Biliary Cirrhosis (Primary and Secondary) Symptoms Diagnosis and Treatment

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.

BILIARY CIRRHOSIS

Biliary Cirrhosis (Primary and Secondary) Symptoms Diagnosis and Treatment


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.

PRIMARBILIARY CIRRHOSIS Symptoms

  • PRIMARBILIARY CIRRHOSIS Clinicafeatures 
  • 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.
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