Other Causes of Chronic Liver Disease


Infections

Schistosomiasis

  • Trematode worms acquired wading contaminated waters
  • Eggs of S. japonicum and S. mansoni lodge in small intrahepatic portal veins & granulomas appear, incr portal HTN is "pre-sinusoidal"
  • Tx: oral praziquantel for acute infection

Echinococcosis

  • Tapeworm infection; usually asymptomatic, but may see large cysts
  • 25% eosinophilia
  • Spread by dogs (sheep-raising countries; Greece, Uruguay, Argentina, Middle East, Australia & NZ)
  • Tx: surgical excision of cysts; acute - mebendazole

Entamoeba histolytica

  • Food borne exposure, some due to fecal contaminated soil for crops, less commonly from water contamination, oral/anal sexual practices
  • Can form large liver abscess, detectable by CT, US
  • Confirmed by serology, PCR, antigen detection in stool
  • Tx: metronidazole or tinidazole, plus luminal agent (iodoquinol, paromomycin, diloxanide furoate)

 

Drugs & Toxins

Pathophysiology

  • Alcohol
    • Fatty liver: deranged lipid metab, & incr TG in liver
    • Alcoholic hepatitis: ballooning degeneration, necrosis, infiltration PMN cells, Mallory bodies, +/- incr fat, fibrosis

Diagnosis

  • Alpha-methyldopa, INH, Nitrofurantoin (> 6 mth), acetaminophen, propylthiouracil, amiodarone, methotrexate, hypervitamin A, TPN & chlorpromazine
  • Alcohol: 80 g/d x 15 yrs will cause liver damage
    • 8 cans beer, 1/2 liter wine, 1/2 pint 80 proof/d
  • Fatty liver: large smooth liver, +/- tender, jaundice 25%, AST usually <300
  • Alcoholic hepatitis: fatigue, anorexia, N/V, decr liver, AST <200-300, GGT very high 600+, abnl PT, UNLIKE viral hepatitis, AST > ALT; occ cholestasis, incr bili

Treatment

  • Alcohol
    • Fatty liver: abstention, support
    • Alcoholic hepatitis: hospitalized mortality 17-40%, (bilirubin >25 mg/dL [428 umol/L], incr PT= poor prognosis); support; encephalopathic give vegetable protein diet; MVI, folate, withhold Fe, Vit K+

 

Metabolic Causes

Pathophysiology

  • Non-alcoholic fatty liver: incr cellular necrosis, inflammation central veins,  cirrhosis
  • Iron: hemosiderin granules deposited & accumulate, portal tract necrosis,  cirrhosis
  • Wilson's dz: copper 1st liver, then brain, corneas & kidneys; due to decr excretion of Cu
  • Alpha-1-Antitrypsin deficiency: protease inhibitor, PAS inclusion bodies periportal & occl bile ducts
  • Porphyrias: enzyme defects in biosynthesis of heme

Diagnosis

  • Non-alcoholic fatty liver: fat; obesity, DM, incr TG, steroids, drugs; valproic acid, IV tetracycline; fatty liver of pregnancy; clinically none to florid symptoms, mild RUQ pain; normal or mild incr LFT
  • Iron:
    • "iron overload"; thalassemia, freq transfusions, diet, idiopathic 40-60 yo,
    • "full blown": cirrhosis, DM, skin pigmentation, CHC incr AST, serum Fe, CT to assess liver density
  • Copper: Wilson's, 1:33,000, autosomal recessive 1:100 carrier, Kayser-Fleischer rings; serum copper, ceruloplasmin levels
  • Alpha-1-Antitrypsin deficiency: liver and/or lung dz, homozygous 1:2000
  • Porphyrias:
    • 4 hepatic subtypes w/varied clinical features;
    • Abdominal pain, peripheral neuropathy, psychiatric problems; Exacerbated by drugs (barbs, anti seizure, OCP), alcohol, fasting, and infection
    • porphyria cutanea tarda: chronic skin lesions, hair poor healing, and liver dz; triggered by OCP hepatocellular carcinoma, SLE

Treatment

  1. Non-alcoholic fatty liver; limit cause, diet
  2. Iron:
    • venesection (phlebotomy)qW, chelators; deferoxamine
  3. Wilsons: 
    • Penicillamine or Trientine, liver transplant
  4. Alpha-1-Antitrypsin deficiency:
    • liver transplant
  5. Porphyrias:
    • 20 g IV glucose/hr, or hematin w/acute episodes; support; beta-carotene, phlebotomy

 

Immunologic

  • Primary Biliary Cirrhosis
  • Autoimmune Chronic Active Hep
    • "Lupoid hepatitis" unrelated to SLE, young women; cirrhosis; very high gama-globulin
    • Similar to chronic active hepatitis; mild to severe
    • Tx:
      • steroids; +/- Zidovudine (AZT) 50 mg/d;
      • hyperviscosity syndrome  plasmapheresis

 

Vascular

  • Chronic Passive Congestion
    • CHF; +/- ascites; mild jaundice incr aminotransferases
    • Due to lack of oxygen and hepatocyte damage
    • Tx: treat CHF; support
       
  • Budd-Chiari Syndrome
  • CT; vena cavography
  • Tx: transplant 

Cancer

  • Benign adenomas
    • Women on OCPs, men on anabolic steroids
    • RUQ mass w/o pain, occ hemoperitoneum s/p rupture
    • Cysts usually discovered incidentally, may be assoc w/renal cysts
    • Adenomas of hepatocytes, kupffer cells absent, cysts and hemangiomas have unclear etiologies
    • Tx:
      • Adenomas  +/- regression after discontinuance of med, surgery for large cysts & occ hemangioma
         
  • Malignancy
    • Hepatocellular CA 1-2% of all tumors US
    • 75% w/cirrhosis, M>F, R/O w/new onset ascites
    • LFTs not useful, high AFP (200-400 ng/mL)
    • Usually or CT abdomen
    • Death <6 mths of diagnosis
    • Assoc w/HepB worldwide, other etiologies: alcohol, hemochromatosis, alpha-1-antitrypsin deficiency
    • HepB DNA integrates into genome of hepatocytes
    • Tx:
      • surgery small mass, chemotherapy (hepatic artery inf)
         
  • Metastatic Cancer
    • Hepatomegaly, jaundice and ascites
    • Liver scan, CT, U/S
    • Most common in GI, breast, lymphoma and melanoma
    • Tx:
      • chemotherapy is palliative