Health-related quality of life questionnaires used in primary biliary
cholangitis: a systematic review
Xin Ai1, Xian Yang1, Jia-min
Xu1, Wen-xia Yang1, Ying-mei
Tang2*
1The Second Affiliated Hospital of Kunming Medical
University;2Department of Gastroenterology, The
Second Affiliated Hospital of Kunming Medical University,Yunnan Research
Center for Liver Diseases;
Abstract
Objective The purpose of this systematic review was to assess
the suitability of HRQOL questionnaires in patients with primary biliary
cholangitis.
Methods Five electronic databases were searched. The validity
of translated questionnaires, floor and ceiling effects, internal
consistency and test-retest reliability were investigated.
Results Forty-four studies were included, of which fifteen
HRQOL questionnaires were identified. The instruments used most
frequently were the PBC-40 (n = 22), followed by the SF-36 (n = 19),
PBC-27(n=4), CLDQ (n = 3) and NIDDK-QA(n=2), the remaining instruments
were uesd only once. Tweenty-six studies used a translated HRQOL
questionnaire and only six reported or referenced a validation of the
translated questionnaire.
Conclusions PBC-specific HRQOL questionnaires used in primary
biliary cholangitis have generally good psychometric properties. But
lots of studies directly applied the HRQOL tools without verifying the
HRQOL tools validity and reliability in PBC patients. Thus, it is better
for clinicians and researchers to test the measurement properties of
HRQOL questionnaires before use it.
Key words: review, primary biliary cholangitis, health-related quality
of life
*Corresponding author: Ying-mei Tang
mail address:
tangyingmei_med@kmmu.edu.cn
Review criteria:
Inclusion criteria
1. Original studies of adult patients (≥18 years old) with primary
biliary cholangitis, studies reporting generic or disease-specific HRQOL
questionnaires as outcome measure.
2. The study had to be a randomized controlled trial, cohort study, case
control study, clinical trial, or validation study of HRQL instruments.
Message for the clinic:
PBC-specific HRQOL questionnaires used in primary biliary cholangitis
have generally good psychometric properties. It is better for clinicians
and researchers to test the measurement properties of HRQOL
questionnaires before use it.
1.Introduction
Primary biliary cholangitis(PBC) is an autoimmune disease, which
characterized by interlobular cholangitis with progressive destruction,
cholestasis, fibrosis and may develop into end-stage liver disease
eventually[1, 2]. High-titer specific
anti-mitochondrial antibodies and anti-nuclear antibodies GP120 and
SP100 appeared in serum of PBC patients, and the level of IgM in
peripheral blood also increased
correspondingly[3]. Fatigue and pruritus are the
most common symptoms of primary biliary cirrhosis, which affects
patients’ quality of life seriously. Ursodeoxycholic acid(UDCA) is the
first valid drug in the treatment of PBC, but 30% of patients with PBC
do not have a biochemical response to UDCA[4,
5].The World Health Organization (WHO) defines quality of life as
individuals’ perception of their position in life in the context of the
culture and value systems in which they live and in relation to their
goals, expectations, standards and concerns[6].
And health-related quality of life (HRQOL) is defined as the influence
of health and disease on quality of life[7].
Health-related quality of life (HRQOL) measures in chronic disease
management is necessary as it assesses the overall impact on health from
the patient’s perspective. A range of different questionnaires are used
to evaluate HRQOL in PBC, including disease-specific and generic
questionnaires, but no guidance of HRQOL questionnaires properties for
clinicians and researchers to choose. The aim of this review was to
evaluate the suitability of questionnaires used to assess HRQOL in
primary biliary cholangitis.
2.Materials and methods
We followed the Preferred Reporting Items for Systematic Reviews and
Meta-Analyses
(PRISMA) statement guidelines for systematic
reviews[8] to ensure a complete reporting in the
development of the study protocol. This systematic review was performed
in January 2020.
2.1 Data sources
We performed electronic searches in the following five databases:
PubMed, the Cochrane Library, Web of Science, Wan Fang Database, and
Chinese National Knowledge Infrastructure. The search was run on 17
January 2020.
2.2 Search strategy
The search strategy included a combination of the following relevant
keywords: “health-related quality of life”, “quality of life”,
“primary biliary cholangitis” and “primary biliary cirrhosis”.
Electronic database searches were limited to English-language
publications.
2.3 Inclusion and exclusion criteria