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Related Experiment Video

Updated: Jul 6, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
04:44

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Published on: June 16, 2020

Interstitial lung disease in systemic sclerosis: a simple staging system.

Nicole S L Goh1, Sujal R Desai, Srihari Veeraraghavan

  • 1Royal Brompton Hospital and National Heart and Lung Institute, London, United Kingdom.

American Journal of Respiratory and Critical Care Medicine
|March 29, 2008
PubMed
Summary

A new staging system for systemic sclerosis-associated interstitial lung disease (SSc-ILD) combines high-resolution computed tomography (HRCT) and pulmonary function tests (PFTs). This approach offers improved mortality prediction compared to using HRCT or PFTs alone.

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Area of Science:

  • Pulmonary Medicine
  • Radiology
  • Rheumatology

Background:

  • Systemic sclerosis-associated interstitial lung disease (SSc-ILD) prognosis is uncertain using standard tests.
  • Optimal integration of pulmonary function tests (PFTs) and high-resolution computed tomography (HRCT) for SSc-ILD prognostication requires clarification.

Purpose of the Study:

  • To develop a practical prognostic algorithm for SSc-ILD by integrating PFTs and HRCT data.
  • To enhance the predictive accuracy of mortality risk in SSc-ILD patients.

Main Methods:

  • Quantified the prognostic value of baseline PFT and HRCT variables in 215 SSc-ILD patients.
  • Assessed outcomes against survival and serial PFT data.
  • Developed and validated a limited/extensive staging system based on HRCT extent and FVC thresholds.

Main Results:

  • HRCT extent >20% strongly predicted mortality (P < 0.0005).
  • In cases with indeterminate HRCT extent (10-30%), an FVC of <70% indicated higher mortality risk.
  • The novel staging system (limited/extensive) showed superior discrimination (HR, 3.46) compared to HRCT extent alone (HR, 2.48) or FVC alone (HR, 2.11).

Conclusions:

  • An accessible limited/extensive staging system integrating HRCT and PFTs provides significant prognostic information for SSc-ILD.
  • This combined approach improves risk stratification for mortality in SSc-ILD patients.
  • The staging system demonstrated predictive accuracy across different levels of clinical experience.