BACKGROUND: Calciphylaxis is a rare disorder predominantly seen in patients with chronic kidney disease. Calcification of cutaneous vessels leads to extremely painful plaques and ulcerations. Mortality is high, sepsis being the leading cause of death. There is no standardized treatment. In the last years, administration of sodium thiosulfate (STS) has yielded some promising results. METHODS: We retrospectively collected data of eight calciphylaxis patients treated with STS at the University Hospital of Leuven between June 2009 and June 2014. RESULTS: Eight patients, of whom two without related renal risk factors, received STS treatment in different dosing schemes. Four patients (50%) achieved complete healing of skin lesions; two patients had only stabilization of the disease while experiencing pain relief. In the two remaining patients who had further progression of the disease, STS treatment led to analgesia in one patient. Overall, seven patients (87.5%) had some benefit from STS treatment. However, four patients (50%) eventually died due to causes related to calciphylaxis. STS was generally well tolerated, although almost all patients (87.5%) had metabolic acidosis. CONCLUSIONS: Treatment of calciphylaxis remains challenging. Although STS therapy, together with other treatment modalities, seems to improve disease outcome, more studies focusing on optimal dosing schemes and duration of treatment are necessary.
BACKGROUND:Calciphylaxis is a rare disorder predominantly seen in patients with chronic kidney disease. Calcification of cutaneous vessels leads to extremely painful plaques and ulcerations. Mortality is high, sepsis being the leading cause of death. There is no standardized treatment. In the last years, administration of sodium thiosulfate (STS) has yielded some promising results. METHODS: We retrospectively collected data of eight calciphylaxispatients treated with STS at the University Hospital of Leuven between June 2009 and June 2014. RESULTS: Eight patients, of whom two without related renal risk factors, received STS treatment in different dosing schemes. Four patients (50%) achieved complete healing of skin lesions; two patients had only stabilization of the disease while experiencing pain relief. In the two remaining patients who had further progression of the disease, STS treatment led to analgesia in one patient. Overall, seven patients (87.5%) had some benefit from STS treatment. However, four patients (50%) eventually died due to causes related to calciphylaxis. STS was generally well tolerated, although almost all patients (87.5%) had metabolic acidosis. CONCLUSIONS: Treatment of calciphylaxis remains challenging. Although STS therapy, together with other treatment modalities, seems to improve disease outcome, more studies focusing on optimal dosing schemes and duration of treatment are necessary.