+ Site Statistics
+ Search Articles
+ PDF Full Text Service
How our service works
Request PDF Full Text
+ Follow Us
Follow on Facebook
Follow on Twitter
Follow on LinkedIn
+ Subscribe to Site Feeds
Most Shared
PDF Full Text
+ Translate
+ Recently Requested

Brief Report: Childhood-Onset Systemic Necrotizing Vasculitides: Long-Term Data From the French Vasculitis Study Group Registry



Brief Report: Childhood-Onset Systemic Necrotizing Vasculitides: Long-Term Data From the French Vasculitis Study Group Registry



Arthritis and Rheumatology 67(7): 1959-1965



To describe the initial features and long-term outcomes of childhood-onset small vessel and medium vessel systemic necrotizing vasculitides (SNVs), including antineutrophil cytoplasmic antibody-associated vasculitides (AAVs) and polyarteritis nodosa (PAN). Data on patients with childhood-onset SNV registered in the French Vasculitis Study Group database were reviewed for demographic characteristics, clinical, laboratory, and histologic features, and outcomes. Disease activity was assessed with the Birmingham Vasculitis Activity Score and the Paediatric Vasculitis Activity Score, and damage was scored using the Vasculitis Damage Index. Relapse and survival rates and causes of death were analyzed. Fifty-six patients (35 with AAV and 21 with PAN) (median age at database enrollment 14 years [range 2-17]) were included in the study. The median duration of followup was 96 months (range 1-336); two-thirds of the patients were followed up beyond 18 years of age. Six patients (11%) died, mostly of SNV-related causes. Relapse rates ranged from 33% for microscopic polyangiitis to 50% for eosinophilic granulomatosis with polyangiitis (Churg-Strauss) and 83% for granulomatosis with polyangiitis (Wegener's), with similar rates among AAV and PAN patients (76% and 75%, respectively); neither overall survival nor relapse-free survival differed significantly between the 2 disease groups. Rates of relapse increased after 18 years of age, both among patients with AAV and among patients with PAN. At the last followup evaluation, AAV patients had more major flares and more severe accrued damage compared with PAN patients. Despite similar relapse rates, patients with childhood-onset AAVs experienced more major flares with more cumulative damage than those with pediatric PAN. Treatments aimed at reducing the rates of mortality and relapse in this patient group need to be developed and assessed.

Please choose payment method:






(PDF emailed within 0-6 h: $19.90)

Accession: 057326694

Download citation: RISBibTeXText

PMID: 25808634

DOI: 10.1002/art.39122


Related references

Mortality in systemic necrotizing vasculitides: A retrospective analysis of the French Vasculitis Study Group registry. Autoimmunity Reviews 17(7): 653-659, 2018

Childhood- versus adult-onset ANCA-associated vasculitides: A nested, matched case-control study from the French Vasculitis Study Group Registry. Autoimmunity Reviews 17(2): 108-114, 2018

Plasma exchanges for the treatment of severe systemic necrotizing vasculitides in clinical daily practice: Data from the French Vasculitis Study Group. Journal of Autoimmunity 65: 49-55, 2015

The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort. Medicine 90(1): 19-27, 2011

Childhood- versus Adult-Onset Polyarteritis Nodosa Results from the French Vasculitis Study Group Registry. Autoimmunity Reviews 17(10): 984-989, 2018

Damage in the anca-associated vasculitides: long-term data from the European vasculitis study group (EUVAS) therapeutic trials. Annals of the Rheumatic Diseases 74(1): 177-184, 2015

Temporal artery biopsy: a diagnostic tool for systemic necrotizing vasculitis. French Vasculitis Study Group. Arthritis and Rheumatism 42(12): 2674-2681, 1999

Rituximab: Recommendations of the French Vasculitis Study Group (FVSG) for induction and maintenance treatments of adult, antineutrophil cytoplasm antibody-associated necrotizing vasculitides. Presse Medicale 42(10): 1317-1330, 2013

Glucocorticoid treatment and damage in the anti-neutrophil cytoplasm antibody-associated vasculitides: long-term data from the European Vasculitis Study Group trials. Rheumatology 54(3): 471-481, 2015

Factors associated with major cardiovascular events in patients with primary systemic necrotizing vasculitides: Results of a longitudinal long-term follow-up study. La Presse Médicale 42(4): 674-675, 2013

FRI0343 Factors associated with major cardiovascular events in patients with primary systemic necrotizing vasculitides: results of a longitudinal long-term follow-up study. Annals of the Rheumatic Diseases 72(Suppl 3): A490-A490, 2013

Acute necrotizing retinal vasculitis as onset of systemic lupus erythematosus: A case report. Medicine 96(2): E5754, 2017

Inflammatory bowel diseases in anti-neutrophil cytoplasmic antibody-associated vasculitides: 11 retrospective cases from the French Vasculitis Study Group. Rheumatology 54(11): 1970-1975, 2015

Vasculitis activity score for Wegeners Granulomatosis A report from the International Network for the Study of Systemic Vasculitides. Arthritis & Rheumatism 42(9 Suppl. ): S317, 1999

A long-term immunological study of childhood onset systemic lupus erythematosus. Annals of the Rheumatic Diseases 51(1): 45-51, 1992