|Diagnosis in short|
Autoimmune hepatitis. H&E stain.
|LM||interface hepatitis with plasma cells, +/- bile duct injury|
|LM DDx||viral hepatitis, primary biliary cholangitis, AIH-PBC overlap, PSC-AIH overlap, drug reaction|
|Clinical history||usu. women - young or middle-aged|
|Blood work||ANA +ve, IgG elevated, LKM-1 +ve, SMA +ve, HBV -ve, HCV -ve, HIV -ve|
|Prognosis||poor without treatment|
|Clin. DDx||primary biliary cholangitis, primary sclerosing cholangitis|
|Treatment||corticosteroids, immunosuppression, liver transplant (end stage)|
Autoimmune hepatitis, abbreviated AIH, in an uncommon medical liver disease that occurs in adults and children.
- Several criteria exist to diagnose and histology (alone) is not sufficient.
- Classically, young and middle aged women.
- May occur in men.
- Often a fluctuating course.
- Untreated AIH has a poor outcome.
- Leads to cirrhosis and liver failure.
Simplifed diagnostic criteria (2008):
- Antibody titer.
- Elevated IgG.
- Liver pathology.
- Exclusion of viral hepatitis.
|ANA or SMA 1:40||1 point|
|ANA or SMA 1:80||2 points|
|LKM-1 1:40||2 points|
|IgG upper normal||1 point|
|IgG 1.1x upper limit||2 points|
|Histology compatible||1 point|
|Typical AIH histo.||2 points|
|No viral hepatitis||2 points|
Interpretation: Definite >= 7 points. Probable = 6 points.
- Autoantibodies may be seen in HCV.
- A normal IgG is very unusual in AIH - but may be seen in atypical variants with zone III involvment.
- ANA = anti-nuclear antibody.
- SMA = smooth muscle antibody.
- LKM-1 = liver kidney microsomal type 1 antibody.
- Chronic hepatitis - lymphocytic dominant.
- Signs of an other disease.
- PAS stain may be useful - find plasma cells.
- Lots of plasma cells should prompt consideration of AIH.
- Atypical Autoimmune hepatitis may have zone III involvment (lymphoplasmacytic infiltrate) and a normal IgG.
- Viral hepatitis - should have plasma cells.
- Drug-induced liver injury - milder changes than AIH, portal neutrophils and cholestasis.
- Primary biliary cholangitis.
- AIH-PBC overlap.
- PSC-AIH overlap.
Table show AIH-nonCIR
Autoimmune hepatitis. Expanded portal regions with dull edges suggestive of interface hepatitis (UL 40X). PAS without diastase shows interface hepatitis (UR 200X). Abundant plasma cells seen at higher power (LL 400X). PAS with diastase shows intense inflammation of portal triad stroma, with some reduplication of ductal epithelium. The patient’s vial serology and anti-microbial antibody were negative. (LR 200X).
Table show AIH-CIR
Chronic hepatitis with changes of autoimmune hepatitis and with cirrhosis and steatosis. Trichrome shows blue fibrosis about hepatocyte nodules with steatosis (UL, 40X). Inflammation includes occasional plasma cells (UR, 400X, yellow arrowhead at plasma cell), consistent with history of autoimmune hepatitis. Ballooning degeneration is present (LL, 400X). PAS without diastase shows piecemeal necrosis, (LR, 400X). The patient had also had diabetes.
LIVER, RIGHT LOBE, CORE BIOPSY: - LIVER WITH INTERFACE HEPATITIS (GRADE 2/4) WITH PLASMA CELLS. - MODERATE FIBROSIS (STAGE 2/4). COMMENT: The histologic findings are compatible with autoimmune hepatitis. Serologic findings (ANA positive, IgG positive, viral serology negative) are noted. Correlation with LKM-1 serology is suggested, if not done. The histology is not suggestive of primary biliary cholangitis.
The sections show two cores of liver with an interface hepatitis with plasma cells. Numerous eosinophils are seen focally in the portal tract. The number of ducts is focally increased in several portal tracts and associated with neutrophils (ductular reaction). Size of biopsy: adequate. Fragmentation: absent. Fibrosis: Stage 2/4. Fibrous septa: present. Septa with curved contours: present, focally only. Large droplet steatosis (% of hepatocytes): minimal <5%. Ballooning of hepatocytes: absent. Mallory-Denk bodies: absent. Portal inflammation: present (1/4). Interface activity: mild (1/4). Lobular necroinflammation: not identified. Ducts: numbers within normal limits. Duct injury: present. Ductular reaction: present. Cholestasis: not apparent. Terminal hepatic venules: present. Iron stain: absent. Ground glass cells with routine stains: absent.
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