Suspect-and-test checklist
Primary biliary cholangitis (PBC)
Autoimmune bile duct disease. Start from a persistently raised ALP with no other cause, then check AMA.
ICD-10-CMK74.3Trial Friend for Clinicians
1Red flags present
Labs
Symptoms
Skin and examination
Autoimmune disease and family
2Already labeled as
3Family to test
4Testing, in the order the sources describe
- Repeat ALP with GGTGGT or conjugated bilirubin supports a liver source. Over 6 months counts as chronic.Result
- History and drug reviewPrescribed, herbal and anabolic agents; alcohol.Result
- Abdominal ultrasoundFirst imaging test, to exclude extrahepatic cholestasis.Result
- AMA and ANAAMA above 1 in 40 with cholestatic tests is usually sufficient, per BSG.Result
- sp100 and gp210 if AMA negativeSpecific but not sensitive. No biopsy needed if positive, per AASLD.Result
- AASLD criteria: 2 of 3Cholestatic ALP; AMA or sp100 or gp210; compatible histology.Result
- MRCPUnexplained cholestasis, especially if antibodies are negative.Result
- Liver biopsyNo PBC-specific antibodies, ALT over 5 times normal, or suspected AIH or steatohepatitis.Result
- Thyroid, celiac and Sjögren screenBSG suggests it at baseline once PBC is diagnosed.Result
5Results the sources say do not rule it out
- Negative AMA. Absent or low titer in 5% to 10%; check sp100 and gp210.
- Gallstones on ultrasound. Common and usually silent in PBC.
- No itch. Many patients never have it.
- Positive AMA with normal ALP. Not PBC by itself; guidelines advise repeat liver tests.
- Raised ALT or ANA. Common in PBC and not proof of overlap.
6Specialists the sources name
Diagnosis code: K74.3 Primary biliary cirrhosisCodes patients often carry before diagnosis are listed in the online guide.
Full guide and sourcesEvery point here is sourced in the online guide, which also has a patient note to print.
Trial Friend, trialfriend.com/for-clinicians. Key sources: AASLD 2018 guidance; EASL 2017 guideline; BSG/UK-PBC 2018 guideline; AGA/AASLD 2026 guideline; Dahlqvist 2017 AMA study; Zein 2003 biopsy study; all 24 with links in the online guide. Checked November 11, 2026. A reference checklist for licensed clinicians that summarizes published sources. It is not a diagnostic tool, a score or a protocol, and it does not replace clinical judgment, local practice or the prescribing information. Not coding advice. Trial Friend never receives anything written or typed on this sheet.