AUTOIMMUNE HEPATITIS
Diagnosis
- A chronic, inflammatory hepatitis
- If untreated, usually progresses to hepatic cirrhosis and failure
- Clinical findings:
- Elevated transaminases
- Presence of autoantibodies and elevated IgG
- Typically ANA and anti-smooth muscle antibodies are used
- However, about 30% of pts with autoimmune hepatitis have normal
results on both, and no correlation was seen between such results and
disease severity, histological findings, or results to treatment in one
retrospective study (Clin. Gastroent. 7:98, 2009-JW)
- Diagnosis generally made using a scoring system that includes
serologic testing and liver biopsy; A cuttoff of > 5 has
sensitivity 88% and specificity 97% for AIH; a cutoff of >
6 has specificity 99% and sensitivity 81%
| Hepatitis B AND Hepatitis C-negative |
2 |
IgG > 1.0 but ≤ 1.1 x upper limit of normal
IgG > 1.1 x upper limit of normal |
1
2 |
SMA or ANA ≥ 1:40
(SMA or ANA ≥ 1:80 OR LKM ≥ 1:40 OR SLA/LP positive) |
1
2 |
Liver biopsy compatible with AIH
Liver biopsy typical for AIH |
1
2 |
SMA = smooth muscle actin
ANA = antinuclear antivody
AMA = antimitochondrial antibody
LKM = liver-kidney microsomal antibodies
SLA/LP = soluble liver/liver-pancreas antibodies (highly specific to
AIH)
Treatment
- Immunosuppressants are frequently used, e.g. prednisone 60mg/d
(or 1-2mg/kg/d for children), prednisone 30mg/d + azathioprine 50mg/d,
etc.
- Consider treatment for transaminases > 10x upper limit of
normal, (transaminases > 5x upper limit of normal + gamma
globulin ≥ 2x normal). liver bx showing bridging necrosis or
multiacinar necrosis, most children
(Sources include Core Content Review of Family Medicine, 2012)