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. 2022 Feb 22;17(1):72.
doi: 10.1186/s13023-022-02201-3.

Porphyria cutanea tarda and patterns of long-term sick leave and disability pension: a 24-year nationwide matched-cohort study

Carl Michael Baravelli  1   2   3 , Aasne Karine Aarsand  4   5 , Sverre Sandberg  4   6   5 , Mette Christophersen Tollånes  5   7
Affiliations

Porphyria cutanea tarda and patterns of long-term sick leave and disability pension: a 24-year nationwide matched-cohort study

Carl Michael Baravelli et al. Orphanet J Rare Dis. .

Erratum in

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 and 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 and 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.

Keywords: Disability pension; Long-term sick leave; Porphyria cutanea tarda; Sick leave absences.

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Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
Overview of eligibility criteria and study sample
Fig. 2
Fig. 2
Kaplan–Meier estimates and subgroup analyses of long-term sick leave (LTSL) and disability pension
See this image and copyright information in PMC

References

    1. Puy H, Gouya L, Deybach JC. Porphyrias. Lancet. 2010;375(9718):924–937. doi: 10.1016/S0140-6736(09)61925-5. - DOI - PubMed
    1. Fontanellas A, Martinez-Fresno M, Garrido-Astray MC, Perucho T, Moran-Jimenez MJ, Garcia-Bravo M, et al. Smoking but not homozygosity for CYP1A2 g-163A allelic variant leads to earlier disease onset in patients with sporadic porphyria cutanea tarda. Exp Dermatol. 2010;19(8):e326–e328. doi: 10.1111/j.1600-0625.2009.01040.x. - DOI - PubMed
    1. Aarsand AK, Boman H, Sandberg S. Familial and sporadic porphyria cutanea tarda: characterization and diagnostic strategies. Clin Chem. 2009;55(4):795–803. doi: 10.1373/clinchem.2008.117432. - DOI - PubMed
    1. Elder G. Porphyria: genetics—encyclopedia of life sciences (ELS) Chichester: Wiley; 2010.
    1. Elder GH. Alcohol intake and porphyria cutanea tarda. Clin Dermatol. 1999;17(4):431–436. doi: 10.1016/S0738-081X(99)00029-2. - DOI - PubMed

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