Table 4 Annual incidence/mortality rates and subdistribution hazard ratios for malignancies and causes of death in persons with porphyria cutanea tarda (2000–2016, 18 years or older)
Incidence cases/no. at risk | Person-years; annual mortality rate per 100 (95% CI) | Subdistribution hazard ratios (95% confidence intervals) | ||
|---|---|---|---|---|
Crude SHR (95% CI) | Adjusted SHR (95% CI) | |||
All sites – first primary C (C00–96, D45–47) | ||||
Reference | 3549/30468 | 393,347; 0.902 (0.873–0.932) | 1.0 | 1.0 |
PCT | 78/572 | 8132; 0.959 (0.768–1.198) | 0.7 (0.5–0.8) | 0.7 (0.6–0.9) |
HCC (155.0; C22.0) | ||||
Reference | 80/30,468 | 402,342; 0.020; 0.020 (0.16–0.025) | 1.0 | 1.0 |
PCT | 5/572 | 8330; 0.060 (0.025–0.144) | 2.2 (0.9–5.5) | 3.1 (1.2–7.7) |
Gallbladder & biliary tract (156; C23–24) | ||||
Reference | 19/30,468 | 402,403; 0.003 (0.003–0.007) | 1.0 | 1.0 |
PCT | 3/572 | 8339; 0.036 (0.012–0.112) | 4.4 (1.3–15.0) | 4.0 (1.1–14.4) |
All-cause mortality | ||||
Reference | 11,756/30468 | 402,757; 2.919 (2.867–2.972) | 1.0 | 1.0 |
PCT | 150/595 | 8684; 1.728 (1.472–2.027) | 0.4 (0.3–0.4) | 0.4 (0.4–0.5) |
Diseases of the liver (K70–77, B15–19, E83.1) | ||||
Reference | 735/30468 | 402,746; 0.182 (0.170–0.196) | 1.0 | 1.0 |
PCT | 4/595 | 8683; 0.046 (0.017–0.123) | 0.2 (0.1–0.6) | 0.2 (0.1–0.6) |
Malignant neoplasms (C00–96) | ||||
Reference | 2323/30,468 | 402,746; 0.577 (0.554–0.601) | 1.0 | 1.0 |
PCT | 45/595 | 8683; 0.518 (0.387–0.694) | 0.5 (0.4–0.7) | 0.6 (0.4–0.8) |
Drug and alcohol overdose (X40–49) | ||||
Reference | 484/30,468 | 402,746; 0.134 (0.134–0.157) | 1.0 | 1.0 |
PCT | 5/597 | 8.683; 0.058 (0.024–0.138) | 0.5 (0.2–1.1) | 0.5 (0.2–1.2) |