Autoimmune liver diseases include: autoimmune hepatitis (AIH); primary biliary cirrhosis (PBC); primary sclerosing cholangitis (PSC); autoimmune cholangitis (AIC); and overlap syndromes (OCS).
I. Autoimmune hepatitis (AIH)
Basis of diagnosis.
Prevalent in women (90%)
Clinical manifestations: malaise, gastrointestinal symptoms, jaundice, hepatosplenomegaly
Biochemical examination: transaminases, elevated Tbil, elevated gamma globulin or IgG greater than 1.5 times normal
Autoantibodies: ANA, SMA, anti-LKM1, anti-SLA/LP, pANCA, anti-ASGPR and anti-LC1. adults > 1:80; children > 1:20. AMA negative.
Liver biopsy pathology: interfacial hepatitis with plasma cell infiltration in the confluent area. No biliary tract damage, no nodules, etc.
Exclusion of other causes of liver damage: hereditary disease, viral hepatitis, alcoholic liver disease, drug-related liver disease.
Autoimmune hepatitis treatment.
1, anti-inflammatory, antioxidant and anti-fibrotic therapy
2.Supportive symptomatic treatment
3.Immunosuppressive therapy: prednisone
4.Liver transplantation
II. Primary biliary cirrhosis
(PBC) diagnostic points:
Middle-aged and elderly women
Clinical manifestations: weakness, itching, jaundice
Biochemical examination: ALP, GGT elevated, immunoglobulin especially IgM elevated
Autoantibodies: AMA and/or M2 positive, ANA and SMA
Ultrasound: Echo enhancement along the bile ducts in the liver and bile duct occlusion
Pathology: cholangitis and granuloma formation with ductal narrowing
Primary biliary cirrhosis treatment: ursodeoxycholic acid, hepatoprotection, antifibrotic therapy, liver transplantation
C. Diagnostic points of primary sclerosing cholangitis (PSC).
Prevalent in males
Progressive aggravated cholestasis
Autoantibodies: ANCA
ERCP shows occlusion of intra- and extra-hepatic bile ducts with bead-like changes
Pathology shows inflammatory cell infiltration in the confluent area, interfacial hepatitis, onion-like fibrosis, bile duct occlusion and disappearance
Treatment of primary sclerosing cholangitis: surgical treatment and liver transplantation
IV. Diagnostic points of autoimmune cholangitis (AIC).
Combination of PBC and AIH manifestations
Negative AMA but high ANA titer
Insignificant IgM elevation
Biochemical changes and pathology similar to PBC
Pathology shows massive plasma cell infiltration; no rosette-like hepatocyte or multinucleated cell infiltration
Autoimmune cholangitis treatment: UCDA combined with hormones (5-15 mg/day)
V. Overlap syndrome (OLS):
AIH and PBC overlap common in adults (10% ~ 20%); AIH and PSC overlap (2% ~ 8%)
AIH and PSC overlap is common in children.
Treatment: Comprehensive treatment according to overlapping diseases.