Austrian Society of Gastroenterology and Hepatology (ÖGGH) consensus on primary biliary cholangitis

ÖGGH · 2026 · Austria

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Authors: Emina Halilbasic, Elisabeth Tatscher, Elmar Aigner et al.
Title: Austrian Society of Gastroenterology and Hepatology (ÖGGH) consensus on primary biliary cholangitis
Journal: Wiener Klinische Wochenschrift, 2026
DOI: 10.1007/s00508-026-02798-6
Licence: CC BY 4.0
Changes: Verbatim excerpts; formatting adapted

Full text retrieved 2026-10-27 from Europe PMC · PMC13601090

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Epidemiology, natural history and diagnosis of PBC

Primary biliary cholangitis (PBC) is a rare autoimmune cholestatic liver disease predominantly affecting women between 40 and 70 years of age. The female-to-male ratio has decreased in recent decades.
The disease can progress without clinical symptoms for years; however, fatigue and pruritus are highly prevalent and represent the most burdensome symptoms, substantially impairing health-related quality of life (HrQoL) independent of disease stage.
The diagnosis of PBC is established when at least two of the following are present: (A1) Persistently elevated parameters of cholestasis, in particular alkaline phosphatase (ALP), in the absence of biliary obstruction on imaging (e.g., abdominal ultrasound or magnetic resonance cholangiopancreatography, MRCP). PBC-specific autoantibodies, e.g., anti-mitochondrial antibodies (AMA) or PBC-specific anti-nuclear antibodies (ANA), i.e. gp210 and sp100. Characteristic histology showing chronic granulomatous, nonsuppurative and destructive cholangitis of interlobular and septal bile ducts, with lymphocytic infiltrates and bile duct loss in advanced stages.
Persistently elevated parameters of cholestasis, in particular alkaline phosphatase (ALP), in the absence of biliary obstruction on imaging (e.g., abdominal ultrasound or magnetic resonance cholangiopancreatography, MRCP).
PBC-specific autoantibodies, e.g., anti-mitochondrial antibodies (AMA) or PBC-specific anti-nuclear antibodies (ANA), i.e. gp210 and sp100.
Characteristic histology showing chronic granulomatous, nonsuppurative and destructive cholangitis of interlobular and septal bile ducts, with lymphocytic infiltrates and bile duct loss in advanced stages.
Particularly in patients with positive PBC-specific antibodies, polyclonal IgM elevation, isolated gamma-glutamyl transferase (GGT) elevation, and other indicative findings, such as hypercholesterolemia or symptoms including fatigue, pruritus, upper abdominal discomfort, sicca syndrome, or arthralgia may raise clinical suspicion of PBC, although not part of the standard diagnostic criteria.
Liver biopsy is not routinely required for diagnosis, if PBC-specific autoantibodies and typical cholestatic biochemistry are present.B1
Liver biopsy is recommended when: PBC-specific autoantibodies are absent despite biochemical cholestasis with exclusion of biliary obstruction. (C1) There is suspicion of PBC-autoimmune hepatitis (AIH) variant syndrome or a co-existing liver disease (e.g., metabolic dysfunction-associated steatotic liver disease (MASLD)) which would affect patient management.C1
PBC-specific autoantibodies are absent despite biochemical cholestasis with exclusion of biliary obstruction.C1
There is suspicion of PBC-autoimmune hepatitis (AIH) variant syndrome or a co-existing liver disease (e.g., metabolic dysfunction-associated steatotic liver disease (MASLD)) which would affect patient management.C1
In individuals with PBC-specific autoantibodies and normal ALP, liver biopsy should not be routinely performed solely to establish a diagnosis of PBC; however, when GGT is elevated and alternative causes (e.g. MASLD) have been excluded, liver biopsy for establishing a diagnosis of PBC can be considered in selected cases.D2
In individuals with PBC-specific autoantibodies and normal liver biochemistry, periodic biochemical reassessment for the development of liver disease can be considered every 1–2 years as a small proportion of these patients will develop PBC.B2
Genetic testing for inherited cholestatic disorders can be considered to support the diagnostic process in patients lacking PBC-specific autoantibodies and if liver histology is not diagnostic of PBC.C2
Disproportionate elevations of ALT to ALP should prompt consideration of an PBC-AIH variant syndrome or the suspicion of another concomitant liver disorder.B1
The Paris criteria requiring at least two of the following: (1) ALT > 5× ULN, (2) IgG > 2× ULN and/or positive anti-SMA, and (3) moderate-to-severe interface hepatitis on liver biopsy remain the so far best-validated and most commonly used tools for diagnosing PBC-AIH variant syndrome.C1
At diagnosis and during follow-up of PBC, associated symptoms, particularly pruritus, fatigue, sicca syndrome, arthralgia and other common extrahepatic autoimmune conditions, should be systematically assessed.C1

Risk stratification, monitoring and follow-up

Accurate disease staging at baseline and during follow-up is recommended to identify patients at risk of progression and to guide individualized therapeutic strategies.A1
Risk stratification in PBC should be based on a combination of clinical (age), biochemical (bilirubin, ALP, AST, albumin, platelet count), immunological (anti-gp-210), and (where available) histological parameters (advanced fibrosis, ductopenia, and interface hepatitis), as well as noninvasive tests (elastography and/or ELF test) and assessment of portal hypertension. (B1) Younger age at diagnosis (< 45–50 years) should be considered a risk factor for poorer outcomes and reduced likelihood of biochemical response. Male sex and the presence of anti-gp210 antibodies have also been associated with unfavorable prognosis, although the supporting evidence is less consistent. Anti-centromere antibodies (ACA) may be associated with disease progression and portal hypertension.
Younger age at diagnosis (< 45–50 years) should be considered a risk factor for poorer outcomes and reduced likelihood of biochemical response.
Male sex and the presence of anti-gp210 antibodies have also been associated with unfavorable prognosis, although the supporting evidence is less consistent.
Anti-centromere antibodies (ACA) may be associated with disease progression and portal hypertension.
In PBC without cirrhosis or advanced fibrosis serum ALP and bilirubin levels are key prognostic markers: ALP > 2 × ULN and/or bilirubin > 1 × ULN after 1 year of therapy are strongly associated with adverse outcomes. Achieving normalization of ALP and low-normal bilirubin (≤ 0.6× ULN) is associated with improved survival.
ALP > 2 × ULN and/or bilirubin > 1 × ULN after 1 year of therapy are strongly associated with adverse outcomes.
Achieving normalization of ALP and low-normal bilirubin (≤ 0.6× ULN) is associated with improved survival.
Clinical and laboratory follow-up should be performed at 3, 6, and 12 months during the first year after diagnosis. Thereafter, follow-up intervals should be individualized according to disease activity, therapeutic response, and, where appropriate, additional risk factors.B1
Non-invasive fibrosis assessment is recommended at diagnosis and during follow-up for prognostic evaluation. (B1) Liver stiffness measurement (LSM) using vibration-controlled transient elastography (VCTE) is recommended as a key noninvasive tool for baseline risk assessment and monitoring. (B1) LSM should be used for risk stratification, with thresholds (< 8 kPa, 8–15 kPa, > 15 kPa) identifying low, intermediate, and high-risk groups. (C1) A current LSM > 10 kPa defines compensated advanced chronic liver disease (cACLD) and identifies those at risk for hepatic decompensation, irrespective of biochemical response. Longitudinal changes in LSM are prognostically relevant, with decreasing values associated with improved outcomes but the most recent value should guide clinical decision making. The enhanced liver fibrosis (ELF) score can be used for risk stratification, with higher values indicating increased risk and values < 9.8 suggesting low risk of liver-related events.C2
Liver stiffness measurement (LSM) using vibration-controlled transient elastography (VCTE) is recommended as a key noninvasive tool for baseline risk assessment and monitoring.B1
LSM should be used for risk stratification, with thresholds (< 8 kPa, 8–15 kPa, > 15 kPa) identifying low, intermediate, and high-risk groups.C1
A current LSM > 10 kPa defines compensated advanced chronic liver disease (cACLD) and identifies those at risk for hepatic decompensation, irrespective of biochemical response.
Longitudinal changes in LSM are prognostically relevant, with decreasing values associated with improved outcomes but the most recent value should guide clinical decision making.
The enhanced liver fibrosis (ELF) score can be used for risk stratification, with higher values indicating increased risk and values < 9.8 suggesting low risk of liver-related events.C2
Periodic reassessment of LSM using VCTE is recommended. (B1) Annual assessment should be considered in patients with LSM > 10 kPa, persistent symptoms (pruritus) and/or inadequate biochemical response. (C1) Longer intervals (2–3 years) might be appropriate in asymptomatic or minimally symptomatic low-risk patients with stable disease and optimal or adequate biochemical response.C2
Annual assessment should be considered in patients with LSM > 10 kPa, persistent symptoms (pruritus) and/or inadequate biochemical response.C1
Longer intervals (2–3 years) might be appropriate in asymptomatic or minimally symptomatic low-risk patients with stable disease and optimal or adequate biochemical response.C2
Non-invasive criteria combining LSM and platelet count (i.e. applying the BAVENO VII CSPH rule-out criteria LSM < 15 kPa combined with a platelet count ≥ 150 G/L) should be used to identify patients at low risk of varices/decompensation, although caution is warranted due to limited validation in PBC.C1
Clinically significant portal hypertension (CSPH) should be considered even in non-cirrhotic PBC patients and is associated with increased risk of decompensation and mortality.B1
Regarding management of portal hypertension and its complications, the Billroth IV recommendations and subsequent updates can be applied; although PBC-specific evidence is limited.B2
Cirrhosis, defined by an LSM > 15 kPa or histology, should be considered the principal risk factor for HCC in PBC.A1
Risk factors for HCC include male sex, older age, inadequate biochemical response, and advanced disease stage.B2
HCC surveillance should be performed according to current guidelines, with liver ultrasound every 6 months and optional serum alpha-fetoprotein (AFP) measurement, in patients with PBC and cirrhosis.B1
Ultrasound surveillance can also be considered in patients with suspected advanced fibrosis on elastography (≥ 10 kPa) and/or an inadequate biochemical response to treatment.C2

Pharmacological treatment of PBC

Management of PBC should focus on preventing disease progression, improving transplant-free survival, optimizing symptom control and quality of life, and managing comorbidities.A1
All patients with PBC, including those with compensated or decompensated cirrhosis, should be treated promptly after diagnosis with oral ursodeoxycholic acid (UDCA) at a dose range of 13–15 mg/kg/day.A1
UDCA can be administered as a single daily dose or in divided doses depending on tolerability.C2
In patients with suspected gastrointestinal intolerance, dose adjustment or gradual dose re-escalation should be considered.C1
Response to UDCA treatment should be assessed using established biochemical criteria (e.g. Paris II, Toronto) as early as 6 months (particularly in patients at higher risk of disease progression) with formal evaluation no later than 12 months after treatment initiation.B1
A complete biochemical response (normal ALP and bilirubin) or adequate biochemical response (e.g. Paris II, Toronto) should be considered an optimal or acceptable treatment targetB1
In patients with an inadequate biochemical response to UDCA, adherence, correct dosing, PBC-AIH variant syndrome, and concomitant liver diseases should be systematically evaluated.B1
UDCA should be continued even in patients with inadequate response, as it provides clinical benefits.B1
Achieving normalization of ALP and bilirubin < 0.6 × ULN (deep response) may be the optimal treatment goal associated with improved clinical outcomes and reduced progression of liver disease, although this strategy requires further validation.
Treatment targets in PBC should be individualized based on risk stratification; the benefits of ALP normalization appear to be most pronounced in younger patients and those with a LSM ≥ 10 kPa.C1
In patients with inadequate response or intolerance to UDCA initiation of an add-on second-line treatment should be implemented, preferably after referral to expert care.B1
At present, PPAR agonists are recommended as add-on second-line treatment in patients with insufficient response or intolerance to UDCA. (A1) The selective PPAR agonists, elafibranor (80 mg/day) and seladelpar (10 mg/day), are EMA-approved treatment options. Bezafibrate (200–400 mg/day) is an off-label treatment alternative. Currently, the lack of head-to-head data does not enable firm conclusions on comparative efficacy between drugs.
The selective PPAR agonists, elafibranor (80 mg/day) and seladelpar (10 mg/day), are EMA-approved treatment options.
Bezafibrate (200–400 mg/day) is an off-label treatment alternative.
Currently, the lack of head-to-head data does not enable firm conclusions on comparative efficacy between drugs.
PPAR agonists are not recommended in decompensated cirrhosis and should be used with caution in compensated cirrhosis (Child Pugh A and B)B1
Potential adverse effects of PPAR agonists, particularly hepatotoxicity, myopathy, and renal dysfunction, warrant careful monitoring and consideration, especially in light of the differing safety profiles of individual agonists.C1
In cases of inadequate response to one PPAR agonist patients can be offered an alternative PPAR agonist.D2
The combination of different PPAR agonists is not recommended.B1
Where available, triple therapy combining a PPAR agonist, FXR agonists such as obeticholic acid (OCA), and UDCA could represent a treatment option for selected patients.D2
In patients with PBC and features of interface hepatitis or persistently elevated transaminases despite anticholestatic therapy, add-on treatment with budesonide can be considered, provided cirrhosis, portal hypertension and MASH comorbidity have been excluded.B2
In patients with PBC-AIH variant syndrome, combined treatment with UDCA and immunosuppressive therapy is recommended.B1

Treatment of symptoms and extrahepatic manifestations

Symptoms of PBC, particularly pruritus and fatigue, should be assessed at each visit using standardized tools, e.g., a numeric rating scale (NRS) for pruritus and treated if clinically significant.B1
Patients with pruritus should be advised on general supportive measures, including skin care with emollients, avoidance of skin irritation, cooling strategies, and appropriate clothing.C1
First-line treatment with UDCA should not be expected to improve pruritus.
PPAR agonists currently available as add-on second line treatment have shown promising effects on pruritus.
Bezafibrate may be considered (off-label) for the treatment of pruritus, particularly in patients not receiving other PPAR agonists.C2
IBAT inhibitors can be considered in patients with moderate-to-severe pruritus.C2
Cholestyramine can be used as an alternative first-line treatment in mild pruritus, ensuring appropriate timing relative to other medications.C2
Rifampicin can be considered as an off-label second-line treatment of pruritus with close monitoring of liver function.C2
Naltrexone or sertraline can be considered as further off-label options in refractory cases.C2
Patients with refractory pruritus should be referred to specialized centers for consideration of nonpharmacological therapies (e.g. UVB therapy, extracorporeal liver support systems, external or nasobiliary drainage).C1
Evaluation of fatigue should include screening for alternative conditions, such as anemia, hypothyroidism, metabolic disturbances, sleep disorders, and depression.B1
Management should focus on treating underlying causes and contributing factors, as well as supportive and behavioral strategies.B1
Currently, no pharmacological treatment can be recommended for fatigue in PBC as no therapies are formally approved. Selective PPAR agonists have demonstrated some efficacy on fatigue.
Non-pharmacological interventions (e.g. physical activity, especially aerobic and resistance-based exercise, behavioral therapies) can be considered, although evidence remains limited.C2
Comorbidities should be systematically assessed, as they impact prognosis, treatment tolerability, and quality of life.B1
Patients with PBC should be considered at increased risk of osteoporosis and fractures and should undergo osteoporosis risk assessment and treatment according to current national clinical practice guidelines. (B1) Fracture risk should be assessed (e.g. using FRAX) at diagnosis. (B1) Bone mineral density assessment (DEXA) should be performed in patients with intermediate or high fracture risk. (B1) Serum calcium, phosphate, vitamin D, and parathyroid hormone should be measured at diagnosis and monitored regularly.B1
Fracture risk should be assessed (e.g. using FRAX) at diagnosis.B1
Bone mineral density assessment (DEXA) should be performed in patients with intermediate or high fracture risk.B1
Serum calcium, phosphate, vitamin D, and parathyroid hormone should be measured at diagnosis and monitored regularly.B1
Prevention and management of osteoporosis in patients with PBC should follow established national clinical practice guidelines, including fall prevention, cessation of smoking, adequate calcium, vitamin D, and protein intake. Antiresorptive or osteoanabolic therapy should be considered in patients with moderate and initiated in those with high fracture risk.B1
Testing for thyroid disease (TSH, thyroid stimulating hormone) is recommendedB1
Symptoms of sicca syndrome should be actively assessed in patients with PBC.B1
Artificial tears and saliva substitutes should be used as first-line therapy for mild sicca syndrome.B1
Referral to a rheumatologist should be considered for severe sicca syndrome, suspected Sjögren’s disease and arthralgia.C1
Lipid profile should be assessed in all patients with PBC.B1
Cardiovascular risk should be evaluated individually, as PBC-related hypercholesterolemia alone does not necessarily increase risk.B1
Decision on initiating lipid lowering therapy should be based on the risk for the development of major cardiovascular events calculated by the appropriate scores (e.g. SCORE or SCORE-2).B2

Liver transplantation in PBC

Indications for liver transplantation in PBC should follow general criteria, while considering disease-specific features.A1
Referral for liver transplantation assessment should be considered in PBC patients with: Persistently elevated or progressively rising bilirubin (≥ 3 mg/dL) (B1) Development of complications of cirrhosis (B1) Severe pruritus refractory to pharmacotherapyB1
Persistently elevated or progressively rising bilirubin (≥ 3 mg/dL)B1
Development of complications of cirrhosisB1
Severe pruritus refractory to pharmacotherapyB1
Recurrent PBC should be recognized as a common long-term phenomenon affecting a substantial proportion of transplant recipients; younger age at diagnosis or transplantation, tacrolimus use, and post-transplant cholestasis should be considered risk factors for recurrence, and histology remains the gold standard for diagnosis when recurrence is suspectedB1
All patients undergoing liver transplantation for PBC should receive pre-emptive treatment with UDCA at 10–15 mg/kg/day, as it reduces the risk of recurrence, graft loss, and mortality.B1
In histologically confirmed recurrent PBC, PPAR agonists can be considered; however, evidence supporting this strategy remains limited, and transplant-specific safety concerns including potential drug-drug interactions should be carefully evaluated.D2

Pregnancy in PBC

Pregnancy in women with well-controlled disease and without advanced fibrosis is not associated with adverse maternal or fetal outcome.
Pregnant patients with PBC should be closely monitored by a multidisciplinary team, including obstetricians and hepatologists.C1
Caution and close surveillance are recommended in patients with cirrhosis.C1
Postpartum follow-up should be performed, as cholestatic flares can occur after delivery.C1
UDCA should be continued during pregnancy, as it is considered safe and non-teratogenic at recommended doses.C1
UDCA can be used throughout all trimesters and during breastfeeding.C2
Pruritus during pregnancy should be actively managed, and specialist input should be considered.B1
Cholestyramine or rifampicin (off-label) can be considered for treatment of pruritus if symptoms are not adequately controlled with UDCA.B2
Fibrates (e.g. bezafibrate off-label) can be considered after the first trimester on a case-by-case basis, if potential benefits outweigh risks.C2
Selective PPAR agonists (elafibranor, seladelpar) should not be used during pregnancy or breastfeeding, due to lack of safety data.C1

Abstract

This consensus document of the Austrian Society of Gastroenterology and Hepatology (ÖGGH) is intended to provide practical guidance for the management of individuals with primary biliary cholangitis (PBC).

PBC is a chronic inflammatory, autoimmune-mediated disease of the intrahepatic bile ducts that can lead to fibrosis and ultimately cirrhosis. Middle-aged women are significantly more frequently affected than men. The pathogenesis is currently not fully understood. Based on the presence of disease-specific autoantibodies, it is classified as an autoimmune liver disease, although a combination of genetic predisposition and environmental factors contribute to disease development and progression.

The diagnosis of PBC is based on a cholestatic enzyme pattern together with the presence of anti-mitochondrial antibodies (AMA) or PBC-specific anti-nuclear antibodies (sp100, gp210). A liver biopsy is rarely required to establish the diagnosis; exceptions are the suspicion of a PBC autoimmune hepatitis (AIH) variant syndrome or the absence of the abovementioned antibodies.

The therapeutic goal is to reduce cholestatic injury thereby preventing disease progression and to reduce symptoms. Approximately 60–70% of patients achieve clinical and biochemical remission with first-line treatment, i.e., ursodeoxycholic acid (UDCA). Recently, the therapeutic paradigm has shifted from achieving certain predefined response criteria to a normalization of alkaline phosphatase (ALP) together with a low-normal bilirubin level, as the latter was linked to improved outcomes in some subgroups. For patients who do not sufficiently respond to UDCA, the newly approved peroxisome proliferator-activated receptor (PPAR) agonists elafibranor and seladelpar, as well as bezafibrate (off-label use), should be used as a combination treatment with UDCA. In patients with decompensated cirrhosis, liver transplantation has been associated with good long-term outcomes, albeit disease recurrence occurs in up to 50% by 15 years.

Contents of the original publication

The section headings of the original.

Statements and recommendations
Epidemiology, natural history and diagnosis of PBC
Risk stratification, monitoring and follow-up
Pharmacological treatment of PBC
Treatment of symptoms and extrahepatic manifestations
Liver transplantation in PBC
Pregnancy in PBC
Introduction
Epidemiology, natural history and diagnosis of PBC
Epidemiology, symptoms and natural history of PBC
Diagnosis of PBC
Methodology and quality requirements of serological diagnostics
Role of liver biopsy
Risk stratification, monitoring and follow-up
Pharmacological treatment of PBC
Ursodeoxycholic acid
Add-on second line treatment in PBC patients with inadequate response to UDCA or UDCA intolerance
Selective PPAR agonists elafibranor and seladelpar (licensed)
Fibrates (off label)
Immunosuppressants
Obeticholic acid (OCA)
Management of symptoms and extrahepatic manifestations
Pruritus
Fatigue
Associated diseases
Liver transplantation in PBC
PBC and pregnancy

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