19. Autoimmune hepatitis Flashcards
what is autoimmune hepatitis ?
chronic hepatitis with
unknown etiology
which can progress into cirrhosis or acute liver failure
affects YOUNG women more than men
what re the clinical manifestations of AH?
fatigue pruritis / jaundice upper abdominal discomfort anorexia arthralgia and myalgia
what is the classification of autoimmune hep and how can you differentiate ?
type 1 ANA ( negative in severe form) ( anti SLA - anti soluble liver antigen antibody occurs in severe form) anti SMA atypical p-ANCA ANTI actin = AAA
association with hla haplotypes = b8 , dr3
igG increase is pronounced
occurs in age 10-20
45-70
type 2
anti - SLA
LKM-1 - liver kidney microbe 1 ANTIBODY
anti liver cytosol 1
association with haplotypes b14 and d3
igG increase moderate
occurs at age 2-14
what are the usual associated autoimmune conditions with autoimmune hepatitis ?
hashimoto thyroiditis / thyroid abnormalities
endocrine = cushing syndrome appearance = acne and hirtuism
polyarthrits of large joints
diagnosis of autoimmune hepatitis is by excluding what ?
similar features
chronic viral hepatitis
drug induced
non alcoholic fatty liver disease
what is the diagnostic algorithm ?
simple scoring system
lab :
elevated AST
elevated gamma globulin levels ( upper limit of norm 1pt , 1.5 times upper limit = 2pt)
AST : alkaline phosphatase ration more than 3
hyperbilirubinemia
liver biopsy = INTERFACE HEPATITIS +/- = HALLMARK LOBULAR HEPATITIS ( 2pt / compatible with AIH = 1pt)
piecemeal necrosis / interface he numerous plasma cells evidence of hepatocellular regeneration =rosette formation / psudolobules necroinflammotry changes --------
ANA , SMA = 1:40 / more than 1:80 (1pt / 2pt)
anti -SLA positive (2pt)
anti-LKM1 over 1:40 (2pt)
alcohol intake of less than 25g / day
------- no viral markers (+2pt) -------- definite diagnosis if points 7 or more probable if 6
anti - SLA is an indicator for ?
poor outcome and severe disease
what are the variant forms of AIH ?
autoimmun hepatitis with + primary billary cirrhosis (anti mitochondrial antibodies present )
+ primary sclerosing cholangitis (anti mitochondria antibodies absent
but positive inflammatory bowel )
+ cholestatic features
what is the treatment ?
AIH + primary billary cirrhosis
if alkaline phosphatase is equal to or less than 2 times the upper limits of normal = prednisone
if AP more than 2 times the UPN = prednisone and UDCA
with primary sclerosis cholangitis
= prednisone and UDCA
with cholestatic features
= prednisone and UDCA
60g prednisone a day step wise reduction by 5g
mainentance with more than 10mg a day
azathioprine is also added
how can you find cholangic involvement ?
MRCP - magnetic resonance cholangiopancreatography
ERCP= endoscopic retrograde choalnmiopacreatography
= BAND LIKE strictures with dilation of bead like appearance or divertcicular out pouching
what’re the side effects of prednisone
osteoporosis - prophylaxis with calcium and vit d
what is the treatmnet if there is liver cirrhosis
liver transplantation is indicated
what lab reveals treatment failure ?
higher serum AST
and bilirubin levels
and progressive histological actvity
onset of ascitis and encephalopathy
