The authors of a new review published in the American Journal of Kidney Diseases urge nephrologists, dermatologists, and other clinicians to be vigilant and proactive in recognizing and treating calciphylaxis.
The condition, also called calcific uremic arteriolopathy, is usually associated with chronic kidney disease, especially end-stage kidney disease, and results from the deposition of calcium in skin and adipose. It is difficult to diagnose and can be life-threatening.
According to Leslie Robinson-Bostom, MD, of Brown University in Providence, Rhode Island, and colleagues, “it is imperative that calciphylaxis is always kept in a clinician’s differential when managing patients with kidney disease who present with new painful skin lesions — even in the setting of acute kidney injury. A dermatologic evaluation, including a histopathological analysis, as well the use of radiographic tools, may be necessary to readily diagnose calciphylaxis.”
Calciphylaxis rates have been increasing in the hemodialysis population, the reviewers noted. A US nationwide study estimated an incidence rate of 3.49 per 1000 patient-years among patients on hemodialysis. Secondary hyperparathyroidism promotes bone remodeling and increasing serum calcium levels and may lead to calcification of arterioles, the authors explained. Patients with earlier stages of chronic kidney disease or acute kidney injury can also experience calciphylaxis.
Clinicians should suspect calciphylaxis after the appearance of painful nodules, indurated plaques, dusky livedoid plaques and/or non-blanching retiform purpura on skin, Dr Robinson-Bostom’s team noted. Ulceration increases the risk for death. Skin biopsy, particularly punch biopsy, may be useful for diagnosis. Visceral calciphylaxis, such as in mesenteric and colonic arteries, is also possible without skin manifestation. Plain radiography and ultrasonography may aid diagnosis.
It is important to reduce risk factors for calciphylaxis. According to the reviewers, strategies potentially include withdrawal of warfarin (a vitamin K antagonist) and vitamin D and calcium-based phosphate binders. Attaining target ranges of calcium, phosphorus, and parathyroid hormone is key and may require replacing activated vitamin D with cinacalcet or parathyroidectomy.
According to Dr Robinson-Bostom’s team, management may include cautious surgical debridement, optimal dialysis clearance (by increasing dialysis dose or kidney transplantation), and systemic medical therapies (prioritizing sodium thiosulfate) and analgesia.
For further reference, a team of experts collaborated last year to provide calciphylaxis prevention and treatment strategies.
Marin BG, Aghagoli G, Hu SL, Massoud CM, Robinson-Bostom L. Calciphylaxis and kidney disease: a review. Am J Kidney Dis. Published online August 12, 2022. doi:10.1053/j.ajkd.2022.06.011