Combining Clinical and Biological Data to Predict Progressive Pulmonary Fibrosis in Patients With Systemic Sclerosis Despite Immunomodulatory Therapy.
Journal
ACR open rheumatology
ISSN: 2578-5745
Titre abrégé: ACR Open Rheumatol
Pays: United States
ID NLM: 101740025
Informations de publication
Date de publication:
Oct 2023
Oct 2023
Historique:
revised:
04
07
2023
received:
10
04
2023
accepted:
13
07
2023
pubmed:
18
8
2023
medline:
18
8
2023
entrez:
18
8
2023
Statut:
ppublish
Résumé
Progressive pulmonary fibrosis (PPF) is the leading cause of death in systemic sclerosis (SSc). This study aimed to develop a clinical prediction nomogram using clinical and biological data to assess risk of PPF among patients receiving treatment of SSc-related interstitial lung disease (SSc-ILD). Patients with SSc-ILD who participated in the Scleroderma Lung Study II (SLS II) were randomized to treatment with either mycophenolate mofetil (MMF) or cyclophosphamide (CYC). Clinical and biological parameters were analyzed using univariable and multivariable logistic regression, and a nomogram was created to assess the risk of PPF and validated by bootstrap resampling. Among 112 participants with follow-up data, 22 (19.6%) met criteria for PPF between 12 and 24 months. An equal proportion of patients randomized to CYC (n = 11 of 56) and mycophenolate mofetil (n = 11 of 56) developed PPF. The baseline severity of ILD was similar for patients who did, compared to those who did not, experience PPF in terms of their baseline forced vital capacity percent predicted, diffusing capacity for carbon monoxide percent predicted, and quantitative radiological extent of ILD. Predictors in the nomogram included sex, baseline CXCL4 level, and baseline gastrointestinal reflux score. The nomogram demonstrated moderate discrimination in estimating the risk of PPF, with a C-index of 0.72 (95% confidence interval 0.60-0.84). The SLS II data set provided a unique opportunity to investigate predictors of PPF and develop a nomogram to help clinicians identify patients with SSc-ILD who require closer monitoring while on therapy and potentially an alternative treatment approach. This nomogram warrants external validation in other SSc-ILD cohorts to confirm its predictive power.
Identifiants
pubmed: 37592449
doi: 10.1002/acr2.11598
pmc: PMC10570669
doi:
Types de publication
Journal Article
Langues
eng
Pagination
547-555Subventions
Organisme : NHLBI NIH HHS
ID : K23 HL150237
Pays : United States
Organisme : NHLBI NIH HHS
ID : U01 HL060606
Pays : United States
Organisme : NHLBI NIH HHS
ID : U01 HL 60606
Pays : United States
Organisme : NHLBI NIH HHS
ID : U01 HL 60587
Pays : United States
Informations de copyright
© 2023 The Authors. ACR Open Rheumatology published by Wiley Periodicals LLC on behalf of American College of Rheumatology.
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