Global incidence, prevalence and features of primary sclerosing cholangitis: A systematic review and meta-analysis.
Liver Int
; 41(10): 2418-2426, 2021 10.
Article
em En
| MEDLINE
| ID: mdl-34224208
BACKGROUND & AIMS: Primary sclerosing cholangitis (PSC) is an idiopathic, cholestatic liver disease with a diverse range of clinical manifestations. Inter-regional data on PSC are variable, but its global geoepidemiology has not been well-studied. We aimed to examine the worldwide incidence, prevalence and features of PSC and PSC-inflammatory bowel disease (PSC-IBD). METHODS: A systematic search of multiple databases was conducted to identify all original, full-text studies until December 2020 with data regarding the incidence rate (IR) and/or prevalence of PSC. Outcomes were PSC IR, prevalence, features and IBD concurrence. Additionally, a meta-analysis of PSC IR was performed. The study was registered in PROSPERO (CRD42021224550). RESULTS: Of the 1003 studies identified, 17 studies spanning three continents were included. PSC IR was 0.60 per 100 000 person-years (PY) (95% confidence interval: 0.37-0.88 per 100 000 PY). In pooled subgroup analysis for studies conducted in Europe and North America, PSC IR was 0.62 and 0.53 per 100 000 PY, respectively. PSC prevalence ranged 0-31.7 per 100 000 persons, with notable inter-regional differences. Mean age at PSC diagnosis was bimodally distributed, with relative peaks at 15 and 35 years. Mean concurrence of IBD with PSC was 50%, with 76% having ulcerative colitis, 17% Crohn's disease and 8% indeterminate/unspecified IBD. CONCLUSION: While considerable heterogeneity exists in the geoepidemiology of PSC, overall, the classical dogmata of male predilection, bimodal distribution of mean age and high PSC-IBD concurrence appear to hold true. Despite a seemingly stable IR over time, further studies are needed to better understand the geoepidemiology of PSC.
Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Colangite Esclerosante
/
Doenças Inflamatórias Intestinais
/
Colite Ulcerativa
/
Doença de Crohn
Tipo de estudo:
Incidence_studies
/
Prevalence_studies
/
Prognostic_studies
/
Risk_factors_studies
/
Systematic_reviews
Limite:
Humans
/
Male
Idioma:
En
Revista:
Liver Int
Ano de publicação:
2021
Tipo de documento:
Article