Review: update on the management of calciphylaxis

Seethapathy, Harish; Brandenburg, Vincent; Sinha, Smeeta; el‐Azhary, Rokea A.; Nigwekar, Sagar U.

doi:10.1093/qjmed/hcy234

Cited by 21 publications

(32 citation statements)

References 24 publications

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“…A small Phase 2 open-label study investigation suggested a potential benefit of SNF472 in calciphylaxis and a Phase 3 multi-centre study is currently in progress (NCT04195906) [31,32]. Until evidence is available from RCT, the management strategy for CUA should essentially be a multifaceted approach which includes medical modification of risk factors, wound care, pain management, surgery and advanced care planning [33]. Both univariable and multivariable cox-regression analysis have shown calciphylaxis as a strong and independent risk factor associated with all-cause mortality.…”

Section: Discussionmentioning

confidence: 99%

Calciphylaxis in end-stage kidney disease: outcome data from the United Kingdom Calciphylaxis Study

et al. 2021

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Background and aims Calciphylaxis is a rare condition associated with very high mortality in patients with end-stage kidney disease. Data from country-based registries have been an invaluable resource for a better understanding of the natural history and management for this condition. This study aimed to investigate the current management strategies and outcomes of patients enrolled in the United Kingdom Calciphylaxis study (UKCS). Methods The study was conducted on 89 patients registered in the UKCS since 2012. The initial analysis included a description of the baseline characteristics, management strategies and outcomes on follow-up until May 2020. Further analysis included a comparison of the mortality outcome of the UKCS patients who were receiving haemodialysis with a propensity score matched cohort of haemodialysis patients from the Chronic Renal Insufficiency Standards Implementation Study- Haemodialysis (CRISIS-HD). Results Median age of the cohort was 59 years, with a predominance of females (61%) and Caucasian (95%) ethnicity. About 54% of the patients were diabetic and 70% were receiving haemodialysis at study entry. The skin lesions were mostly distributed in the lower extremities (48%). Sodium thiosulphate and calcimimetic were the most widely used management strategies. The mortality rate was 72 deaths per hundred patient-years (50 deaths observed in 69.5 patient years). Complete wound healing was noted in 17% and bacteraemia was reported in 26% of patients. In a comparative analysis of the matched haemodialysis patients, the presence of calciphylaxis in 62 patients showed a strong association with all-cause mortality (HR 6.96; p < 0.001), with annual mortality 67% versus 10.2% in haemodialysis patients without calciphylaxis. Conclusions This UK wide study strengthens the evidence that calciphylaxis is a strong and independent risk factor associated with all-cause mortality; no significant benefit was shown with any individual treatment modality. Until further evidence becomes available, a multifaceted approach would be the appropriate treatment strategy in the management of this extremely serious condition. Graphic abstract

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Section: Discussionmentioning

confidence: 99%

Calciphylaxis in end-stage kidney disease: outcome data from the United Kingdom Calciphylaxis Study

et al. 2021

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“…Hence, recognizing the clinical picture in which patients present with, that is, subcutes lesions that are indurated plaques and nodules with a pattern of livedo racemosa (reflecting the underlying vascular insult), these lesions are usually located in areas with abundant adipose tissue (eg, abdomen, thighs, and buttocks) in the uremic‐induced calciphylaxis, opposite to the nonuremic warfarin‐induced type where these have a predominance toward the lower extremities, particularly below the knees. If the primary cause was not addressed, these lesions can ulcerate 9,10 . Furthermore, BFN can also manifest as a breast mass within a complex cyst or as a nodular mass with an overlying skin changes that might be misinterpreted as breast cancer 1 …”

Section: Discussionmentioning

confidence: 99%

“…Management of BFN secondary to warfarin‐induced calciphylaxis can be quite challenging not only to diagnose but also to manage 1,7 . However, once the diagnosis is established a case‐by‐case approach based on a multi‐disciplinary decision‐making should be adapted to provide patients with the best possible outcomes 1,2,9,13 . The management should address three main aspects, namely addressing (a) the primary cause of calciphylaxis, (b) the pre‐existing calciphylaxis and prevention of further thrombosis, and (c) local wound care 2,5,9 …”

Section: Discussionmentioning

confidence: 99%

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Breast fat necrosis secondary to warfarin‐induced calciphylaxis, a rare mimicker of breast cancer: A case report and a review of literature

et al. 2021

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Breast fat necrosis (BFN) is usually a benign inflammatory response to breast trauma. However, an extremely rare cause of fat necrosis is calciphylaxis, a calcification of small‐ and medium‐sized arteries causing thrombosis and ischemia. It is classified into (A) uremic (B) nonuremic‐induced calciphylaxis. Calciphylaxis has been reported to be encountered in different parts of the body. However, to the best of our knowledge there is only one case in the English literature of BFN 2ry to warfarin‐induced calciphylaxis. We report a 65‐year‐old female, known case of atrial fibrillation on warfarin, presented with a left breast mass of 4‐month duration. The mass was painful and progressively enlarging. Examination of the left breast showed 7 × 4 cm mass, spanning from 10‐2 o'clock, free from surrounding structures, with preserved overlying skin. However, the mass was not visualized on mammogram. Ultrasound showed a left breast lobulated hypoechoic mass containing a hyperechoic component. Biopsy showed fat necrosis. After 1 month, she presented with ulceration of the overlying skin. After wide local excision, histopathology demonstrated a calciphylaxis‐induced fat necrosis. Considering the patient's background, the diagnosis was BFN secondary to warfarin‐induced calciphylaxis. Hence, the warfarin was shifted to Rivaroxaban, 6 months follow‐up showed no evidence of recurrence. In conclusion, the rarity of nonuremic calciphylaxis is reflected on the delay of diagnosis in some of the reported cases and the lack of grading system used to guide the management of such difficult wounds. However, keeping a high index of suspicion is important whenever such wounds are encountered with presence of risk factors other than end‐stage kidney disease.

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“…21 Stopping warfarin, vitamin D, calcium supplements and iron intake is recommended by the majority when treating CP patients. 18,22,23 Direct thrombin inhibitors have been shown to be safe and well tolerated in smaller case series and lead to improvement of CP. 24 Another case series with 15 patients observed a positive effect for tissue plasminogen activator as an adjunctive treatment option in CP.…”

Section: Risk Factors and Co-morbiditiesmentioning

confidence: 99%

<p>Management of patients with calciphylaxis: current perspectives</p>

Erfurt‐Berge¹,

Renner²

2019

CWCMR

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Calciphylaxis (CP) is a rare disorder presenting with painful ischemic skin ulcerations. Its association with end-stage renal disease, but also non-uremic cases have been described. Several risk factors, like metabolic syndrome, calcium-phosphate imbalance, anticoagulative medication and female gender have been discussed. Multidisciplinary therapeutic approaches are necessary since evidence-based guidelines are missing due to lack of pathophysiological understanding of the disease and the absence of studies with large patient cohorts. However, strategies for local wound therapy, reduction of risk factors and systemic therapies are being developed. Changes in duration, frequency and method of hemodialysis and specific medication to lower the calcium upload can be effective in uremic CP cases. Systemic treatments with cinacalcet, sodium thiosulfate or bisphosphonates have been applied successfully in CP patients, but large placebo-controlled randomized trials are still needed to gather better insight into this fatal disease.

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Review: update on the management of calciphylaxis

Cited by 21 publications

References 24 publications

Calciphylaxis in end-stage kidney disease: outcome data from the United Kingdom Calciphylaxis Study

Calciphylaxis in end-stage kidney disease: outcome data from the United Kingdom Calciphylaxis Study

Breast fat necrosis secondary to warfarin‐induced calciphylaxis, a rare mimicker of breast cancer: A case report and a review of literature

<p>Management of patients with calciphylaxis: current perspectives</p>

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