Researchers in Italy have reported on a case series showing favorable results with a multimodal intervention for treating calciphylaxis, a rare but potentially life-threatening syndrome for which no established treatment protocol exists.

Calciphylaxis, also known as calcific uremic arteriolopathy (CUA), is characterized by progressive calcification of arterioles leading to local ischemia and thrombosis. It is often diagnosed only at the advanced, ulcerative stage rather than at the nodular stage characterized by non-ulcerated calcified skin plaques, investigators led by Domenico Russo, MD, of the University of Naples, explained in paper published in Clinical Kidney Journal (2016;9:108-112). Hyperparathyroidism, excessive vitamin D administration, hyperphosphatemia, inflammation, and deficiency of inhibitors of vascular calcification are contributing factors.

The intervention is based on wound care, antibiotics, and surgical debridement, and combined use of sodium thiosulfate and and cinacalcet. Dr. Russo’s team used the intervention to treat 5 dialysis patients (4 women and 1 man) with advanced late-diagnosed calciphylaxis. All patients had severe hyperparathyroidism. The treatment was associated with full regression of skin lesions in 4 cases after variable treatment protocols ranging from 4–33 months, according to the investigators.

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“This favourable clinical course paralleled with almost full regression of hyperparathyroidism,” the researchers wrote. “The patient unresponsive to this multimodal therapy was overtly non-compliant and presented unabated hyperparathyroidism across a 30-month follow-up.”

Dr. Russo’s group concluded that the present case series further confirms that the diagnosis of CUA is dialysis patients is rarely made at the nodular, non-ulcerative phase of the disease. “This series adds to previous observations and contributes to the information build-up that will hopefully serve as a basis for well-conceived comparative effectiveness studies investigating the value of the combined interventions applied so far.”

The study “raises hopes for a more effective treatment of CUA, although the results have to be validated within bigger studies,” the journal’s editor-in-chief, Alberto Ortiz, MD, said in a statement from the European Renal Association-European Dialysis and Transplant Association, which publishes the journal.

At Kidney Week 2015 in San Diego, researchers who conducted a retrospective study of 45 hemodialysis patients with biopsy-confirmed calciphylaxis reported that sodium thiosulfate may confer a survival advantage. Of the 45 patients, 23 (51%) received sodium thiosulfate and 22 (49%) did not. The 1-year mortality rate was 22% in the sodium thiosulfate recipients compared with 50% in those who did not receive the drug, investigators Chamberlain I. Obialo, MBBS, and Alexander Quarshie, MbChB, of the Morehouse School of Medicine in Atlanta, reported.