

Summary: Stratifying patients based on key SSc-ILD characteristics (e.g. severity, risk of progression, comorbid disease presentation) may provide a useful guide for practitioners treating SSc-ILD.

Citing Articles

Systemic sclerosis-associated interstitial lung disease: How to manage in 2024?.

Bautista-Sanchez R, Khanna D Rheumatol Immunol Res. 2024; 5(3):157-165.

PMID: 39439972 PMC: 11492822. DOI: 10.2478/rir-2024-0022.

[Interdisciplinary centers for autoimmune diseases in Germany].

Worm M, Gunther C, Claussen M, Keysser G, Kotter I, Riemekasten G Z Rheumatol. 2024; 83(10):844-851.

PMID: 39052075 PMC: 11614946. DOI: 10.1007/s00393-024-01542-7.

Connective tissue disease-associated interstitial lung disease.

Storrer K, Muller C, Pessoa M, Pereira C J Bras Pneumol. 2024; 50(1):e20230132.

PMID: 38536980 PMC: 11095924. DOI: 10.36416/1806-3756/e20230132.

Immune profiling analysis of double-negative T cells in patients with systemic sclerosis.

Zhang D, Alip M, Chen H, Wu D, Zhu H, Han Y Clin Rheumatol. 2024; 43(5):1623-1634.

PMID: 38436769 DOI: 10.1007/s10067-024-06920-9.

A Multidisciplinary Approach as a Goal for the Management of Complications in Systemic Scleroderma: A Literature Review and Case Scenario.

Patrintasu D, Sarkozi H, Lupusor E, Vlangar I, Rotariu G, Renta I Diagnostics (Basel). 2023; 13(21).

PMID: 37958228 PMC: 10648338. DOI: 10.3390/diagnostics13213332.

References

Sullivan K, Goldmuntz E, Keyes-Elstein L, McSweeney P, Pinckney A, Welch B . Myeloablative Autologous Stem-Cell Transplantation for Severe Scleroderma. N Engl J Med. 2018; 378(1):35-47. PMC: 5846574. DOI: 10.1056/nejmoa1703327. View

Highland K, Distler O, Kuwana M, Allanore Y, Assassi S, Azuma A . Efficacy and safety of nintedanib in patients with systemic sclerosis-associated interstitial lung disease treated with mycophenolate: a subgroup analysis of the SENSCIS trial. Lancet Respir Med. 2021; 9(1):96-106. DOI: 10.1016/S2213-2600(20)30330-1. View

Wells A, Margaritopoulos G, Antoniou K, Denton C . Interstitial lung disease in systemic sclerosis. Semin Respir Crit Care Med. 2014; 35(2):213-21. DOI: 10.1055/s-0034-1371541. View

Miller M, Hankinson J, Brusasco V, Burgos F, Casaburi R, Coates A . Standardisation of spirometry. Eur Respir J. 2005; 26(2):319-38. DOI: 10.1183/09031936.05.00034805. View

Jaeger V, Wirz E, Allanore Y, Rossbach P, Riemekasten G, Hachulla E . Incidences and Risk Factors of Organ Manifestations in the Early Course of Systemic Sclerosis: A Longitudinal EUSTAR Study. PLoS One. 2016; 11(10):e0163894. PMC: 5051961. DOI: 10.1371/journal.pone.0163894. View

Nagaraja V, Matucci-Cerinic M, Furst D, Kuwana M, Allanore Y, Denton C . Current and Future Outlook on Disease Modification and Defining Low Disease Activity in Systemic Sclerosis. Arthritis Rheumatol. 2020; 72(7):1049-1058. PMC: 7329619. DOI: 10.1002/art.41246. View

Goh N, Desai S, Veeraraghavan S, Hansell D, Copley S, Maher T . Interstitial lung disease in systemic sclerosis: a simple staging system. Am J Respir Crit Care Med. 2008; 177(11):1248-54. DOI: 10.1164/rccm.200706-877OC. View

Das A, Kumar A, Arrossi A, Ghosh S, Highland K . Scleroderma-related interstitial lung disease: principles of management. Expert Rev Respir Med. 2019; 13(4):357-367. DOI: 10.1080/17476348.2019.1575732. View

Tashkin D, Elashoff R, Clements P, Goldin J, Roth M, Furst D . Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med. 2006; 354(25):2655-66. DOI: 10.1056/NEJMoa055120. View

10.

Khanna D, Denton C, Jahreis A, van Laar J, Frech T, Anderson M . Safety and efficacy of subcutaneous tocilizumab in adults with systemic sclerosis (faSScinate): a phase 2, randomised, controlled trial. Lancet. 2016; 387(10038):2630-2640. DOI: 10.1016/S0140-6736(16)00232-4. View

11.

Perelas A, Silver R, Arrossi A, Highland K . Systemic sclerosis-associated interstitial lung disease. Lancet Respir Med. 2020; 8(3):304-320. DOI: 10.1016/S2213-2600(19)30480-1. View

12.

Walker U, Saketkoo L, Distler O . Haematopoietic stem cell transplantation in systemic sclerosis. RMD Open. 2018; 4(1):e000533. PMC: 6045702. DOI: 10.1136/rmdopen-2017-000533. View

13.

Goswami R, Ray A, Chatterjee M, Mukherjee A, Sircar G, Ghosh P . Rituximab in the treatment of systemic sclerosis-related interstitial lung disease: a systematic review and meta-analysis. Rheumatology (Oxford). 2020; 60(2):557-567. DOI: 10.1093/rheumatology/keaa550. View

14.

Bernstein E, Peterson E, Sell J, DOvidio F, Arcasoy S, Bathon J . Survival of adults with systemic sclerosis following lung transplantation: a nationwide cohort study. Arthritis Rheumatol. 2015; 67(5):1314-22. DOI: 10.1002/art.39021. View

15.

Sircar G, Goswami R, Sircar D, Ghosh A, Ghosh P . Intravenous cyclophosphamide vs rituximab for the treatment of early diffuse scleroderma lung disease: open label, randomized, controlled trial. Rheumatology (Oxford). 2018; 57(12):2106-2113. DOI: 10.1093/rheumatology/key213. View

16.

Hatabu H, Hunninghake G, Richeldi L, Brown K, Wells A, Remy-Jardin M . Interstitial lung abnormalities detected incidentally on CT: a Position Paper from the Fleischner Society. Lancet Respir Med. 2020; 8(7):726-737. PMC: 7970441. DOI: 10.1016/S2213-2600(20)30168-5. View

17.

Tyndall A, Bannert B, Vonk M, Airo P, Cozzi F, Carreira P . Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Ann Rheum Dis. 2010; 69(10):1809-15. DOI: 10.1136/ard.2009.114264. View

18.

Kuwana M, Azuma A . Nintedanib: New indication for systemic sclerosis-associated interstitial lung disease. Mod Rheumatol. 2019; 30(2):225-231. DOI: 10.1080/14397595.2019.1696505. View

19.

Goh N, Hoyles R, Denton C, Hansell D, Renzoni E, Maher T . Short-Term Pulmonary Function Trends Are Predictive of Mortality in Interstitial Lung Disease Associated With Systemic Sclerosis. Arthritis Rheumatol. 2017; 69(8):1670-1678. DOI: 10.1002/art.40130. View

20.

Sundaram S, Chung L . An Update on Systemic Sclerosis-Associated Pulmonary Arterial Hypertension: a Review of the Current Literature. Curr Rheumatol Rep. 2018; 20(2):10. DOI: 10.1007/s11926-018-0709-5. View