Outcomes and mortality in calciphylaxis: A multicenter update

Abstract

Background: Current understanding of calciphylaxis pathogenesis and treatment has advanced, with lower mortality.

Objective: To provide an updated report of calciphylaxis risk factors and outcomes in a large and diverse multicenter American validated cohort.

Methods: This was a multicenter, retrospective observational study across 6 major academic institutions. Adult patients with a clinical diagnosis of calciphylaxis from January 1, 2006, to December 31, 2022, were included.

Results: A total of 268 patients were included. Nephrogenic cases comprised 84.62% (209) of patients, while non-nephrogenic cases comprised 15.38% (38) of patients. Seventy-five patients (27.98%) died of calciphylaxis-related causes. One-year and 2-year disease-specific mortality was 28.88% and 29.92%, respectively. Penile lesions (penile vs distal: hazard ratio [HR]: 11.90, 95% CI: 2.44-44.85, P = .04), history of atrial fibrillation (HR: 2.41, 95% CI: 1.36-4.35, P < .01), and dialysis at diagnosis (HR: 2.02, 95% CI: 1.10-3.95, P = .03) were associated with higher mortality, while distal lesions were associated with lower mortality (distal vs proximal: HR: 0.34, 95% CI: 0.18-0.62, P = .04).

Limitations: Limitations of this study included its retrospective nature, limited representation of non-White or Black ethnicities, and outsized representation of 1 academic center.

Conclusion: While differences in survival emerged between institutions, the overall 1-year disease-specific mortality of 28.88% is lower than historic (45% to 80%), suggesting better patient outcomes with time.

Keywords: atrial fibrillation; calciphylaxis; lesion distribution; mortality; multicenter; nephrogenic; non-nephrogenic; outcomes; race.