Primary biliary cholangitis (PBC) is a chronic autoimmune cholestatic liver disease that selectively destroys intrahepatic small bile ducts. Destruction leads to bile retention, hepatocyte damage, and progressive fibrosis. Onset is insidious; many patients are diagnosed while still asymptomatic through routine elevation of alkaline phosphatase. Recognising the evolving clinical picture allows early therapy and monitoring of complications.

1. Early-stage signals
   Fatigue: the most frequent first complaint, unrelated to disease severity, often fluctuating and out of proportion to liver biochemistry.
   Pruritus: initially nocturnal, localized to palms or soles, later generalized; can be intermittent or persistent, sometimes worsened by heat, stress, or pregnancy.
   Dry eyes and dry mouth: part of associated Sjögren-like exocrine involvement, causing gritty sensation and difficulty swallowing dry foods.
   Right upper quadrant discomfort: dull, vague heaviness rather than sharp pain, from liver capsule stretching.
2. Cholestatic progression
   Hyperpigmentation: patchy bronze discoloration on trunk, limbs, or scars due to melanin deposition in cholestasis.
   Xanthelasma and tendon xanthomas: yellowish lipid deposits around eyelids, elbows, or hands from altered cholesterol and bile acid metabolism.
   Steatorrhea: bulky, pale, greasy stools with increased frequency; results from reduced bile acid pool and fat malabsorption.
   Weight loss: gradual, often unnoticed, related to fat malabsorption and anorexia.
3. Metabolic bone and blood effects
   Bone pain or fragility fractures: chronic cholestasis reduces vitamin D absorption and impairs osteoblast activity, leading to osteopenia/osteoporosis.
   Easy bruising: from vitamin K malabsorption and subsequent coagulopathy.
   Anemia or mild icterus: may appear once advanced cholestasis impairs erythropoiesis and bilirubin excretion.
4. Decompensated-stage features
   Jaundice: progressive yellowing of sclera and skin, indicating rising conjugated bilirubin.
   Ascites and lower limb edema: portal hypertension and hypoalbuminemia.
   Spider nevi, palmar erythema, splenomegaly: signs of cirrhotic transformation.
   Hepatic encephalopathy: sleep inversion, confusion, or asterixis when liver failure supervenes.
5. Associated extrahepatic conditions
   Thyroid dysfunction: hypothyroidism or Hashimoto thyroiditis.
   Raynaud phenomenon and arthralgias: overlapping autoimmune features.
   Celiac disease or inflammatory bowel disease: warrant screening when suggestive symptoms arise.

Red-flag combinations demanding immediate review
Rapid deepening of jaundice, new ascites with fever, upper gastrointestinal bleeding, or sudden altered mental state.

Summary table