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Updated: Feb 2, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Mycophenolate therapy in interstitial pneumonia with autoimmune features: a cohort study
Sara S McCoy1, Zubin Mukadam2, Keith C Meyer2
1Division of Rheumatology, Department of Internal Medicine, School of Medicine and Public Health, University of Wisconsin, Madison, WI 53705, USA, ssmccoy@medicine.wisc.edu.
Interstitial pneumonia with autoimmune features (IPAF) patients may benefit from mycophenolate therapy, though further research is needed. This study observed a trend toward improved lung function in treated individuals.
Area of Science:
- Pulmonology
- Rheumatology
- Immunology
Background:
- Interstitial pneumonia with autoimmune features (IPAF) is a provisional diagnosis for interstitial lung disease (ILD) patients with autoimmune characteristics who don't meet criteria for specific autoimmune diseases.
- Understanding IPAF's clinical features and treatment responses is crucial for managing this complex condition.
Purpose of the Study:
- To describe the clinical characteristics of patients diagnosed with IPAF.
- To evaluate the therapeutic response to mycophenolate in IPAF patients.
Main Methods:
- Retrospective cohort study of adult patients meeting European Respiratory Society/American Thoracic Society IPAF criteria.
- Longitudinal analysis of sociodemographic, clinical, and pulmonary function test data, comparing mycophenolate-treated and untreated groups.
Main Results:
- 52 IPAF patients were identified; 28 received mycophenolate, 24 did not.
- No significant difference in FVC% or DLCO% change between groups (P=0.08, P=0.17).
- A trend towards improved FVC% and DLCO% slopes was observed after mycophenolate initiation in the treated group.
Conclusions:
- Mycophenolate therapy may offer potential benefits for patients with IPAF.
- Larger prospective clinical trials are necessary to definitively establish mycophenolate's efficacy in IPAF.
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