Increased mortality in patients with porphyria cutanea tarda—A nationwide cohort study - 19/08/20
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.
Le texte complet de cet article est disponible en PDF.Key words : epidemiology, liver, mortality, porphyria, porphyria cutanea tarda, skin
Abbreviations used : AMI, CI, COPD, HIV, HR, ICD, PCT, UROD
Plan
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. |
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Conflicts of interest: None disclosed. |
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Reprints not available from the authors. |
Vol 83 - N° 3
P. 817-823 - septembre 2020 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.
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