Calciphylaxis in the Absence of Renal Disease: Secondary Hyperparathyroidism and Systemic Lupus Erythematosus

Abstract

To the Editor: Calciphylaxis is a rare disorder characterized by calcification of small and medium-size arteries, causing thrombosis, tissue necrosis, and painful, nonhealing skin ulcerations. The preponderance of cases occurs in patients undergoing dialysis due to endstage renal disease. Prognosis is generally poor, with one-year mortality ranging from 45% to 80%1,2. A recent report noted that even among nondialysis patients, renal function was moderately to severely impaired in 69% [glomerular filtration rate (GFR) < 40 ml/min]1. Autoimmune or inflammatory conditions were present in 60%, including systemic lupus erythematosus (SLE), polymyositis, sarcoidosis, hepatitis, ulcerative colitis, rheumatoid arthritis, Sjogren’s syndrome, and pemphigus. Various other risk factors are documented, including hyperparathyroidism, elevated serum calcium and phosphate, hypoalbuminemia, obesity, diabetes mellitus, and female sex, and use of warfarin, vitamin D3 analogs and calcium-containing phosphate binders1,2. Couto, et al described a patient with secondary hypoparathyroidism due to vitamin D deficiency, with low calcium/phosphorus product, who developed calciphylaxis in the absence of endstage renal disease3. Case reports of calciphylaxis with normal calcium/phosphorus product … Address correspondence to J.J. Zechlinski; E-mail:jzechlin{at}mcw.edu