Table 2.

Prevalence of Extrahepatic Manifestations of Primary Biliary Cholangitis²⁰–²⁹,³⁹,⁴⁰,⁴⁴,⁴⁵

Reference	Region	No. of patients	Cohort	Prevalence, %
Sjogren’s syndrome
20	Italy	170	Consecutive patients with PBC evaluated at the University of Milan	3.5
21	United States	1,032	Patients with PBC at 23 tertiary referral medical centers for liver diseases	10
22	Japan	874	Patients with PBC included in a national multicenter survey	20.8
23	United States	67	Patients with PBC evaluated at the Mayo Clinic in Rochester, MN	24
24	United States	38	Outpatients with PBC seen at the National Institute of Diabetes and Digestive and Kidney Diseases	47.4
25	Italy	361	Patients with PBC at a referral tertiary care center	56.1
26	United States	113	Patients with PBC participating in a therapeutic trial of D-penicillamine at the Mayo Clinic	66
27	Canada	95	Patients with PBC evaluated at The Toronto Hospital either on the phone or as outpatients	68.4
28	United Kingdom	18	Patients with PBC attending hospital at the London, St Bartholomew’s and Southampton general hospitals with less than 10 mm moistening of the filter paper during Schirmer’s type I and II tests together with corneal or conjunctival staining with rose bengal	72
29	Sweden	26	Consecutive patients with PBC who were evaluated at the University of Umea with the presence of a Schirmer’s test of less than 5 mm and corneal staining with rose bengal and/or radiological findings of sialectasia	73
Thyroid disease
39	United Kingdom	95	Patients with PBC at the King’s College Hospital	13.7
40	United States	58	Patients with PBC at New England Medical Center Hospital	12
22	Japan	874	Patients with PBC included in a national multicenter survey	5.6
21	United States	1,032	Patients with PBC at 23 tertiary referral medical centers for liver diseases	9
23	United States	67	Patients with PBC evaluated at the Mayo Clinic in Rochester, MN	13
25	Italy	361	Patients with PBC at a referral tertiary care center	23.6
Systemic sclerosis
22	Japan	874	Patients with PBC included in a national multicenter survey	1.4
21	United States	1,032	Patients with PBC at 23 tertiary referral medical centers for liver diseases	2
44	Japan	5,805	Cross-sectional study of patients with PBC registered to receive public financial aid from the Ministry of Health, Labour and Welfare	2
45	United States	558	Patients with PBC and scleroderma seen at the Mayo Clinic	3.9
25	Italy	361	Patients with PBC at a referral tertiary care center	9.9
20	Italy	170	Patients with PBC seen consecutively at the University of Milan	12.3

PBC, primary biliary cholangitis.