Lack of evidence does not justify neglect: how can we address unmet medical needs in calciphylaxis?

Area of Science:

• Nephrology
• Dermatology
• Vascular Medicine

Background:

• Calcific uraemic arteriolopathy (CUA), also known as calciphylaxis, is a rare and severe condition often associated with dialysis.
• The low incidence of CUA limits prospective studies, resulting in scarce evidence for effective management strategies.
• CUA presents a significant clinical challenge with a poor prognosis, necessitating improved diagnostic and therapeutic approaches.

Purpose of the Study:

• To address critical gaps in evidence regarding the diagnosis and management of dialysis-associated calciphylaxis.
• To consolidate expert opinions on controversial aspects of CUA treatment and diagnosis.
• To provide practical guidance for clinicians managing patients with calciphylaxis in the absence of evidence-based medicine.

Main Methods:

• Convened the 1st Consensus Conference on CUA, involving 13 international experts.
• Utilized a 9-point Likert scale questionnaire covering 20 unresolved issues in CUA diagnosis and management.
• Anonymously collected and analyzed expert opinions to identify areas of consensus and divergence.

Main Results:

• Identified areas of general consensus among experts, offering valuable support for less experienced physicians.
• Highlighted divergent opinions on specific diagnostic methods, such as skin biopsy, and the administration of certain treatments.
• The consensus process provided a framework for approaching complex cases of calciphylaxis.

Conclusions:

• The consensus report offers preliminary guidance for managing calcific uraemic arteriolopathy.
• Further research is needed to resolve controversial aspects of CUA diagnosis and treatment.
• Collaboration and shared expertise are crucial for improving outcomes in patients with this rare disease.