Contents
Disease of the bile ducts that causes inflammation and obliterative fibrosis of bile ducts inside and/or outside of the liver.
- Type of Chronic Liver Disease (CLD)
- 30-60 year age group
Etiology
Autoimmune associations:
- IBD (1⁄3 PSC patients are affected with IBD): Ulcerative colitis (3-10%) > Crohn’s disease (~1%)
- HLA association: HLA-B8, HLA-DR3, HLA-DRw52a
- Hypergammaglobulinemia (↑ IgM)
- ↑ p-ANCA association (80%)

Secondary sclerosing cholangitis:
Typical bile duct changes (of PSC) with a clear predisposing factor for duct fibrosis.
- Previous bile duct surgery with stricturing & cholangitis
- Bile duct stones causing cholangitis
- Intrahepatic infusion of 5-fluorodeoxyuridine
- Insertion of formalin into hepatic hydatid cysts
- Insertion of alcohol into hepatic tumors
- Parasitic infections (eg. Clonorchis)
- Autoimmune pancreatitis/Ig64-associated cholangitis
- AIDS (infective due to CMV or Cryptosporidium)
Presentation
Inflammation
↓
Portal fibrosis
↓
Gradual obliteration of intrahepatic & extrahepatic bile ducts
↓
Biliary cirrhosis
↓
Portal hypertension
↓
Hepatic failure

Complications
- Liver:
- Portal hypertension → Hepatosplenomegaly → Cirrhosis
- Biliary system:
- Cholangiocarcinoma (10-30% cases)
- Gallbladder cancer
- Recurrent cholangitis
- Metabolic bone disease (eg. Osteoporosis)
Diagnosis
Diagnostic criteria:
- (Cholangiography) Generalized beading & stenosis of the biliary system
- (History) Absence of choledocholithiasis (or history of bile duct surgery)
- (Follow-up) Exclusion of bile duct cancer
Lab studies:
- LFT: ↑ ALP, GGT, ↑ Serum bilirubin, absent urobilinogen
- ↑ Atypical anti-p-ANCA antibodies
- AMA titre (-)
Imaging:
- ERCP (Endoscopic Retrograde Cholangiopancreatography) (M/accurate)
- MRCP (Magnetic Resonance Cholangiopancreatography) (IOC)
- Beading of ducts

Tissue biopsy:
- Onion-skin fibrosis & inflammation around bile duct
- Portal oedema
- Bile ductular proliferation
- Obliterative cholangitis: Vanishing bile duct syndrome

Differential diagnosis:
- Primary Biliary Cholangitis (PBC)
- AMA titre (+)
- Secondary sclerosing cholangitis
- Cholangiocarcinoma
Management
No cure. Only symptomatic management
Medical management:
- Broad-spectrum antibiotics
- Fat-soluble vitamin (A, D, E, K) replacement
- Cholestyramine: Bile acid sequestrant
- Large dose steroids
- Immunosuppression therapy: Methotrexate, azathioprine, tacrolimus, cyclosporine
- Ursodeoxycholic acid (UCDA)
Surgical management:
- Stenting
- T-tube drainage
- Orthoropic liver transplantation (DEFINITIVE TREATMENT): In advanced liver disease
- C/I: Cholangiocarcinoma