Mortality Prediction in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis With Kidney Involvement: Validation of the DANGER Score.

Background/objective: The DANGER (Death in ANCA Glomerulonephritis-Estimating the Risk) score was developed to assess mortality risk in patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV). This study aimed to validate score in a cohort of Latin American patients.

Methods: This cohort study included patients with AAV evaluated between 2000 and 2022. The DANGER score was calculated, and its performance evaluated using the c-statistic and time-dependent area under the receiver operating characteristic curve. Multivariable Cox regression analysis was performed to identify variables that could enhance the score's predictive accuracy.

Results: We included 154 patients, 104 (68%) female, with a median age of 52 years (interquartile range [IQR], 38-61 years) and creatinine of 2.5 mg/dL (IQR, 1.7-2.5 mg/dL). Over a median follow-up of 74 months (IQR, 32-126 months), 24 patients died, with mortality rates of 6.5%, 8.6%, and 11.9% at 1, 2, and 5 years, respectively. The leading cause of death was infection. Mortality rates at 1 and 3 years in the low-, intermediate-, and high-risk categories were 1.0% and 3.1%, 14.0% and 16.8%, and 40.0% and 70.0%, respectively. The overall c-statistic for the DANGER model was 0.81 (95% confidence interval [CI], 0.73-0.90), with areas under the receiver operating characteristic curve of 0.81 (95% CI, 0.70-0.91), 0.78 (95% CI, 0.67-0.89), and 0.80 (95% CI, 0.70-0.90) at 1, 3, and 5 years, respectively. A revised model incorporating age, creatinine, C-reactive protein, and pulmonary involvement had a c-statistic of 0.86 (95% CI, 0.79-0.94).

Conclusions: The DANGER score has good predictive accuracy for mortality in AAV patients with kidney involvement. In younger patients, the score may be modified to include variables such as C-reactive protein and severe pulmonary involvement to enhance its performance.