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Background <p>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.</p> Methods <p>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.</p> Results <p>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&#xa0;also common among cases (15,20.5%).</p> Conclusions <p>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.</p>

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

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2025年2月26日

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  • Mohammadreza Shafiei,
  • Solale Hosseini,
  • Soodeh Ghadimi,
  • Mahbubeh Mirzaee,
  • Mohammadamin Keikhah,
  • Nastaran Ardalan,
  • Masoumeh Mohkam,
  • Mehrdad Tamiji,
  • Mahnaz Jamee

摘要

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.