Calciphylaxis in Patients With Normal Renal Function: A Case Series and Systematic Review

Area of Science:

• Dermatology
• Nephrology
• Pathology

Background:

• Nonnephrogenic calciphylaxis (NNC) is a rare and severe condition.
• Understanding its risk factors and outcomes is crucial for patient management.

Purpose of the Study:

• To define concomitant risk factors, treatment, and outcomes for patients with nonnephrogenic calciphylaxis (NNC).

Main Methods:

• Retrospective review of Massachusetts General Hospital (MGH) medical records.
• Systematic literature review of multiple databases (PubMed, Google Scholar, EMBASE, MEDLINE, CENTRAL).
• Analysis of demographic characteristics, concomitant features, lesion characteristics, outcomes (lesion improvement and mortality), and predictors.

Main Results:

• Nine NNC patients identified via MGH records; 107 identified through literature review.
• Common concomitant factors included vitamin K antagonism and obesity.
• Lower age and higher BMI were associated with central lesions; vitamin K antagonism with peripheral lesions.
• Median survival in MGH series: 24.0 months; 33% lesion improvement by 6 months.
• Median survival in literature review: 4.2 months; median time to lesion improvement: 5.9 months.
• No treatments were associated with lesion improvement or survival in the literature review.

Conclusions:

• Concomitant traits identified may aid in earlier recognition of NNC.
• Further research is needed to investigate NNC pathogenesis and effective treatments.