Isolated Mesenteric Calciphylaxis With Ischemic Colitis in a Hemodialysis Patient Without Active Cutaneous Calciphylaxis: A Case Report of Calcific Uremic Arteriolopathy

Abstract

Calciphylaxis, also known as calcific uremic arteriolopathy, is a devastating systemic disease most commonly associated with chronic kidney failure. Its hallmark histopathologic features of small-vessel calcification, intimal hyperplasia, and microthrombi lead to microvascular occlusion and tissue necrosis. Clinically, it typically presents with painful cutaneous lesions that may be distal or proximal, with proximal lesions associated with higher mortality. Visceral involvement in this disease process is rare and in such case reports, all patients have coincident active cutaneous lesions. We present a case of a man in his 40s receiving hemodialysis presenting with mesenteric calciphylaxis complicated by ischemic colitis without active cutaneous lesions. Treatment consisted of sodium thiosulfate, vitamin K, and surgical resection. He previously had penile calciphylaxis treated with 3 months of sodium thiosulfate therapy and optimization of his serum calcium, phosphate, and parathyroid hormone levels. His penile calciphylaxis healed 12 months before his presentation with mesenteric calciphylaxis. This is the first known case report of isolated mesenteric calciphylaxis. It raises a number of clinical dilemmas, including duration of sodium thiosulfate use, monitoring for disease activity, and suitability for future kidney transplantation.

Calciphylaxis and Kidney Disease: A Review

“…31 Visceral calciphylaxis without primary cutaneous manifestations has also been reported, such as in mesenteric and colonic arteries . 32,33 A recent retrospective chart review of 145 patients identified that in 22 patients, the first calciphylaxis lesions appeared in areas of previous trauma including direct injury from hitting an object (n = 4), abrasions (n = 3), insulin injections (n = 3), mechanical falls (n =3), toe clipping or stubbed toe (n = 2), catheter placement (n = 1) and peritoneal dialysis catheter removal (n = 1). 34 These findings support the hypothesis that calciphylaxis may be associated with Koebnerization, a phenomenon in which dermatologic lesions emerge in areas of cutaneous trauma in otherwise intact skin.…”

“…63 Another study of 53 hemodialysis-dependent patients with J o u r n a l P r e -p r o o f 12 calciphylaxis who were treated with intravenous sodium thiosulfate showed that 26% of the patients had complete resolution and 19% had marked improvement in their skin lesions. 32 Additionally, a recent case report presented management of cutaneous calciphylaxis with smalldose fractionated sodium thiosulfate in a patient with peritoneal dialysis. 64 Intravenous STS has also demonstrated therapeutic success for calciphylaxis in patients with normal kidney function.…”

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“…Visceral calciphylaxis without primary cutaneous manifestations has also been reported, such as in mesenteric and colonic arteries [14,15]. The one-year mortality in calciphylaxis patients is reported at 45-80% with ulcerated lesions associated with higher mortality compared to non-ulcerated lesions.…”

"Exploring Novel Therapeutic Strategies for Calciphylaxis: A Focus on Vitamin K2 Supplementation"

“…Visceral calciphylaxis without primary cutaneous manifestations has also been reported, such as in mesenteric and colonic arteries [14,15]. The one-year mortality in calciphylaxis patients is reported at 45-80% with ulcerated lesions associated with higher mortality compared to non-ulcerated lesions.…”