Clinical characteristics and treatment modalities in uremic and non uremic calciphylaxis - a dermatological single-center experience

Calciphylaxis (CP) is a serious, potentially life-threatening disease that presents with medial calcification of small-sized vessels and painful ischemic ulcerations. Although calciphylaxis is frequently seen in patients with end-stage kidney disease on dialysis (calcific uremic arteriolopathy, CUA)...

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Main Authors: Sabine Yousuf, Dorothee Busch, Regina Renner, Stefan Schliep, Cornelia Erfurt-Berge
Format: Article
Language:English
Published: Taylor & Francis Group 2024-12-01
Series:Renal Failure
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Online Access:https://www.tandfonline.com/doi/10.1080/0886022X.2023.2297566
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author Sabine Yousuf
Dorothee Busch
Regina Renner
Stefan Schliep
Cornelia Erfurt-Berge
author_facet Sabine Yousuf
Dorothee Busch
Regina Renner
Stefan Schliep
Cornelia Erfurt-Berge
author_sort Sabine Yousuf
collection DOAJ
description Calciphylaxis (CP) is a serious, potentially life-threatening disease that presents with medial calcification of small-sized vessels and painful ischemic ulcerations. Although calciphylaxis is frequently seen in patients with end-stage kidney disease on dialysis (calcific uremic arteriolopathy, CUA), there are reported cases of nonuremic calciphylaxis (NUC), which often remain undiagnosed. We conducted a retrospective chart review at our dermatological hospital and evaluated data concerning the epidemiology, comorbidities, medication, laboratory abnormalities, and therapeutic approaches of 60 patients diagnosed with calciphylaxis between 01/2012 and 12/2022. We identified 21 patients diagnosed with NUC and 39 with kidney disease. The predilection sites of skin lesions were the lower legs in 88% (n = 53), followed by the thigh and gluteal regions in 7% (n = 4). Significant differences were identified in comorbidities, such as atrial fibrillation (p < 0.001) and hyperparathyroidism (p < 0.01) accounting for CUA patients. Medication with vitamin K antagonists (p < 0.001), phosphate binders (p < 0.001), and loop diuretics (p < 0.01) was found to be associated with the onset of calciphylaxis. Hyperphosphatemia (p < 0.001), increased parathyroid hormone (p < 0.01) and triglyceride levels (p < 0.01), hypoalbuminemia (p < 0.01) and decreased hemoglobin values (p < 0.001) in the CUA cohort were significantly different from those in the NUC group. All patients with CUA received systemic medication. In contrast, only 38% (n = 8) of patients with NUC received systemic treatment. Striking discrepancies in the treatment of both cohorts were detected. In particular, NUC remains a disease pattern that is still poorly understood and differs from CUA in several important parameters.
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spelling doaj-art-ee4090f8c89d47b1bed5d20ce7d4709f2025-01-23T04:17:48ZengTaylor & Francis GroupRenal Failure0886-022X1525-60492024-12-0146110.1080/0886022X.2023.2297566Clinical characteristics and treatment modalities in uremic and non uremic calciphylaxis - a dermatological single-center experienceSabine Yousuf0Dorothee Busch1Regina Renner2Stefan Schliep3Cornelia Erfurt-Berge4Hautklinik, Uniklinikum Erlangen, Friedrich-Alexander-Universität Erlangen-Nürnberg, Erlangen, GermanyHautklinik, Uniklinikum Erlangen, Friedrich-Alexander-Universität Erlangen-Nürnberg, Erlangen, GermanyPrivate Practice, Esslingen, GermanyHautklinik, Uniklinikum Erlangen, Friedrich-Alexander-Universität Erlangen-Nürnberg, Erlangen, GermanyHautklinik, Uniklinikum Erlangen, Friedrich-Alexander-Universität Erlangen-Nürnberg, Erlangen, GermanyCalciphylaxis (CP) is a serious, potentially life-threatening disease that presents with medial calcification of small-sized vessels and painful ischemic ulcerations. Although calciphylaxis is frequently seen in patients with end-stage kidney disease on dialysis (calcific uremic arteriolopathy, CUA), there are reported cases of nonuremic calciphylaxis (NUC), which often remain undiagnosed. We conducted a retrospective chart review at our dermatological hospital and evaluated data concerning the epidemiology, comorbidities, medication, laboratory abnormalities, and therapeutic approaches of 60 patients diagnosed with calciphylaxis between 01/2012 and 12/2022. We identified 21 patients diagnosed with NUC and 39 with kidney disease. The predilection sites of skin lesions were the lower legs in 88% (n = 53), followed by the thigh and gluteal regions in 7% (n = 4). Significant differences were identified in comorbidities, such as atrial fibrillation (p < 0.001) and hyperparathyroidism (p < 0.01) accounting for CUA patients. Medication with vitamin K antagonists (p < 0.001), phosphate binders (p < 0.001), and loop diuretics (p < 0.01) was found to be associated with the onset of calciphylaxis. Hyperphosphatemia (p < 0.001), increased parathyroid hormone (p < 0.01) and triglyceride levels (p < 0.01), hypoalbuminemia (p < 0.01) and decreased hemoglobin values (p < 0.001) in the CUA cohort were significantly different from those in the NUC group. All patients with CUA received systemic medication. In contrast, only 38% (n = 8) of patients with NUC received systemic treatment. Striking discrepancies in the treatment of both cohorts were detected. In particular, NUC remains a disease pattern that is still poorly understood and differs from CUA in several important parameters.https://www.tandfonline.com/doi/10.1080/0886022X.2023.2297566Calciphylaxiscalcific uremic arteriolopathynonuremic calciphylaxisleg ulcer
spellingShingle Sabine Yousuf
Dorothee Busch
Regina Renner
Stefan Schliep
Cornelia Erfurt-Berge
Clinical characteristics and treatment modalities in uremic and non uremic calciphylaxis - a dermatological single-center experience
Renal Failure
Calciphylaxis
calcific uremic arteriolopathy
nonuremic calciphylaxis
leg ulcer
title Clinical characteristics and treatment modalities in uremic and non uremic calciphylaxis - a dermatological single-center experience
title_full Clinical characteristics and treatment modalities in uremic and non uremic calciphylaxis - a dermatological single-center experience
title_fullStr Clinical characteristics and treatment modalities in uremic and non uremic calciphylaxis - a dermatological single-center experience
title_full_unstemmed Clinical characteristics and treatment modalities in uremic and non uremic calciphylaxis - a dermatological single-center experience
title_short Clinical characteristics and treatment modalities in uremic and non uremic calciphylaxis - a dermatological single-center experience
title_sort clinical characteristics and treatment modalities in uremic and non uremic calciphylaxis a dermatological single center experience
topic Calciphylaxis
calcific uremic arteriolopathy
nonuremic calciphylaxis
leg ulcer
url https://www.tandfonline.com/doi/10.1080/0886022X.2023.2297566
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