open access

Vol 5, No 1 (2019)
Prace poglądowe
Published online: 2019-04-04
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Calciphylaxis — pathogenesis and clinical picture

Beata Dubiel-Braszczok, Magdalena Włoch- targońska, Przemysław Jacek Kotyla
DOI: 10.5603/FR.2019.0003
·
Forum Reumatologiczne 2019;5(1):14-20.

open access

Vol 5, No 1 (2019)
Prace poglądowe
Published online: 2019-04-04

Abstract

Calciphylaxis, also known as calcific uremic arteriolopathy (CUA), is a rare vascular disease caused by deposition of calcium salts in vessel walls, accompanied by coagulation disorders, which leads to extensive skin ulceration and soft tissue necrosis. Typical cases of calciphylaxis are usually described in patients with renal failure, though it is currently known that approx. 1/10th of all cases stem from other causes, including systemic inflammation, metabolic disorders and endocrine disorders. This article shows an overview of the most important pathophysiological phenomena which accompany calciphylaxis, characterizes the clinical picture of the disease and presents available approaches to therapeutic management.


Forum Reumatol. 2019, tom 5, nr 1: 14–20

Abstract

Calciphylaxis, also known as calcific uremic arteriolopathy (CUA), is a rare vascular disease caused by deposition of calcium salts in vessel walls, accompanied by coagulation disorders, which leads to extensive skin ulceration and soft tissue necrosis. Typical cases of calciphylaxis are usually described in patients with renal failure, though it is currently known that approx. 1/10th of all cases stem from other causes, including systemic inflammation, metabolic disorders and endocrine disorders. This article shows an overview of the most important pathophysiological phenomena which accompany calciphylaxis, characterizes the clinical picture of the disease and presents available approaches to therapeutic management.


Forum Reumatol. 2019, tom 5, nr 1: 14–20

Get Citation

Keywords

calciphylaxis; vasculitis; end-stage renal disease

About this article
Title

Calciphylaxis — pathogenesis and clinical picture

Journal

Forum Reumatologiczne

Issue

Vol 5, No 1 (2019)

Pages

14-20

Published online

2019-04-04

DOI

10.5603/FR.2019.0003

Bibliographic record

Forum Reumatologiczne 2019;5(1):14-20.

Keywords

calciphylaxis
vasculitis
end-stage renal disease

Authors

Beata Dubiel-Braszczok
Magdalena Włoch- targońska
Przemysław Jacek Kotyla

References (31)
  1. Selye H, Gentile G, Prioreschi P. Cutaneous Molt Induced by Calciphylaxis in the Rat. Science. 1961; 134(3493): 1876–1877.
  2. Rees JK, Coles GA. Calciphylaxis in man. Br Med J. 1969; 2(5658): 670–672.
  3. Wilmer WA, Magro CM. Calciphylaxis: emerging concepts in prevention, diagnosis, and treatment. Semin Dial. 2002; 15(3): 172–186.
  4. Fernández M, Morales E, Gutierrez E, et al. Calciphylaxis: Beyond CKD-MBD. Nefrologia. 2017; 37(5): 501–507.
  5. Lee JL, Naguwa SM, Cheema G, et al. Recognizing calcific uremic arteriolopathy in autoimmune disease: an emerging mimicker of vasculitis. Autoimmun Rev. 2008; 7(8): 638–643.
  6. Daudén E, Oñate MJ. Calciphylaxis. Dermatol Clin. 2008; 26(4): 557–68, ix.
  7. Nigwekar SU, Kroshinsky D, Nazarian RM, et al. Calciphylaxis: risk factors, diagnosis, and treatment. Am J Kidney Dis. 2015; 66(1): 133–146.
  8. Nigwekar SU, Wolf M, Sterns RH, et al. Calciphylaxis from nonuremic causes: a systematic review. Clin J Am Soc Nephrol. 2008; 3(4): 1139–1143.
  9. Ghosh T, Winchester DS, Davis MDP, et al. Early clinical presentations and progression of calciphylaxis. Int J Dermatol. 2017; 56(8): 856–861.
  10. Kossard S, Winkelmann RK. Vascular calcification in dermatopathology. Am J Dermatopathol. 1979; 1(1): 27–34.
  11. Fischer AH, Morris DJ. Pathogenesis of calciphylaxis: study of three cases with literature review. Hum Pathol. 1995; 26(10): 1055–1064.
  12. Nigwekar SU. Calciphylaxis. Curr Opin Nephrol Hypertens. 2017; 26(4): 276–281.
  13. Cassius C, Moguelet P, Monfort JB, et al. Calciphylaxis in haemodialysed patients: diagnostic value of calcifications in cutaneous biopsy. Br J Dermatol. 2018; 178(1): 292–293.
  14. Nigwekar SU, 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.
  15. Brandenburg VM, Kramann R, Rothe H, et al. Calcific uraemic arteriolopathy (calciphylaxis): data from a large nationwide registry. Nephrol Dial Transplant. 2017; 32(1): 126–132.
  16. Fernández E. [Uremic calcifying ateriolopathy (calciphylaxis) with metabolic syndrome and diabetes mellitus. The current perspective]. Nefrologia. 2008; 28(1): 20–24.
  17. Dhanjal TS, Babu SB, Beevers G, et al. Calciphylaxis associated with widespread pulmonary calcification. BMJ Case Rep. 2009; 2009.
  18. Dominguez AR, Goldman SE. Nonuremic calciphylaxis in a patient with rheumatoid arthritis and osteoporosis treated with teriparatide. J Am Acad Dermatol. 2014; 70(2): e41–e42.
  19. Fink NR, Pedersen FK, Gyhrs AV. [Symptoms of severe calciphylaxis in a girl with X-linked hypophosphataemia]. Ugeskr Laeger. 2014; 176(21).
  20. Hayashi M. Calciphylaxis: diagnosis and clinical features. Clin Exp Nephrol. 2013; 17(4): 498–503.
  21. Yalin AS, Altiparmak MR, Trabulus S, et al. Calciphylaxis: a report of six cases and review of literature. Ren Fail. 2013; 35(1): 163–169.
  22. Marshall BJ, Johnson RE. Case Report on Calciphylaxis: An Early Diagnosis and Treatment May Improve Outcome. J Am Coll Clin Wound Spec. 2012; 4(3): 67–70.
  23. Burnie R, Smail S, Javaid MM. Calciphylaxis and sodium thiosulphate: a glimmer of hope in desperate situation. J Ren Care. 2013; 39(2): 71–76.
  24. Cai MMX, Smith ER, Brumby C, et al. Fetuin-A-containing calciprotein particle levels can be reduced by dialysis, sodium thiosulphate and plasma exchange. Potential therapeutic implications for calciphylaxis? Nephrology (Carlton). 2013; 18(11): 724–727.
  25. Ketteler M, Brandenburg VM. K-alcification Protection in Dialysis Patients: The Underestimated Phenomenon of Vitamin K Deficiency. J Am Soc Nephrol. 2017; 28(6): 1667–1668.
  26. Bechara FG, Altmeyer P, Kreuter A. Should we perform surgical debridement in calciphylaxis? Dermatol Surg. 2009; 35(3): 554–555.
  27. An J, Devaney B, Ooi KY, et al. Hyperbaric oxygen in the treatment of calciphylaxis: A case series and literature review. Nephrology (Carlton). 2015; 20(7): 444–450.
  28. Ackermann F, Levy A, Daugas E, et al. Sodium thiosulfate as first-line treatment for calciphylaxis. Arch Dermatol. 2007; 143(10): 1336–7; author reply 1338.
  29. Aihara S, Yamada S, Uchida Y, et al. The Successful Treatment of Calciphylaxis with Sodium Thiosulfate and Hyperbaric Oxygen in a Non-dialyzed Patient with Chronic Kidney Disease. Intern Med. 2016; 55(14): 1899–1905.
  30. Arenas MD, Gil MT, Gutiérrez MD, et al. Management of calcific uremic arteriolopathy (calciphylaxis) with a combination of treatments, including hyperbaric oxygen therapy. Clin Nephrol. 2008; 70(3): 261–264.
  31. Borges L, Rosa P, Dias E, et al. Successful treatment of calciphylaxis by a multidisciplinary approach. BMJ Case Rep. 2014; 2014.

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