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Patients With APECED Have Increased Early Mortality Due to Endocrine Causes, Malignancies and infections
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...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Oxford University Press
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7150614/ https://www.ncbi.nlm.nih.gov/pubmed/32185376 http://dx.doi.org/10.1210/clinem/dgaa140 |
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author | Borchers, Joonatan Pukkala, Eero Mäkitie, Outi Laakso, Saila |
author_facet | Borchers, Joonatan Pukkala, Eero Mäkitie, Outi Laakso, Saila |
author_sort | Borchers, Joonatan |
collection | PubMed |
description | 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 < 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. CONCLUSIONS: Patients 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. |
format | Online Article Text |
id | pubmed-7150614 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | Oxford University Press |
record_format | MEDLINE/PubMed |
spelling | pubmed-71506142020-04-15 Patients With APECED Have Increased Early Mortality Due to Endocrine Causes, Malignancies and infections Borchers, Joonatan Pukkala, Eero Mäkitie, Outi Laakso, Saila J Clin Endocrinol Metab Online Only Articles 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 < 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. CONCLUSIONS: Patients 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. Oxford University Press 2020-03-18 /pmc/articles/PMC7150614/ /pubmed/32185376 http://dx.doi.org/10.1210/clinem/dgaa140 Text en © Endocrine Society 2020. http://creativecommons.org/licenses/by/4.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted reuse, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Online Only Articles Borchers, Joonatan Pukkala, Eero Mäkitie, Outi Laakso, Saila Patients With APECED Have Increased Early Mortality Due to Endocrine Causes, Malignancies and infections |
title | Patients With APECED Have Increased Early Mortality Due to Endocrine Causes, Malignancies and infections |
title_full | Patients With APECED Have Increased Early Mortality Due to Endocrine Causes, Malignancies and infections |
title_fullStr | Patients With APECED Have Increased Early Mortality Due to Endocrine Causes, Malignancies and infections |
title_full_unstemmed | Patients With APECED Have Increased Early Mortality Due to Endocrine Causes, Malignancies and infections |
title_short | Patients With APECED Have Increased Early Mortality Due to Endocrine Causes, Malignancies and infections |
title_sort | patients with apeced have increased early mortality due to endocrine causes, malignancies and infections |
topic | Online Only Articles |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7150614/ https://www.ncbi.nlm.nih.gov/pubmed/32185376 http://dx.doi.org/10.1210/clinem/dgaa140 |
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