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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
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[Systemic sclerosis].

Susanne Schinke1, Gabriela Riemekasten2

  • 1Klinik für Rheumatologie und Immunologie, Universitätsklinikum Schleswig-Holstein, Campus Lübeck, Ratzeburger Allee 160, 23562, Lübeck, Deutschland. s.schinke@uksh.de.

Der Internist
|November 23, 2019
PubMed
Summary

Systemic sclerosis (SSc) is a rare fibrosing rheumatic disease. Early, intensive treatment can stabilize SSc, and this review covers its pathophysiology, classification, manifestations, and therapies.

Keywords:
Acral necrosisCREST syndromeDigital ulcersPulmonary arterial hypertensionPulmonary fibrosis

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Area of Science:

  • Rheumatology and immunology
  • Fibrosing rheumatic diseases
  • Multi-systemic autoimmune disorders

Background:

  • Systemic sclerosis (SSc) is a rare, multi-systemic fibrosing rheumatic disease.
  • Key mortality factors include lung fibrosis, pulmonary arterial hypertension, and cardiac involvement.
  • Early and intensive treatment is crucial for disease stabilization and symptom relief.

Purpose of the Study:

  • To review current understanding of Systemic Sclerosis (SSc) pathophysiology.
  • To summarize SSc classification, clinical features, and established therapies.
  • To highlight recent advancements in immunosuppressant, biological, and vasoactive treatments for SSc.

Main Methods:

  • Literature review of Systemic Sclerosis (SSc).
  • Synthesis of data on pathophysiology and clinical manifestations.
  • Analysis of current and emerging therapeutic strategies.

Main Results:

  • SSc pathophysiology involves complex fibrotic and vascular mechanisms.
  • Lung fibrosis, pulmonary hypertension, and cardiac issues significantly impact SSc mortality.
  • Effective management relies on early intervention and multi-specialty care.

Conclusions:

  • Understanding SSc pathophysiology guides treatment strategies.
  • Comprehensive management, including novel therapies, offers improved outcomes for SSc patients.
  • Ongoing research into immunosuppressants, biologics, and vasoactive drugs is vital for advancing SSc care.