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Updated: Sep 28, 2025

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Systemic sclerosis and primary biliary cholangitis: An overlapping entity?

Gemma Lepri1, Silvia Bellando Randone1, Marco Matucci Cerinic1

  • 1Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Florence, Italy.

Journal of Scleroderma and Related Disorders
|April 6, 2022
PubMed
Summary
This summary is machine-generated.

Systemic sclerosis (SSc) and primary biliary cholangitis (PBC) share autoimmune and fibrotic pathways. Recent findings highlight shared antibodies and cytokines, suggesting these conditions may represent an overlapping autoimmune entity.

Keywords:
Systemic sclerosisautoimmune diseasesfibrotic disordersoverlap entityoverlap syndromeprimary biliary cholangitis

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Area of Science:

  • Rheumatology
  • Hepatology
  • Immunology

Background:

  • Systemic sclerosis (SSc) is an autoimmune disease causing fibrosis in skin and organs.
  • SSc has limited cutaneous and diffuse cutaneous subsets.
  • SSc frequently co-occurs with other autoimmune diseases, notably primary biliary cholangitis (PBC).

Purpose of the Study:

  • To review recent findings on the association between SSc and PBC.
  • To explore shared etiological factors and immunological mechanisms.
  • To clarify the prevalence and diagnostic implications of this association.

Main Methods:

  • Literature review of recent studies on SSc and PBC.
  • Analysis of shared immunological markers, including T lymphocyte subpopulations (Th17 and regulatory T cells).
  • Examination of antibody presence and prevalence data in co-occurring SSc and PBC patients.

Main Results:

  • SSc and PBC share fibrogenic cytokines and immune cell involvement.
  • Presence of disease-specific antibodies in patients with the other condition suggests a close relationship.
  • Overt PBC affects approximately 2% of SSc patients, particularly those with limited cutaneous SSc and anticentromere antibodies.

Conclusions:

  • The association between SSc and PBC is supported by shared immunological and fibrotic mechanisms.
  • The presence of specific antibodies strengthens the evidence for a link between these conditions.
  • SSc and PBC may represent facets of a single, overlapping autoimmune and fibrotic disorder.