Benign Recurrent Intrahepatic Cholestasis (BRIC) is a rare, autosomal recessive liver disorder.
Characterised by recurrent episodes of jaundice and severe pruritus, with complete clinical and biochemical resolution between attacks.
Does not cause progressive liver damage or cirrhosis → distinguishes it from PFIC.

🧬 Aetiology

Linked to mutations in ATP8B1 (BRIC type 1) and ABCB11 (BRIC type 2) – same genes involved in Progressive Familial Intrahepatic Cholestasis (PFIC).
Onset often in adolescence or early adulthood.
Episodes may be triggered by infection, stress, pregnancy (intrahepatic cholestasis of pregnancy), or oral contraceptives.
Inheritance is usually autosomal recessive.

🩺 Clinical Features – Cholestatic Picture

Symptoms during attacks:
- Pruritus: Intense itching, often the most disabling feature.
- Jaundice: Fluctuating, with dark urine and pale stools.
- Malaise, anorexia, weight loss, and fat malabsorption (→ steatorrhea).
Signs:
- Xanthelasma (eyelid cholesterol deposits).
- Xanthomas (tendon/skin cholesterol deposits in chronic cases).

🔎 Investigations

LFTs: Cholestatic pattern (↑ ALP, ↑ bilirubin) during attacks; normal between episodes.
Serum bile acids: Elevated during episodes.
Liver biopsy: Shows intrahepatic cholestasis (bile plugs) but no fibrosis; biopsy is normal between attacks.
Genetic testing: Confirms diagnosis and distinguishes BRIC from PFIC.

💊 Management

Symptom control:
- Bile acid sequestrants (e.g., cholestyramine).
- Rifampicin (induces alternative bile acid metabolism).
- Antihistamines (for pruritus relief).
Vitamin supplementation: Fat-soluble vitamins (A, D, E, K) due to malabsorption.
Supportive care: Address steatorrhea, weight loss, and osteoporosis risk.
Exclude other causes of cholestasis before confirming diagnosis.
Prognosis: Favourable – unlike PFIC, BRIC does not progress to cirrhosis or liver failure.

⚖️ Distinction from PFIC

Feature	BRIC	PFIC
Onset	Adolescence/early adulthood	Infancy/early childhood
Pattern	Recurrent episodes with full remission	Persistent, progressive cholestasis
Fibrosis	Absent	Present → cirrhosis, liver failure
Genetics	ATP8B1, ABCB11 (milder effect)	ATP8B1, ABCB11 (severe loss of function)
Prognosis	Benign, normal life expectancy	Poor without transplant

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Benign recurrent intrahepatic cholestasis

📌 About

🧬 Aetiology

🩺 Clinical Features – Cholestatic Picture

🔎 Investigations

💊 Management

⚖️ Distinction from PFIC

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