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Journal of the American Academy of Dermatology
Sous presse. Epreuves corrigées par l'auteur. Disponible en ligne depuis le lundi 3 février 2020
Doi : 10.1016/j.jaad.2019.07.082
accepted : 24 July 2019
Increased mortality in patients with porphyria cutanea tarda—A nationwide cohort study
 

Anne Lindegaard Christiansen, MD a, , Axel Brock, MD, DMSc b, Anette Bygum, MD, DMSc c, Lars Melholt Rasmussen, MD, DMSc a, Peter Jepsen, MD, PhD d, e
a Department of Clinical Biochemistry and Pharmacology, Odense University Hospital, Odense, Aalborg, Denmark 
b Department of Clinical Biochemistry, Aalborg South University Hospital, Odense, Aalborg, Denmark 
c Department of Dermatology and Allergy Centre, Odense University Hospital, Odense, Aalborg, Denmark 
d Departments of Hepatology and Gastroenterology, Aarhus University Hospital, Aarhus, Denmark 
e Clinical Epidemiology, Aarhus University Hospital, Aarhus, Denmark 

Correspondence to: Anne Lindegaard Christiansen, MD, Department of Clinical Biochemistry and Pharmacology, Odense University Hospital, J. B. Winslows Vej 4, Indgang 18, Penthouse 1st Floor, 5000 Odense C, Denmark.Department of Clinical Biochemistry and PharmacologyOdense University HospitalJ. B. Winslows Vej 4, Indgang 18Penthouse 1st FloorOdense C5000Denmark
Abstract
Background

Porphyria cutanea tarda (PCT) is a rare hepatocutaneous disease for which the prognosis is largely unknown.

Objective

To compare all-cause and cause-specific mortality between a nationwide cohort of patients with PCT and a matched population sample.

Methods

We included all Danish patients who received a diagnosis of PCT from 1989 through 2012. Each patient was matched by age and sex to 10 random population control individuals. We compared survival and cause-specific mortality between patients and control individuals and adjusted for confounding from alcohol-related diseases, hepatitis, hemochromatosis, HIV, diabetes, acute myocardial infarction, stroke, cancer, chronic obstructive pulmonary disease, and cirrhosis.

Results

The 20-year survival was 42.9% (95% confidence interval [CI], 36.9-48.7) for patients with PCT compared with 60.5% (95% CI, 58.6-62.4) for matched control individuals. All-cause mortality hazard ratio (HR) was 1.80 (95% CI, 1.56-2.07) before adjustment and 1.22 (95% CI, 1.04-1.44) after adjustment. The cause-specific mortality was markedly increased for nonmalignant gastrointestinal diseases (HR, 5.32; 95% CI, 2.71-10.43) and cancers of the gut (HR, 2.05; 95% CI, 1.24-3.39), liver/gallbladder (HR, 11.24; 95% CI, 4.46-28.29), and lungs (HR, 2.17; 95% CI, 1.41-3.33).

Limitations

We had no data on lifestyle factors.

Conclusions

Patients with PCT have increased mortality, primarily explained by an increased mortality from gastrointestinal diseases and from cancers of the gut, liver/gallbladder, and lungs.

The full text of this article is available in PDF format.

Key words : epidemiology, liver, mortality, porphyria, porphyria cutanea tarda, skin

Abbreviations used : AMI, CI, COPD, HIV, HR, ICD, PCT, UROD



 Supported by the University of Southern Denmark and Region of Southern Denmark. The funders have not been involved in study design, data collection, data analysis, manuscript preparation, or publication decisions.
 Conflicts of interest: None disclosed.
 Reprints not available from the authors.



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