Calcific Uremic Arteriolopathy in End Stage Renal Disease: Pathophysiology and Management

Overview

Journal Ochsner J

Specialty General Medicine

Date 2014 Sep 25

PMID 25249804

Citations 7

Authors

Preethi Yerram

Kunal Chaudhary

Affiliations

Soon will be listed here.

Abstract

Background: Calcific uremic arteriolopathy (CUA), a debilitating condition with high morbidity and mortality, is most commonly seen in patients with kidney disease. The pathophysiology of CUA is multifactorial, leading to a disruption in the balance between factors that promote and those that inhibit calcification, although the exact pathophysiological mechanisms of CUA remain to be elucidated.

Methods: This review provides an overview of the pathophysiology, clinical presentation and diagnosis, and treatment of CUA.

Results: Diagnosis of CUA requires a high degree of suspicion; skin biopsy with histological examination remains the gold standard to confirm the diagnosis. Treatment of CUA requires a multidisciplinary approach.

Conclusion: With a high degree of clinical suspicion and early diagnosis, an aggressive multifactorial treatment approach involving optimal wound management, minimization/avoidance of risk factors and precipitating causes, and correction of calcium-phosphorus abnormalities can significantly improve patient outcomes.

Citing Articles

Successful Management of Calciphylaxis with Sodium Thiosulfate in End-Stage Renal Disease: A Case Report.

Albekery M, Alkulaib M, Alanazi A, Alturki L, Al Sahlawi M, Abulikailik R Healthcare (Basel). 2025; 13(3).

PMID: 39942471 PMC: 11817084. DOI: 10.3390/healthcare13030282.

An Alternate Explanation.

Alsaigh T, Dhaliwal G, Fukaya E, Leeper N, Sayed N N Engl J Med. 2023; 388(14):1318-1324.

PMID: 37018496 PMC: 10409491. DOI: 10.1056/NEJMcps2210419.

Non-uraemic calciphylaxis (NUC) postliver transplantation.

Frunza-Stefan S, Poola-Kella S, Silver K BMJ Case Rep. 2018; 2018.

PMID: 30361453 PMC: 6203001. DOI: 10.1136/bcr-2018-226537.

Gastric Pneumatosis from Isolated Gastric Calciphylaxis.

Mulgund A, Razeghi S, Poreddy S ACG Case Rep J. 2017; 4:e91.

PMID: 28761894 PMC: 5519402. DOI: 10.14309/crj.2017.91.

Metabolic acidosis status and mortality in patients on the end stage of renal disease.

Raikou Md PhD V J Transl Int Med. 2017; 4(4):170-177.

PMID: 28191541 PMC: 5290893. DOI: 10.1515/jtim-2016-0036.

References

Mataic D, Bastani B . Intraperitoneal sodium thiosulfate for the treatment of calciphylaxis. Ren Fail. 2006; 28(4):361-3. DOI: 10.1080/08860220600583781. View

Coates T, Kirkland G, Dymock R, Murphy B, Brealey J, MATHEW T . Cutaneous necrosis from calcific uremic arteriolopathy. Am J Kidney Dis. 1998; 32(3):384-91. DOI: 10.1053/ajkd.1998.v32.pm9740153. View

Hayden M, Tyagi S, Kolb L, Sowers J, Khanna R . Vascular ossification-calcification in metabolic syndrome, type 2 diabetes mellitus, chronic kidney disease, and calciphylaxis-calcific uremic arteriolopathy: the emerging role of sodium thiosulfate. Cardiovasc Diabetol. 2005; 4:4. PMC: 1079905. DOI: 10.1186/1475-2840-4-4. View

Wallin R, Cain D, Hutson S, Sane D, Loeser R . Modulation of the binding of matrix Gla protein (MGP) to bone morphogenetic protein-2 (BMP-2). Thromb Haemost. 2001; 84(6):1039-44. View

Nigwekar S, Wolf M, Sterns R, Hix J . Calciphylaxis from nonuremic causes: a systematic review. Clin J Am Soc Nephrol. 2008; 3(4):1139-43. PMC: 2440281. DOI: 10.2215/CJN.00530108. View

AlBugami M, Wilson J, Clarke J, Soroka S . Oral sodium thiosulfate as maintenance therapy for calcific uremic arteriolopathy: a case series. Am J Nephrol. 2013; 37(2):104-9. DOI: 10.1159/000346410. View

Rogers N, Teubner D, Coates P . Calcific uremic arteriolopathy: advances in pathogenesis and treatment. Semin Dial. 2007; 20(2):150-7. DOI: 10.1111/j.1525-139X.2007.00263.x. View

Sewell L, Weenig R, Davis M, McEvoy M, Pittelkow M . Low-dose tissue plasminogen activator for calciphylaxis. Arch Dermatol. 2004; 140(9):1045-8. DOI: 10.1001/archderm.140.9.1045. View

Raymond C, Wazny L . Sodium thiosulfate, bisphosphonates, and cinacalcet for treatment of calciphylaxis. Am J Health Syst Pharm. 2008; 65(15):1419-29. DOI: 10.2146/ajhp070546. View

10.

Rogers N, Chang S, Teubner D, Coates P . Hyperbaric oxygen as effective adjuvant therapy in the treatmentof distal calcific uraemic arteriolopathy. NDT Plus. 2015; 1(4):244-9. PMC: 4421205. DOI: 10.1093/ndtplus/sfn036. View

11.

Strazzula L, Nigwekar S, Steele D, Tsiaras W, Sise M, Bis S . Intralesional sodium thiosulfate for the treatment of calciphylaxis. JAMA Dermatol. 2013; 149(8):946-9. DOI: 10.1001/jamadermatol.2013.4565. View

12.

Gordillo G, Sen C . Revisiting the essential role of oxygen in wound healing. Am J Surg. 2003; 186(3):259-63. DOI: 10.1016/s0002-9610(03)00211-3. View

13.

Picazo M, Bover J, de la Fuente J, Sans R, Cuxart M, Matas M . [Sterile maggots as adjuvant procedure for local treatment in a patient with proximal calciphylaxis]. Nefrologia. 2006; 25(5):559-62. View

14.

Norris B, Vaysman V, Line B . Bone scintigraphy of calciphylaxis: a syndrome of vascular calcification and skin necrosis. Clin Nucl Med. 2005; 30(11):725-7. DOI: 10.1097/01.rlu.0000182215.97219.72. View

15.

Shiraishi N, Kitamura K, Miyoshi T, Adachi M, Kohda Y, Nonoguchi H . Successful treatment of a patient with severe calcific uremic arteriolopathy (calciphylaxis) by etidronate disodium. Am J Kidney Dis. 2006; 48(1):151-4. DOI: 10.1053/j.ajkd.2006.04.062. View

16.

Tittelbach J, Graefe T, Wollina U . Painful ulcers in calciphylaxis - combined treatment with maggot therapy and oral pentoxyfillin. J Dermatolog Treat. 2002; 12(4):211-4. DOI: 10.1080/09546630152696035. View

17.

Mazhar A, Johnson R, Gillen D, Stivelman J, Ryan M, Davis C . Risk factors and mortality associated with calciphylaxis in end-stage renal disease. Kidney Int. 2001; 60(1):324-32. DOI: 10.1046/j.1523-1755.2001.00803.x. View

18.

Hayashi M, Takamatsu I, Kanno Y, Yoshida T, Abe T, Sato Y . A case-control study of calciphylaxis in Japanese end-stage renal disease patients. Nephrol Dial Transplant. 2011; 27(4):1580-4. DOI: 10.1093/ndt/gfr658. View

19.

Kiechl S, Werner P, Knoflach M, Furtner M, Willeit J, Schett G . The osteoprotegerin/RANK/RANKL system: a bone key to vascular disease. Expert Rev Cardiovasc Ther. 2006; 4(6):801-11. DOI: 10.1586/14779072.4.6.801. View

20.

Baldwin C, Farah M, Leung M, Taylor P, Werb R, Kiaii M . Multi-intervention management of calciphylaxis: a report of 7 cases. Am J Kidney Dis. 2011; 58(6):988-91. DOI: 10.1053/j.ajkd.2011.06.022. View