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Updated: Jun 10, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Connective tissue disease-associated interstitial lung disease: a call for clarification
Aryeh Fischer1, Sterling G West, Jeffrey J Swigris
1Division of Rheumatology, National Jewish Health and University of Colorado, Denver, CO 80206, USA. fischera@njhealth.org
Patients with interstitial pneumonia and suspected connective tissue disease (CTD) face classification challenges. A new "lung-dominant CTD" phenotype is proposed for better understanding and treatment.
Area of Science:
- Pulmonology
- Rheumatology
- Medical Classification
Background:
- Interstitial pneumonia (IP) patients often present with features suggestive of connective tissue disease (CTD).
- Current rheumatologic classification systems struggle to diagnose CTD-associated interstitial lung disease (CTD-ILD) in these cases.
- This diagnostic ambiguity hinders appropriate patient management and research.
Purpose of the Study:
- To highlight the limitations in classifying patients with IP and overlapping CTD features.
- To discuss the challenges of redefining undifferentiated CTD to include IP.
- To propose a distinct phenotype for improved classification and research.
Main Methods:
- Commentary and critical analysis of current classification systems.
- Discussion of diagnostic dilemmas in patients with IP and suspected CTD.
- Proposal for a new classification approach.
Main Results:
- Current classification systems are inadequate for patients with IP and suggestive but insufficient CTD features.
- Redefining undifferentiated CTD to include IP presents significant challenges.
- A multidisciplinary approach to evaluation and classification is advocated.
Conclusions:
- A new phenotype, 'lung-dominant CTD', is proposed for patients with IP and overlapping CTD features.
- This distinct classification may allow for targeted prognostic, therapeutic, and pathobiologic studies.
- Future multi-institutional research is needed to validate this proposed phenotype.
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