Biliary Cirrhosis (Primary and Secondary) Symptoms Diagnosis and Treatment

BILIARY CIRRHOSIS

• Biliary cirrhosis occurs due to injury or prolonged obstruction of intrahepatic or extrahepatic biliary sys­tem. There is destruction of liver cells and progres­sive fibrosis with biliary obstruction.
• In primary biliary cirrhosis there is intrahepatic bile ductules obstruction.
  • progressive jaundice, consequences of impaired bile excretion, and ultimately cirrhosis and liver failure.
  • Presents as asymptomatic elevation in alkaline phosphatase (better prognosis) or with pruritus,
• Secondary biliary cirrhosis is due to long-stand­ing obstruction, of larger extrahepatic ducts.

PRIMARY BILIARY CIRRHOSIS Etiology

• May be associated with:
• · CREST syndrome – (Calcinosis, Raynaud’s phe­nomenon, Esophageal dysmotility, Sclerodactyly, Telangiectasia)
• · Diabetes type I
• · Sicca syndrome.
• Cause is unknown. It may be autoimmune.
• IgG mitochondrial antibody (AMA) is detected. Se­rum levels of IgM is elevated
• Liver biopsy most important in AMA-negative PBC.
• Biopsies identify 4 stages:
  • stage 1—destruction of interlobular bile ducts, granulomas;
  • stage 2—ductular proliferation;
  • stage 3—fibrosis; stage 4—cirrhosis.

Manifestations of PBC

• Specific to PBC
  • Fatigue
  • Pruritus
  • Portal hypertension
  • Metabolic bone disease
  • Xanthomata
  • Fat soluble vitamin malabsorption
  • Urinary tract infection
  • Malignancy
• Associated disorders
  • Thyroid dysfunction
  • Sicca syndrome
  • CREST
  • Raynaud’s syndrome
  • Rheumatoid arthritis
  • Celiac disease
  • Inflammatory bowel disease

PRIMARY BILIARY CIRRHOSIS Pathology

• · There is chronic non-suppurative destructive cholangitis.
• · Cirrhosis may be micronodular or macronodular.

PRIMARY BILIARY CIRRHOSIS Symptoms

• PRIMARY BILIARY CIRRHOSIS Clinical features 
• 90% of patients are females of age 35 – 60 years. May be asymptomatic and detected on routine screen­ing with elevated serum alkaline phosphatase.
• There is progressive liver injury.
• There is pruritus, especially of palms and soles. There is fatigue, jaundice, and darkening of the ex­posed areas of skin (melanosis).
• There is steatorrhoea and malabsorption offat solu’ble vitamins due to impaired bile excretion.
• Xanthelasma can be seen over eyes, joints, tendons. There is portal hypertension-ascites, variceal haemorrhage.
• Death occurs due to hepatic insufficiency in 5 – 10 years.

Physical examination:

• May be normal (no finding) Jaundice
• Melanosis
• Xanthomas
• Hepatomegaly Splenomegaly
• Clubbing
• Bone tenderness
• Sicca syndrome (drying up of mucus membranes) CREST syndrome.

PRIMARY BILIARY CIRRHOSIS Diagnosis

•  Lab findings
• · Serum alkaline phosphatase is elevated
• · Serum 5 nucleotidase activity and gamma glutamyl transpeptidase (GTT) levels are el­evated
• · Serum bilirubin is normal.
• · ALT and AST are increased
• · Positive AMA test
• · Hyperlipidemia
• · Steatorrhea
• · Hypoprothrombinemia
• · Liver biopsy confirms the diagnosis.

PRIMARY BILIARY CIRRHOSIS Treatment

• No specific treatment for PBC Liver transplantation
• Ursodiol – 13 to 15 mg/kg/day single dose Colchicine
• Methotrexate
• Cholestyramine orally helps to relieve pruritus For steatorrhoea-Iow fat diet
• Vitamin A, 0, E, K
• Zinc and calcium supplementation Biphosphonate (Alendronate) for osteoporosis.

SECONDARY BILIARY CIRRHOSIS

• Etiology:
• · Partial or total obstruction of common bile duct
• · Cause of obstruction may be strictures, gall stones, chronic pancreatitis
• · Autoimmune causes
• · Congenital biliary atresia
• · Cystic fibrosis.

SECONDARY BILIARY CIRRHOSIS Symptoms 

• Clinical features
• · Jaundice
• · Pruritus
• · Biliary colics.

SECONDARY BILIARY CIRRHOSIS Diagnosis

•  Investigation
• · Cholangiography
• · Liver biopsy.

SECONDARY BILIARY CIRRHOSIS Treatment

• Relief of obstruction by surgery or endoscopy Antibiotics.