Nephrogenic systemic fibrosis: A 15-year retrospective study at a single tertiary care center.

BACKGROUND: Despite multiple therapeutic approaches for nephrogenic systemic fibrosis (NSF), no single treatment has convincingly shown consistent benefit. The most successful outcomes have been associated with recovery of renal function, although evidence remains limited and past studies have been inconclusive.

OBJECTIVE: We sought to investigate whether improvement of renal function via successful transplantation or via return of renal function after acute kidney injury correlates with improvement of NSF, and to further characterize the clinical features and progression of NSF.

METHODS: A retrospective medical chart review led to the identification of patients (n = 8) diagnosed with NSF who presented to a single academic tertiary referral center over a 15-year period. These 8 patients were contacted by phone to obtain information related to treatment and clinical course of their NSF and renal function. Statistical analysis was performed using Fisher's exact test.

RESULTS: There is a significant correlation (P = .0286) of improved renal function with improvement of NSF. All 4 patients who had improvement of renal function also had improvement of NSF. Two of these patients had end-stage renal disease and a successful kidney transplant, and two had acute kidney injury that resolved. No improvement in NSF was observed without kidney function resolution.

LIMITATIONS: Our study is limited by a small sample size (n = 8) and a retrospective study design, which increased its potential for selection and recall bias.

CONCLUSION: Improvement of renal function through either transplantation or resolution of acute kidney injury with medical management is significantly associated with improvement of NSF.