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

IF 2 3区医学 Q2 PEDIATRICS BMC Pediatrics Pub Date : 2025-02-26 DOI:10.1186/s12887-025-05458-2

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

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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.

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自身免疫性多内分泌病念珠菌病外胚层营养不良（APECED）肾病：系统综述。

背景：自身免疫性多内分泌病变念珠菌病外胚层营养不良（APECED），又称自身免疫性多内分泌综合征I型（APS-I），是一种先天性免疫错误（IEI），具有免疫失调表型，主要以肾上腺功能不全、慢性粘膜皮肤念珠菌病、外胚层营养不良等内分泌和非内分泌表现为特征。肾脏疾病似乎是APECED患者的重要发病率，需要进一步的调查。方法：采用相关关键词在PubMed、Web of Science和Scopus数据库中进行文献检索，对纳入的文献进行临床和免疫学特征的系统综述。纳入了至少有一种肾病并发症的APECED患者。结果：从30项研究中确定了93例APECED患者。超过一半的患者（38.52%）表现为肾钙质沉着症。第二和第三常见的肾脏并发症分别是小管间质性肾炎（TIN）（23.31%）和高血压（13.18%）。其他不常见的肾脏疾病包括肾小管酸中毒（RTA）肾小球肾炎也有报道。此外，尿路感染（UTI）也很常见（15.20.5%）。结论：APECED患者的肾脏并发症是一个重要的问题，在管理这些患者以保持肾功能和改善患者预后时应予以监测和考虑。

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来源期刊

BMC Pediatrics PEDIATRICS-

CiteScore

3.70

自引率

4.20%

发文量

683

审稿时长

3-8 weeks

期刊介绍： BMC Pediatrics is an open access journal publishing peer-reviewed research articles in all aspects of health care in neonates, children and adolescents, as well as related molecular genetics, pathophysiology, and epidemiology.