Quality of Life in Patients With Variant Syndromes of Autoimmune Liver Diseases-A Cross-Sectional Multicentre Study

Natalie Uhlenbusch; Romée J A L M Snijders; Meike Mund; Maciej K Janik; Piotr Milkiewicz; Bernd Löwe; Claudia Kroll; Joanna Raszeja-Wyszomirska; Alessio Gerussi; Francesca Bolis; Laura Cristoferi; Pietro Invernizzi; Patricia Kovats; Mária Papp; Lisbet Grønbæk; Henning Grønbæk; Eric T T L Tjwa; Luise Aamann; Henriette Ytting; Vincenzo Ronca; Kathryn Olsen; Ye H Oo; Adriaan J van der Meer; João Madaleno; Bernardo Canhão; Bastian Engel; Alejandro Campos-Murguia; Richard Taubert; Özgür M Koc; Matthijs Kramer; José A Willemse; Ansgar W Lohse; Joost P H Drenth; Tom J G Gevers; Christoph Schramm

doi:10.1111/liv.70526

Quality of Life in Patients With Variant Syndromes of Autoimmune Liver Diseases-A Cross-Sectional Multicentre Study

Natalie Uhlenbusch, Romée J A L M Snijders, Meike Mund, Maciej K Janik, Piotr Milkiewicz, Bernd Löwe, Claudia Kroll, Joanna Raszeja-Wyszomirska, Alessio Gerussi, Francesca Bolis, Laura Cristoferi, Pietro Invernizzi, Patricia Kovats, Mária Papp, Lisbet Grønbæk, Henning Grønbæk, Eric T T L Tjwa, Luise Aamann, Henriette Ytting, Vincenzo RoncaKathryn Olsen, Ye H Oo, Adriaan J van der Meer, João Madaleno, Bernardo Canhão, Bastian Engel, Alejandro Campos-Murguia, Richard Taubert, Özgür M Koc, Matthijs Kramer, José A Willemse, Ansgar W Lohse, Joost P H Drenth, Tom J G Gevers, Christoph Schramm

2 Citations (Scopus)

Abstract

BACKGROUND & AIMS: Primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC) and autoimmune hepatitis (AIH) go along with reduced health-related quality of life (HRQOL). Variant syndromes, that is, conditions with features of both PBC/PSC and AIH, are associated with higher clinical complexity and worse prognosis. Studies on HRQOL in patients with variant syndromes are lacking. We aimed to provide large-scale evidence addressing this gap.

METHODS: We included adult patients with clinical diagnoses of autoimmune liver diseases across nine countries in a cross-sectional study. We descriptively compared demographical, clinical and patient-reported outcomes between the conditions and investigated whether additional AIH contributes to reduced HRQOL compared to the cholestatic liver disease alone. Further, we explored the role of fatigue, cirrhosis and depression severity regarding HRQOL.

RESULTS: N = 1275 patients were included (PBC: n = 342, PBC-AIH: n = 160, PSC: n = 305, PSC-AIH: n = 121, AIH: n = 347). Patients with variant syndromes showed high rates of cirrhosis and increased depressive or anxiety symptoms. Additional AIH was associated with further reduction in physical and mental HRQOL in people with PSC (physical: ΔR 2 = 0.012, p = 0.041; mental: ΔR 2 = 0.016, p = 0.020), but not with PBC (physical: ΔR 2 = 0.008, p = 0.081; mental: ΔR 2 = 0.001, p = 0.609). Physical HRQOL was associated with higher age and fatigue, while mental HRQOL was associated with lower age, fatigue, and depression severity.

CONCLUSIONS: Patients with variant syndromes of autoimmune liver diseases show high physical and mental burden, with fatigue as the main contributor. Particularly PSC-AIH goes along with more severely reduced HRQOL compared to the cholestatic liver disease alone, which is attributable to a higher symptom burden.

Original language	English
Article number	e70526
Journal	Liver international : official journal of the International Association for the Study of the Liver
Volume	46
Issue number	3
Pages (from-to)	e70526
ISSN	1478-3223
DOIs	https://doi.org/10.1111/liv.70526
Publication status	Published - Mar 2026

Keywords

Humans
Quality of Life
Cross-Sectional Studies
Male
Female
Middle Aged
Hepatitis, Autoimmune/psychology
Liver Cirrhosis, Biliary/psychology
Cholangitis, Sclerosing/psychology
Adult
Fatigue/epidemiology
Aged
Depression/epidemiology
Syndrome
Severity of Illness Index
Anxiety/epidemiology
Liver Cirrhosis/epidemiology
overlap syndrome
variant syndrome
autoimmune liver diseases
cholestatic liver diseases
autoimmune hepatitis
quality of life

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Uhlenbusch, N., Snijders, R. J. A. L. M., Mund, M., Janik, M. K., Milkiewicz, P., Löwe, B., Kroll, C., Raszeja-Wyszomirska, J., Gerussi, A., Bolis, F., Cristoferi, L., Invernizzi, P., Kovats, P., Papp, M., Grønbæk, L., Grønbæk, H., Tjwa, E. T. T. L., Aamann, L., Ytting, H., ... Schramm, C. (2026). Quality of Life in Patients With Variant Syndromes of Autoimmune Liver Diseases-A Cross-Sectional Multicentre Study. Liver international : official journal of the International Association for the Study of the Liver, 46(3), e70526. Article e70526. https://doi.org/10.1111/liv.70526

Quality of Life in Patients With Variant Syndromes of Autoimmune Liver Diseases-A Cross-Sectional Multicentre Study. / Uhlenbusch, Natalie; Snijders, Romée J A L M; Mund, Meike et al.
In: Liver international : official journal of the International Association for the Study of the Liver, Vol. 46, No. 3, e70526, 03.2026, p. e70526.

Research output: Contribution to journal › Journal article › Research › peer-review

Uhlenbusch, N, Snijders, RJALM, Mund, M, Janik, MK, Milkiewicz, P, Löwe, B, Kroll, C, Raszeja-Wyszomirska, J, Gerussi, A, Bolis, F, Cristoferi, L, Invernizzi, P, Kovats, P, Papp, M, Grønbæk, L, Grønbæk, H, Tjwa, ETTL, Aamann, L, Ytting, H, Ronca, V, Olsen, K, Oo, YH, van der Meer, AJ, Madaleno, J, Canhão, B, Engel, B, Campos-Murguia, A, Taubert, R, Koc, ÖM, Kramer, M, Willemse, JA, Lohse, AW, Drenth, JPH, Gevers, TJG & Schramm, C 2026, 'Quality of Life in Patients With Variant Syndromes of Autoimmune Liver Diseases-A Cross-Sectional Multicentre Study', Liver international : official journal of the International Association for the Study of the Liver, vol. 46, no. 3, e70526, pp. e70526. https://doi.org/10.1111/liv.70526

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title = "Quality of Life in Patients With Variant Syndromes of Autoimmune Liver Diseases-A Cross-Sectional Multicentre Study",

abstract = "BACKGROUND \& AIMS: Primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC) and autoimmune hepatitis (AIH) go along with reduced health-related quality of life (HRQOL). Variant syndromes, that is, conditions with features of both PBC/PSC and AIH, are associated with higher clinical complexity and worse prognosis. Studies on HRQOL in patients with variant syndromes are lacking. We aimed to provide large-scale evidence addressing this gap.METHODS: We included adult patients with clinical diagnoses of autoimmune liver diseases across nine countries in a cross-sectional study. We descriptively compared demographical, clinical and patient-reported outcomes between the conditions and investigated whether additional AIH contributes to reduced HRQOL compared to the cholestatic liver disease alone. Further, we explored the role of fatigue, cirrhosis and depression severity regarding HRQOL.RESULTS: N = 1275 patients were included (PBC: n = 342, PBC-AIH: n = 160, PSC: n = 305, PSC-AIH: n = 121, AIH: n = 347). Patients with variant syndromes showed high rates of cirrhosis and increased depressive or anxiety symptoms. Additional AIH was associated with further reduction in physical and mental HRQOL in people with PSC (physical: ΔR 2 = 0.012, p = 0.041; mental: ΔR 2 = 0.016, p = 0.020), but not with PBC (physical: ΔR 2 = 0.008, p = 0.081; mental: ΔR 2 = 0.001, p = 0.609). Physical HRQOL was associated with higher age and fatigue, while mental HRQOL was associated with lower age, fatigue, and depression severity. CONCLUSIONS: Patients with variant syndromes of autoimmune liver diseases show high physical and mental burden, with fatigue as the main contributor. Particularly PSC-AIH goes along with more severely reduced HRQOL compared to the cholestatic liver disease alone, which is attributable to a higher symptom burden.",

keywords = "Humans, Quality of Life, Cross-Sectional Studies, Male, Female, Middle Aged, Hepatitis, Autoimmune/psychology, Liver Cirrhosis, Biliary/psychology, Cholangitis, Sclerosing/psychology, Adult, Fatigue/epidemiology, Aged, Depression/epidemiology, Syndrome, Severity of Illness Index, Anxiety/epidemiology, Liver Cirrhosis/epidemiology, overlap syndrome, variant syndrome, autoimmune liver diseases, cholestatic liver diseases, autoimmune hepatitis, quality of life",

author = "Natalie Uhlenbusch and Snijders, \{Rom{\'e}e J A L M\} and Meike Mund and Janik, \{Maciej K\} and Piotr Milkiewicz and Bernd L{\"o}we and Claudia Kroll and Joanna Raszeja-Wyszomirska and Alessio Gerussi and Francesca Bolis and Laura Cristoferi and Pietro Invernizzi and Patricia Kovats and M{\'a}ria Papp and Lisbet Gr{\o}nb{\ae}k and Henning Gr{\o}nb{\ae}k and Tjwa, \{Eric T T L\} and Luise Aamann and Henriette Ytting and Vincenzo Ronca and Kathryn Olsen and Oo, \{Ye H\} and \{van der Meer\}, \{Adriaan J\} and Jo{\~a}o Madaleno and Bernardo Canh{\~a}o and Bastian Engel and Alejandro Campos-Murguia and Richard Taubert and Koc, \{{\"O}zg{\"u}r M\} and Matthijs Kramer and Willemse, \{Jos{\'e} A\} and Lohse, \{Ansgar W\} and Drenth, \{Joost P H\} and Gevers, \{Tom J G\} and Christoph Schramm",

note = "{\textcopyright} 2026 The Author(s). Liver International published by John Wiley \& Sons Ltd.",

year = "2026",

month = mar,

doi = "10.1111/liv.70526",

language = "English",

volume = "46",

pages = "e70526",

journal = "Liver international : official journal of the International Association for the Study of the Liver",

issn = "1478-3223",

publisher = "Wiley-Blackwell",

number = "3",

}

TY - JOUR

T1 - Quality of Life in Patients With Variant Syndromes of Autoimmune Liver Diseases-A Cross-Sectional Multicentre Study

AU - Uhlenbusch, Natalie

AU - Snijders, Romée J A L M

AU - Mund, Meike

AU - Janik, Maciej K

AU - Milkiewicz, Piotr

AU - Löwe, Bernd

AU - Kroll, Claudia

AU - Raszeja-Wyszomirska, Joanna

AU - Gerussi, Alessio

AU - Bolis, Francesca

AU - Cristoferi, Laura

AU - Invernizzi, Pietro

AU - Kovats, Patricia

AU - Papp, Mária

AU - Grønbæk, Lisbet

AU - Grønbæk, Henning

AU - Tjwa, Eric T T L

AU - Aamann, Luise

AU - Ytting, Henriette

AU - Ronca, Vincenzo

AU - Olsen, Kathryn

AU - Oo, Ye H

AU - van der Meer, Adriaan J

AU - Madaleno, João

AU - Canhão, Bernardo

AU - Engel, Bastian

AU - Campos-Murguia, Alejandro

AU - Taubert, Richard

AU - Koc, Özgür M

AU - Kramer, Matthijs

AU - Willemse, José A

AU - Lohse, Ansgar W

AU - Drenth, Joost P H

AU - Gevers, Tom J G

AU - Schramm, Christoph

PY - 2026/3

Y1 - 2026/3

N2 - BACKGROUND & AIMS: Primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC) and autoimmune hepatitis (AIH) go along with reduced health-related quality of life (HRQOL). Variant syndromes, that is, conditions with features of both PBC/PSC and AIH, are associated with higher clinical complexity and worse prognosis. Studies on HRQOL in patients with variant syndromes are lacking. We aimed to provide large-scale evidence addressing this gap.METHODS: We included adult patients with clinical diagnoses of autoimmune liver diseases across nine countries in a cross-sectional study. We descriptively compared demographical, clinical and patient-reported outcomes between the conditions and investigated whether additional AIH contributes to reduced HRQOL compared to the cholestatic liver disease alone. Further, we explored the role of fatigue, cirrhosis and depression severity regarding HRQOL.RESULTS: N = 1275 patients were included (PBC: n = 342, PBC-AIH: n = 160, PSC: n = 305, PSC-AIH: n = 121, AIH: n = 347). Patients with variant syndromes showed high rates of cirrhosis and increased depressive or anxiety symptoms. Additional AIH was associated with further reduction in physical and mental HRQOL in people with PSC (physical: ΔR 2 = 0.012, p = 0.041; mental: ΔR 2 = 0.016, p = 0.020), but not with PBC (physical: ΔR 2 = 0.008, p = 0.081; mental: ΔR 2 = 0.001, p = 0.609). Physical HRQOL was associated with higher age and fatigue, while mental HRQOL was associated with lower age, fatigue, and depression severity. CONCLUSIONS: Patients with variant syndromes of autoimmune liver diseases show high physical and mental burden, with fatigue as the main contributor. Particularly PSC-AIH goes along with more severely reduced HRQOL compared to the cholestatic liver disease alone, which is attributable to a higher symptom burden.

AB - BACKGROUND & AIMS: Primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC) and autoimmune hepatitis (AIH) go along with reduced health-related quality of life (HRQOL). Variant syndromes, that is, conditions with features of both PBC/PSC and AIH, are associated with higher clinical complexity and worse prognosis. Studies on HRQOL in patients with variant syndromes are lacking. We aimed to provide large-scale evidence addressing this gap.METHODS: We included adult patients with clinical diagnoses of autoimmune liver diseases across nine countries in a cross-sectional study. We descriptively compared demographical, clinical and patient-reported outcomes between the conditions and investigated whether additional AIH contributes to reduced HRQOL compared to the cholestatic liver disease alone. Further, we explored the role of fatigue, cirrhosis and depression severity regarding HRQOL.RESULTS: N = 1275 patients were included (PBC: n = 342, PBC-AIH: n = 160, PSC: n = 305, PSC-AIH: n = 121, AIH: n = 347). Patients with variant syndromes showed high rates of cirrhosis and increased depressive or anxiety symptoms. Additional AIH was associated with further reduction in physical and mental HRQOL in people with PSC (physical: ΔR 2 = 0.012, p = 0.041; mental: ΔR 2 = 0.016, p = 0.020), but not with PBC (physical: ΔR 2 = 0.008, p = 0.081; mental: ΔR 2 = 0.001, p = 0.609). Physical HRQOL was associated with higher age and fatigue, while mental HRQOL was associated with lower age, fatigue, and depression severity. CONCLUSIONS: Patients with variant syndromes of autoimmune liver diseases show high physical and mental burden, with fatigue as the main contributor. Particularly PSC-AIH goes along with more severely reduced HRQOL compared to the cholestatic liver disease alone, which is attributable to a higher symptom burden.

KW - Humans

KW - Quality of Life

KW - Cross-Sectional Studies

KW - Male

KW - Female

KW - Middle Aged

KW - Hepatitis, Autoimmune/psychology

KW - Liver Cirrhosis, Biliary/psychology

KW - Cholangitis, Sclerosing/psychology

KW - Adult

KW - Fatigue/epidemiology

KW - Aged

KW - Depression/epidemiology

KW - Syndrome

KW - Severity of Illness Index

KW - Anxiety/epidemiology

KW - Liver Cirrhosis/epidemiology

KW - overlap syndrome

KW - variant syndrome

KW - autoimmune liver diseases

KW - cholestatic liver diseases

KW - autoimmune hepatitis

KW - quality of life

UR - https://www.scopus.com/pages/publications/105029067508

U2 - 10.1111/liv.70526

DO - 10.1111/liv.70526

M3 - Journal article

C2 - 41623132

SN - 1478-3223

VL - 46

SP - e70526

JO - Liver international : official journal of the International Association for the Study of the Liver

JF - Liver international : official journal of the International Association for the Study of the Liver

IS - 3

M1 - e70526

ER -

Quality of Life in Patients With Variant Syndromes of Autoimmune Liver Diseases-A Cross-Sectional Multicentre Study

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