Scleroderma-related interstitial lung disease
- PMID: 28761806
- PMCID: PMC5524221
- DOI: 10.1016/j.rmcr.2017.07.007
Scleroderma-related interstitial lung disease
Abstract
Scleroderma-related interstitial lung disease (SSc-ILD) is a pulmonary fibrosing disorder characterized by systemic inflammation and progressive scarring of the lungs that leads to respiratory failure. Although certain immunosuppressive therapies may slow disease progression, current treatment strategies are not curative; consequently, SSc-ILD continues to be a major cause of morbidity and mortality. We present four cases of SSc-ILD that emphasize the importance of early screening and detection, close follow-up, and aggressive management. We also highlight the need for well-conducted clinical trials designed to identify new and effective treatments.
Keywords: Immunosuppression; Progressive systemic sclerosis; Pulmonary fibrosis.
Figures
Similar articles
-
Systemic Sclerosis Associated Interstitial Lung Disease: New Directions in Disease Management.Front Med (Lausanne). 2019 Oct 31;6:248. doi: 10.3389/fmed.2019.00248. eCollection 2019. Front Med (Lausanne). 2019. PMID: 31737640 Free PMC article. Review.
-
Intravenous cyclophosphamide pulse therapy in interstitial lung disease associated with systemic sclerosis in a retrospective open-label study: influence of the extent of inflammation on pulmonary function.Clin Rheumatol. 2018 Oct;37(10):2715-2722. doi: 10.1007/s10067-018-4171-6. Epub 2018 Jul 10. Clin Rheumatol. 2018. PMID: 29987427 Free PMC article.
-
[Interstitial lung disease in systemic sclerosis].Rev Mal Respir. 2007 Oct;24(8):1035-46. doi: 10.1016/s0761-8425(07)92767-9. Rev Mal Respir. 2007. PMID: 18033190 Review. French.
-
Interstitial Lung Disease in Systemic Sclerosis: Focus on Early Detection and Intervention.Open Access Rheumatol. 2019 Dec 9;11:283-307. doi: 10.2147/OARRR.S226695. eCollection 2019. Open Access Rheumatol. 2019. PMID: 31849543 Free PMC article.
-
Tracking Impact of Interstitial Lung Disease in Systemic Sclerosis in a Complete Nationwide Cohort.Am J Respir Crit Care Med. 2019 Nov 15;200(10):1258-1266. doi: 10.1164/rccm.201903-0486OC. Am J Respir Crit Care Med. 2019. PMID: 31310156
Cited by
-
Radiology of fibrosis. Part I: Thoracic organs.J Transl Med. 2024 Jul 2;22(1):609. doi: 10.1186/s12967-024-05244-1. J Transl Med. 2024. PMID: 38956586 Free PMC article. Review.
-
Alveolar epithelial-to-mesenchymal transition in scleroderma interstitial lung disease: Technical challenges, available evidence and therapeutic perspectives.J Scleroderma Relat Disord. 2024 Feb;9(1):7-15. doi: 10.1177/23971983231181727. Epub 2023 Jun 20. J Scleroderma Relat Disord. 2024. PMID: 38333528 Free PMC article. Review.
-
The Role of Autophagy and Apoptosis in Affected Skin and Lungs in Patients with Systemic Sclerosis.Int J Mol Sci. 2023 Jul 7;24(13):11212. doi: 10.3390/ijms241311212. Int J Mol Sci. 2023. PMID: 37446389 Free PMC article. Review.
-
Impact of Innovative Treatment Using Biological Drugs for the Modulation of Diffuse Cutaneous Systemic Sclerosis: A Systematic Review.Medicina (Kaunas). 2023 Jan 27;59(2):247. doi: 10.3390/medicina59020247. Medicina (Kaunas). 2023. PMID: 36837449 Free PMC article. Review.
-
The Role of Bronchoalveolar Lavage in Systemic Sclerosis Interstitial Lung Disease: A Systematic Literature Review.Pharmaceuticals (Basel). 2022 Dec 19;15(12):1584. doi: 10.3390/ph15121584. Pharmaceuticals (Basel). 2022. PMID: 36559035 Free PMC article. Review.
References
-
- Giacomelli R., Liakouli V., Berardicurti O., Ruscitti P., Di Benedetto P., Carubbi F., Guggino G., Di Bartolomeo S., Ciccia F., Triolo G., Cipriani P. Interstitial lung disease in systemic sclerosis: current and future treatment. Rheumatol. Int. 2017 - PubMed
-
- Rodnan G.P., Benedek T.G. A historical account of the study of progressive systemic sclerosis (diffuse scleroderma) Ann. Intern Med. 1962;57:305–319. - PubMed
-
- Furst D.E., Fernandes A.W., Iorga S.R., Greth W., Bancroft T. Epidemiology of systemic sclerosis in a large US managed care population. J. Rheumatol. 2012;39:784–786. - PubMed
-
- Hoyles R.K., Ellis R.W., Wellsbury J., Lees B., Newlands P., Goh N.S., Roberts C., Desai S., Herrick A.L., McHugh N.J., Foley N.M., Pearson S.B., Emery P., Veale D.J., Denton C.P., Wells A.U., Black C.M., du Bois R.M. A multicenter, prospective, randomized, double-blind, placebo-controlled trial of corticosteroids and intravenous cyclophosphamide followed by oral azathioprine for the treatment of pulmonary fibrosis in scleroderma. Arthritis Rheum. 2006;54:3962–3970. - PubMed
-
- Morales-Cardena A., Perez-Madrid C., Arias L., Ojeda P., Mahecha M.P., Rojas-Villarraga A., Carrillo-Bayona J.A., Anaya J.M. Pulmonary involvement in systemic sclerosis. Autoimmun. Rev. 2016;15:1094–1108. - PubMed
Publication types
LinkOut - more resources
Full Text Sources
Other Literature Sources
