Primary Biliary Cholangitis (PBC)
Primary Biliary Cholangitis (PBC) is a chronic autoimmune liver disease in which the body’s immune system slowly destroys the small bile ducts inside the liver. These ducts normally carry bile, a fluid that helps digest fats and remove toxins. When the ducts are damaged, bile builds up and injures liver tissue, leading to scarring (fibrosis) and potentially cirrhosis or liver failure over time. PBC affects mostly middle-aged women but can occur in men and younger people too. Common early symptoms include fatigue, itching, and dry eyes or mouth, while jaundice, dark urine, and abdominal swelling appear in advanced stages. Although there is no complete cure, medications such as Ursodeoxycholic acid (UDCA) and Obeticholic acid (OCA) can slow disease progression, and liver transplant remains a curative option in severe cases. With early diagnosis and treatment, many patients can maintain good quality of life and near-normal life expectancy.
(For simplicity, we will use MASLD to refer to this condition, in line with the newer terminology, but many doctors and patients may still know it as NAFLD.)
Symptoms
In the early stages, up to half of people with PSC have no symptoms at diagnosis; the disease might be found via abnormal liver tests on routine bloodwork my.clevelandclinic.org . When symptoms do occur, they can include:
- Fatigue – very common and often severe.
- Pruritus (itchy skin) – may precede other symptoms.
- Dry eyes and dry mouth (sicca symptoms).
- Jaundice (yellowing of skin/eyes) – usually later stage.
- Dark urine and pale stools.
- Upper right abdominal discomfort.
- Enlarged liver or spleen in advanced disease.
- Bone/joint pain or brittle bones (osteoporosis/osteopenia).
Causes
- Autoimmune mechanism: Immune system attacks small intrahepatic bile ducts.
- Genetic predisposition: Family clustering increases risk.
- Environmental factors: Possible triggers include infections, smoking, toxins (not fully proven).
- Not alcohol-related: PBC is distinct from alcoholic liver disease.
Diagnosis
PSC is usually diagnosed through a combination of lab tests and imaging:
- Liver blood tests: Cholestatic pattern – elevated alkaline phosphatase (ALP) and gamma-GT.
- Autoantibodies:
- Anti-mitochondrial antibody (AMA) – positive in ~90–95% of PBC patients (highly specific).
- ANA or other atypical autoantibodies may be present.
- Immunoglobulins: Elevated IgM is characteristic.
- Liver biopsy: Sometimes done to confirm diagnosis or assess staging; shows bile duct injury and fibrosis.
- Imaging: Ultrasound or MRCP to rule out bile duct obstruction from stones/tumors.
Treatment
- Ursodeoxycholic acid (UDCA): First-line; slows disease progression and improves survival if started early.
- Obeticholic acid (OCA): For patients not responding to or intolerant of UDCA.
- Fibrates (e.g., bezafibrate): Emerging therapy for UDCA non-responders (off-label in some regions).
- Symptom management:
- Itching: Cholestyramine, rifampin, naltrexone, or antihistamines.
- Dry eyes/mouth: Artificial tears, saliva substitutes.
- Osteoporosis: Vitamin D, calcium, bisphosphonates if indicated.
- Liver transplant: Only curative option; indicated for advanced cirrhosis, liver failure, or intractable symptoms.
FAQs
No. PBC affects small intrahepatic bile ducts; PSC affects both intra- and extrahepatic ducts and is linked to IBD.
No. Despite the name, alcohol is not a cause.
No cure exists yet. Medications (like UDCA, OCA) slow progression and improve survival.
With early diagnosis and treatment, many patients live near-normal lifespans. Without treatment, risk of cirrhosis and complications increases.
Yes, recurrence can happen in 10–30% of patients, but outcomes post-transplant are usually excellent.