VASCULAR CALCIFICATION IN PATIENTS WITH KIDNEY DISEASE: Calcific Uremic Arteriolopathy: Advances in Pathogenesis and Treatment

Abstract

Calcific uremic arteriolopathy (CUA) is a rare but serious life-threatening complication of CRF that manifests as painful nonhealing eschars in association with panniculitis and dermal necrosis. This condition is being increasingly recognized and reported as a contributing factor to death in dialysis patients. The pathognomic lesion is vascular calcification with intimal arterial hypertrophy and superimposed small vessel thrombosis. Hyperparathyroidism and elevated concentrations of serum phosphate remain consistent clinical features of most cases reported. Controversy still exists regarding the role of parathyroidectomy in this condition with some studies suggesting improved outcome with surgical intervention. A number of potential new etiological factors have been identified including reduced serum levels of a calcification inhibitory protein alpha,2-Heremans-Schmid glycoprotein (Fetuin-A) and abnormalities in smooth muscle cell biology in uremia. Promising new treatment options including hyperbaric oxygen therapy and sodium thiosulfate infusion have been reported in case series. Benefits from biphosphonates and tissue plasminogen activator have also been reported. Overall these new treatment approaches and understanding of potential mechanisms underlying this important severe clinical condition offer new hope in the diagnosis and management of this severely morbid and often fatal condition.

Patient Outcomes and Factors Associated with Healing in Calciphylaxis Patients Undergoing Adjunctive Hyperbaric Oxygen Therapy

“…10 However, neither gender nor the number of treatments received have been reported as prognostic factors in overall healing. In our study, six patients were female and 2 were male.…”

“…In two of the largest case series reported, Podymow et al had four out of five patients who were female and Basile et al had six out of eleven patients who were female. 10,11 In both of these studies, all patients received at least twenty HBO 2 treatments. In our study, the majority of the patients were females and this is consistent with what has been reported in the literature.…”

“…7 The benefits of HBO 2 in the treatment of calciphylaxis have been reported in many case reports and a few retrospective case series. 2,[8][9][10] In Podymow et al 2 out of 5 patients showed complete resolution of extensive necrotic ulcers and improvement in wound area transcutaneous oxygen pressure. 2 Dean and Werman report a case of a female patient with end-stage renal disease and diabetes mellitus who presented with lower extremity calciphylaxis who responded to 7 weeks of HBO 2 treatments after failing to respond to standard care including parathyroidectomy and wound debridement.…”

Femenina de 52 años con arteriolopatía cálcica urémica.

“…Aparte de dichos factores de riesgo, los análogos de vitamina D y la terapia de sustitución con hierro se han descrito como contribuyentes al desarrollo de la calcifilaxis (3) .…”

Cardiomyopathy and Calciphylaxis in a Patient With Normal Renal Function: A Case Report

“…3 Risk factors associated with nonuremic calciphylaxis include obesity, rapid weight loss, liver disease, malnutrition, protein C and protein S deficiency, primary hyperparathyroidism, connective tissue diseases, malignancies, polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes (POEMS) syndrome, vitamin D deficiency, systemic corticosteroid use, and warfarin therapy. [2][3][4] We found only one reported case of cardiomyopathy associated with calciphylaxis and normal kidney function. 5 …”