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Updated: Jun 17, 2026
04:44
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Undifferentiated connective tissue disease-associated interstitial lung disease: changes in lung function.
Brent W Kinder1, Cyrus Shariat, Harold R Collard
1Division of Pulmonary, Critical Care and Sleep Medicine, Department of Medicine, University of Cincinnati College of Medicine, 231 Albert Sabin Way, Cincinnati, OH 45267-0564, USA. brent.kinder@uc.edu
Lung
|January 14, 2010
Summary
Patients with undifferentiated connective tissue disease and interstitial lung disease (UCTD-ILD) show significant lung function improvement compared to idiopathic pulmonary fibrosis (IPF). Identifying UCTD is crucial for managing interstitial pneumonia.
Area of Science:
- Pulmonology
- Rheumatology
- Internal Medicine
Background:
- Undifferentiated connective tissue disease (UCTD) can present with interstitial lung disease (ILD).
- The natural history and prognosis of UCTD-ILD are not well understood.
- Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic lung disease with a generally poor prognosis.
Purpose of the Study:
- To compare the longitudinal changes in lung function in patients with UCTD-ILD versus IPF.
- To determine if UCTD-ILD patients are more likely to experience improvement in forced vital capacity (FVC) compared to IPF patients.
- To highlight the clinical significance of differentiating UCTD-ILD from IPF.
Main Methods:
- Retrospective analysis of patients within the UCSF ILD cohort study.
- Inclusion criteria: diagnosis of IPF or UCTD with available longitudinal pulmonary function data.
- Primary outcome: > or = 5% increase in percent predicted FVC during follow-up. Regression models used to adjust for confounders.
Main Results:
- Fifty-nine subjects (30 IPF, 29 UCTD) were analyzed.
- UCTD patients demonstrated a significant improvement in FVC (OR=8.23, p=0.03) over a median follow-up of 8 months, after adjusting for baseline FVC, treatment, and study duration.
- IPF patients did not show similar significant improvements in FVC.
Conclusions:
- Patients with UCTD-ILD exhibit a higher likelihood of pulmonary function improvement during follow-up compared to those with IPF.
- Early identification of UCTD is critical for patients presenting with interstitial pneumonia that might otherwise be misdiagnosed as IPF.
- This finding underscores the importance of distinguishing UCTD in the management of interstitial lung diseases.
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