Abstract
Background. Calcific uremic arteriolopathy (CUA) or calciphylaxis though generally noted for its high mortality, recent case reports have shown promising results using single agent therapies. However, it is not clear whether combination therapeutic agents will improve course of the disease. Objective. To determine clinical outcome in subjects with CUA on multimodal treatment. Methods. All patients with end-stage renal failure (ESRF) at The Townsville Hospital, Australia, from April 1, 2006, to March 31, 2011, with diagnosis of CUA were retrospectively studied. Results. Six subjects with CUA (4 females and 2 males) were on various combination therapeutic agents comprising sodium thiosulphate, hyperbaric oxygen, prednisolone, cinacalcet, and parathyroidectomy in addition to intensified haemodialysis, specialist local wound care, and antibiotics. The wounds failed to heal in 3 patients while 5 of the 6 subjects died; cause of death being sepsis in 3 and myocardial infarction in 2. Conclusion. Prognosis of CUA remains poor in spite of multimodal combination therapy. Further prospective studies on a larger population are needed to verify our findings.
Highlights
Calcific uremic arteriolopathy (CUA) or calciphylaxis is a syndrome of painful skin necrosis and vascular calcification with high morbidity and mortality
Modalities of treatment of the calciphylaxis apart from wound care and administration of antibiotics in all included cinacalcet in patient 1, 3, and 6; prednisolone was used in patient 3 and 4, while parathyroidectomy was emergently done in patient 2 and 3 with marked hyperparathyroidism and progressive disease despite medical therapy
Knee amputation was performed in only one subject, patient 5, who did not respond to local wound care, antibiotics, hyperbaric oxygen therapy, and administration of sodium thiosulphate
Summary
Calcific uremic arteriolopathy (CUA) or calciphylaxis is a syndrome of painful skin necrosis and vascular calcification with high morbidity and mortality. A common factor linking non-renal-failure-related presentation of the same disease is elevated parathyroid hormone with or without elevated calcium-phosphate product leading to calcification of small vessels The consequences of these are significant mortality of 80% principally from multiple end organ damage due to ischemia and infarction commonly complicated by infection; sepsis being the principal cause of death [1,2,3]. Calcific uremic arteriolopathy (CUA) or calciphylaxis though generally noted for its high mortality, recent case reports have shown promising results using single agent therapies. It is not clear whether combination therapeutic agents will improve course of the disease. Further prospective studies on a larger population are needed to verify our findings
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