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Published in: BMC Nephrology 1/2017

Open Access 01-12-2017 | Case report

A case report of severe calciphylaxis – suggested approach for diagnosis and treatment

Authors: Margret Patecki, Gabriele Lehmann, Jan Hinrich Bräsen, Jessica Schmitz, Anna Bertram, Lars Daniel Berthold, Hermann Haller, Wilfried Gwinner

Published in: BMC Nephrology | Issue 1/2017

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Abstract

Background

Calciphylaxis is a serious complication in patients with chronic kidney disease associated mineral and bone disorder. It can occur in conditions with low and high bone turnover. So far, there are no definite diagnostic and therapeutic guidelines which may prevent the devastating outcome in many calciphylaxis patients. We report a case which clearly illustrates that knowledge of the underlying bone disorder is essential for a directed treatment. Based on this experience we discuss a systematic diagnostic and therapeutic approach in patients with calciphylaxis.

Case presentation

We report a patient with severe calciphylaxis. Initial evaluation showed an elevated serum parathormone concentration and a bone-specific alkaline phosphatase activity in the upper normal range; however, the bone biopsy clearly showed adynamic bone disease. Extended dialysis with low calcium dialysate concentration and citrate anticoagulation, and administration of teriparatide led to a further increase in bone-specific alkaline phosphatase activity and most importantly, resulted in an activated bone turnover as confirmed by a second bone biopsy 11 weeks later.

Conclusions

This case illustrates that laboratory tests cannot reliably differentiate between high and low bone turnover in calciphylaxis patients. More importantly, this case highlights the fact that specific therapies that alter bone metabolism cannot be applied without knowledge of the bone status. On this background, we suggest that bone biopsies should be an integral part in the diagnosis and therapeutic decision in these patients and should be evaluated in further studies.
Literature
1.
go back to reference Sprague SM. Painful skin ulcers in a hemodialysis patient. Clin J Am Soc Nephrol. 2014;9(1):166–73.CrossRefPubMed Sprague SM. Painful skin ulcers in a hemodialysis patient. Clin J Am Soc Nephrol. 2014;9(1):166–73.CrossRefPubMed
2.
go back to reference Nigwekar SU, Kroshinsky D, Nazarian RM, Goverman J, Malhotra R, Jackson VA, Kamdar MM, Steele DJ, Thadhani RI. Calciphylaxis: risk factors, diagnosis, and treatment. Am J Kidney Dis. 2015;66(1):133–46.CrossRefPubMedPubMedCentral Nigwekar SU, Kroshinsky D, Nazarian RM, Goverman J, Malhotra R, Jackson VA, Kamdar MM, Steele DJ, Thadhani RI. Calciphylaxis: risk factors, diagnosis, and treatment. Am J Kidney Dis. 2015;66(1):133–46.CrossRefPubMedPubMedCentral
3.
go back to reference Nigwekar SU, Zhao S, Wenger J, Hymes JL, Maddux FW, Thadhani RI, Chan KE. A Nationally Representative Study of Calcific Uremic Arteriolopathy Risk Factors. J Am Soc Nephrol. 2016. doi:10.1681/ASN.2015091065.PubMed Nigwekar SU, Zhao S, Wenger J, Hymes JL, Maddux FW, Thadhani RI, Chan KE. A Nationally Representative Study of Calcific Uremic Arteriolopathy Risk Factors. J Am Soc Nephrol. 2016. doi:10.​1681/​ASN.​2015091065.PubMed
4.
go back to reference Latus J, Kimmel M, Ott G, Ting E, Alscher MD, Braun N. Early stages of calciphylaxis: are skin biopsies the answer? Case Rep Dermatol. 2011;3(3):201–5.CrossRefPubMedPubMedCentral Latus J, Kimmel M, Ott G, Ting E, Alscher MD, Braun N. Early stages of calciphylaxis: are skin biopsies the answer? Case Rep Dermatol. 2011;3(3):201–5.CrossRefPubMedPubMedCentral
5.
go back to reference Shmidt E, Murthy NS, Knudsen JM, Weenig RH, Jacobs MA, Starnes AM, Davis MD. Net-like pattern of calcification on plain soft-tissue radiographs in patients with calciphylaxis. J Am Acad Dermatol. 2012;67(6):1296–301.CrossRefPubMed Shmidt E, Murthy NS, Knudsen JM, Weenig RH, Jacobs MA, Starnes AM, Davis MD. Net-like pattern of calcification on plain soft-tissue radiographs in patients with calciphylaxis. J Am Acad Dermatol. 2012;67(6):1296–301.CrossRefPubMed
6.
go back to reference Bonchak JG, Park KK, Vethanayagamony T, Sheikh MM, Winterfield LS. Calciphylaxis: a case series and the role of radiology in diagnosis. Int J Dermatol. 2016;55(5):e275–9.CrossRefPubMed Bonchak JG, Park KK, Vethanayagamony T, Sheikh MM, Winterfield LS. Calciphylaxis: a case series and the role of radiology in diagnosis. Int J Dermatol. 2016;55(5):e275–9.CrossRefPubMed
7.
go back to reference Brandenburg VM, Sinha S, Specht P, Ketteler M. Calcific uraemic arteriolopathy: a rare disease with a potentially high impact on chronic kidney disease-mineral and bone disorder. Pediatr Nephrol. 2014;29:2289–98.CrossRefPubMed Brandenburg VM, Sinha S, Specht P, Ketteler M. Calcific uraemic arteriolopathy: a rare disease with a potentially high impact on chronic kidney disease-mineral and bone disorder. Pediatr Nephrol. 2014;29:2289–98.CrossRefPubMed
8.
go back to reference Brandenburg V, Adragao T, Van Dam B, Evenepoel P, Frazão JM, Ketteler M, et al. Blueprint for a european calciphylaxis registry initiative: the European Calciphylaxis Network (EuCalNet). Clin Kidney J. 2015;8(5):567–71.CrossRefPubMedPubMedCentral Brandenburg V, Adragao T, Van Dam B, Evenepoel P, Frazão JM, Ketteler M, et al. Blueprint for a european calciphylaxis registry initiative: the European Calciphylaxis Network (EuCalNet). Clin Kidney J. 2015;8(5):567–71.CrossRefPubMedPubMedCentral
9.
go back to reference Mawad HW, Sawaya BP, Sarin R, Malluche HH. Calcific uremic ateriolopathy in association with low turnover uremic bone disease. Clin Nephrol. 1999;52(3):160–6.PubMed Mawad HW, Sawaya BP, Sarin R, Malluche HH. Calcific uremic ateriolopathy in association with low turnover uremic bone disease. Clin Nephrol. 1999;52(3):160–6.PubMed
10.
go back to reference Russo D, Capuano A, Cozzolino M, Napolitano P, Mosella F, Russo L, Saviano C, Zoccali C. Multimodal treatment of calcific uraemic arteriolopathy [calciphylaxis]: a case series. Clin Kidney J. 2016;9(1):108–12.CrossRefPubMed Russo D, Capuano A, Cozzolino M, Napolitano P, Mosella F, Russo L, Saviano C, Zoccali C. Multimodal treatment of calcific uraemic arteriolopathy [calciphylaxis]: a case series. Clin Kidney J. 2016;9(1):108–12.CrossRefPubMed
11.
12.
go back to reference Brandenburg VM, Kramann R, Rothe H, Kaesler N, Korbiel J, Specht P, et al. Calcific uraemic arteriolopathy (calciphylaxis): data from a large nationwide registry. Nephrol Dial Transplant. 2016. doi:10.1093/ndt/gfv438. Brandenburg VM, Kramann R, Rothe H, Kaesler N, Korbiel J, Specht P, et al. Calcific uraemic arteriolopathy (calciphylaxis): data from a large nationwide registry. Nephrol Dial Transplant. 2016. doi:10.​1093/​ndt/​gfv438.
13.
go back to reference Cai MM, Smith ER, Brumby C, McMahon LP, Holt SG. Fetuin-A-containing calciprotein particle levels can be reduced by dialysis, sodium thiosulfate and plasma exchange. Potential therapeutic implications for calciphylaxis? Nephrology. 2013;18:724–7.CrossRefPubMed Cai MM, Smith ER, Brumby C, McMahon LP, Holt SG. Fetuin-A-containing calciprotein particle levels can be reduced by dialysis, sodium thiosulfate and plasma exchange. Potential therapeutic implications for calciphylaxis? Nephrology. 2013;18:724–7.CrossRefPubMed
14.
go back to reference Ceijka D. Renale osteodystrophie. Wien Med Wochenschr. 2013;163(17–18):403–8.CrossRef Ceijka D. Renale osteodystrophie. Wien Med Wochenschr. 2013;163(17–18):403–8.CrossRef
15.
go back to reference Behets GJ, Spasovski G, Sterling LR, Goodman WG, Spiegel DM, De Broe ME, D’Haese PC. Bone histomorphometry before and after long-term treatment with cinacalcet in dialysis patients with secondary hyperparathyroidism. Kidney Int. 2015;87:846–56.CrossRefPubMed Behets GJ, Spasovski G, Sterling LR, Goodman WG, Spiegel DM, De Broe ME, D’Haese PC. Bone histomorphometry before and after long-term treatment with cinacalcet in dialysis patients with secondary hyperparathyroidism. Kidney Int. 2015;87:846–56.CrossRefPubMed
16.
go back to reference Schlieper G, Brandenburg V, Ketteler M, Floege J. Sodium thiosulfate in the treatment of calcific uremic arteriolopathy. Nat Rev Nephrol. 2009;5:539–43.CrossRefPubMed Schlieper G, Brandenburg V, Ketteler M, Floege J. Sodium thiosulfate in the treatment of calcific uremic arteriolopathy. Nat Rev Nephrol. 2009;5:539–43.CrossRefPubMed
17.
go back to reference Oliveira TM, Frazao JM. Calciphylaxis: from the disease to the diseased. Journal of Nephrology. 2015;28(5):531–40.CrossRefPubMed Oliveira TM, Frazao JM. Calciphylaxis: from the disease to the diseased. Journal of Nephrology. 2015;28(5):531–40.CrossRefPubMed
18.
go back to reference Blick SK, Dhillon S, Keam SJ. Teriparatide: a systemic review of its use in osteoporosis. Drugs. 2008;68(18):2709–37.CrossRefPubMed Blick SK, Dhillon S, Keam SJ. Teriparatide: a systemic review of its use in osteoporosis. Drugs. 2008;68(18):2709–37.CrossRefPubMed
19.
go back to reference Palcu P, Dion N, Ste-Marie LG, et al. Teriparatide and bone turnover and formation in a hemodialysis patient with low-turnover bone disease: a case report. Am J Kidney Dis. 2015;65(6):933–6.CrossRefPubMed Palcu P, Dion N, Ste-Marie LG, et al. Teriparatide and bone turnover and formation in a hemodialysis patient with low-turnover bone disease: a case report. Am J Kidney Dis. 2015;65(6):933–6.CrossRefPubMed
20.
go back to reference Cejka D, Kodras K, Bader T, Haas M. Treatment of hemodialysis-associated adynamic bone disease with teriparatide (PTH1–34): a pilot study. Kidney Blood Press Res. 2010;33:221–6.CrossRefPubMed Cejka D, Kodras K, Bader T, Haas M. Treatment of hemodialysis-associated adynamic bone disease with teriparatide (PTH1–34): a pilot study. Kidney Blood Press Res. 2010;33:221–6.CrossRefPubMed
21.
go back to reference Kidney Disease: Improving Global Outcomes (KDIGO) CKD–MBD Work Group. KDIGO clinical practice guideline for the diagnosis, evaluation, prevention, and treatment of chronic kidney disease–mineral and bone disorder (CKD–MBD). Kidney International. 2009;76(Suppl 113):S1–S130. Kidney Disease: Improving Global Outcomes (KDIGO) CKD–MBD Work Group. KDIGO clinical practice guideline for the diagnosis, evaluation, prevention, and treatment of chronic kidney disease–mineral and bone disorder (CKD–MBD). Kidney International. 2009;76(Suppl 113):S1–S130.
22.
go back to reference Sumida K, Ubara Y, Hoshino J, Mise K, Hayami N, Suwabe T, Kawada M, et al. Once weekly teriparatide in hemodialysis patients with hypoparathyreoidism and low bone mass: a prospective study. Osteoporos Int. 2016;27(4):1441–50.CrossRefPubMed Sumida K, Ubara Y, Hoshino J, Mise K, Hayami N, Suwabe T, Kawada M, et al. Once weekly teriparatide in hemodialysis patients with hypoparathyreoidism and low bone mass: a prospective study. Osteoporos Int. 2016;27(4):1441–50.CrossRefPubMed
23.
go back to reference Monegal A, Peris P, Alsina M, Colmenero J, Guanabens N. Development of multiorganic calciphylaxis during teriparatide, vitamin D, and calcium treatment. Osteoporos Int. 2016;27(8):2631–4.CrossRefPubMed Monegal A, Peris P, Alsina M, Colmenero J, Guanabens N. Development of multiorganic calciphylaxis during teriparatide, vitamin D, and calcium treatment. Osteoporos Int. 2016;27(8):2631–4.CrossRefPubMed
24.
go back to reference Sugimoto T, Nakamura T, Nakamura Y, Isogai Y, Shiraki M. Profile of changes in bone turnover markers during once-weekly teriparatide administration for 24 weeks in postmenopausal women with osteoporosis. Osteoporos Int. 2014;25:1173–80.CrossRefPubMed Sugimoto T, Nakamura T, Nakamura Y, Isogai Y, Shiraki M. Profile of changes in bone turnover markers during once-weekly teriparatide administration for 24 weeks in postmenopausal women with osteoporosis. Osteoporos Int. 2014;25:1173–80.CrossRefPubMed
25.
go back to reference Hocher B, Oberthür D, Slowinski T, Querfeld U, Schaefer F, Doyon A, Tepel M, Roth HJ, Grön HJ, Reichetzeder C, Betzel C, Armbruster FP. Model of oxidized PTH (oxPTH) and non-oxidized PTH (n-oxPTH) receptor binding and releationship of oxidized to non-oxidized PTH in children with chronic renal failure, adult patients on hemodialysis and kidney transplant recipients. Kidney Blood Press Res. 2013;37:240–51.CrossRefPubMed Hocher B, Oberthür D, Slowinski T, Querfeld U, Schaefer F, Doyon A, Tepel M, Roth HJ, Grön HJ, Reichetzeder C, Betzel C, Armbruster FP. Model of oxidized PTH (oxPTH) and non-oxidized PTH (n-oxPTH) receptor binding and releationship of oxidized to non-oxidized PTH in children with chronic renal failure, adult patients on hemodialysis and kidney transplant recipients. Kidney Blood Press Res. 2013;37:240–51.CrossRefPubMed
26.
go back to reference Lindsay R, Nieves J, Henneman E, Shen V, Cosman F. Subcutaneous administration of the amino-terminal fragment of human parathyreoid hormone-(1–34): kinetics and biochemical response in estrogenized osteoporotic patients. J Clin Endocrinol Metab. 1993;77(6):1535–9.PubMed Lindsay R, Nieves J, Henneman E, Shen V, Cosman F. Subcutaneous administration of the amino-terminal fragment of human parathyreoid hormone-(1–34): kinetics and biochemical response in estrogenized osteoporotic patients. J Clin Endocrinol Metab. 1993;77(6):1535–9.PubMed
27.
go back to reference Sprague SM, Bellorin-Font E, Jorgetti V, Carvalho AB, Malluche HH, Ferreira A, D’Haese PC, Drüeke TB, Du H, Manley T, Rojas E, Moe SM. Diagnostic accuracy of bone turnover markers and bone histology in patients with CKD treated by dialysis. Am J Kidney Dis. 2016;67(4):559–66.CrossRefPubMed Sprague SM, Bellorin-Font E, Jorgetti V, Carvalho AB, Malluche HH, Ferreira A, D’Haese PC, Drüeke TB, Du H, Manley T, Rojas E, Moe SM. Diagnostic accuracy of bone turnover markers and bone histology in patients with CKD treated by dialysis. Am J Kidney Dis. 2016;67(4):559–66.CrossRefPubMed
Metadata
Title
A case report of severe calciphylaxis – suggested approach for diagnosis and treatment
Authors
Margret Patecki
Gabriele Lehmann
Jan Hinrich Bräsen
Jessica Schmitz
Anna Bertram
Lars Daniel Berthold
Hermann Haller
Wilfried Gwinner
Publication date
01-12-2017
Publisher
BioMed Central
Published in
BMC Nephrology / Issue 1/2017
Electronic ISSN: 1471-2369
DOI
https://doi.org/10.1186/s12882-017-0556-z

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