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

Area of Science:

• Immunology
• Nephrology
• Genetics

Background:

• Autoimmune polyendocrinopathy candidiasis ectodermal dystrophy (APECED), or autoimmune polyendocrine syndrome type I (APS-I), is an inborn error of immunity.
• It presents with immune dysregulation, endocrine issues, and non-endocrine manifestations like chronic mucocutaneous candidiasis.
• Renal disorders are a significant, under-investigated comorbidity in APECED patients.

Purpose of the Study:

• To systematically review the literature on renal complications in APECED patients.
• To identify the prevalence and types of kidney disorders associated with APECED.
• To highlight the importance of renal monitoring in APECED management.

Main Methods:

• A comprehensive literature search was performed across PubMed, Web of Science, and Scopus.
• Articles were systematically reviewed for clinical and immunological features of APECED.
• Studies including APECED patients with at least one nephrological complication were selected.

Main Results:

• Ninety-three APECED patients from 30 studies were analyzed.
• Nephrocalcinosis was present in over 38% of patients.
• Tubulointerstitial nephritis (23%), hypertension (13%), and urinary tract infections (20.5%) were also common renal complications.

Conclusions:

• Renal complications are a significant concern in APECED patients.
• Regular monitoring of kidney function is essential for managing APECED.
• Addressing renal issues can improve patient outcomes and preserve renal function.