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Kalziphylaxie

Eine nephrologisch-dermatologische Herausforderung

Calciphylaxis

A call for interdisciplinary cooperation

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Zusammenfassung

Die Kalziphylaxie ist eine seltene, oft sehr schmerzhafte und potenziell lebensbedrohliche Erkrankung an der Schnittstelle zwischen Dermatologie und Nephrologie. Das Vollbild besteht aus kutanen Nekrosen, die auf dem Boden kalzifizierter kutaner Arteriolen entstehen. Oft sind Patienten mit chronischer Niereninsuffizienz oder Dialysepatienten betroffen. Die Pathophysiologie ist unvollständig verstanden; mehrere lokale und systemische Faktoren müssen zusammenkommen. Hinsichtlich der Therapie fehlen prospektive, kontrollierte Interventionsstudien, sodass sich die aktuellen interdisziplinären Therapiestrategien entweder an der vermuteten Pathophysiologie ausrichten und/oder sich auf kasuistisch geschilderte Therapieerfolge stützen. Zu den Behandlungskonzepten gehören u. a. eine Normalisierung des Mineralstoffwechsels, eine Intensivierung des Dialyseregimes, Vermeidung von Vitamin-K-Antagonisten, Gabe von Calcimimetika, Bisphosphonaten und Natriumthiosulfat sowie hyperbare O2-Therapie. Supportive Maßnahmen wie analgetische Therapie, antibiotische Therapie und lokales Wundmanagement sind ebenfalls Eckpfeiler der Therapie. Wir haben zur Verbesserung der Patientenbetreuung mit Unterstützung der Firma Amgen ein Online-Kalziphylaxie-Register (www.calciphylaxie.de) ins Leben gerufen.

Abstract

Calciphylaxis is a rare, often very painful and potentially life-threatening disorder at the interface between nephrology and dermatology. It is characterized by skin lesions and ulcerations following calcification and occlusion of cutaneous arterioles. Most patients have chronic kidney disease or are on dialysis. A concert of various, still incompletely understood local and systemic risk factors is necessary to cause the development of calciphylaxis. Since randomized prospective trials are missing, interdisciplinary treatment is based on pathophysiological considerations as well as evidence derived from case reports or case series. Normalization of mineral metabolism, intensifying dialysis and avoidance of coumarins, as well as administration of calcimimetics, bisphosphonates and sodium thiosulfate and hyperbaric oxygen therapy are often used. Supportive measures include analgesics, antibiotics and local wound care. We have initiated an internet-based registry for patients with calciphylaxis in order to collect data for improved patient care (with support from Amgen) (www.calciphylaxie.de).

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Literatur

  1. Selye H, Grasso G, Dieudonne J (1961) On the role of adjuvants in calciphylaxis. Rev Allergy 15:461–465

    PubMed  CAS  Google Scholar 

  2. Selye H, Gabbiani G, Strebel R (1962) Sensitization to calciphylaxis by endogenous parathyroid hormone. Endocrinology 71:554–558

    Article  PubMed  CAS  Google Scholar 

  3. Hafner J, Keusch G, Wahl C et al (1995) Uremic small-artery disease with medial calcification and intimal hyperplasia (so-called calciphylaxis): a complication of chronic renal failure and benefit from parathyroidectomy. J Am Acad Dermatol 33(6):954–962

    Article  PubMed  CAS  Google Scholar 

  4. Gipstein RM, Coburn JW, Adams DA et al (1976) Calciphylaxis in man. A syndrome of tissue necrosis and vascular calcification in 11 patients with chronic renal failure. Arch Intern Med 136(11):1273–1280

    Article  PubMed  CAS  Google Scholar 

  5. Angelis M, Wong LL, Myers SA, Wong LM (1997) Calciphylaxis in patients on hemodialysis: a prevalence study. Surgery 122(6):1083–1089

    Article  PubMed  CAS  Google Scholar 

  6. Kalajian AH, Malhotra PS, Callen JP, Parker LP (2009) Calciphylaxis with normal renal and parathyroid function: not as rare as previously believed. Arch Dermatol 145(4):451–458

    Article  PubMed  Google Scholar 

  7. Brandenburg V, Floege J, Ketteler M (2008) Vorstellung eines Online-Calciphylaxie-Registers des International Collaborative Calciphylaxis Network [Abstrakt]. Nieren Hochdruckkrankheiten 37(9):440–441

    Google Scholar 

  8. Weenig RH (2008) Pathogenesis of calciphylaxis: Hans Selye to nuclear factor kappa-B. J Am Acad Dermatol 58(3):458–471

    Article  PubMed  Google Scholar 

  9. Franks AG Jr (2009) Skin manifestations of internal disease. Med Clin North Am 93(6):1265–1282

    Article  PubMed  CAS  Google Scholar 

  10. Fine A, Zacharias J (2002) Calciphylaxis is usually non-ulcerating: risk factors, outcome and therapy. Kidney Int 61(6):2210–2217

    Article  PubMed  Google Scholar 

  11. Rogers NM, Coates PT (2008) Calcific uraemic arteriolopathy: an update. Curr Opin Nephrol Hypertens 17(6):629–634

    Article  PubMed  Google Scholar 

  12. Mazhar AR, Johnson RJ, Gillen D et al (2001) Risk factors and mortality associated with calciphylaxis in end-stage renal disease. Kidney Int 60(1):324–332

    Article  PubMed  CAS  Google Scholar 

  13. Nigwekar SU, Wolf M, Sterns RH, Hix JK (2008) Calciphylaxis from nonuremic causes: a systematic review. Clin J Am Soc Nephrol 3(4):1139–1143

    Article  PubMed  Google Scholar 

  14. Meissner M, Gille J, Kaufmann R (2006) Calciphylaxis: no therapeutic concepts for a poorly understood syndrome? J Dtsch Dermatol Ges 4(12):1037–1044

    Article  PubMed  Google Scholar 

  15. Au S, Crawford RI (2002) Three-dimensional analysis of a calciphylaxis plaque: clues to pathogenesis. J Am Acad Dermatol 47(1):53–57

    Article  PubMed  Google Scholar 

  16. (2001) Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 31–2001. A 70-year-old woman with end-stage renal disease and cutaneous ulcers. N Engl J Med 345(15):1119–1124

  17. Ketteler M, Westenfeld R, Schlieper G, Brandenburg V (2005) Pathogenesis of vascular calcification in dialysis patients. Clin Exp Nephrol 9(4):265–270

    Article  PubMed  Google Scholar 

  18. Janigan DT, Hirsch DJ, Klassen GA, MacDonald AS (2000) Calcified subcutaneous arterioles with infarcts of the subcutis and skin („calciphylaxis“) in chronic renal failure. Am J Kidney Dis 35(4):588–597

    Article  PubMed  CAS  Google Scholar 

  19. Mathur RV, Shortland JR, El Nahas AM (2001) Calciphylaxis. Postgrad Med J 77(911):557–561

    Article  PubMed  CAS  Google Scholar 

  20. Budisavljevic MN, Cheek D, Ploth DW (1996) Calciphylaxis in chronic renal failure. J Am Soc Nephrol 7(7):978–982

    PubMed  CAS  Google Scholar 

  21. Rogers NM, Teubner DJ, Coates PT (2007) Calcific uremic arteriolopathy: advances in pathogenesis and treatment. Semin Dial 20(2):150–157

    Article  PubMed  Google Scholar 

  22. Krueger T, Westenfeld R, Schurgers L, Brandenburg V (2009) Coagulation meets calcification: the vitamin K system. Int J Artif Organs 32(2):67–74

    PubMed  CAS  Google Scholar 

  23. Brandenburg VM, Floege J, Ketteler M (2009) Kalzifizierende urämische Arteriolopathie. Nephrologe 4(1):65–66

    Article  Google Scholar 

  24. Weenig RH, Sewell LD, Davis MD et al (2007) Calciphylaxis: natural history, risk factor analysis, and outcome. J Am Acad Dermatol 56(4):569–579

    Article  PubMed  Google Scholar 

  25. Velasco N, MacGregor MS, Innes A, MacKay IG (2006) Successful treatment of calciphylaxis with cinacalcet – an alternative to parathyroidectomy? Nephrol Dial Transplant 21(7):1999–2004

    Article  PubMed  Google Scholar 

  26. Raymond CB, Wazny LD (2008) Sodium thiosulfate, bisphosphonates, and cinacalcet for treatment of calciphylaxis. Am J Health Syst Pharm 65(15):1419–1429

    Article  PubMed  CAS  Google Scholar 

  27. Schlieper G, Brandenburg V, Ketteler M, Floege J (2009) Sodium thiosulfate in the treatment of calcific uremic arteriolopathy. Nat Rev Nephrol 5(9):539–543

    Article  PubMed  CAS  Google Scholar 

  28. Pasch A, Schaffner T, Huynh-Do U et al (2008) Sodium thiosulfate prevents vascular calcifications in uremic rats. Kidney Int 74(11):1444–1453

    Article  PubMed  CAS  Google Scholar 

  29. Coates T, Kirkland GS, Dymock RB et al (1998) Cutaneous necrosis from calcific uremic arteriolopathy. Am J Kidney Dis 32(3):384–391

    Article  PubMed  CAS  Google Scholar 

  30. Rogers NM, Chang SH, Teubner DJ, Coates PT (2008) Hyperbaric oxygen as effective adjuvant therapy in the treatmentof distal calcific uraemic arteriolopathy. Nephrol Dial Transplant 1:244–249

    CAS  Google Scholar 

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Authors and Affiliations

  1. Medizinische Klinik I; Kardiologie, Universitätsklinikum der RWTH Aachen, Pauwelsstr. 30, 52057, Aachen, Deutschland

    V.M. Brandenburg

  2. Medizinische Klinik II; Nephrologie, Universitätsklinikum der RWTH Aachen, Aachen, Deutschland

    V.M. Brandenburg, S. Schmitz & J. Floege

  3. Nephropathologische Abteilung im Pathologischen Institut, Universitätsklinikum Erlangen, Erlangen, Deutschland

    K. Amann

  4. Klinik für Nephrologie, Klinikum Coburg, Coburg, Deutschland

    M. Ketteler

Authors
  1. V.M. Brandenburg
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  2. S. Schmitz
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  3. J. Floege
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  4. K. Amann
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  5. M. Ketteler
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Correspondence to V.M. Brandenburg.

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Brandenburg, V., Schmitz, S., Floege, J. et al. Kalziphylaxie. Hautarzt 62, 452–458 (2011). https://doi.org/10.1007/s00105-011-2169-y

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  • DOI: https://doi.org/10.1007/s00105-011-2169-y

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