Table 1. Main clinical, analytical, immunological and histopathological differences among HCV infection, primary biliary cirrhosis, autoimmune hepatitis and sclerosing cholangitis17–19 .
| Hepatitis C virus infection | Primary biliary cirrhosis | Autoimmune hepatitis | Sclerosing cholangitis (SC) | |
| Female:male ratio | 1:2.5 | 9:1 | 3.6:1 | 1:2 |
| Mean age (years) | 30–49 | 35–60 | 15–40 | 25–45 |
| Incidence | Not well known because acute infection is generally asymptomatic | 0.33–5.8 per 100,000 inhabitants/year | 0.08–3 per 100,000 inhabitants/year | 0–1.3 per 100,000 inhabitants/year |
| Prevalence | 2.8% worldwide (predominance in Africa) | 1.91–40.2 per 100,000 inhabitants | 11.6–35.9 per 100,000 inhabitants | 0–16.2 per 100,000 inhabitants |
| Clinical symptoms/signs | Jaundice, non-specific* | Jaundice, non-specific* | Non-specific* | Jaundice, non-specific* |
| Liver profile | Cytolysis/cholestatic pattern | Cholestatic pattern predominance but raised aminotransferases may be present | Cytolysis pattern predominance but cholestatic pattern may also be present | Cholestatic predominance but cytolysis pattern may also be present |
| Hypergammaglobulinemia | + | ++ | +++ | − |
| Other biochemical tests | − | Hyperlipidemia, hypercholesterolemia, ↑IgM | ↑IgG | ↑IgG (IgG4 if associated with IgG4-related disease) |
| Liver biopsy |
Acute hepatitis: hepatocyte ballooning degeneration, Kupffer cell hyperplasia, lobular and sinusoid inflammatory cell infiltrates, acidophil bodies Chronic hepatitis: lymphoid aggregate or follicle in the portal tract (according to severity: necroinflammatory activity or fibrosis) |
Stage I: Portal inflammation; formation of granulomas Stage II: periportal inflammation Stage III: fibrous bridges between portal tracts Stage IV: regenerative nodules |
Portal monocytic infiltrate with scattered eosinophils and “interface hepatitis” | Periductal concentric (“onion-skin”) fibrosis of intra- and extra-hepatic bile ducts |
| Pathognomonic autoantibodies | − | anti-AMA-M2 (others: AMA-M4, -M5, -M8, -M9) | anti-SMA, anti-LKM-1, (others: anti-LC-1, anti-SLA/LPA) | − |
| Other autoantibodies | ANA, RF | ANA, thyroid antibodies, SMA | ANA, ANCA | ANCA, ANA, SMA, ACA, RF |
| Cryoglobulins | + | − | − | − |
| Hypocomplementemia | + | − | − | − |
| Treatment |
Genotypes 2,3,4: Pegylated interferon alpha and ribavirin Genotype 1: Pegylated interferon alpha and ribavirin together with boceprevir or telaprevir |
Ursodeoxycholic acid | Corticoids +/− azathioprine | Ursodeoxycholic acid. Percutaneous or endoscopic placement of biliary stent in dominant biliary strictures. Orthotopic liver transplantation for advanced disease |
| related diseases | Cryoglobulinemia, SS, porphyria cutanea tarda | SS, SSc, RA, SLE, PM, autoimmune thyroiditis | Hemolytic anemia, type-1diabetes, RA, autoimmune thyroiditis | IgG4-related disease, inflammatory bowel disease |
*
Affected patients may be completely asymptomatic, and may be diagnosed after incidental discovery of abnormal liver function tests. Non-specific symptoms: fatigue, nausea, anorexia, weight loss, pruritus, upper abdominal discomfort.
AMA, anti-mitochondrial antibodies; ANA, anti-nuclear antibodies; ANCA, anti-neutrophil cytoplasmic antibodies; LC-1, liver cytosol type 1; LKM-1, liver kidney microsome type 1; PM, polymyositis; RA, rheumatoid arthritis; RF, rheumatoid factor; SLA/LPA, soluble liver antigen/liver-pancreas antigen; SS, Sjögren syndrome; SLE, systemic lupus erythematosus; SMA, smooth muscle antibodies; SSc, systemic sclerosis