The Cytokine Profile of Primary Biliary Cholangitis (PBC): Is PBC an Autoinflammatory vs. Autoimmune Disease?


Üçdal M. T.

41.Ulusal Gastroenteroloji Haftası, Antalya, Turkey, 25 November 2024 - 01 January 2025, vol.1, no.1, pp.95, (Full Text)

  • Publication Type: Conference Paper / Full Text
  • Volume: 1
  • City: Antalya
  • Country: Turkey
  • Page Numbers: pp.95
  • Hacettepe University Affiliated: Yes

Abstract

Objective Primary biliary cholangitis (PBC) is a disease within the spectrum of autoimmune liver disease. However, there is a current debate about whether the underlying mechanism of PBC is an autoinflammatory or autoimmune. This study aimed to elucidate type of immune dysregulation in PBC by evaluating the cytokine and toll-like receptor (TLR) profiles in comparisen with celiac disease (CD) and autoimmune hepatitis (AIH). Methods The serum immunological profiles of patients with PBC (n=45), CD (n=42), and AIH (n=44) were analyzed by using ELISA and flow cytometry for the levels of IL-1, IL-6, TLR-4, and TLR-6 (markers of autoinflammatory in innate immune responses); IL-2, IL-4, IL-12, and IL-13 (markers of autoimmunity associated with adaptive immune responses), and IL-10, IL-17, IL-23, IFN-γ, and TGF-β (mediators of both autoinflammatory and autoimmune processes). Results Classical autoinflammatory cytokines showed no significant elevations in PBC (IL-1β p=0.188, TNF-α p=0.104, IL-6 p=0.790), suggesting a complex immunological profile. Celiac patients exhibited decreased IL-23 levels (p=0.05), indicating Th17-mediated responses. AIH patients demonstrated higher IL-12p70 (p=0.005) and IL-23 (p=0.001) levels, implying Th1/Th17 predominance, yet classical autoimmune cytokines were comparable to other groups. Table 1 summarizes serum cytokine levels, while Figure 1 illustrates immunological profiles across diseases. Discussion In light of these data, we conclude that the immunopathology of these diseases is more nuanced than suggested by the proposed categorical classifications: 1.PBC presents a generally lower cytokine profile, which does not align with the proposed autoinflammatory pattern. 2. AIH shows a Th1/Th17 predominant response with some autoimmune features, but cannot be classified as purely autoimmune. 3. CD exhibits a mixed pro