Calciphylaxis diagnosis, management and future directions: a comprehensive update on behalf of the European Renal Association CKD-MBD Working Group

Huish, Sharon, Moore, Sacha, Alfieri, Carlo, Bellasi, Antonio, Cejka, Daniel, de Borst, Martin H., Diaz-Tocados, Juan Miguel, Ferraro, Pietro Manuel, Fusaro, Maria, Haarhaus, Mathias, Hansen, Ditte, Kanbay, Mehmet, Ketteler, Markus and Sinha, Smeeta 2025. Calciphylaxis diagnosis, management and future directions: a comprehensive update on behalf of the European Renal Association CKD-MBD Working Group. Clinical Kidney Journal , sfaf338. 10.1093/ckj/sfaf338

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Abstract

Calciphylaxis, or calcific uraemic arteriolopathy, is a rare and life-threatening condition predominantly affecting people receiving dialysis. Characterised by painful necrotic skin lesions due to arteriolar calcification and thrombosis, calciphylaxis is associated with high morbidity and mortality. Diagnosis is frequently delayed due to misdiagnosis and an absence of specific diagnostic tests. Current treatment approaches are largely based on registry data and small uncontrolled studies. This update brings together the latest understanding of calciphylaxis pathogenesis, diagnostic approaches, and management, highlighting recent advances and future directions. Pathophysiological mechanisms include vascular smooth muscle cell osteogenic transformation, loss of endogenous calcification inhibitors (fetuin-A, matrix Gla protein, pyrophosphate), systemic inflammation, and thrombosis. The potential prognostic role of biomarkers, including the calciprotein particle crystallisation test (T50) and plasma pyrophosphate, are also discussed. Management remains complex, with no proven treatments. A multifaceted, and multi-professional team, approach is fundamental. Sodium thiosulfate remains widely used despite the lack of trial evidence. Recent investigational therapies, including SNF472 and INZ-701, target key calcification pathways and offer promise. The BEAT-Calci adaptive platform trial represents a landmark step in evaluating multiple therapies systematically. National registries remain vital for informing prevalence estimates and improving real-world outcome data. Looking ahead, future research should prioritise the development and validation of diagnostic criteria, and prognostic tools integrating clinical risk factors with biomarkers. In addition, we propose the routine inclusion of patient-reported experience measures in calciphylaxis studies to better capture treatment impact in this vulnerable population.

Item Type:	Article
Date Type:	Published Online
Status:	In Press
Schools:	Schools > Medicine
Additional Information:	License information from Publisher: LICENSE 1: URL: https://creativecommons.org/licenses/by-nc/4.0/, Start Date: 2025-11-06
Publisher:	Oxford University Press
ISSN:	2048-8505
Date of First Compliant Deposit:	25 November 2025
Last Modified:	25 Nov 2025 12:30
URI:	https://orca.cardiff.ac.uk/id/eprint/182642

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