Pain Management of patients with Calciphylaxis

Abstract

Calciphylaxis, a rare Thrombotic disorder characterized by vascular calcification and excruciating pain, poses significant challenges due to its high mortality rate (40-60%) and limited treatment options. This study aimed to assess pain management strategies for calciphylaxis patients across various clinical settings. A systematic search was conducted using databases - PubMed, Medline, and EMBASE yielding 714 initially identified articles, 351 meeting inclusion criteria after language and publication date, resulting in 28 relevant studies to pain treatment/management. Treatment modalities encompassed sodium thiosulfate, corticosteroid and lidocaine injections, opioids, dialysis, bisphosphonates, parathyroidectomy, and vitamin K supplementation. Opioids, particularly oxycodone, were commonly utilized for pain management, with paracetamol as the preferred adjunct agent. Administration of intralesional sodium thiosulfate showed promising clinical responses regarding pain relief and wound healing within two weeks. Topical cannabis and low-dose ketamine infusion exhibited notable pain alleviation, resulting in a 33% decrease in pain intensity and a reduction of 3 points on the pain scale respectively. Liposomal bupivacaine nerve blocks provided sustained pain relief for 72 hours. Despite numerous options, the absence of a standardized pain management guideline underscores the need for further research. A comprehensive framework tailored to patient needs, integrating pain grading and early palliative care consultation, is crucial for enhancing outcomes in calciphylaxis cases. This study highlights the importance of personalized approaches in managing calciphylaxis-associated pain and emphasizes the necessity of developing evidence-based guidelines to optimize patient care. Addressing pain management in calciphylaxis is not only essential for alleviating suffering but also for potentially improving overall survival rates.