ArticleThe Journal of clinical endocrinology and metabolism2020
Patients With APECED Have Increased Early Mortality Due to Endocrine Causes, Malignancies and infections.
Article in The Journal of clinical endocrinology and metabolism, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 28 papers.
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Who cites it
28 citing papers in PubMed.
- Addison's disease in Italy: mortality and survival by etiology.Journal of endocrinological investigation · 2026Article
- A variant in RESF1 is associated with Addison's disease and multiple autoimmune syndrome in young Nova Scotia Duck Tolling Retrievers.Scientific reports · 2026Article
- Selective JAK Inhibition Reveals Paradoxical and Hierarchical Control of interferon-γ-driven Autoimmunity in AIRE Deficiency.bioRxiv : the preprint server for biology · 2026Article
- Intravenous Glucose Tolerance Tests in Predicting Diabetes Onset in APECED: A Retrospective Cohort Study.Journal of the Endocrine Society · 2026Article
- Head and Neck Malignancies in Autoimmune Polyendocrine Syndrome Type 1 (APS-1/APECED): A Scoping Review of Molecular Pathogenesis, Clinical Features, and Outcomes.International journal of molecular sciences · 2025Article
- Lessons From Prospective Longitudinal Follow-up of a French APECED Cohort.The Journal of clinical endocrinology and metabolism · 2025Observational
- Esophageal Candida Infection and Esophageal Cancer Risk in Patients With Achalasia.JAMA network open · 2025Article
- The Role of Interferon-γ in Autoimmune Polyendocrine Syndrome Type 1.The New England journal of medicine · 2024Article
- Ex Vivo-Generated Tolerogenic Dendritic Cells: Hope for a Definitive Therapy of Autoimmune Diseases.Current issues in molecular biology · 2024Review
- Leber congenital amaurosis as the initial and essential manifestation in a Chinese patient with autoimmune polyglandular syndrome Type 1.Documenta ophthalmologica. Advances in ophthalmology · 2023Article
- Gastrointestinal manifestations in autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) patient: major effect on treatment and prognosis.Endocrinology, diabetes & metabolism case reports · 2023Article
- Unlocking life-threatening COVID-19 through two types of inborn errors of type I IFNs.The Journal of clinical investigation · 2023Review
- Notes for the general paediatrician: managing thyrotoxicosis in children and young people.BMJ paediatrics open · 2022Review
- New Approach to Addison Disease: Oral Manifestations Due to Endocrine Dysfunction and Comorbidity Burden.Diagnostics (Basel, Switzerland) · 2022Review
- Anti-CD45RC antibody immunotherapy prevents and treats experimental autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy syndrome.The Journal of clinical investigation · 2022Article
- Clinical Characteristics in the Longitudinal Follow-Up of APECED Syndrome in Southern Croatia-Case Series.Genes · 2022Article
- Druggable monogenic immune defects hidden in diverse medical specialties: Focus on overlap syndromes.World journal of clinical pediatrics · 2022Review
- Generation and Characterization of iPS Cells Derived from APECED Patients for Gene Correction.Frontiers in endocrinology · 2022Article
- Differentiation of Pluripotent Stem Cells Into Thymic Epithelial Cells and Generation of Thymic Organoids: Applications for Therapeutic Strategies Against APECED.Frontiers in immunology · 2022Review
- Autoimmune polyendocrine syndrome type 1: an Italian survey on 158 patients.Journal of endocrinological investigation · 2021Article
Corrections and comments
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Authors and funding
4 authors.
Funding
No grant is acknowledged in the PubMed record.
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. PATIENTS: Ninety-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.
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