# Primary Biliary Cholangitis > Primary biliary cholangitis for NEET-SS Hepatology: AMA testing, UDCA dosing, Paris II response criteria and the pruritus ladder in concise notes. - Canonical URL: https://prepelephant.com/topics/neet-ss/hepatology/pbc-dm - Exam / course: NEET-SS · Subject: Hepatology - Publisher: PrepElephant (https://prepelephant.com) — Prepared and reviewed by the PrepElephant Academic Review Team - First published: 2026-10-02 - Last updated: 2026-10-02 - How to cite: "Primary Biliary Cholangitis", PrepElephant, https://prepelephant.com/topics/neet-ss/hepatology/pbc-dm ## Direct answer Life-long ursodeoxycholic acid at 13–15 mg/kg/day is the treatment of primary biliary cholangitis, started at diagnosis in every patient, with the one-year response checked against Paris II criteria (ALP at or below 1.5 times the upper limit, AST at or below twice normal, and a normal bilirubin). The diagnosis stands on a cholestatic ALP in a middle-aged woman with a positive antimitochondrial antibody — sensitivity around 95 per cent — and biopsy is reserved for antibody-negative or atypical cases. Inadequate responders step up to obeticholic acid or fibrates, keeping in mind that obeticholic acid is contraindicated in decompensated cirrhosis. Pruritus is treated down a fixed ladder that begins with cholestyramine, and every cirrhotic patient enters six-monthly hepatocellular carcinoma surveillance. ## What you must remember - **Diagnostic pair:** cholestatic biochemistry plus antimitochondrial antibody at a titre of 1:40 or more; the M2 subtype is specific, and sp100 or gp210 antibodies rescue the 5 per cent who are AMA-negative. - **Epidemiology:** nine women for every man, peak age 40–60; fatigue and pruritus precede jaundice by years, and up to half are asymptomatic at detection. - **UDCA dose:** 13–15 mg/kg/day in divided doses — a viva favourite; too small a dose is the commonest reason for "non-response". - **Response check at 12 months:** Paris II for early disease (ALP ≤1.5 × ULN, AST ≤2 × ULN, normal bilirubin); Paris I, UK-PBC and GLOBE scores risk-stratify the inadequate responder. - **Second line:** obeticholic acid 5–10 mg (contraindicated in decompensated cirrhosis and portal hypertension), or bezafibrate 400 mg as an add-on; elafibranor and seladelpar are recent per current guidance. - **Pruritus ladder:** cholestyramine 4 g separated from UDCA by at least four hours, then rifampicin 150–300 mg, then naltrexone, then sertraline; refractory cases go to albumin dialysis or transplant assessment. - **Bone disease:** osteoporosis not osteomalacia is the classical association — dual-energy X-ray absorptiometry every two years with calcium and vitamin D. - **Associated autoimmunity:** sicca, thyroid disease, coeliac disease and vitiligo; check thyroid function at baseline. ## Numbers worth knowing Three clusters of numbers earn marks. The first cluster is diagnostic: an ALP more than 1.5 times normal for over six months with a positive AMA needs no biopsy; if the AMA is negative but clinical suspicion is high, biopsy looking for florid duct lesions — granulomatous destruction of small intrahepatic bile ducts — settles it. The second cluster is therapeutic response: recheck ALP, AST and bilirubin at 12 months. A patient meeting Paris II on UDCA has near-normal survival; one with bilirubin creeping past 2 mg/dL or failing the criteria moves to second-line therapy and transplant discussion, because bilirubin is the strongest survival predictor and the original Mayo risk score was built around it. The third cluster is surveillance: six-monthly ultrasound for hepatocellular carcinoma once cirrhosis or portal hypertension is present, two-yearly bone densitometry for all, and yearly review of itch control — fatigue, unfortunately, responds to no drug. ## Where students slip The classic error is stopping UDCA once the ALP normalises — the drug is for life, and stopping invites biochemical and histological relapse that is harder to recapture. The second is misreading the jaundiced patient: deepening jaundice with itch in PBC can be end-stage disease, but a sudden jump should prompt a search for a dominant stricture or concomitant duct pathology, because PBC does not predispose to cholangiocarcinoma the way PSC does. Finally, do not give obeticholic acid to the ascitic patient — the boxed warning after reports of decompensation is a recognised question stem. ## Frequently asked questions ### Which two findings diagnose primary biliary cholangitis without biopsy? Cholestatic ALP elevation for over six months plus antimitochondrial antibody at 1:40 or higher; biopsy is for antibody-negative or doubtful cases. ### What is the correct dose of ursodeoxycholic acid? 13–15 mg/kg/day lifelong, in divided doses, taken at least four hours apart from cholestyramine. ### How is UDCA response assessed? At 12 months using Paris II criteria — ALP ≤1.5 times normal, AST ≤2 times normal, normal bilirubin; failure defines the inadequate responder needing second-line therapy. ### Why is obeticholic acid contraindicated in decompensated cirrhosis? It worsened liver failure and portal hypertension in advanced disease, prompting regulatory restriction to compensated patients. ### What is the first drug for pruritus in PBC? Cholestyramine 4 g before and after the morning UDCA dose, escalating before rifampicin, naltrexone and sertraline are considered.