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Isolated Mesenteric Calciphylaxis With Ischemic Colitis in a Hemodialysis Patient Without Active Cutaneous
Rachel Yi Ping Tan1,2, Rajiv Juneja1,2, Dimuth Nilanga Gunawardane3
1Renal Unit, Flinders Medical Centre, South Australia, Australia.
Insights
Calciphylaxis, or calcific uremic arteriolopathy, is a severe kidney disease complication. This case highlights isolated mesenteric calciphylaxis, a rare form without skin lesions, presenting treatment and transplant challenges.
Area of Science:
- Nephrology
- Vascular Biology
- Gastroenterology
Background:
- Calciphylaxis (calcific uremic arteriolopathy) is a severe systemic disease linked to chronic kidney failure.
- Histopathology reveals small-vessel calcification, intimal hyperplasia, and microthrombi, causing tissue necrosis.
- Typically presents as painful cutaneous lesions; visceral involvement is rare and usually co-occurs with skin lesions.
Observation:
- A case report of a male patient undergoing hemodialysis who presented with mesenteric calciphylaxis and ischemic colitis.
- Notably, this patient had no active cutaneous lesions, distinguishing it from previously documented cases of visceral calciphylaxis.
- The patient had a history of penile calciphylaxis, which had healed 12 months prior to the mesenteric presentation.
Findings:
- This case represents the first documented instance of isolated mesenteric calciphylaxis.
- Treatment involved sodium thiosulfate, vitamin K, and surgical resection of the affected mesenteric tissue.
- The patient's prior penile calciphylaxis was treated with sodium thiosulfate and management of mineral and bone disorder parameters.
Implications:
- This case highlights the potential for calciphylaxis to manifest in visceral organs without cutaneous involvement, posing diagnostic challenges.
- It raises critical questions regarding optimal duration of sodium thiosulfate therapy, methods for monitoring disease activity, and the implications for kidney transplant eligibility.
- Further research is needed to understand the pathophysiology and management of isolated visceral calciphylaxis.
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.
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