Abstract
ContextAutoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) is an autoimmune endocrinopathy with severe and unpredictable course. The impact of APECED on mortality has not been determined.ObjectiveTo assess overall and cause-specific mortality of patients with APECED.Design and SettingA follow-up study of Finnish patients with APECED from 1971 to 2018. Causes and dates of death were collected from Finnish registries.PatientsNinety-one patients with APECED.Main Outcome MeasureOverall and cause-specific standardized mortality ratios (SMRs) determined by comparing the observed numbers of death and those expected on the basis of respective population death rates in Finland.ResultsThe overall disease mortality was significantly increased (29 deaths, SMR 11; 95% confidence interval [CI] 7.2-16; P < 0.001). The relative risk (SMR) was highest in the youngest age groups but the absolute excess risk was similar (about 10 per 10 000 person-years) in all age categories. The highest SMRs were seen for endocrine and metabolic diseases (SMR 570; 95% CI, 270-1000; P < 0.001) and for oral and esophageal malignancies (SMR 170; 95% CI, 68-360; P < 0.001). Mortality was also increased for infections, diseases of digestive system, alcohol-related deaths, and for accidents. Due to the small number of cases we were unable to evaluate whether mortality was affected by disease severity.ConclusionsPatients with APECED have significantly increased mortality in all age groups. Highest SMRs are found for causes that are directly related to APECED but also for infections. Increased alcohol- and accident-related deaths may be influenced by psychosocial factors.
Highlights
Context: Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) is an autoimmune endocrinopathy with severe and unpredictable course
The highest Standardized mortality ratio (SMR) were seen for endocrine and metabolic diseases (SMR 570; 95% confidence intervals (CIs), 270-1000; P < 0.001) and for oral and esophageal malignancies (SMR 170; 95% CI, 68-360; P < 0.001)
Highest SMRs are found for causes that are directly related to APECED and for infections
Summary
Patients and research permits The Finnish cohort of patients with APECED formed the basis of the study [16, 17]. The cohort was initially recruited from university and central hospitals by contacting the respective endocrine and pediatric endocrine units. The cohort was later complemented by continuous recruitment of subsequently diagnosed patients from tertiary pediatric and adult endocrine centers throughout the country. The study was approved by the Research Ethics Committee of the Hospital District of Helsinki and Uusimaa. Informed written consent was obtained from all study participants or their guardians (for subjects aged < 18 years) at inclusion in the study. Data collection for clinical manifestations and causes of death
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