Condition	How to recognise (features • labs • imaging/biopsy)	First-line & follow-up
🩷 Autoimmune Hepatitis (AIH)	Hepatitic LFTs (ALT/AST ≫ ALP), fatigue/jaundice; can be acute severe. ↑ IgG; ANA/SMA (type 1), anti-LKM1 (type 2), anti-SLA/LP. Biopsy: interface hepatitis with plasma cells.	Prednisolone → taper + azathioprine (or MMF if AZA-intolerant). Aim normal ALT/AST & IgG; monitor q3–6 mo. Cirrhosis → HCC surveillance (US ± AFP 6-monthly).
💚 Primary Biliary Cholangitis (PBC)	Cholestatic pattern (ALP ≫ ALT), pruritus/fatigue, xanthelasma. ↑ IgM; AMA-M2 (~95%); if AMA-neg: ANA gp210/sp100. Imaging to exclude obstruction; biopsy if atypical.	UDCA 13–15 mg/kg/day; check 12-mo biochemical response. Inadequate → consider obeticholic acid* or bezafibrate†. Manage pruritus (cholestyramine → rifampicin → naltrexone). DEXA & fat-soluble vitamins; HCC surveillance if cirrhotic.
🌀 Primary Sclerosing Cholangitis (PSC)	Often men 30–40 yrs; strong UC link; cholestatic LFTs, pruritus/cholangitis. p-ANCA common (non-specific); check IgG4 to exclude IgG4-SC. MRCP: multifocal strictures “beading”; biopsy for small-duct disease.	No proven disease-modifying drug; treat complications, endoscopic therapy for dominant strictures; transplant if advanced/intractable pruritus. Cancer risks: cholangiocarcinoma, gallbladder Ca, colorectal Ca (if IBD) → colonoscopy 1–2-yearly; gallbladder US annually; local CCA surveillance protocols.
🔗 Overlap (AIH–PBC / AIH–PSC)	Mixed cholestatic + hepatitic picture; combined serology (e.g., AMA with ↑IgG/ANA/SMA). Biopsy often needed to confirm.	Combine therapies: steroids ± AZA for AIH component + UDCA for cholestasis. Tailor surveillance to both conditions; specialist management.

* Obeticholic acid is contraindicated in decompensated cirrhosis (Child-Pugh B/C). † Bezafibrate is off-label in the UK; consider in non-responders under specialist care.

🧭 Practical UK notes

📉 Targets: normal ALT/AST (AIH), ALP & bilirubin fall (PBC), symptom control (PSC).
🔁 Monitoring: LFTs & IgG/ALP q3–6 months; bone health (PBC/PSC) with baseline DEXA; fat-soluble vitamins if advanced cholestasis.
🧪 Before AZA: check TPMT; counsel on sun protection & cytopenias.
🧻 Pruritus: cholestyramine (separate from other meds by 4 h), rifampicin, naltrexone, sertraline (specialist if refractory).
🧪 PSC + IBD: colonoscopic surveillance 1–2 yearly from diagnosis regardless of IBD duration.
🏥 Transplant: decompensated cirrhosis; intractable pruritus (PBC/PSC); recurrent cholangitis (PSC); non-response acute severe AIH.

The content on this website is provided for educational and informational purposes only to support exam preparation (e.g., MLA, MRCP, USMLE) and learning. This is NOT medical advice, diagnosis, treatment, or professional guidance. It does not replace consultation with a qualified healthcare professional, official guidelines (e.g., NICE, GMC, BNF), or supervised clinical practice. Always verify information with current, authoritative sources. Makindo and its contributors accept no liability for any reliance on this content, including errors, omissions, or any resulting harm, loss, or consequences. By using this site, you agree to these terms.

Autoimmune Liver Disease

🧠 Autoimmune Liver Disease — 3-Column Quick Guide (UK)

🧭 Practical UK notes

Categories