Back
Calciphylaxis is an obliterative vasculopathy, characterized by medial calcification of small arteries and ischemia of the subcutaneous tissue, which can progress to ischemic necrosis of the adipose tissue and dermis. It has poor outcome and ocurr in ESRD and in non-uremic conditions. The pathogenesis of calciphylaxis is poorly understood, the diagnosis is made mainly by clinical manifestations and later confirmed by radiological or histological studies. There is currently no specific therapy for calciphylaxis that has been validated when systematically analyzed.
Male, black patient, 51 years old, with end-stage renal disease (ESRD) of unknown etiology. He had deceased donor kidney transplant in 2012. Kidney graft rejection in 2020, with no conditions for retransplant due to inferior vena cava thrombosis. Patient returned to hemodialysis (HD) through tunneled catheter in the left femoral vein, last access to his survival. At moment, the patient is in palliative care due to failed vascular and peritoneal access. Report of trauma to the right tibial region in September, 2022 that evolved into a painful lesion measuring approximately 4 cm, difficult to heal with progressive increase in size and presence of necrotic tissue, without infection. He was monitored by the committee on methods related to skin integrity, which advised washing the lesions with 0.9% SF and using 10% papain gel and essential fatty acid (EFA) solution twice a day in an attempt to perform chemical debridement. There was partial improvement of the injury, but other painful lesions with diferent sizes appeared in other locations on both legs. Patient was hospitalized due the need for regular intravenous analgesia and difficulty walking. Dermatology made a clinical diagnosis of calciphylaxis without the need for skin biopsy. Patient had bone mineral disease: Calcium: 10.8 mg/dL, phosphorus: 5.6 mg/dL and PTH 929 pg/ml. X-ray of the legs showed difuse vascular calcifications. After discharge, patient continued on HD, failing to achieve dialysis adequacy, HD was intensified: 4 times/week, 4h 30min/session. He used cinacalcet 30mg daily associated with dialisate calcium concentration:2.5 mEq/L. Due to the spontaneous appearance of new painful lesions, worsening of old lesions and poor quality of life of the patient, we started empirical monthly treatment with pamidronate, at an initial dose of 1 mg/Kg (90 mg) IV in April, 2023.
Results
After the first dose of pamidronate, the patient experienced significant improvement in pain and slight improvement in the lesions. After 5 subsequent doses of pamidronate and daily dressing of the lesions with 10% papain gel and EFA solution, the patient showed complete healing of the lesions. In September, 2023, patient does not use regular analgesia and walks without difficulty. Laboratory tests: Calcium 9.9 mg/dL, phosphorus: 2.8 mg/dL and PTH 694 pg/mL.
Treatment of calciphylaxis is based mainly on the correction of alterations of calcium-phosphate metabolism, intensive wound care, dialysate with low calcium and dialysis intensification. Pamidronate may be an effective treatment of calciphylaxis, but randomized trials are needed to confirm the effectiveness of the treatment.