Table 1.
Features of AIH, PBC, and PSC
| Feature | AIH | PBC | PSC |
|---|---|---|---|
| Sex | Females: 60%‐75% | Females: >90% | Females: 30%‐35% |
| Age | All age groups; median age ∼45 years | Typically 30‐65 years; not diagnosed in children | Typically 30‐50 years, but all age groups |
| Aminotransferases | Markedly elevated, often 3‐ to 10‐fold, but may be normal or only minimally elevated | Normal or slightly elevated | Normal or slightly elevated |
| ALP | Elevated levels may be seen | Moderately to markedly elevated | Moderately to markedly elevated (typically at least 3× ULN; but variable levels, may even be normal) |
| Bilirubin | Variable increase | Variable increase, but normal in majority at diagnosis | Variable increase, but normal in majority at diagnosis |
| Immunoglobulins | Hyper‐gammaglobulinemia, especially elevated IgG (generally elevated 1.2‐3.0× ULN) | IgM increased in most patients | IgG increased in up to 61%; IgM increased in up to 45% |
| Autoantibodies | |||
| ANA, SMA | Significant titers (≥1:40) of ANA and/or SMA in 70%‐80% | ANA in >30% (anti‐gp210 and anti‐Sp100 highly specific); SMA may be present | ANA in 8%‐77%; SMA in 0%‐83% |
| Anti‐LKM | Anti‐LKM in 3%‐4% | ||
| Anti‐SLA/LP | Anti‐SLA/LP in 10%‐30% | Anti‐SLA/LP may be detected | Anti‐SLA/LP may be detected |
| pANCA | pANCA in 50%‐96% (often atypical, pANNA); conventional autoantibodies not detected in up to 10% | pANCA in 26%‐94% | |
| AMA | AMA in low titer occasionally seen (AMA anti–PDC‐E2 pattern rarely detected) | AMA in 90%‐95% (AMA anti–PDC‐E2 pattern highly specific) | AMA occasionally positive |
| Liver biopsy | |||
| Interface hepatitis | Typical findinga | In a proportion of casesb | In a variable number of casesc |
| Portal inflammation | Portal plasma cell infiltrate | Portal lymphocytic infiltrate | Portal lymphocytic infiltrate |
| Biliary changes | In a proportion of cases | Typical | Typical |
| Granulomas | Atypical | Suggestive of PBC, but invariably present | Atypical, but may be observed |
| Cholangiography | Normal or signs of liver cirrhosis | Normal or signs of liver cirrhosis | Characteristic findings, diagnostic of PSC; normal cholangiography in small duct PSC |
| Irritable bowel disease | Rarely associated with AIH; PSC should be excluded | Rarely associated with PBC | Present in up to 80% |
Abbreviations: IgG, immunoglobulin G; IgM, immunoglobulin M; pANCA, perinuclear anti‐neutrophil cytoplasmic antibodies; pANNA, anti‐neutrophil nuclear antibody; PDC‐E2, pyruvate dehydrogenase complex‐E2; LKM, Liver Kidney Microsomal; SLA/LP, soluble liver antigen/liver‐pancreas.
a
A diagnosis of definite AIH should not be concluded without a liver biopsy.
b
A liver biopsy is not required in AMA‐positive cases. In early disease, characteristic features are uncommon.
c
A liver biopsy is not necessary for the diagnosis of large duct PSC but is required for the diagnosis of small duct PSC. Based on Trivedi and Hirschfield.2
Adapted from Reference 2.