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Porphyria cutanea tarda (PCT) is a rare hepatocutaneous disease for which the prognosis is largely unknown.
To compare all-cause and cause-specific mortality between a nationwide cohort of patients with PCT and a matched population sample.
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.
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).
We had no data on lifestyle factors.
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
| 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.