Abstract

Calciphylaxis is a life threatening disease for which patients with chronic kidney disease, particular those on dialysis, are at increased risk. As it is a rare condition, treatment options are limited and duration of treatment is not clear, especially when it comes to patients on renal replacement treatment with peritoneal dialysis.
 We report a case of a 55 year-old woman with end stage renal disease on renal replacement treatment with peritoneal dialysis for five months that presented with severe calciphylaxis of both legs. She had started haemodialysis at the age of 31 and 3 later she received a cadaveric renal transplant that lasted for 20 years. She was treated with warfarin due to atrial fibrillation diagnosis. The treatment approach was multimodal and multidisciplinary and included treatment with sodium thiosulfate and hyperbaric oxygen, warfarin suspension and maintenance of optimized peritoneal dialysis prescription. Additionally, wound dressings were done every other day and antibiotics started whenever there were signs of infection and pain was managed with high doses of opiates and pain adjuvants. There was significant improvement and the patient kept peritoneal dialysis treatment with preserved residual renal function. This raises the question, if there is a need to transition calciphylaxis patients on peritoneal dialysis to haemodialysis, as it is possible to ensure a high efficiency dialysis program with automated peritoneal dialysis.

Highlights

  • La calciphylaxie est une affection rare et potentiellement mortelle résultant de la calcification et de la thrombose des petits et moyens vaisseaux du tissu adipeux souscutané et du derme, conduisant à l’ischémie tissulaire et à la nécrose, avec ulcères extrêmement douloureux et étendus pouvant entraîner des infections sévères et le décès [1].Le diagnostic repose sur la biopsie cutanée, bien qu’une forte suspicion clinique puisse suffire [2-3]

  • Summary Calciphylaxis is a life threatening disease for which patients with chronic kidney disease, particular those on dialysis, are at increased risk. As it is a rare condition, treatment options are limited and duration of treatment is not clear, especially when it comes to patients on renal replacement treatment with peritoneal dialysis

  • We report a case of a 55 year-old woman with end stage renal disease on renal replacement treatment with peritoneal dialysis for five months that presented with severe calciphylaxis of both legs

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Summary

INTRODUCTION

La calciphylaxie est une affection rare et potentiellement mortelle résultant de la calcification et de la thrombose des petits et moyens vaisseaux du tissu adipeux souscutané et du derme, conduisant à l’ischémie tissulaire et à la nécrose, avec ulcères extrêmement douloureux et étendus pouvant entraîner des infections sévères et le décès [1]. Des taux sanguins élevés de PTH sont liés au développement de la calciphylaxie, mais environ 45% des patients atteints présentent des taux de PTH sanguins inférieurs aux recommandations au moment du diagnostic (possiblement liés à l’utilisation de médicaments à base de phosphate de calcium et de vitamine D). L’utilisation d’anticoagulants antagonistes de la vitamine K augmente le risque de 3 à 13 fois ; environ 50% des patients atteints de calciphylaxie urémique ont été traités par ces médicaments [1,8]. Certaines études semblent montrer une incidence et un risque plus élevés de calciphylaxie chez les patients en dialyse péritonéale par rapport aux patients hémodialysés (9 cas versus 3,5 cas par millier d’annéespatients dans l’une des rares revues de cas portant sur ce sujet) [1,8], probablement liés à l’incidence plus élevée d’hypoparathyroïdie et d’hyperphosphatémie et à un bilan calcique positif lié aux solutions de dialyse péritonéale. Les meilleures options thérapeutiques pour les patients en dialyse péritonéale atteints de calciphylaxie, et la justification de les transférer en hémodialyse pour optimiser le contrôle du phosphore et du calcium restent incertaines [9]

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