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. 2025 Feb 26;25(1):139.
doi: 10.1186/s12887-025-05458-2.

Renal disorders in Autoimmune Polyendocrinopathy Candidiasis Ectodermal dystrophy (APECED): a systematic review

Mohammadreza Shafiei  1 , Solale Hosseini  2 , Soodeh Ghadimi  3 , Mahbubeh Mirzaee  4 , Mohammadamin Keikhah  2 , Nastaran Ardalan  2 , Masoumeh Mohkam  4 , Mehrdad Tamiji  5 , Mahnaz Jamee  4
Affiliations

Renal disorders in Autoimmune Polyendocrinopathy Candidiasis Ectodermal dystrophy (APECED): a systematic review

Mohammadreza Shafiei et al. BMC Pediatr. .

Abstract

Background: Autoimmune polyendocrinopathy candidiasis ectodermal dystrophy (APECED), also known as autoimmune polyendocrine syndrome type I (APS-I) is an inborn error of immunity (IEI) with an immune dysregulation phenotype, mainly characterized by endocrine and non-endocrine manifestations including adrenal insufficiency, chronic mucocutaneous candidiasis, and ectodermal dystrophy. Renal disorders seem to be a significant morbidity in APECED patients, requiring further investigations.

Methods: The literature search was conducted in PubMed, Web of Science, and Scopus databases using relevant keywords, and included articles were systematically reviewed regarding the clinical and immunological features. APECED patients with at least one nephrological complication were included.

Results: Ninety-three APECED patients from 30 studies were identified. More than half of the patients (38,52%) presented nephrocalcinosis. The second and third most prevalent renal complications were tubulointerstitial nephritis (TIN) (23,31%), and hypertension (13,18%), respectively. Other less frequent renal disorders including renal tubular acidosis (RTA) glomerulonephritis were also reported among patients. Additionally urinary tract infections (UTI), were also common among cases (15,20.5%).

Conclusions: Renal complications in APECED represent a significant issue that should be monitored and considered in managing these patients to preserve renal function and improve patients' outcomes.

Keywords: AIRE; APECED; Autoimmune polyendocrinopathy; Inborn error of immunity; Nephrocalcinosis; Primary immunodeficiency.

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Conflict of interest statement

Declarations. Ethics approval and consent to participate: Not applicable. Consent for publication: Not applicable. Competing interests: The authors declare no competing interests.

Figures

Fig. 1
Fig. 1
PRISMA diagram summarizing the selection of eligible studies based on the PRISMA guideline
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