Calciphylaxis
Calciphylaxis (calcifying uremic arteriolopathy, CUA) is a rare but very serious condition with a mortality rate of up to 50–80 %, with sepsis usually being the cause of death. It is characterized by painful, deep skin and subcutaneous gangrene with systemic consequences. It predominantly, though not exclusively, affects patients with advanced kidney disease or kidney failure.
Review
The presented algorithm introduces the issue of calciphylaxis to users in an engaging and easily understandable way. Calciphylaxis is a rare but potentially very serious diagnosis that often poses a life-threatening risk due to the development of fatal complications. Despite its potential severity, calciphylaxis is treatable today; however, the greatest challenge lies in the long time delay between the initial skin manifestations and the establishment of the correct diagnosis, which is essential for initiating the appropriate, i.e., specific, treatment.
The case report included in this algorithm is clearly structured. The authors correctly interpret the epidemiology, the accumulation of risk factors, and the individual diagnostic and therapeutic steps. All steps are properly and adequately justified, including the explanations for incorrect answers.
Sources
CHANG, John J. Calciphylaxis: Diagnosis, Pathogenesis, and Treatment. Advances in Skin & Wound Care [online]. 2019, vol. 32, no. 5, pp. 205–215. ISSN 1538-8654. Available from: https://doi.org/10.1097/01.ASW.0000554443.14002.13. [Accessed 23 March 2025].
DUSILOVÁ SULKOVÁ, Sylvie, ŠAFRÁNEK, Roman, POKORNÁ, Anita, SOUKUP, Tomáš, BĚLOBRÁDKOVÁ, Martina, HORÁČEK, Jiří, and PALIČKA, Vladimír. Calciphylaxis as a Multidisciplinary Problem: Important Information for (Not Only) Clinical Osteologists and Case Reports. Clinical Osteology. 2019, vol. 24, no. 4, pp. 205–215.
NIGWEKAR, Sagar U.; KROSHINSKY, Daniela; NAZARIAN, Rosalynn M.; GOVERMAN, Jeremy; MALHOTRA, Rajeev et al. Calciphylaxis: Risk Factors, Diagnosis, and Treatment. American Journal of Kidney Diseases [online]. 2015, vol. 66, no. 1, pp. 133–146. ISSN 0272-6386. Available from: https://doi.org/10.1053/j.ajkd.2015.01.034. [Accessed 23 March 2025].
HU, Lilio; NAPOLETANO, Angelodaniele; PROVENZANO, Michele; GAROFALO, Carlo; BINI, Claudia et al.Mineral Bone Disorders in Kidney Disease Patients: The Ever-Current Topic. International Journal of Molecular Sciences [online]. 2022, vol. 23, no. 20. ISSN 1422-0067. Available from: https://doi.org/10.3390/ijms232012223. [Accessed 23 March 2025].
WEN, Wen; PORTALES-CASTILLO, Ignacio; SEETHAPATHY, Rituvanthikaa; KRINSKY, Scott; KROSHINSKY, Daniela et al. Intravenous Sodium Thiosulfate for Vascular Calcification of Hemodialysis Patients—A Systematic Review and Meta-Analysis. Nephrology Dialysis Transplantation [online]. 2023, vol. 38, no. 3, pp. 733–745. ISSN 0931-0509. Available from: https://doi.org/10.1093/ndt/gfac171. [Accessed 23 March 2025].
Learning targets
2. The candidate is able to list the risk factors for the development of calciphylaxis.
3. The candidate understands the pathophysiology of calcium and phosphorus metabolism disorders in kidney disease.
4. The candidate knows the basics of the diagnostic approach.
5. The candidate has basic knowledge of the correct comprehensive therapeutic approach to treating calciphylaxis.
Key points
2. Calciphylaxis is always associated with a disorder of calcium and phosphorus metabolism; hyperparathyroidism must also be considered.
3. In the presence of hyperparathyroidism, immediate and radical parathyroidectomy is indicated.
4. Warfarin is among the risk factors. It must be discontinued immediately and should not be administered to at-risk patients.
5. The comprehensive treatment of a patient with calciphylaxis includes the administration of sodium thiosulfate.
