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Cystic fibrosis (CF) is an autosomal recessive disorder that predominantly affects individuals of Northern European descent, occurring at a rate of 1 in 3500. It is caused by a genetic mutation in a gene on chromosome 7, most commonly the ΔF508 mutation, that codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. This results in thicker mucus secretions and obstruction pathologies in multiple organs, including the lungs and sinuses.
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Related Experiment Video

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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
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Systemic Sclerosis-Associated Interstitial Lung Disease: Improved Understanding and Advances in Management.

Amit Syal1, Chris T Derk2

  • 1Department of Medicine, Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, PA, USA.

British Journal of Hospital Medicine (London, England : 2005)
|December 24, 2025
PubMed
Summary

Systemic sclerosis-associated interstitial lung disease (SSc-ILD) management is advancing with AI-enhanced imaging, new biomarkers like KL-6, and updated guidelines. Treatment now includes antifibrotics, immunomodulators, and combination therapies for better patient outcomes.

Keywords:
interstitial lung diseasemanagementmonitoringscreeningsystemic sclerosis

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Area of Science:

  • Rheumatology
  • Pulmonology
  • Medical Imaging
  • Biomarkers

Background:

  • Interstitial lung disease (ILD) is a severe complication of systemic sclerosis (SSc), leading to significant morbidity and mortality, especially in diffuse cutaneous SSc (dcSSc).
  • Early detection and monitoring of SSc-ILD are crucial for effective management.
  • Recent therapeutic advancements have expanded treatment options for SSc-ILD.

Purpose of the Study:

  • To synthesize current screening, monitoring, and therapeutic strategies for SSc-ILD.
  • To highlight recent advancements in AI-based diagnostics, serological biomarkers, and updated international treatment guidelines.
  • To discuss emerging combination and triple therapy approaches and propose future research directions.

Main Methods:

  • Review of recent literature on SSc-ILD diagnostics and therapeutics.
  • Analysis of advancements in artificial intelligence (AI)-enhanced high-resolution computed tomography (HRCT).
  • Evaluation of serological biomarkers, including Krebs von den Lungen (KL)-6.
  • Synthesis of updated international guidelines (ACR, CHEST, ATS, EULAR).

Main Results:

  • AI-enhanced HRCT and biomarkers like KL-6 facilitate earlier SSc-ILD detection and monitoring.
  • Antifibrotics (e.g., nintedanib) and immunomodulators (e.g., tocilizumab) are now established therapeutic options.
  • Updated guidelines reflect the evolving treatment landscape, including combination therapies.

Conclusions:

  • The management of SSc-ILD is shifting towards proactive screening, precise monitoring using novel diagnostics, and personalized therapeutic strategies.
  • Combination and potentially triple therapy approaches represent a significant advancement in treating SSc-ILD.
  • Future research should focus on biomarker validation, mechanistic insights, and optimizing personalized treatment plans for SSc-ILD.