Combining Clinical and Biological Data to Predict Progressive Pulmonary Fibrosis in Patients With Systemic Sclerosis Despite Immunomodulatory Therapy
Objective 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 (S...
| Main Authors: | , , , , , , , |
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| Format: | Article |
| Language: | English |
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Wiley
2023-10-01
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| Series: | ACR Open Rheumatology |
| Online Access: | https://doi.org/10.1002/acr2.11598 |
| _version_ | 1797660672623902720 |
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| author | Elizabeth R. Volkmann Holly Wilhalme Shervin Assassi Grace Hyun J. Kim Jonathan Goldin Masataka Kuwana Donald P. Tashkin Michael D. Roth |
| author_facet | Elizabeth R. Volkmann Holly Wilhalme Shervin Assassi Grace Hyun J. Kim Jonathan Goldin Masataka Kuwana Donald P. Tashkin Michael D. Roth |
| author_sort | Elizabeth R. Volkmann |
| collection | DOAJ |
| description | Objective 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). Methods 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. Results 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). Conclusion 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. |
| first_indexed | 2024-03-11T18:34:17Z |
| format | Article |
| id | doaj.art-c6aa75ec170c44a7ad6960cb9f59e514 |
| institution | Directory Open Access Journal |
| issn | 2578-5745 |
| language | English |
| last_indexed | 2024-03-11T18:34:17Z |
| publishDate | 2023-10-01 |
| publisher | Wiley |
| record_format | Article |
| series | ACR Open Rheumatology |
| spelling | doaj.art-c6aa75ec170c44a7ad6960cb9f59e5142023-10-13T07:11:06ZengWileyACR Open Rheumatology2578-57452023-10-0151054755510.1002/acr2.11598Combining Clinical and Biological Data to Predict Progressive Pulmonary Fibrosis in Patients With Systemic Sclerosis Despite Immunomodulatory TherapyElizabeth R. Volkmann0Holly Wilhalme1Shervin Assassi2Grace Hyun J. Kim3Jonathan Goldin4Masataka Kuwana5Donald P. Tashkin6Michael D. Roth7University of California, Los Angeles David Geffen School of MedicineUniversity of California, Los Angeles David Geffen School of MedicineUniversity of Texas Health Science Center at HoustonUniversity of California, Los Angeles David Geffen School of MedicineUniversity of California, Los Angeles David Geffen School of MedicineNippon Medical School Tokyo JapanUniversity of California, Los Angeles David Geffen School of MedicineUniversity of California, Los Angeles David Geffen School of MedicineObjective 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). Methods 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. Results 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). Conclusion 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.https://doi.org/10.1002/acr2.11598 |
| spellingShingle | Elizabeth R. Volkmann Holly Wilhalme Shervin Assassi Grace Hyun J. Kim Jonathan Goldin Masataka Kuwana Donald P. Tashkin Michael D. Roth Combining Clinical and Biological Data to Predict Progressive Pulmonary Fibrosis in Patients With Systemic Sclerosis Despite Immunomodulatory Therapy ACR Open Rheumatology |
| title | Combining Clinical and Biological Data to Predict Progressive Pulmonary Fibrosis in Patients With Systemic Sclerosis Despite Immunomodulatory Therapy |
| title_full | Combining Clinical and Biological Data to Predict Progressive Pulmonary Fibrosis in Patients With Systemic Sclerosis Despite Immunomodulatory Therapy |
| title_fullStr | Combining Clinical and Biological Data to Predict Progressive Pulmonary Fibrosis in Patients With Systemic Sclerosis Despite Immunomodulatory Therapy |
| title_full_unstemmed | Combining Clinical and Biological Data to Predict Progressive Pulmonary Fibrosis in Patients With Systemic Sclerosis Despite Immunomodulatory Therapy |
| title_short | Combining Clinical and Biological Data to Predict Progressive Pulmonary Fibrosis in Patients With Systemic Sclerosis Despite Immunomodulatory Therapy |
| title_sort | combining clinical and biological data to predict progressive pulmonary fibrosis in patients with systemic sclerosis despite immunomodulatory therapy |
| url | https://doi.org/10.1002/acr2.11598 |
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