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Multi-site Calciphylaxis With Pulmonary Calcification in a Young Haemodialysis Patient
Vismaya Pillai1, Charles Hall1
1Nephrology, Royal Free Hospital, London, GBR.
Abstract:
Calciphylaxis is a rare and life-threatening complication of end-stage renal failure (ESRF), typically presenting with painful cutaneous necrosis but occasionally involving the visceral organs. Metastatic pulmonary calcification (MPC) results from calcium deposition within the alveolar walls due to severe derangements in mineral metabolism, whereas pulmonary calciphylaxis is even rarer and characterised by vascular calcification with thrombosis and tissue ischaemia. We describe a 34-year-old man with congenital horseshoe kidney and recurrent papillary urothelial carcinoma who developed ESRF secondary to obstructive uropathy and demonstrated profound haemodialysis (HD) non-adherence. He presented, following three weeks of missed dialysis, with lethargy, weakness, and severe hyperkalaemia (8.6 mmol/L). Within days, he developed painful violaceous plaques on the thighs and penile shaft, which rapidly progressed to necrosis, consistent with aggressive calciphylaxis. He subsequently developed breathlessness and cough; CT pulmonary angiography showed bilateral ground-glass opacities with septal thickening. Despite antibiotics, opacities persisted, and bone scintigraphy revealed increased tracer uptake in the lungs and subcutaneous tissues, supporting a diagnosis of MPC rather than pulmonary calciphylaxis. Management included intensified HD with low-calcium dialysate, intravenous sodium thiosulphate, non-calcium phosphate binders, analgesia, and anticoagulation for pulmonary embolism. Although oxygenation improved, cutaneous and penile lesions remained painful with poor healing. Given extensive calcification, malignancy, and dialysis dependence, he was referred to palliative care. This case highlights the diagnostic role of bone scintigraphy in distinguishing MPC from pulmonary calciphylaxis and underscores the importance of HD adherence and multidisciplinary management in preventing fatal outcomes.
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