Mortality in ANCA-Associated Vasculitis: A Retrospective Study from a Tertiary Vasculitis Center

Abstract

Objectives Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), comprising granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), remains a life-threatening disease despite therapeutic advances.[1] Although immunosuppressive treatments have improved survival, treatment-related complications, particularly infections, are an increasing concern.[2,3] We described the clinical evolution, treatment exposures, cause of death and potential contributors in mortality in patients with GPA or MPA in a real-world tertiary care setting. Methods We conducted a retrospective study of 21 deceased patients with GPA or MPA followed in our vasculitis center between 2001 and 2022. Clinical characteristics, treatment regimens, organ involvement, infections, and causes of death were extracted from medical records. Cumulative prednisone doses were estimated and described in relation to outcomes. Results Among the 21 patients included, 67% had GPA and 33% had MPA. The median follow-up was 986 days. Renal and/or pulmonary involvement occurred in most patients. While 50% of patients had active vasculitis at death, infection was the most common cause of death (52%), exceeding vasculitis-related mortality (19%) (Figure 1). Patients who died from infection had received higher cumulative prednisone doses (median 10.2 grams[g], IQR 4.9–14.1 g) than those who died from other causes (median 5.6 g, IQR 2.5–28.3 g). Long-term glucocorticoid use was associated with metabolic complications and repeated infections. Conclusion Infections, rather than active vasculitis, were the leading cause of death in our cohort. These findings underscore the need for individualized immunosuppressive regimens, judicious steroid tapering, and proactive infection risk mitigation in the management of AAV. Future studies should aim to identify predictors and preventive measures of infection-related mortality, while optimizing steroid-sparing strategies. References [1.] Jennette JC. N Engl J Med 1997;337:1512-23. [2.] Stone JH. N Engl J Med 2010;363:221-32. [3.] Wallace ZS. Semin Arthritis Rheum 2016;45:483-9.