chronic active autoimmune hepatitis

Question 1

etiology

Answer 1

• Actual causative factor unknown
• hepatotropic viruses
• metabolic / genetic derangements
• hepatototoxic drugs
• known to mainly affect women
  3 types of autoimmune hepatitis (acc to Abs):
• anti- smooth mm – common
• antinuclear Ab – in children & old ppl
• cellular liver & kidney – for old people

Question 2

pathogenesis

Answer 2

• If T lymphocytes ↓ → autoimmunological rXn against liver specific lipoproteins → auto Abs against own liver structure
• Viral dependent changes & autoimmunological changes present together in practice → hepatocellular necrosis, inflmtn, fibrosis of liver (intralobular necrosis) → leads to cirrhosis & liver failure
• Progressive disease, recovery is impossible
• When body exposed to triggering factor, it actives host cell-mediated immune response.
• HLA on the surface of hepatocytes causes immune response to form antibodies against it.
• The antibodies then cause T-cytotoxic cell & plasma cells to infiltrate healthy tissue, release cytokines and destroy the tissue

Question 3

clinical picture

Answer 3

Symptoms
- Abdmnl, chest pain, severe acne, joint affection, cessation of menses, diarrhea, fever, large abdomen, hepatic signs, ascites, edema, polyserositis, myocarditis and echymosis.
- asymptomatic, fatigue, anorexia, dark colour of urine, jaundice, hepatomegaly, itching

Question 4

main syndrome

Answer 4

• Asthenic
• Hepato-splenomegaly, hypersplenism
• Extrahepatic signs – liver hand, vascular stasis, trophic disorders
• Hemorrhagic syndrome
• Cytolysis
• Hepatodepression
• Mesenchymal inflammation
• systemic inflmtn (leucopenia, increased ESR, anemia, fever)

Question 5

investigation

Answer 5

1. Gen blood analysis
   - Pancytopenia
   - ↑ bleeding time & high prothrombin time
   - normochromic normocystic anemia,
   - mild leucopenia w eosinophilia,
   - thrombocytopenia
   - ↑ESR
2. Biochemical of blood
   - changes in γ globulins
   - electrolyte tests
   - increased ALT, AST
   - increased serum bilirubun
   - increased alkaline phosphotase
   - hypoalbuminemia
3. Immunology
   - high level Ig G
   - viral markers in blood dependent on phase of activity of viruses
   - specific cells – Lupus Erythematosus Test (LE cells) – positive
   - Absence of viral serological markers
   - Serum protein electrophoresis – presence of IgG
4. U/S - Changes of tissue density, bile ducts, diameter of liver
5. Liver biopsy – piece-meal necrosis, bridging necrosis, fibrosis, lobular collapse

Question 6

treatment

Answer 6

1. Diet No. 5 – no fatty food, spices, alcohol. High calorie diet ( protein rich)
2. Antiviral drugs
3. Drugs that ↑ level of immune syst –interferon (reaferon)
4. Hepatoprotectors – Riboxin, acid glutaminic acid, Vipamic
5. vitamins
6. Immunodepression drugs – Prednisolone, cytostatics, corticosteroids
7. liver transplantation if liver cirrhosis - prednisolone with azathioprine given daily for 2 weeks

Question 7

prognosis

Answer 7

• Good prognosis if there is adequate treatment and management; spontaneous remission
• Prognosis is very poor if patient develops liver cirrhosis & if patient suffers from multiple relapses & inability to gain remission