Z Gastroenterol 2020; 58(05): e102
DOI: 10.1055/s-0040-1712321
Hepatologie

Features of the portal hypertensive syndrome in patients with advanced PBC

L Burghart
1   Medical University of Vienna, Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Vienna, Austria
2   Vienna Hepatic Hemodynamic Lab, Medical University of Vienna, Vienna, Austria
,
B Simbrunner
1   Medical University of Vienna, Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Vienna, Austria
2   Vienna Hepatic Hemodynamic Lab, Medical University of Vienna, Vienna, Austria
3   RALID Center of the ERN Rare Liver, Medizinischer Universitätscampus Wien, Vienna, Austria
4   Ludwig Boltzmann Institute for Rare and Undiagnosed Diseases, Vienna, Austria
5   CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria
,
P Schwabl
1   Medical University of Vienna, Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Vienna, Austria
2   Vienna Hepatic Hemodynamic Lab, Medical University of Vienna, Vienna, Austria
,
E Halilbasic
1   Medical University of Vienna, Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Vienna, Austria
3   RALID Center of the ERN Rare Liver, Medizinischer Universitätscampus Wien, Vienna, Austria
,
AF Stättermayer
1   Medical University of Vienna, Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Vienna, Austria
3   RALID Center of the ERN Rare Liver, Medizinischer Universitätscampus Wien, Vienna, Austria
,
M Mandorfer
1   Medical University of Vienna, Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Vienna, Austria
2   Vienna Hepatic Hemodynamic Lab, Medical University of Vienna, Vienna, Austria
3   RALID Center of the ERN Rare Liver, Medizinischer Universitätscampus Wien, Vienna, Austria
,
M Trauner
1   Medical University of Vienna, Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Vienna, Austria
3   RALID Center of the ERN Rare Liver, Medizinischer Universitätscampus Wien, Vienna, Austria
,
T Reiberger
1   Medical University of Vienna, Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Vienna, Austria
2   Vienna Hepatic Hemodynamic Lab, Medical University of Vienna, Vienna, Austria
3   RALID Center of the ERN Rare Liver, Medizinischer Universitätscampus Wien, Vienna, Austria
4   Ludwig Boltzmann Institute for Rare and Undiagnosed Diseases, Vienna, Austria
5   CeMM Research Center for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria
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Background and Aims Primary biliary cholangitis (PBC) is a rare liver disease that may progress to cirrhosis and portal hypertension. Therefore, we aimed to characterize the prevalence and features of the portal hypertensive syndrome (PH) in patients with PBC.

Methods PBC patients presenting at the Medical University of Vienna were screened for features of clinically significant portal hypertension (CSPH): (i) gastroesophageal varices, (ii) splenomegaly  > 11cm, (iii) ascites (excluding non-hepatic causes), (iv) portosystemic collaterals, (v) hepatic venous pressure gradient (HVPG) ≥ 10 mmHg, or (vi) medical record documented a serious complication attributed to portal hypertension.

Results N = 249 PBC patients were included, of whom 22.1 % had PBC-AIH Overlap. 97 patients (39 %) showed features of CSPH: varices in 18.5 % (N = 46), splenomegaly in 30.5 % (N = 76), ascites in 18.9 % (N = 47), HVPG ≥ 10mmHg in 4.4 % (N = 11) and 11.6 % had a liver stiffness measurement  > 15kPa. The median duration between PBC diagnosis and development of CSPH was 1.51 years (IQR -0.1 to -8.93), and in 39 patients only the occurrence of CSPH-related complications led to the diagnosis of PBC. No statistically significant differences were found between patients with and without CSPH, regarding age, AMA-M2(+) positivity, and PBC-specific ANAs. However, thrombocytopenia ( < 150G/L), was more frequent (29.2 % vs. 2.7 %, p < 0.0001) and median LSM was higher (13.4 vs. 5.9 kPa, p < 0.001) in patients with CSPH. N = 17 (6.8 %) of the patients developed variceal bleeding and N = 30 (12.0 %) required endoscopic variceal ligation (EVL). During a median follow-up of 6.1 years, n = 40 (16.1 %) patients died - including n = 20 (8 %) liver-related deaths of which n = 16 were attributed to complications of CSPH.

Conclusion Features of CSPH in patients with PBC are frequent and may occur early after diagnosis. Splenomegaly is the most prevalent and earliest feature of CSPH in PBC. CSPH-related complications accounted for 40 % of the deaths in our PBC cohort.



Publication History

Article published online:
26 May 2020

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