Autoimmune liver diseases in systemic rheumatic diseases

Table 2 Demographic, clinical, laboratory, pathological, therapeutic and prognostic profiles in three common autoimmune liver diseases

Category	AIH	PBC	PSC
Demographic
Sex	Predominant F, 4:1	Predominant F, 10:1	Predominant M, 2:1
Age	Any, median 45 yr	Common above 40 yr	Any, typical 30-50 yr
Prevalence	Rare, 4-25 per 100000	Rare, 2-40 per 100000	Rare, 4-16 per 100000
Laboratory
Abnormal LFT	Majorly AST/ALT	Majorly ALP/GGT	Majorly ALP/GGT
Serum Ig	Elevated IgG	Elevated IgM	Elevated IgG, IgM
Autoantibody	I: ANA, ASMA; II: anti-LKM, -LC	ANA, AMA	ANCA
HLA-DR	DR3, DR4	DR8	DR52
Liver biopsy
Interface HA	Typical finding	Occasional	Occasional
Portal infiltrate	Lymphoplasmacytic	Lymphocytic	Lymphocytic
Bile duct lesion	Occasional	Florid duct lesion	Obliterative duct
Granuloma	Rare	Typical finding	Rare
Diagnosis	AIH score for definite diagnosis	AMA, liver biopsy, Cholestatic LFT	Cholangiography, Cholestatic LFT
Coexistent SRD
SLE	0.7%-2.8%	1.3%-3.7%	1.70%
SS	1.4%-35%	3.5%-38%	CR
SSc	0.80%	2.3%-12%	CR
RA	1.6%-5.4%	1.8%-13%	1.2%-3.4%
IIM	CR	0.6%-3.1%	CR
MCTD	CR	0.60%	NA
SV	1.60%	2.20%	CR
Sarcoidosis	0.60%	2.70%	0.80%
First-line Tx	CS or CS plus AZA	UDCA	No effective therapy
Prognosis	Generally responsive to IS, poor prognosis if untreated	Excellent prognosis if responsive to UDCA	Median survival without LT 12-16 yr after diagnosis

AIH: Autoimmune hepatitis; ALP: Alkaline phosphatase ALT: Alanine aminotransferase; AMA: Antimitochondrial autoantibody; ANA: Anti-nuclear antibody; ANCA: Perinuclear antineutrophil cytoplasmic antibody; APS: Antiphospholipid syndrome; ASMA: Anti-smooth muscle antibody; AST: Aspartate aminotransferase; AZA: Azathioprine; CR: Case report; CS: Corticosteroids; EHAID: Extra-hepatic autoimmune disease; HA: Hepatitis; Ig: Immunoglobulin; IIM: Idiopathic inflammatory myopathies; IS: Immunosuppressants; GGT: Gamma-glutamyl transferase; LC: Liver cytosol; LKM: Liver kidney microsomal; LFT: Liver function test; LT: Liver transplantation; MCTD: Mixed connective tissue disease; NA: Not available; PBC: Primary biliary cholangitis; PSC: Primary sclerosing cholangitis; RA: Rheumatoid arthritis; SLE: Systemic lupus erythematosus; SS: Sjögren syndrome; SSc: Systemic sclerosis; Tx: Treatment; UDCA: Ursodeoxycholic acid.