Calciphylaxis in end-stage renal disease prior to dialytic treatment: a case report and literature review

Abstract

IntroductionCalciphylaxis is a rare medical condition that is usually diagnosed in patients suffering from end-stage renal disease who are already receiving renal replacement therapy and in those post-transplantation. The pathogenesis still remains to be fully elucidated; hence, the treatment is not uniform. The prognosis is generally poor. The ulcerative stage exhibits a worse prognosis than the nonulcerative one. Calciphylaxis presenting in terminal kidney disease prior to dialytic treatment has only rarely been reported.Case presentationA 32-year-old female Caucasian clerk sought medical attention for increasing tiredness and lower limb skin ulcers. Polycystic kidney disease was diagnosed in her late father and two of her siblings. At the first nephrology consultation, obesity, pallor, bilateral flank masses with ballottement, and two ulcers with a dark necrotic center on the distal left leg were noted. In addition, another indurated light bluish lesion of 5 cm just above the right knee with intact skin was observed. All lesions were very tender and warm on touch. Laboratory results yielded hypercalcemia, hyperphosphatemia, anemia, and parathyroid hormone levels that were more than ten times the normal values in the patient, and with a glomerular filtration rate of 4 mL/minute. Skin biopsy confirmed the suspicion of calciphylaxis. The patient was placed on peritoneal dialysis with low Ca concentration baths, cinacalcet, and aluminum hydroxide. The results included correction of hypercalcemia, improvement of phosphate levels, and the product of both Ca and phosphate, but only a transitory decrease in serum parathyroid hormone levels. The ulcerations were completely healed after 2 months of treatment. Cinacalcet was discontinued after 18 months, but multiple large-size, nonulcerative indurated areas appeared 3 months later in the lower limbs after discontinuation of the drug. A parathyroidectomy performed 17 months later revealed a four-gland hyperplastic disease. The patient experienced relief of skin symptoms soon after the procedure and remains in a very satisfactory condition.ConclusionCalciphylaxis is a very complex clinical entity. Calciphylaxis presenting prior to dialytic treatment in end-stage renal disease is rare in the absence of a trigger. Cinacalcet and parathyroidectomy should be considered in selected patients.

Calciphylaxis due to metastatic well-differentiated neuroendocrine carcinoma

“…For treatment-resistant patients, bisphosphonates and hyperbaric oxygen are also considered. Cinacalcet and parathyroidectomy can also be used in selected cases especially in the setting of end stage renal disease 15…”

Gastrointestinal Bleeding Secondary to Calciphylaxis

“…However, the proposed mechanism is that the presence of hyperparathyroidism, hyperphosphatemia, and high calcium-phosphate product in the uremic milieu down-regulates the inhibitors of vascular calcification. The ensuing vascular calcification leads to vasoconstriction, which ultimately leads to decreased distal perfusion and ischemic manifestations [ 12 ]. Secondary hyperparathyroidism and disturbances in the metabolism of calcium and phosphate are thought to be the risk factors for calciphylaxis; therefore, it is more prevalent in patients with chronic renal failure on hemodialysis [ 1 ].…”

“…Supportive treatment is the mainstay of management of systemic calciphylaxis [ 12 ]. Sodium thiosulphate has emerged as a novel treatment modality for the management of calciphylaxis.…”

“…Pamidronate has also been used for managing calciphylaxis [ 16 – 18 ]. Hyperbaric treatment has been tried in patients to assist with wound healing [ 19 ], and parathyroidectomy has also been attempted [ 9 , 12 ]. Normalization of the abnormal calcium and phosphorus levels can also be helped by diet, binding agents, and low-calcium bath dialysate [ 9 ].…”

The use of cinacalcet for the treatment of calciphylaxis in patients with chronic kidney disease: A comprehensive review

“…Calciphylaxis typically affects end‐stage renal failure patients undergoing haemodialysis or those who have received a kidney transplant. It carries significant morbidity from severe pain, surgical resections and amputations and has a high mortality rate (60–80%); mainly secondary to gangrene and sepsis …”