A New Vasculitis Activity Score for Predicting Death in Myeloperoxidase-Antineutrophil Cytoplasmic Antibody-Associated Vasculitis Patients

Abstract

Background/Aims: Myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA)-positive microscopic polyangiitis patients with renal involvement have been shown to have a progressive clinical course. In this study, we compared the clinical utility of the Japanese Vasculitis Activity Score (JVAS) with the Birmingham Vasculitis Activity Score (BVAS) for predicting death in patients with MPO-ANCA-associated renal involvement. Methods: Sixty-nine patients with MPO-ANCA-associated vasculitis with renal involvement (22 males and 47 females, age 69.8 ± 8.7 years) were enrolled in this study. We retrospectively investigated which score was better for predicting the poor prognosis of patients. Results: The mortality rate of the patients within 2 years after disease onset was 33% (23/69). JVAS was not correlated with BVAS. Univariate logistic regression analysis for death showed that the odds ratio (OR) of JVAS was statistically significant (OR 1.76, 95% confidence interval, CI, 1.29–2.41, p < 0.001), while that of BVAS was not (OR 1.07, 95% CI 0.98–1.16, p = 0.14). Moreover, a multivariate model showed that JVAS was an independent determinant of death (OR 1.59, 95% CI 1.12–2.25, p = 0.009). The area under the receiver operating characteristic curve for JVAS was 0.778, which was significantly larger (p = 0.02) than that for BVAS (0.586). The estimated optimal cut-off point of JVAS for the prediction of death was 5. At this point, the sensitivity was 82.6% and the specificity was 60.9%. Conclusion: We demonstrated that compared with BVAS, JVAS was a simpler and more reliable measure for predicting death in patients with MPO-ANCA-associated vasculitis with renal involvement.

Respiratory manifestations of ANCA‐associated vasculitis

“…This study demonstrates the importance of investigating for pulmonary manifestations of AAV in all patients, regardless of disease type or severity. While previous studies have focused on one particular condition within the spectrum of AAV , this study has shown that this is a significant problem even in those conditions which have not been classically associated with pulmonary involvement. The results demonstrate that such complications could be associated with an adverse effect on mortality.…”

“…This study goes further in that it has also incorporated patients with CSS and MPA, giving an overall idea of the effects of pulmonary involvement of AAV, rather than being limited to one condition. The effect of pulmonary vasculitis on mortality was also considered in a recent trial of a vasculitis activity score in patients with MPA . This study compared the Birmingham Vasculitis Activity Score (BVAS) with the Japanese Vasculitis Activity Score (JVAS) and found respiratory involvement to be a greater predictor of death.…”

“…This study compared the Birmingham Vasculitis Activity Score (BVAS) with the Japanese Vasculitis Activity Score (JVAS) and found respiratory involvement to be a greater predictor of death. In comparison to BVAS, which uses involvement of nine organ systems, JVAS uses four parameters: age, serum creatinine, serum CRP and the presence of a pulmonary lesion . In this score, the importance placed on the presence of pulmonary involvement and its effectiveness in predicting mortality suggests that pulmonary vasculitis may have a profound effect on mortality.…”

Comparison of severity classification in Japanese patients with antineutrophil cytoplasmic antibody-associated vasculitis in a nationwide, prospective, inception cohort study

“…The overall and ESRD-free survival rates significantly differed between grades I/II, III, and IV in this study even though some patients did not have renal involvement. A previous report also showed that score based on this system was a good predictor of mortality in patients with MPO-AAV [19]. As for the EUVAS-defined disease severity, severe disease was a good predictor of 6-month overall and ESRD-free survival, but the difference of these outcomes was not apparent among limited, early systemic, and generalized diseases.…”

Simple, readily available clinical indices predict early and late mortality among patients with ANCA-associated vasculitis

“…Bakoush and coworkers followed 83 patients; neither survival nor ESKD was predicted by BVAS in their cohort, with less severe renal failure compared to our patients [ 9 ]. In Japanese patients with MPO-ANCA disease, no association was found between BVAS and mortality during the two years follow-up [ 20 ]. In another investigation there was no difference between the baseline BVAS of survivors and non-survivors; baseline BVAS did not, but BVAS at 1 and 3 months predicted survival [ 21 ].…”