Calciphylaxis diagnosis, management and future directions: a comprehensive update on behalf of the European Renal

Sharon Huish1,2, Sacha Moore3,4, Carlo Alfieri5,6

  • 1Department of Renal Dietetics, Royal Devon University Healthcare NHS Foundation Trust, and University of Exeter, Exeter, UK.

Clinical Kidney Journal
|December 10, 2025
PubMed

Insights

Calciphylaxis (calcific uraemic arteriolopathy) is a severe condition causing painful skin lesions in dialysis patients. Recent research explores its causes, diagnostic biomarkers, and promising new treatments, emphasizing a multidisciplinary approach.

Area of Science:

  • Nephrology
  • Dermatology
  • Vascular Biology

Background:

  • Calciphylaxis, or calcific uraemic arteriolopathy, is a rare, life-threatening condition primarily affecting dialysis patients.
  • It presents as painful, necrotic skin lesions resulting from arteriolar calcification and thrombosis, leading to high morbidity and mortality.
  • Diagnosis is often delayed due to misdiagnosis and a lack of specific diagnostic tests.

Purpose of the Study:

  • To provide an update on the latest understanding of calciphylaxis pathogenesis, diagnostic approaches, and management strategies.
  • To highlight recent advances and future research directions in the field.
  • To emphasize the need for systematic evaluation of novel therapies and improved diagnostic tools.

Main Methods:

  • Review of current literature and registry data on calciphylaxis pathogenesis, diagnosis, and treatment.
  • Discussion of emerging biomarkers such as the calciprotein particle crystallization test (T50) and plasma pyrophosphate.
  • Highlighting the Better Evidence and Translation for Calciphylaxis (BEAT-Calci) adaptive platform trial for systematic therapy evaluation.

Main Results:

  • Pathophysiological mechanisms involve vascular smooth muscle cell osteogenic transformation, loss of calcification inhibitors, inflammation, and thrombosis.
  • Sodium thiosulfate is commonly used, but evidence is limited; investigational therapies like SNF472 and INZ-701 show promise.
  • National registries and adaptive trials like BEAT-Calci are crucial for data collection and therapy evaluation.

Conclusions:

  • Management of calciphylaxis is complex, requiring a multifaceted, multidisciplinary team approach.
  • Future research should focus on validating diagnostic criteria, prognostic tools, and patient-reported outcomes.
  • Developing and systematically evaluating new therapies is essential for improving outcomes in this vulnerable patient population.

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