Patients With APECED Have Increased Early Mortality Due to Endocrine Causes, Malignancies and infections

Abstract Context Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) is an autoimmune endocrinopathy with severe and unpredictable course. The impact of APECED on mortality has not been determined. Objective To assess overall and cause-specific mortality of patients with APECED....

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Published in:The journal of clinical endocrinology and metabolism 2020-06, Vol.105 (6), p.e2207-e2213
Main Authors: Borchers, Joonatan, Pukkala, Eero, Mäkitie, Outi, Laakso, Saila
Format: Article
Language:English
Subjects:
Adolescent
Adult
Age
Aged
Autoimmune diseases
Candidiasis
Cause of Death
Child
Child, Preschool
Death
Dystrophy
Endocrine disorders
Endocrine System Diseases - complications
Esophagus
Female
Follow-Up Studies
Humans
Infant
Infant, Newborn
Infections
Infections - complications
Male
Medicin och hälsovetenskap
Metabolic disorders
Middle Aged
Mortality
Neoplasms - complications
Online Only
Polyendocrinopathies, Autoimmune - etiology
Polyendocrinopathies, Autoimmune - mortality
Polyendocrinopathies, Autoimmune - pathology
Prognosis
Prospective Studies
Survival Rate
Young Adult
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Summary:Abstract Context Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) is an autoimmune endocrinopathy with severe and unpredictable course. The impact of APECED on mortality has not been determined. Objective To assess overall and cause-specific mortality of patients with APECED. Design and Setting A follow-up study of Finnish patients with APECED from 1971 to 2018. Causes and dates of death were collected from Finnish registries. Patients Ninety-one patients with APECED. Main Outcome Measure Overall 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. Results The overall disease mortality was significantly increased (29 deaths, SMR 11; 95% confidence interval [CI] 7.2-16; P 
ISSN:0021-972X
1945-7197
1945-7197
DOI:10.1210/clinem/dgaa140