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Related Experiment Video

Updated: Sep 30, 2025

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

Published on: June 16, 2020

Comorbidities in unclassifiable interstitial lung disease.

Thomas Skovhus Prior¹, Charlotte Hyldgaard², Sebastiano Emanuele Torrisi³

¹Centre for Rare Lung Diseases, Department of Respiratory Diseases and Allergy, Aarhus University Hospital, Aarhus, Denmark. thbjer@rm.dk.

Respiratory Research

|March 17, 2022

Summary

Comorbidities are common in unclassifiable interstitial lung disease (uILD), but their impact on survival requires further study. Three patient clusters with distinct comorbidity profiles were identified, with one showing slower disease progression.

Keywords:

Cluster analyses Comorbidities Disease course Mortality Unclassifiable interstitial lung disease

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Area of Science:

Pulmonology
Internal Medicine
Clinical Research

Background:

Comorbidities are prevalent in interstitial lung diseases (ILD) and affect survival.
The frequency and prognostic impact of comorbidities in unclassifiable interstitial lung disease (uILD) are not well understood.

Purpose of the Study:

To determine the prevalence of comorbidities in uILD patients.
To assess the impact of comorbidities on survival and disease progression in uILD.
To identify potential uILD phenotypes based on comorbidity clusters.

Main Methods:

Retrospective analysis of incident uILD patients from Danish and German ILD centers (2003-2018).
Diagnosis of uILD confirmed by multidisciplinary team meetings.
Comorbidity data extracted from ILD registries and patient files; survival analyzed using Cox regression; disease progression by linear mixed effects models; comorbidity clusters identified using self-organizing maps.

Main Results:

249 uILD patients identified; 85% had at least one comorbidity.
Dyslipidemia was the only comorbidity linked to increased mortality; no association found between overall comorbidity burden and survival.
Three distinct comorbidity clusters were identified; clusters 1 and 2 showed significant decline in FVC and DLCO, while cluster 3 exhibited slower progression without increased mortality.

Conclusions:

The comorbidity burden in uILD appears lower than in other ILDs, and its impact on mortality needs further investigation.
Three identified clusters may represent distinct uILD phenotypes, with cluster 3 showing a potentially better prognosis.
Further research with longer observation periods is needed to clarify disease behavior and mortality in uILD subgroups.