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Updated: Jul 12, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Mycophenolate and Azathioprine in Fibrotic Interstitial Lung Disease
Janelle Vu Pugashetti1, Adelle S Jee2,3, Andrew R Yu4
1Division of Pulmonary and Critical Care Medicine, University of Michigan, Ann Arbor, MI, United States.
Immunosuppression did not improve survival or lung function in fibrotic interstitial lung diseases (ILDs). It was linked to worse outcomes in non-IPF IIP and fibrotic hypersensitivity pneumonitis, highlighting the need for clinical trials.
Area of Science:
- Pulmonology
- Immunology
- Clinical Trials
Background:
- Immunosuppression is common for non-idiopathic pulmonary fibrosis (IPF) interstitial lung diseases (ILDs).
- Evidence supporting its efficacy and long-term impact is limited.
- This study addresses the use of immunosuppressants in fibrotic ILDs.
Purpose of the Study:
- To assess if mycophenolate or azathioprine improves transplant-free survival at three years.
- To evaluate the impact on lung function trajectory in fibrotic ILDs.
- Included non-IPF idiopathic interstitial pneumonia (IIP), fibrotic hypersensitivity pneumonitis (fHP), and connective-tissue disease (CTD)-ILD.
Main Methods:
- Emulated a randomized controlled trial using a clone-censor-weighting framework.
- Patients were assigned to immunosuppression initiation or no initiation, with censoring for strategy deviation.
- Stabilized inverse probability treatment and censoring weights were applied in this multi-center retrospective study.
Main Results:
- Immunosuppression initiation was not linked to improved survival in any ILD subtype.
- Worse survival was observed in non-IPF IIP (HR 1.38) and fHP (HR 1.62).
- No significant impact on lung function trajectory was found across ILD subtypes.
Conclusions:
- Immunosuppression initiation demonstrated no benefit for three-year transplant-free survival or lung function.
- Associated with increased mortality in specific fibrotic ILD subtypes.
- Urgent need for prospective randomized controlled trials to guide clinical practice.
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