Z Gastroenterol 2016; 54 - KV208
DOI: 10.1055/s-0036-1586984

Serum IgG4 and long term outcome in patients with primary biliary cholangitis treated with ursodeoxycholic acid

ME Pascu 1, M Riedel 2, E Schott 3, B Wiedenmann 3, T Berg 2, T Müller 3
  • 1Martin-Luther Krankenhaus, Innere Medizin, Berlin, Deutschland
  • 2Universitätsklinikum Leipzig, Klinik für Gastroenterologie und Rheumatologie – Sektion Hepatologie, Leipzig, Deutschland
  • 3Charité – Universitätsmedizin Berlin, Campus Charité Mitte und Virchow Klinikum, Medizinische Klinik mit Schwerpunkt Hepatologie und Gastroenterologie, Berlin, Deutschland

Background and Aims:

The aim of this study was to assess the potential influence of serum immunglobulin G4 (IgG4) on the long term outcome of patients with primary biliary cholangitis (PBC) receiving ursodeoxycholic acid (UDCA).

Methods:

IgG4 levels were measured in sera of 229 patients with PBC prior to the initiation of treatment with UDCA. Endpoints were liver related death or orthotopic liver transplantation (OLT). Kaplan-Meier estimates were compared with expected survival of matched German controls. Cox proportional hazard regression analyses were performed to identify prognostic factors for the endpoints.

Results:

The liver biochemistry (serum bilirubin, AP, GGT, AST, ALT) was significantly increased in PBC patients with elevated serum IgG4 levels (N = 10, 4.4%) in comparison to those with normal (N = 219, 95.6%) serum IgG4 levels. At the end of the follow-up no liver related death had occurred, but significantly more OLT was encountered (p 0.011 Fisher's exact test) after a significantly longer UDCA treatment time (p 0.003 Mann-Whithney-U-test) in patients with elevated IgG4 levels in comparison to those with normal IgG4 levels. Serum bilirubin at baseline was an independent prognostic factor regularly associated with OLT-free survival regardless IgG4 levels.

Conclusions:

PBC patients with elevated serum IgG4 levels exhibit enhanced cholestasis prior to the initiation of UDCA treatment. In addition, pre-treatment serum IgG4 level seems to be associated with a higher risk for OLT. However, because of the small number of PBC patients with elevated IgG4 serum levels, further studies are needed to confirm a causal association between elevated serum IgG4 levels and risk for liver related death or OLT in patients with PBC on UDCA.