Liakouli, V. orcid.org/0000-0002-7392-9334, Ciancio, A., Del Galdo, F. orcid.org/0000-0002-8528-2283 et al. (2 more authors) (2024) Systemic sclerosis interstitial lung disease: unmet needs and potential solutions. Nature Reviews Rheumatology, 20 (1). pp. 21-32. ISSN 1759-4790
Abstract
Systemic sclerosis (SSc), or scleroderma, is a rare, complex, systemic autoimmune disease of unknown aetiology, characterized by high morbidity and mortality often resulting from cardiopulmonary complications such as interstitial lung disease and pulmonary arterial hypertension. Despite substantial progress in unravelling the pathways involved in the pathogenesis of SSc and the increasing number of therapeutic targets tested in clinical trials, there is still no cure for this disease, although several proposed treatments might limit the involvement of specific organs, thereby slowing the natural history of the disease. A specific focus of recent research has been to address the plethora of unmet needs regarding the global management of SSc-related interstitial lung disease, including its pathogenesis, early diagnosis, risk stratification of patients, appropriate treatment regimens and monitoring of treatment response, as well as the definition of progression and predictors of progression and mortality. More refined stratification of patients on the basis of clinical features, molecular signatures, identification of subpopulations with distinct clinical trajectories and implementation of outcome measures for future clinical trials could also improve therapeutic management strategies, helping to avoid poor outcomes related to lung involvement.
Metadata
Item Type: | Article |
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Authors/Creators: |
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Keywords: | Lung; Humans; Lung Diseases, Interstitial; Scleroderma, Systemic |
Dates: |
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Institution: | The University of Leeds |
Academic Units: | The University of Leeds > Faculty of Medicine and Health (Leeds) > School of Medicine (Leeds) > Institute of Rheumatology & Musculoskeletal Medicine (LIRMM) (Leeds) > Inflammatory Arthritis (Leeds) |
Depositing User: | Symplectic Publications |
Date Deposited: | 23 Apr 2024 11:19 |
Last Modified: | 23 Apr 2024 11:19 |
Status: | Published |
Publisher: | Springer |
Identification Number: | 10.1038/s41584-023-01044-x |
Related URLs: | |
Open Archives Initiative ID (OAI ID): | oai:eprints.whiterose.ac.uk:211703 |