Vincent M Brandenburg1,2, Rafael Kramann2,3, Hansjörg Rothe4, Nadine Kaesler3, Joanna Korbiel5, Paula Specht1, Sophia Schmitz6, Thilo Krüger3, Jürgen Floege2,3, Markus Ketteler4. 1. Department of Cardiology, University Hospital of the RWTH Aachen, Aachen, Germany. 2. Center for Rare Diseases, University Hospital of the RWTH Aachen (ZSEA), Aachen, Germany. 3. Department of Nephrology, University Hospital of the RWTH Aachen, Aachen, Germany. 4. Department of Nephrology, Klinikum Coburg, Coburg, Germany. 5. Clinical Trial Center-Aachen, RWTH Aachen, Aachen, Germany. 6. Department of General, Visceral and Transplantation Surgery, University Hospital of the RWTH Aachen, Aachen, Germany.
Abstract
Background: Calcific uraemic arteriolopathy (CUA, calciphylaxis) is a rare disease predominantly in dialysis patients and associated with high mortality. Painful skin ulcerations and calcification of cutaneous arterioles characterize calciphylaxis. Methods: We established an observational, Internet-based registry allowing online notification for all German CUA cases. The registry recorded data about patient characteristics, biochemistry and therapies. Blood samples were stored in a central biobank. Results: Between 2006 and 2015, 253 CUA patients were recorded: median age 70 [interquartile range (IQR) 61-76] years, 60% females and 86% ( n = 207) dialysis patients, translating into an estimated annual incidence rate of 0.04% in German dialysis patients. Fifty-two per cent received vitamin K antagonists (VKAs) prior to CUA. Skin lesions were localized in 71% on the legs or gluteal region. In dialysis CUA patients median total serum calcium was 2.20 (IQR 2.06-2.37) mmol/L, phosphorus 1.67 (IQR 1.35-2.03) mmol/L, intact parathyroid hormone 147 (IQR 72-276) pg/mL and fetuin-A 0.21 (IQR 0.16-0.26) g/L (normal range 0.35-0.95). Median sclerostin, osteoprotegerin, TRAP5b, bone-specific alkaline phosphatase and c-terminal FGF23 levels were all elevated. The most frequently recorded therapeutic procedures in dialysis CUA patients were as follows: wound debridement (29% of cases), stopping VKA (25%), lowering calcium supply (24%), sodium thiosulphate (22%), application of vitamin K (18%), increase of dialysis duration/frequency (17%) and stoping active vitamin D (16%). Conclusions: Approximately 50% of CUA patients used VKA. Our data suggest that uncontrolled hyperparathyroidism is not the key determinant of calciphylaxis. Therapeutic strategies were heterogeneous. The experience of the German registry will help substantially to initiate a large-scale multinational CUA registry.
Background: Calcific uraemic arteriolopathy (CUA, calciphylaxis) is a rare disease predominantly in dialysis patients and associated with high mortality. Painful skin ulcerations and calcification of cutaneous arterioles characterize calciphylaxis. Methods: We established an observational, Internet-based registry allowing online notification for all German CUA cases. The registry recorded data about patient characteristics, biochemistry and therapies. Blood samples were stored in a central biobank. Results: Between 2006 and 2015, 253 CUA patients were recorded: median age 70 [interquartile range (IQR) 61-76] years, 60% females and 86% ( n = 207) dialysis patients, translating into an estimated annual incidence rate of 0.04% in German dialysis patients. Fifty-two per cent received vitamin K antagonists (VKAs) prior to CUA. Skin lesions were localized in 71% on the legs or gluteal region. In dialysis CUA patients median total serum calcium was 2.20 (IQR 2.06-2.37) mmol/L, phosphorus 1.67 (IQR 1.35-2.03) mmol/L, intact parathyroid hormone 147 (IQR 72-276) pg/mL and fetuin-A 0.21 (IQR 0.16-0.26) g/L (normal range 0.35-0.95). Median sclerostin, osteoprotegerin, TRAP5b, bone-specific alkaline phosphatase and c-terminal FGF23 levels were all elevated. The most frequently recorded therapeutic procedures in dialysis CUA patients were as follows: wound debridement (29% of cases), stopping VKA (25%), lowering calcium supply (24%), sodium thiosulphate (22%), application of vitamin K (18%), increase of dialysis duration/frequency (17%) and stoping active vitamin D (16%). Conclusions: Approximately 50% of CUA patients used VKA. Our data suggest that uncontrolled hyperparathyroidism is not the key determinant of calciphylaxis. Therapeutic strategies were heterogeneous. The experience of the German registry will help substantially to initiate a large-scale multinational CUA registry.
Authors: Sagar U Nigwekar; Donald B Bloch; Rosalynn M Nazarian; Cees Vermeer; Sarah L Booth; Dihua Xu; Ravi I Thadhani; Rajeev Malhotra Journal: J Am Soc Nephrol Date: 2017-01-03 Impact factor: 10.121
Authors: Sagar U Nigwekar; Pawina Jiramongkolchai; Florian Wunderer; Emily Bloch; Rika Ichinose; Rosalynn M Nazarian; Ravi I Thadhani; Rajeev Malhotra; Donald B Bloch Journal: Am J Nephrol Date: 2017-11-09 Impact factor: 3.754
Authors: Vincent M Brandenburg; Smeeta Sinha; Jose-Vicente Torregrosa; Rekha Garg; Stephan Miller; Ana-Zeralda Canals; Daun Bahr; Pieter H Joubert; Carolina Salcedo; Kevin J Carroll; Alex Gold; Joan Perelló Journal: J Nephrol Date: 2019-08-10 Impact factor: 3.902