Abstract
Primary biliary cholangitis (PBC) has been regarded as female-predominant without evidence of gender difference in survival. We aimed to compare the overall survival, incidence and prevalence of PBC in two well defined population-based studies over a recent decade, considering also sex ratios and mortality. We have taken advantage of population-wide records, during 2000–2009, in Lombardia, Northern Italy, and Denmark. We focused on the incident cases of PBC, including gender and outcome, among 9.7 million inhabitants of Lombardia and 5.5 million of Denmark. In Lombardia there were 2,970 PBC cases with a female:male ratio of 2.3:1. The age/sex-adjusted annual incidence of PBC was 16.7 per million. Point prevalence was 160 per million on January 1st 2009. In Denmark there were 722 cases of incident PBC, female:male ratio was 4.2:1, and the annual incidence was 11.4 per million, a point prevalence of 115 per million in 2009. Cox regression multivariate analysis identified male sex as an independent predictor of all-cause mortality in both Italian (HR 2.36) and Danish population (HR 3.04). Our data indicate for PBC a sex ratio significantly lower than previously cited, a reversal of the usual latitudinal difference in prevalence and a surprisingly higher overall mortality for male patients.
Highlights
Autoimmune diseases include more than 70 different conditions affecting approximately 5% of the population of developed countries[1]; they are a major health problem and can greatly impair the quality of life of affected subjects
Administrative databases are an alternative data source that should overcome some of the weaknesses evident in previous studies[39,40] and we assess data derived from two such databases, for Lombardia, Italy and for Denmark, regions located at latitudinal extremes of continental Europe
The administrative database contains information on all patients discharged from any hospital in the region, and includes sex, date of birth, discharge diagnoses based on the WHO International Classification of Diseases 9th Edition, Clinical Modification (ICD-9-CM)[41], dates of hospitalization and discharge, and date and cause of death for patients who died in hospital; data were recorded since 2000
Summary
Autoimmune diseases include more than 70 different conditions affecting approximately 5% of the population of developed countries[1]; they are a major health problem and can greatly impair the quality of life of affected subjects. According to earlier data[24,31,32] PBC is considered a disease predominantly affecting women with female:male (F:M) ratios of up to 10:1 and is a prime example of the characteristic sexual dimorphism in autoimmunity. The annual incidence and prevalence rates of PBC per 100,000 inhabitants have ranged from 0.33–5.8 per 100,000 and 1.91–40.2, respectively[34]; attributed to ethnic differences in study populations, methods, and case ascertainment. We aimed to compare the overall mortality, incidence and prevalence of PBC in two well-defined population-based studies over a recent decade, 2000–2009, considering sex ratios and mortality. Lombardia, is a Northern Italian province with a population of some 9.7 million, Denmark counts nearly 5.5 million inhabitants
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