The prevalence and disease course of autoimmune liver diseases in Greenland

Figures & data

Figure 1. Flowchart of in- and exclusions.

Abbreviations: ICD-10, International Classification of Diseases – Tenth Revision; ATC, Anatomical Therapeutic Chemical.

Table 1. Cohort background characteristics at the disease level.

Variable	AIH n = 7	PBC n = 3	AIH/PBC overlap n = 4
Age (years), median (range)	64 (60–70)	69 (56–71)	61 (48–69)
Female sex, n (%)	4 (57%)	3 (100%)	3 (75%)
Time since diagnosis (years), median (range)	5.8 (2.1–8.2)	10.7 (2.5–15.4)	14.7 (14.6–18.8)
Liver histopathology, n (%) Interphase hepatitis Lymphocytic inflammation Rosette formation Portal inflammation or granulomas Fibrosis Steatosis	4 (57%) 5 (71%) 4 (57%) 1 (14%) 4 (57%) 3 (43%)	1 (33%) 2 (67%) 0 (0%) 3 (100%) 2 (67%) 0 (0%)	4 (100%) 4 (100%) 1 (25%) 3 (75%) 4 (100%) 2 (50%)
More than one liver biopsy, n (%)	1 (14%)	1 (33%)	3 (75%)
Cirrhosis at diagnosis, n (%)	1 (14%)	0%)	0 (0%)
Cirrhosis at present, n (%)	1 (14%)	1 (33%)	1 (25%)
Pharmacotherapies, n (%) Prednisolone Azathioprine Mycophenolic acid Ursodeoxycholic acid	7 (100%) 5 (71%) 1 (14%) 0 (0%)	0 (0%) 0 (0%) 0 (0%) 3 (100%)	2 (50%) 4 (100%) 0 (0%) 4 (100%)
Disease course, n (%) Complete treatment response Partial treatment response Relapsing Non-response	6 (86%) 0 (0%) 1 (14%) 0 (0%)	1 (33%) 1 (33%) 0 (0%) 1 (33%)	1 (25%) 2 (50%) 1 (25%) 0 (0%)
Anthropometrics, median (range) Height (cm) Weight (kg) BMI (kg/m^2)	158 (154–179) 87 (64–110) 31.5 (25.0–45.2)	153 (146–160) 50 (48–53) 21.8 (18.7–24.9)	169 (151–182) 83 (52–104) 28.9 (22.8–31.4)
Biochemistry, median (range) ALT (U/l) AST (U/l) Alkaline phosphatase (U/l) Bilirubin (µmol/l) FIB4 score	33 (28–53) 30 (25–43) 91 (35–114) 27 (7–34) 1.34 (1.12–2.69)	58 (12–111) 52 (21–176) 318 (70–521) 9 (7–155) 2.91 (1.39–4.43)	80 (52–125) 50 (42–81) 155 (129–175) 21 (8–32) 1.94 (1.00–2.41)
Birthplace, n (%) Greenland Denmark Unknown	6 (86%) 0 (0%) 1 (14%)	3 (100%) 0 (0%) 0 (0%)	3 (75%) 1 (25%) 0%)

Abbreviations: AIH, autoimmune hepatitis; PBC, primary biliary cholangitis; BMI, body mass index; ALT, alanine aminotransferase; AST, aspartate aminotransferase; FIB4, fibrosis 4.

Table 2. Cohort characteristics at the individual level. The cross-sectional anthropometric and biochemistry background information were extracted from the most recent hospital contact, i.e. between January 1, 2019, and May 31, 2023. Subsequently, the pharmacotherapy data were extracted through the medical chart reviews. The term present treatment states that the treatment was ongoing at the most recent hospital contact.

Cross-sectional background anthropometric and biochemistry information												Follow-up on pharmacotherapies
No.	Disease	Sex	Age group(yr)	Time sincediagnosis(yr)	Birth-place	ALT (U/l)	AST (U/l)	Alkaline phosphatase (U/L)	Bilirubin (µmol/l)	FIB4	BMI (kg/m2)	prednisolone	azathioprine	mycophenolic acid	UDCA	Treatment response
1 2 3 4 5 6 7 8 9 10 11 12 13 14	AIH AIH AIH AIH AIH AIH AIH AIH/PBC AIH/PBC AIH/PBC AIH/PBC PBC PBC PBC	M M F F M F F M F F F F F F	≥60 ≥60 ≥60 ≥60 ≥60 ≥60 ≥60 <60 ≥60 <60 ≥60 ≥60 ≥60 <60	6.6 5.4 7.5 8.4 2.3 5.9 2.8 18.9 14.8 14.7 14.8 15.5 2.7 10.8	GR GR GR GR GR N/R GR GR GR GR DK GR GR GR	43 33 28 39 53 28 28 91 69 125 52 111 58 12	43 25 N/R 29 31 31 29 55 42 81 45 176 52 21	52 88 109 114 35 91 109 157 175 129 153 521 318 70	27 7 8 27 34 14 31 24 8 17 32 155 9 7	2.69 1.12 N/R 1.33 1.35 2.09 1.18 1.00 2.28 1.61 2.41 4.43 1.39 N/R	29.1 25.0 45.2 N/R 32.3 44.0 30.8 31.4 22.8 28.9 N/R 24.9 18.7 N/R	present present present previously present previously present present present N/R N/R never never never	present present never present present previously never present present present present never never never	never never never never never present never never never never never never never never	never never never never never never never present present present present present present present	Complete Relapsing Complete Complete Complete Complete Complete Relapsing Partial Complete Partial Non-resp. Non-resp. Responder

Abbreviations: AIH, autoimmune hepatitis; PBC, primary biliary cholangitis; M, male; F, female; yr, years; GR, Greenland; DK, Denmark; N/R, not reported; ALT, alanine aminotransferase; AST, aspartate aminotransferase; FIB4, fibrosis 4; BMI, body mass index; UDCA, ursodeoxycholic acid; Non-resp., non-responder.

Table 3. Liver histology examinations at the individual level.

Histopathology at diagnosis								Histopathology follow-up
No.	Date (yr)	Interphase hepatitis	lymphocyte infiltration	rosette formation	biliary changes	fibrosis	steatosis	Date (yr)	Interphase hepatitis	lymphocyte infiltration	rosette formation	biliary changes	fibrosis	steatosis
1 2 3 4 5 6 7 8 9 10 11 12 13 14	2017 2018 2016 2015 2021 2017 2021 2004 2009 2011 2009 2008 2010 2013	yes no no yes N/R yes yes yes yes yes yes yes no no	yes no yes yes N/R yes yes yes yes yes yes yes yes no	no N/R yes yes N/R yes yes no N/R N/R N/R no no no	no no no no N/R no yes no yes yes no yes no yes	grade 1 no no grade 1 N/R no grade 4 grade 1 no grade 2 grade 1 grade 2 no no	yes yes no no N/R no yes no no no yes no no no	- - - - - 2020 - 2016 2017 2019 - - 2021 -	- - - - - yes - yes yes no - - no -	- - - - - yes - yes yes yes - - no -	- - - - - no - no yes no - - no -	- - - - - no - no no no - - yes -	- - - - - grade 3 - grade 1 grade 4 grade 1 - - grade 2 -	- - - - - no - no no yes - - no -

Abbreviations: N/R, not reported; yr, year.