皮肤卟啉病(PCT)是一种由肝酶尿卟啉原脱羧酶缺陷引起的皮肤疾病,与丙型肝炎病毒感染有关,高酒精摄入量,吸烟和铁过载。缺乏PCT长期发病率的数据。
我们在24年的时间里进行了一项全国性的配对队列研究。研究样本包括534名年龄在18-67岁之间并经生化确认的PCT诊断的人,以及从工作年龄人群中随机选择的21,360人样本,年龄相匹配,性和教育程度。我们调查了散发性和家族性PCT患者长期病假(LTSL)或伤残抚恤金的风险是否增加。我们在(PCT之前)之前进一步评估了风险,在(PCT期间)和(PCT后)首次发病至诊断的典型时期期间,治疗和缓解。
总的来说,PCT患者LTSL风险增加40%(风险比[HR]=1.4,95%置信区间[CI]=1.3,1.5),残疾抚恤金风险增加50%(HR=1.5,CI=1.3,1.7).残疾抚恤金的风险在PCT之前增加(HR=1.3,CI1.3(1.0,1.6),PCT期间(HR1.5,CI1.0,2.2)和PCT后(HR=2.0,CI1.5,2.6)。对于LTSL,PCT前(HR=1.3,CI1.1,1.4)和PCT期间(HR=1.5,CI1.1,2.1)的风险增加,但不是PCT后。偶发性PCT患者的风险最大。与匹配的对照组相比,残疾抚恤金增加的诊断原因是PCT或199例中的11例皮肤病(PCT:n=7,发生率[IRR]=49.2,CI=38.8,62.4;皮肤和皮下组织疾病,n=4,IRR=4.2,CI=1.6,11.0)。获得残疾养恤金的绝大多数诊断原因与合并症有关,PCT易感性因素和更一般的健康问题,例如:恶性肿瘤(n=12,IRR=2.4,CI=1.4,4.2),物质和酒精依赖(n=7,IRR=5.0,CI=2.5,10.1),神经症和情绪障碍(n=21,IRR=1.7,CI=1.1,2.6),以及肌肉骨骼系统和结缔组织疾病(n=71,IRR=2.5,CI=1.9,3.2)。
PCT患者患LTSL和残疾养老金的风险增加,表明该患者组发病率显著。建议对复发和共病进行适当的长期随访和监测。
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.
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.
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).
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.