Severe extraossous calcification in chronic kidney disease

Abstract

Background. The most frequent cause of death in patients with chronic kidney disease (CKD) is a cardiovascular event. Disturbances of mineral and bone metabolism result in vascular calcification, which partly accounts for the increased cardiovascular morbidity and mortality. Extraosseous calcification in CKD can produce a range of pathologies including calcific uremic arteriolopathy, extraosseous soft-tissue calcification, calcification involving solid organs, and corneal, conjunctival, peritoneal, vascular, and valvular calcification. In rare instances, calcification can involve all of these structures. Patient. We report a CKD patient with extensive extraosseous calcification and calciphylaxis. A 45-year-old woman, known to have CKD since 2001, presented to us with painful ulcers in both great toes and exertional dyspnea. Results. Investigations revealed severe renal failure, anemia, hypoalbuminemia, hyperphosphatemia, and normal corrected calcium. Her imaging studies showed diffuse uniform calcification of femoral, posterior tibial, radial, ulnar, and digital arteries. Ophthalmological consultation obtained for red eye suggested this condition was a result of conjunctival and corneal calcification. In a plain abdominal x-ray she had visceral calcification, subcutaneous calcification, and calcification of her abdominal arteries. A Doppler study of both upper-and lower-limb arteries was normal. Biopsy from the skin adjacent to the ulcer was suggestive of calciphylaxis. A superficial temporal artery biopsy showed medial calcification and subintimal fibrosis. Her iPTH was 5,560 pg/mL, and ultrasound of the neck revealed diffusely enlarged parathyroid glands. Alcohol ablation was attempted but failed. The patient was treated with daily dialysis, aluminum-based phosphate binder, and aggressive wound care, and a parathyroidectomy was planned. The patient had sudden cardiac death on dialysis.Conclusions. This case is presented to highlight all components of extraosseous calcification in a single patient including soft-tissue, visceral, and vascular calcification, and calciphylaxis.