A case report of severe calciphylaxis – suggested approach for diagnosis and treatment

Abstract

Background: Calciphylaxis is a serious complication in patients with chronic kidney disease associated mineral and bone disorder. It can occur in conditions with low and high bone turnover. So far, there are no definite diagnostic and therapeutic guidelines which may prevent the devastating outcome in many calciphylaxis patients. We report a case which clearly illustrates that knowledge of the underlying bone disorder is essential for a directed treatment. Based on this experience we discuss a systematic diagnostic and therapeutic approach in patients with calciphylaxis. Case presentation: We report a patient with severe calciphylaxis. Initial evaluation showed an elevated serum parathormone concentration and a bone-specific alkaline phosphatase activity in the upper normal range; however, the bone biopsy clearly showed adynamic bone disease. Extended dialysis with low calcium dialysate concentration and citrate anticoagulation, and administration of teriparatide led to a further increase in bone-specific alkaline phosphatase activity and most importantly, resulted in an activated bone turnover as confirmed by a second bone biopsy 11 weeks later. Conclusions: This case illustrates that laboratory tests cannot reliably differentiate between high and low bone turnover in calciphylaxis patients. More importantly, this case highlights the fact that specific therapies that alter bone metabolism cannot be applied without knowledge of the bone status. On this background, we suggest that bone biopsies should be an integral part in the diagnosis and therapeutic decision in these patients and should be evaluated in further studies.

Digital gangrene and pneumatosis intestinalis associated with calciphylaxis

“… 6 Most commonly, these lesions occur in adipose-rich areas, such as trunk, buttocks, and thighs, although acral and penile involvement has also been reported. 7 , 8 , 9 Ocular, cardiovascular, pulmonary, and gastrointestinal system complications are extremely rare, with fewer than 10 reported cases of gastrointestinal involvement in calciphylaxis. 7 , 8 , 10 , 11 , 12 , 13 Risk factors for calciphylaxis include hyperparathyroidism, elevated calcium-phosphorus product, chronic kidney disease, diabetes, female sex, warfarin use, and obesity.…”

“… 7 , 8 , 10 , 11 , 12 , 13 Risk factors for calciphylaxis include hyperparathyroidism, elevated calcium-phosphorus product, chronic kidney disease, diabetes, female sex, warfarin use, and obesity. 9 …”

“…Current mainstays of treatment are mostly supportive, including pain control, wound care, intravenous sodium thiosulfate, correction of calcium and phosphorus levels, bisphosphonates, vitamin K, and full-intensity anticoagulation. 2 Patecki et al 9 recommended bone biopsy in patients with calciphylaxis to dictate therapy after determination of the level of bone turnover. The benefit of kidney transplantation in calciphylaxis remains unclear, and diverging results after renal transplantation have been reported.…”

Treatment of Calciphylaxis in CKD: A Systematic Review and Meta-analysis

“…Although bisphosphonates have been used for the treatment of calciphylaxis, their mechanism of action is unknown, and includes inhibition of calcium crystallization and prevention of hydroxyapatite formation 10, 11. Other treatments described in case reports have included the use of apheresis 12 and tissue plasminogen activator 13, 14. At present, there are no approved treatments for calciphylaxis, and all drug therapies that have been tested fall under the off-label use.…”

3DTEE imaging of a descending aorta floating thrombus in a patient with calciphylaxis

“…Thus, patients present with pain and nonhealing skin ulcers . Furthermore, patients with calciphylaxis can develop vascular calcification of other organs including the heart, the lungs, and the pancreas (visceral calciphylaxis) or calcification of big vessels like the aorta . Calciphylaxis, apart from the impaired renal function, is attributed to many risk factors such as secondary hyperparathyroidism and disturbances in calcium‐phosphate metabolism, inflammatory states, diabetes mellitus, therapy with vitamin K antagonists, obesity, and female gender .…”