Analysis of the clinical course, complications and causes of mortality at the onset of the primary necrotizing systemic vasculitis

BACKGROUND: Primary necrotizing systemic vasculitis is a significant problem of practical healthcare due to a severe progressive clinical course and a high risk of complications, including life-threatening damage. AIM: To evaluate the features of the clinical course of primary necrotizing systemic vasculitis during the first 3 years of the disease. MATERIALS AND METHODS: The study included 232 patients with primary necrotizing systemic vasculitis, who were hospitalized in Saint Petersburg clinics from 2010 to 2018. The features of the clinical course, complications, causes of mortality during the first 3 years of the disease were assessed. RESULTS: The cohort of patients with рrimary necrotizing systemic vasculitis consisted of patients with granulomatosis with polyangiitis (n = 94), microscopic polyangiitis (n = 46), eosinophilic granulomatosis with polyangiitis (n = 69), polyarteritis nodosa (n = 23). The highest values of the Birmingham Vasculitis Activity Score index were noted among patients with granulomatosis with polyangiitis and microscopic polyangiitis (21.4 and 21.5 points, respectively). Patients with microscopic polyangiitis were significantly more likely to develop chronic kidney disease (C3aC5 stages) compared to other primary necrotizing systemic vasculitis (p 0.001). We established that the average value of the Vasculitis Damage Index after 36 months of the disease was highest in the group of patients with granulomatosis with polyangiitis (4.7 points) and significantly exceeded the corresponding indicator at the same period of the disease in the group of patients with microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis, polyarteritis nodosa (3.8, 2.7 and 3.1 points, respectively). CONCLUSIONS: The most unfavorable prognosis among primary necrotizing systemic vasculitis during the first 3 years of the disease was noted in patients with granulomatosis with polyangiitis, which is reflected in the high level of the Vasculitis Damage Index that shows the number of irreversible organ lesions.