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

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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 (C3a–C5 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.

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作者简介

Anton Chudinov

North-Western State Medical University named after I.I. Mechnikov; Clinical Rheumatology Hospital No. 25, Saint Petersburg

编辑信件的主要联系方式.
Email: anton-chudinov@mail.ru
ORCID iD: 0000-0002-7675-5683
SPIN 代码: 5771-8320
Scopus 作者 ID: 57189099891

MD, Cand. Sci. (Med.)

俄罗斯联邦, Saint Petersburg; Saint Petersburg

Irina Belyaeva

North-Western State Medical University named after I.I. Mechnikov; Clinical Rheumatology Hospital No. 25, Saint Petersburg

Email: belib@mail.ru
ORCID iD: 0000-0002-7981-6349
SPIN 代码: 3136-9062

MD, Dr. Sci. (Med.), Assistant Professor

俄罗斯联邦, Saint Petersburg; Saint Petersburg

Vadim Mazurov

North-Western State Medical University named after I.I. Mechnikov; Clinical Rheumatology Hospital No. 25, Saint Petersburg

Email: rectorat@szgmu.ru
ORCID iD: 0000-0002-0797-2051
SPIN 代码: 6823-5482
Scopus 作者 ID: 16936315400
Researcher ID: J-9643-2014

MD, Dr. Sci. (Med.), Professor, Academician of the RAS, Honoured Science Worker

俄罗斯联邦, Saint Petersburg; Saint Petersburg

Oksana Inamova

North-Western State Medical University named after I.I. Mechnikov; Clinical Rheumatology Hospital No. 25, Saint Petersburg

Email: b25@zdrav.spb.ru
ORCID iD: 0000-0001-9126-3639
SPIN 代码: 8841-5496

MD, Cand. Sci. (Med.)

俄罗斯联邦, Saint Petersburg; Saint Petersburg

参考

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  8. Luqmani RA, Bacon PA, Moots RJ, et al. Birmingham vasculitis activity score (BVAS) in systemic necrotizing vasculitis. QJM. 1994;87(11):671–678.
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2. Fig. 1. Distribution of patients with systemic vasculitis depending on the nosological affiliation. ANCA-SV — systemic vasculitis associated with antineutrophil cytoplasmic antibodies

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3. Fig. 2. The period of time from the onset of clinical manifestations to the appointment of pathogenetic therapy. Data are presented as median, upper and lower quartiles. GPA — granulomatosis with polyangiitis; MPA — microscopic polyangiitis; EGPA — eosinophilic granulomatosis with polyangiitis; PN — polyarteritis nodosa

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4. Fig. 3. The mean value of the index according to the Birmingham vasculitis activity scale at the time of the appointment of pathogenetic therapy. GPA — granulomatosis with polyangiitis; MPA — microscopic polyangiitis; EGPA — eosinophilic granulomatosis with polyangiitis; PN — polyarteritis nodosa

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5. Fig. 4. The mean value of the Vasculitis Damage Index after 12 and 36 months from the onset of the disease. GPA — granulomatosis with polyangiitis; MPA — microscopic polyangiitis; EGPA — eosinophilic granulomatosis with polyangiitis; PN — polyarteritis nodosa

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