Visceral calciphylaxis

Visceral calciphylaxis is a rare and life-threatening condition characterized by calcification and thrombotic occlusion of small blood vessels supplying internal organs, leading to ischemic tissue injury and organ damage. Unlike the more commonly recognized cutaneous calciphylaxis (calcific uremic arteriolopathy), which primarily affects the skin and subcutaneous tissues, visceral calciphylaxis involves internal organs such as the gastrointestinal tract, lungs, kidneys, mesentery, and other visceral structures. The condition is most frequently seen in patients with end-stage renal disease (ESRD) and is associated with disturbances in calcium and phosphate metabolism, secondary hyperparathyroidism, and the use of calcium-based phosphate binders or warfarin therapy. Clinical manifestations of visceral calciphylaxis depend on the organs involved and may include gastrointestinal bleeding, bowel ischemia and infarction, pulmonary hemorrhage, and multi-organ failure. The diagnosis is often challenging and may require imaging studies showing vascular calcification or histopathological confirmation demonstrating medial calcification and intimal fibrosis of small arterioles. The prognosis is extremely poor, with high mortality rates, often exceeding those of cutaneous calciphylaxis alone. Treatment is largely supportive and aimed at correcting underlying metabolic derangements. Strategies include normalization of calcium-phosphate balance, discontinuation of calcium-based phosphate binders and vitamin D analogs, parathyroidectomy in cases of severe hyperparathyroidism, and administration of sodium thiosulfate, which acts as a calcium chelator and antioxidant. Wound care, nutritional support, and management of sepsis are also critical. Despite these interventions, outcomes remain poor, and early recognition is essential to improve survival.

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