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|July 7, 2025
PubMed
Summary

Systemic sclerosis with interstitial lung disease (SSc-ILD) shows elevated pro-inflammatory and profibrotic markers. Increased triglyceride levels correlate with ILD progression, potentially via endoplasmic reticulum stress.

Keywords:
interstitial lung fibrosislung fibrosismulti‐omicssclerodermasystemic sclerosis

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Area of Science:

  • Autoimmune diseases
  • Connective tissue disorders
  • Pulmonary medicine

Background:

  • Systemic sclerosis (SSc) is a rare autoimmune disorder.
  • Interstitial lung disease (ILD) is the main cause of mortality in SSc patients.
  • Identifying biomarkers for ILD progression in SSc is crucial.

Purpose of the Study:

  • To identify markers associated with ILD progression in SSc patients.
  • To compare biological profiles of SSc patients with and without ILD.
  • To investigate the multi-level biological picture of SSc-related ILD.

Main Methods:

  • Analysis of plasma, whole blood, and skin biopsies from 52 SSc patients and 5 healthy volunteers.
  • Utilized metabolomics, proteomics, cytokine quantification, gene expression profiling, and immunophenotyping.
  • Compared patients with progressive ILD, no ILD, and healthy volunteers.

Main Results:

  • Elevated pro-inflammatory markers (interferon-1, fractalkine, CCL2) and profibrotic markers (CXCL17, thrombospondin, LTBP1) were found in SSc-ILD.
  • Lower cellular inflammatory activity observed in SSc-ILD patients, possibly due to immunosuppressive therapies.
  • ILD progression correlated with increased plasma levels of cytoskeletal proteins and triglycerides.

Conclusions:

  • Pro-inflammatory and profibrotic mechanisms are involved in SSc-related ILD.
  • Elevated triglyceride levels represent a novel potential biomarker for ILD progression in SSc.
  • Triglycerides may contribute to fibrogenesis through endoplasmic reticulum stress.