Successful Treatment of Non-Uremic Calciphylaxis with Pamidronate and Sodium Thiosulfate

Program: Abstracts - Orals, Poster Previews, and Posters
Session: FRI 368-390-Metabolic Bone Disease Case Reports (posters)
Clinical
Friday, April 1, 2016: 1:15 PM-3:15 PM
Exhibit/Poster Hall (BCEC)

Poster Board FRI 372
Kacy Church*1, Chrysoula Dosiou2, Tammy Sirich3 and Jennifer S. Lee4
1Stanford University Medical Center, Stanford, CA, 2Stanford University School of Medicine, Stanford, CA, 3Stanford University Medical Center, 4Stanford University Medical Center VA Palo Alto Health Care System
Background:  Calciphylaxis involves subcutaneous arteriolar calcification and painful overlying cutaneous necrosis, typically occurring in individuals with end-stage renal disease and secondary hyperparathyroidism. The mortality is high at 50-80%. The mainstays of therapy are wound care and sodium thiosulfate (STS), with or without bisphosphonate and/or hyperbaric oxygen. Less recognized is non-uremic calciphylaxis (NUC), for which there is no known effective pharmacological treatment. STS has produced mixed results, and there has been only one case report of successful treatment with a bisphosphonate.  We describe a patient with NUC, which resolved with the addition of weekly pamidronate to STS.

Clinical Case:  A 65-year old Caucasian man presented with new hypercalcemia and a painful 1.2-cm area of indurated, dusky plaque with central necrosis on his posterior left calf. The lesion expanded the week prior despite antibiotics. He had type 2 DM, HTN, CKD stage 3, obesity, CHF, paroxysmal afib, and OSA on CPAP. His medications included insulin, losartan, metoprolol, pravastatin, amiodarone, and warfarin. A skin punch biopsy showed perivascular calcium deposits in small vessels around adipose tissue and non-inflammatory thrombi, consistent with calciphylaxis. On admission, his corrected calcium was 13.3 mg/dl, creatinine was 2.5 mg/dL (baseline of 1.5), with normal phosphate, an appropriately low PTH of 4 pg/ml, a normal PTHrP, a normal 25-OH vitamin D, an elevated 1,25-OH vitamin D of 92 pg/ml, and HbA1c of 7.6%. CRP was elevated at 1.83 mg/dl and peaked to 2.02 mg/dl (n<1 mg/dl).  Frequent wound care and STS infusion (12.5 g every other day) were started. Evaluation of hypercalcemia revealed numerous non-caseating granulomas on bone marrow biopsy, which was deemed to be mostly likely due to amiodarone. Amiodarone was discontinued, and with isotonic fluids and a single dose of pamidronate (30 mg IV), hypercalcemia resolved within a few days. The left calf lesion improved by discharge 2 weeks later, but its size increased and a new skin lesion appeared on his right calf after 5 weeks of STS and wound care, Therefore, weekly pamidronate (30 mg IV) was initiated, resulting in normalization of CRP after 3 months and gradual resolution of all skin lesions at 5.5 months after initial presentation. After the skin lesions resolved, STS was continued for 2 weeks (a total of 6 months) and pamidronate for another 4 weeks (a total of 5 months)  The patient has remained normocalcemic and without skin lesions one year after conclusion of all therapy. 

Conclusion: This patient’s presentation of calciphylaxis is notable for the atypical features: non-uremia and hypercalcemia (without hyperparathyroidism) likely secondary to a complication of amiodarone use. In this setting, we observed that weekly pamidronate in conjunction with STS was more effective in managing NUC than STS alone.

Nothing to Disclose: KC, CD, TS, JSL

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