{"doi":"10.1210/clinem/dgab705","title":"Pregnancy Outcome in Women With APECED (APS-1): A Multicenter Study on 43 Females With 83 Pregnancies","abstract":"CONTEXT: Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED; also known as autoimmune polyendocrine syndrome type 1) has a severe, unpredictable course. Autoimmunity and disease components may affect fertility and predispose to maternal and fetal complications, but pregnancy outcomes remain unknown. OBJECTIVE: To assess fetal and maternal outcomes and course of clinical APECED manifestations during pregnancy in women with APECED. DESIGN AND SETTING: A multicenter registry-based study including 5 national patient cohorts. PATIENTS: 321 females with APECED. MAIN OUTCOME MEASURE: Number of pregnancies, miscarriages, and deliveries. RESULTS: Forty-three patients had altogether 83 pregnancies at median age of 27 years (range, 17-39). Sixty (72%) pregnancies led to a delivery, including 2 stillbirths (2.4%) and 5 (6.0%) preterm livebirths. Miscarriages, induced abortions, and ectopic pregnancies were observed in 14 (17%), 8 (10%), and 1 (1.2%) pregnancies, respectively. Ovum donation resulted in 5 (6.0%) pregnancies. High maternal age, premature ovarian insufficiency, primary adrenal insufficiency, or hypoparathyroidism did not associate with miscarriages. Women with livebirth had, on average, 4 APECED manifestations (range 0-10); 78% had hypoparathyroidism, and 36% had primary adrenal insufficiency. APECED manifestations remained mostly stable during pregnancy, but in 1 case, development of primary adrenal insufficiency led to adrenal crisis and stillbirth. Birth weights were normal in >80% and apart from 1 neonatal death of a preterm baby, no serious perinatal complications occurred. CONCLUSIONS: Outcome of pregnancy in women with APECED was generally favorable. However, APECED warrants careful maternal multidisciplinary follow-up from preconceptual care until puerperium.","journal":"The Journal of Clinical Endocrinology & Metabolism","year":2021,"id":181529,"datarank":0.0,"base_score":0.0,"endowment":0.0,"self_citation_contribution":0.0,"citation_network_contribution":0.0,"self_endowment_contribution":0.0,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":16,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":0.9563,"is_data_producer":false,"deposit_databanks":null,"is_oa":true,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":"2021-01-01","fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":732474,"name":"Elina Holopainen","orcid":"0000-0003-3572-1337","position":1,"is_corresponding":false},{"id":732475,"name":"Corrado Betterle","orcid":"0000-0002-8996-410X","position":2,"is_corresponding":false},{"id":732476,"name":"Viivi Saari","orcid":"0000-0003-1784-9193","position":3,"is_corresponding":false},{"id":732477,"name":"Elinor Vogt","orcid":"0000-0001-9810-987X","position":4,"is_corresponding":false},{"id":390349,"name":"Monica M. Schmitt","orcid":"0000-0002-0726-5170","position":5,"is_corresponding":false},{"id":298528,"name":"Karen K. Winer","orcid":"0000-0001-8261-5188","position":6,"is_corresponding":false},{"id":618890,"name":"Maria Kareva","orcid":"0000-0003-1320-6561","position":7,"is_corresponding":false},{"id":732478,"name":"Chiara Sabbadin","orcid":"0000-0003-1526-6832","position":8,"is_corresponding":false},{"id":104202,"name":"Eystein S. Husebye","orcid":"0000-0002-7886-2976","position":9,"is_corresponding":false},{"id":618887,"name":"Elizaveta Orlova","orcid":"0000-0001-6196-5322","position":10,"is_corresponding":false},{"id":104225,"name":"Michail S. Lionakis","orcid":"0000-0003-4994-9500","position":11,"is_corresponding":false},{"id":449956,"name":"Outi Mäkitie","orcid":"0000-0002-4547-001X","position":12,"is_corresponding":false},{"id":732473,"name":"Saila Laakso","orcid":"0000-0002-5588-7855","position":0,"is_corresponding":true}],"reference_count":35,"raw_metadata":null,"created_at":"2026-07-18T23:48:05.146883Z","pmid":"34570215","pmcid":null,"fwci":null,"citation_percentile":null,"influential_citations":0,"oa_status":null,"license":null,"views":0,"total_file_size_bytes":0,"version_count":0,"fair_f":null,"fair_a":null,"fair_i":null,"fair_r":null,"fair_zscore":null,"fair_rationale":null,"fair_model":null,"fair_agent_version":null,"fair_fulltext_source":null,"fair_has_llm":null,"fair_computed_at":null,"clinical_trials":[],"software_tools":[],"db_accessions":[],"linked_datasets":[],"topics":[]}