scholarly journals Porphyria Cutanea Tarda and Patterns of Long-term Sick Leave and Disability Pension: a 24-year Nationwide Matched Cohort Study

Author(s):  
Carl Michael Baravelli ◽  
Aasne Karine Aarsand ◽  
Sverre Sandberg ◽  
Mette Christophersen Tollånes

Abstract Background: Porphyria cutanea tarda (PCT) is a skin disorder caused by a defect in the liver enzyme uroporphyrinogen decarboxylase and is associated with hepatitis C virus infection, high alcohol intake, smoking and iron overload. Data on the long-term morbidity of PCT is lacking. Methods: We conducted a nationwide matched cohort study over a 24-year period. The study sample included 534 persons aged 18-67 years with a biochemically confirmed PCT diagnosis and a sample of 21,360 persons randomly selected from the working age population, matched on age, sex and educational attainment. We investigated if persons with sporadic and familial PCT had an increased risk of long-term sick leave (LTSL) or disability pension. We further assessed risk before (pre-PCT), during (during-PCT) and after (post-PCT) the typical period of first onset to diagnosis, treatment and remission. Results: Overall, persons with PCT had a 40% increased risk (hazard ratio [HR]=1.4, 95% confidence interval [CI]=1.3, 1.5) of LTSL and a 50% increased risk (HR=1.5, CI=1.3, 1.7) of disability pension. Risk of disability pension was increased pre-PCT (HR=1.3, CI: 1.3 (1.0, 1.6), during-PCT (HR: 1.5, CI: 1.0, 2.2) and post-PCT (HR=2.0, CI: 1.5, 2.6). For LTSL, risk was increased pre-PCT (HR=1.3, CI: 1.1, 1.4) and during-PCT (HR=1.5, CI: 1.1, 2.1), but not post-PCT. Risk was greatest in persons with sporadic than familial PCT. Diagnostic reasons for disability pension that were increased compared to matched controls were PCT or skin disease in 11 of 199 cases (PCT: n=7, incident rate ratios [IRR]=49.2, CI=38.8, 62.4; diseases of the skin & subcutaneous tissue, n=4, IRR=4.2, CI=1.6, 11.0). The vast majority of diagnostic reasons for accessing disability pension were related to comorbidities, PCT susceptibility factors and more general health issues such as: malignant neoplasms (n=12, IRR=2.4, CI=1.4, 4.2), substance and alcohol dependence (n=7, IRR=5.0, CI=2.5, 10.1), neurotic and mood – disorders (n=21, IRR=1.7, CI=1.1, 2.6), and diseases of the musculoskeletal system & connective tissue (n=71, IRR=2.5, CI=1.9, 3.2).Conclusions: Persons with PCT have an increased risk of LTSL and disability pension indicating significant morbidity in this patient group. Appropriate long-term follow-up and monitoring for relapses and co-morbid diseases are recommended.

2018 ◽  
Vol 21 (9) ◽  
pp. 1716-1722 ◽  
Author(s):  
Kao-Chih Hsu ◽  
Chia-Hung Sun ◽  
Yin-Yin Wu ◽  
Liang-Cheng Chen ◽  
Yung-Tsan Wu ◽  
...  

2019 ◽  
Vol 6 (Supplement_2) ◽  
pp. S510-S510
Author(s):  
Jacob Bodilsen ◽  
Michael Dalager-Pedersen ◽  
Diederik van de Beek ◽  
Matthijs C Brouwer ◽  
Henrik Nielsen

Abstract Background The long-term outcome of brain abscess is unclear. Methods We used medical registries to conduct a nationwide population-based matched cohort study to examine the long-term risks of mortality and new-onset epilepsy in patients hospitalized with brain abscess in Denmark from 1982 through 2016. Comparison cohorts from the same population individually matched on age, sex, and residence were identified, as were siblings of all study participants (Figure 1). We computed cumulative incidences and hazard rate ratios (HRRs) for mortality and new-onset epilepsy among brain abscess patients, comparison cohorts and siblings. Population and appendicitis controls had similar characteristics and prognosis why only comparisons between brain abscess patients and population controls are detailed here. Results We identified 1,384 brain abscess patients with a median follow-up time of 5.9 years (IQR 1.1–14.2). The 1-year, 2–5 year, and 6–30-year mortality of patients after brain abscess was 21%, 16% and 27% when compared with 1%, 6% and 20% for matched population controls (Figure 2). Cox regression analyses adjusted for Charlson comorbidity index score showed 1-year, 2–5 year, and 6- to 30-year HRRs of 17.5 (95% CI 13.9–22.2), 2.61 (95% CI 2.16–3.16) and 1.94 (95% CI 1.62–2.31). The mortality in brain abscess patients compared with population controls was significantly increased regardless of sex or age group except among subjects 80 years or older, and in both previously healthy individuals and immuno-compromised persons. Among the 30-day survivors of brain abscess (median follow-up 7.6 years [IQR 2.2–15.5]), new-onset epilepsy occurred in 32% compared with 2% in matched population controls. Cause-specific Cox regression analysis adjusted for stroke, head trauma, alcohol abuse, and cancer showed 1-year, 2–5-year, and 6–30-year HRRs for new-onset epilepsy of 155 (95% CI 78.8–304), 37.7 (95% CI 23.0–59.9), and 8.93 (95% CI 5.62–14.2) (Figure 3). Comparisons between sibling cohorts suggested no substantial effect of family-related factors on the long-term risk of death or epilepsy after brain abscess (Figure 4). Conclusion Brain abscess is associated with an increased long-term risk of mortality and new-onset epilepsy for several years after the acute infection. Disclosures All authors: No reported disclosures.


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