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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
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[Updates in systemic sclerosis pathogenesis: Toward new therapeutic opportunities]

K Didier1, A Robbins1, F Antonicelli2

  • 1Service de médecine interne, maladies infectieuses et immunologie clinique, faculté de médecine de Reims, CHU de Reims, 51100 Reims, France; EA 7509 Immuno-régulation et remodelage tissulaire dans le cancer et dans les maladies auto-immunes et inflammatoires, université Reims Champagne Ardenne, 51100 Reims, France.

La Revue De Medecine Interne
|July 15, 2019
PubMed

Insights

Systemic sclerosis involves fibrosis and immune issues, with oxidative stress a key factor. While treatments are limited, autologous stem cell transplants show promise for severe cases, despite risks.

Area of Science:

  • Rheumatology
  • Immunology
  • Fibrosis Research

Background:

  • Systemic sclerosis is a rare connective tissue disease featuring fibrosis, vasculopathy, and immune abnormalities.
  • The precise origin and chronological development of systemic sclerosis pathogenesis remain incompletely understood.
  • Oxidative stress, influenced by genetic and environmental factors, is implicated in early disease phases.

Purpose of the Study:

  • To review recent advancements in understanding systemic sclerosis pathogenesis.
  • To explore the implications of new pathogenic insights for current and future medical care.
  • To highlight novel therapeutic opportunities arising from a deeper knowledge of the disease.

Main Methods:

  • Literature review focusing on recent research in systemic sclerosis.
  • Analysis of studies investigating the role of oxidative stress and immune cells.
  • Evaluation of current and emerging therapeutic strategies.

Main Results:

  • Fibroblast and endothelial cell dysfunction, alongside immune cell involvement, are key features.
  • Oxidative stress appears crucial in early fibrosis, vasculopathy, and immune tolerance.
  • Autologous hematopoietic stem cell transplantation offers lasting fibrosis regression in severe cases but carries significant toxicity.

Conclusions:

  • Recent discoveries have illuminated new mechanisms in systemic sclerosis pathogenesis.
  • These insights are paving the way for improved therapeutic strategies.
  • Further research into pathogenic pathways is essential for developing effective treatments.
Keywords:
PathogenesisPhysiopathologieSclerodermaSclérodermie systémiqueSystemic sclerosisTraitementTreatment

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