Table 1.

Incidence of concomitant EHAIDs in AILDs.

	EHAIDs	Sjogren's syndrome	Autoimmune thyroid disease	Systemic lupus erythematosus	Rheumatoid arthritis	Systemic sclerosis or scleroderma	Inflammatory bowel disease	Dermatomyositis or polymyositis	Raynaud's phenomenon	Mixed connective tissue disease	Autoimmune thrombocytopenic purpura	Pernicious anemia	References
AIH	29.9–61.8%	1.4–34.5%	10.0–23.0%	0.7–18.8%	1.8–12.9%	1.2–3.5%	2.0–8.0%	3.6%	—	2.0–4.0%	1 case	—	[3–7]
PBC	36.5–67.4%	3.5–47.4%	14.4–23.8%	1.0–5.2%	1.8–17.0%	0.8–12.3%	2.0–7.5%	0.6–3.1%	18.0–24.0%	0.6–0.8%	1.0%	4.0%	[3, 8–11]
PBC-AIH OS	25.0–43.7%	8.5–20.8%	18.3%	2.8%	4.2%	1.4%	—	1 case	—	—	1 case	1.4%	[12–14]
PSC	60.0–80.0%	2 cases	7.6%	2 cases	5.6%	1 case	1.7–70.0%	—	—	—	—	—	[15–18]

EHAID: extrahepatic autoimmune disease; AILD: autoimmune liver disease; AIH: autoimmune hepatitis; PBC: primary biliary cirrhosis; PSC: primary sclerosing cholangitis; OS: overlap syndrome.