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

Cover Page


Cite item

Full Text

Open Access Open Access
Restricted Access Access granted
Restricted Access Subscription or Fee Access

Abstract

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.

Full Text

Restricted Access

About the authors

Anton L. Chudinov

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

Author for correspondence.
Email: anton-chudinov@mail.ru
ORCID iD: 0000-0002-7675-5683
SPIN-code: 5771-8320
Scopus Author ID: 57189099891

MD, Cand. Sci. (Med.)

Russian Federation, Saint Petersburg; Saint Petersburg

Irina B. 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-code: 3136-9062

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

Russian Federation, Saint Petersburg; Saint Petersburg

Vadim I. 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-code: 6823-5482
Scopus Author ID: 16936315400
ResearcherId: J-9643-2014

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

Russian Federation, Saint Petersburg; Saint Petersburg

Oksana V. 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-code: 8841-5496

MD, Cand. Sci. (Med.)

Russian Federation, Saint Petersburg; Saint Petersburg

References

  1. Klinicheskaya revmatologiya. Rukovodstvo dlya vrachei. Ed. by V.I. Mazurov. 3rd ed. Moscow: E-noto; 2021. 696 p. (In Russ.)
  2. Mazurov VI, Lesnyak OM. Revmatologiya. Farmakoterapiya bez oshibok. Rukovodstvo dlya vrachei. Moscow: E-noto; 2017. P. 479–505. (In Russ.)
  3. Mahr A, Guillevin L, Poissonnet M, Aymé S. Prevalences of polyarteriitis nodosa, microscopic polyangiitis, Wegener’s granulomatosis and Churg-Strauss syndrome in a French urban multiethnic population in 2000: a capture-recapture estimate. Arthritis Rheum. 2004;51(1):92–99. doi: 10.1002/art.20077
  4. Watts RA, Scott DGI. Epidemiology of vasculitis. Oxford: Oxford University Press; 2008. P. 7–22.
  5. Vasculitis in Clinical Practice. Ed. by R.A Watts, D.G.I. Scott. New York: Springer-Verlag; 2010. P. 71–77.
  6. Nasonov EL, Baranov AA, Shilkina NP. Vaskulity i vaskulopatii. Yaroslavl’: Verkhnyaya Volga; 1999. P. 340–347. (In Russ.)
  7. Fries JF, Hunder GG, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of vasculitis. Summary. Arthritis Rheum. 1990;33(8):1135–1136. doi: 10.1002/art.1780330812
  8. Luqmani RA, Bacon PA, Moots RJ, et al. Birmingham vasculitis activity score (BVAS) in systemic necrotizing vasculitis. QJM. 1994;87(11):671–678.
  9. Exley AR, Bacon PA, Luqmani RA, et al. Development and initial validation of the Vasculitis Damage Index for the standardized clinical assessment of damage in the systemic vasculitides. Arthritis Rheum. 1997;40(2):371–380. doi: 10.1002/art.1780400222
  10. Booth AD, Almond MK, Burns A, et al. Outcome of ANCA-associated renal vasculitis: a 5-year retrospective study. Am J Kidney Dis. 2003;41:776–784. doi: 10.1016/s0272-6386(03)00025-8
  11. Drooger JC, Dees A, Swaak AJG. ANCA-positive patients: The Influence of PR3 and MPO antibodies on survival rate and the association with clinical and laboratory characteristics. Open Rheumatol J. 2009;3:14–17. doi: 10.2174/1874312900903010014

Supplementary files

Supplementary Files
Action
1. JATS XML
2. Fig. 1. Distribution of patients with systemic vasculitis depending on the nosological affiliation. ANCA-SV — systemic vasculitis associated with antineutrophil cytoplasmic antibodies

Download (233KB)
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

Download (83KB)
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

Download (50KB)
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

Download (86KB)

Copyright (c) 2023 Eco-Vector



СМИ зарегистрировано Федеральной службой по надзору в сфере связи, информационных технологий и массовых коммуникаций (Роскомнадзор).
Регистрационный номер и дата принятия решения о регистрации СМИ: серия ПИ № ФС 77 - 70763 от 21.08.2017 г.


This website uses cookies

You consent to our cookies if you continue to use our website.

About Cookies