LAUSR

BACKGROUND Porphyria cutanea tarda (PCT) is a rare hepatocutaneous disease whose prognosis is largely unknown. OBJECTIVES 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 diagnosed with PCT from 1989-2012. Each patient was matched by age and gender to ten random population controls. We compared survival and cause-specific mortality between patients and controls and adjusted for confounding from alcohol-related diseases, hepatitis, hemochromatosis, HIV, diabetes, AMI, stroke, cancer, COPD, and cirrhosis. RESULTS The 20-year survival was 42.9% (95% CI 36.9-48.7%) for patients with PCT compared to 60.5% (95% CI 58.6-62.4%) for matched controls. 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 non-malignant 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.