From despair to results: Clinical experience and effective strategies in the treatment of calciphylaxis
Authors:
Krošlák Oliver 1; Sulková Dusilová Sylvie 1; Šafránek Roman 1; Halouzka Tomáš 1; Pokorná Anita 1; Chrobok Viktor 2; Horáček Jiří 3; Pavlíková Ladislava 4; Palička Vladimír 4
Authors‘ workplace:
Nefrologická klinika, FN Hradec Králové a LF UK v Hradci Králové
1; Klinika otorinolaryngologie a chirurgie hlavy a krku, FN Hradec Králové a LF UK v Hradci Králové
2; IV. interní hematologická klinika, FN Hradec Králové a LF UK v Hradci Králové
3; Ústav klinické biochemie a diagnostiky, FN Hradec Králové a LF UK v Hradci Králové
4
Published in:
Clinical Osteology 2025; 30(1): 14-20
Category:
Overview
Calciphylaxis (CUA) represents a rare but serious complication in patients with chronic kidney disease and less frequently in other conditions. The aim of our study is to assess our experiences with the diagnosis and treatment of CUA. We present the clinical characteristics, the prevalence of key risk factors, and the favorable prognosis of patients with calciphylaxis treated at our institution between 2014 and 2021. The cohort consisted of 23 patients who were diagnosed with calciphylaxis based on clinical presentation. In most cases, the lesions were multiple, painful, and led to significant mobility limitations. Important risk factors included the presence of diabetes mellitus (43 %), obesity (61 %), and the use of warfarin (48 %). The early mortality rate in the cohort was less than 5 %, one-year mortality was 15 %. Our results emphasize the importance of early diagnosis and individualized multidisciplinary therapy for this life-threatening condition.
Keywords:
calciphylaxis, mortality, warfarin, sodium thiosulphate, hyperparatyroidism, paratyreoi-dectomy
Sources
1. Gallo Marin B, Aghagoli G, Hu SL, et al. Calciphylaxis and Kidney Disease: A Review. Am J Kidney Dis. 2023;81(2):232-239.
2. Brandenburg VM, Evenepoel P, Floege J, et al. ERA-EDTA Working Group on CKD-MBD and EUCALNET. Lack of evidence does not justify neglect: how can we address unmet medical needs in calciphylaxis? Nephrol Dial Transplant. 2016;31(8):1211-1219.
3. García-Lozano JA, Ocampo-Candiani J, Martínez-Cabriales SA, et al. An Update on Calciphylaxis. Am J Clin Dermatol. 2018;19:599-608.
4. Nigwekar SU, Thadhani R, Brandenburg VM. Calciphylaxis. N Engl J Med 2018; 378(18):1704-1714.
5. Portales-Castillo I, Kroshinsky D, Malhotra CK, et al. Calciphylaxis-as a drug induced adverse event. Expert Opin Drug Saf. 2019;18(1):29-35.
6. Nigwekar SH, Zhao S, Wenger J, et al. A nationally representative study of calcific uremic arteriolopathy risk factors. J Am Soc Nephrol. 2016;27(11):3421-3429.
7. Nigwekar SU. Multidisciplinary approach to calcific uremic arteriolopathy. Curr Opin Nephrol Hypertens. 2015;24(6):531-537.
8. Soloway AM, Arkebauer MR, Soloway S. Nonuremic Calciphylaxis. J Clin Rheumatol. 2020;26(4):e83-e84.
9. Randall DP, Fisher MA, Thomas C. Rhabdomyolysis as the presenting manifestation of calciphylaxis. Muscle Nerve. 2000;23(2):289-293.
10. Dusilová Sulková S, Pokorná A, Horáček J, et al. Kalcifylaxe: klinický obraz, systémová léčba a hojení ran. Hojení ran.
11. Chen TY, Lehman LJ, Gibson JE, et al. Histopathology of Calciphylaxis: Cohort Study With Clinical Correlations. Am J Dermatopathol. 2017;39(11):795-802.
12. Ellis CL, O’Neill WC. Questionable specificity of histologic findings in calcific uremic arteriolopathy. Kidney Int. 2018;94(2):390-395.
13. Williams EA, Moy AP, Cipriani NA, et al. Factors associated with false negative pathologic diagnosis of calciphylaxis. J Cutan Pathol. 2019;46(1):16-25.
14. Ruderman I, Toussaint ND, Hawley CM, et al. The Australian Calciphylaxis Registry: reporting clinical features and outcomes of patients with calciphylaxis. Nephrol Dial Transplant. 2021;36(4):649-656.
15. Voelkl J, Lang F, Eckardt KU, et al. Signaling pathways involved in vascular smooth muscle cell calcification during hyperphosphatemia. Cell Mol Life Sci. 2019;76(11):2077-2091.
16. Dusilová Sulková S, Horáček J, Vykoukalová E, et al. Parathyroidectomy in hyperparathyroidism Associated Calciphylaxis in End-Stage Renal Disease Should be Prompt and Radical a Case Report with Two Original Therapeutic Modifications and Successful Outcome. Acta Medica (Hradec Kralove). 2017;60(2):85-88.
17. Drüeke TB, Massy ZA. Role of vitamin D in vascular calcification: bad guy or good guy? Nephrol Dial Transplant. 2012;27(5):1704-1707.
18. Razzaque MS. The dualistic role of vitamin D in vascular calcifications. Kidney Int. 2011; 79(7):708-714.
19. Mao M, Lee S, Kashani K, et al. Severe anion gap acidosis associated with intravenous sodium thiosulfate administration. J Med Toxi col. 2013;9(3):274-247.
20. Wen W, Portales-Castillo I, Seethapathy R, et al. Intravenous sodium thiosulphate for vascular calcification of hemodialysis patients-a systematic review and meta-analysis. Nephrol Dial Transplant. 2023;38(3):733-745.
21. Peng T, Zhuo L, Wang Y, et al. Systematic review of sodium thiosulfate in treating calciphylaxis in chronic kidney disease patients. Nephrology (Carlton). 2018;23(7):669-675.
22. Vyskočilová M, Svojanovský J, Blaštíková J, Dvořáková G, Souček M. Kalcifikující uremická arteriolopatie léčba tiosulfátem sodným [Calcific uremic arteriolopathy treatment with sodium thiosulfate]. Vnitr Lek. 2015;61(2):166-172.
23. Shanahan CM. Vascular calcification. Curr Opin Nephrol Hypertens. 2005;14(4):361-367.
24. Siracusa C, Carabetta N, Morano MB, et al. Understanding Vascular Calcification in Chronic Kidney Disease: Pathogenesis and Therapeutic Implications. Int J Mol Sci. 2024;25(23):13096.
25. Garza-Mayers AC, Shah R, Sykes DB, et al. The Successful Use of Apixaban in Dialysis Patients with Calciphylaxis Who Require Anticoa gulation: A Retrospective Analysis. Am J Nephrol. 2018;48(3):168-171.
26. Moe SM, Drüeke T, Lameire N, Eknoyan G. Chronic kidney disease-mineral-bone disorder: a new paradigm. Adv Chronic Kidney Dis. 2007;14(1):3-12.
27. Dusilová Sulková S, Šafránek R, Pokorná A: Kalcifylaxe jako multioborový problém: důležité informace (nejen) pro klinického osteologa a kasuistiky. Clin Osteol. 2019;24(4):205-215
28. Cozzolino M, Bernard L, Csomor PA. Active vitamin D increases the risk of hypercalcaemia in non-dialysis chronic kidney disease patients with secondary hyperparathyroidism: a systematic review and meta-analysis. Clin Kidney J. 2021;14(11):2437-2443.
29. KDIGO 2017 Clinical Practice Guideline update for the diagnosis, evaluation, prevention, and treatment of chronic kidney disease-mineral and bone disorder (CKD-MBD). Kidney Int Suppl. 2017;7:1-59.
30. Asobie N, Wong E, Cook M. Calciphylaxis in a Diabetic Patient Provoked by Warfarin Therapy. Clin Exp Dermatol. 2008;33(3):342-344.
31. Hasegava H. Clinical Assessment of Warfarin Therapy in Patients with Maintenance Dialysis Clinical Efficacy, Risks and Development of Calciphylaxis. Ann Vasc Dis. 2017;10(3):170-177.
32. Kazanji N, Falatko J, Neupane S, et al. Calciphylaxis presenting as digital ischemia. Intern Emerg Med. 2015;10(4):529-530.
33. Nigwekar SU, Sprague SM. We Do Too Many Parathyroidectomies for Calciphylaxis. Semin Dial. 2016;29(4):312-314.
34. Mao M, Lee S, Kashani K, et al. Severe anion gap acidosis associated with intravenous sodium thiosulfate administration. J Med Toxicol. 2013;9(3):274-277.
Labels
Clinical biochemistry Paediatric gynaecology Paediatric radiology Paediatric rheumatology Endocrinology Gynaecology and obstetrics Internal medicine Orthopaedics General practitioner for adults Radiodiagnostics Rehabilitation Rheumatology Traumatology OsteologyArticle was published in
Clinical Osteology
2025 Issue 1
Most read in this issue
- From despair to results: Clinical experience and effective strategies in the treatment of calciphylaxis
- Vitamin D – current overview of the issue and specifics in nephrological practice
- A completely now approach to the issue of bone disease in patients with chronic kidney disease – what KDIGO practice recommendations can we expect? Commentary on the KDIGO 2025 Controversies Conference.
- Několik slov úvodem