Early Outcomes in Children With Antineutrophil Cytoplasmic Antibody–Associated Vasculitis

for the ARChiVe Investigators Network within the PedVas Initiative

Research output: Contribution to journalArticle

16 Citations (Scopus)

Abstract

Objective: To characterize the early disease course in childhood-onset antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV) and the 12-month outcomes in children with AAV. Methods: Eligible subjects were children entered into the Pediatric Vasculitis Initiative study who were diagnosed before their eighteenth birthday as having granulomatosis with polyangiitis (Wegener's), microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis (Churg-Strauss), or ANCA-positive pauci-immune glomerulonephritis. The primary outcome measure was achievement of disease remission (Pediatric Vasculitis Activity Score [PVAS] of 0) at 12 months with a corticosteroid dosage of <0.2 mg/kg/day. Secondary outcome measures included the rates of inactive disease (PVAS of 0, with any corticosteroid dosage) and rates of improvement at postinduction (4–6 months after diagnosis) and at 12 months, presence of damage at 12 months (measured by a modified Pediatric Vasculitis Damage Index [PVDI]; score 0 = no damage, score 1 = one damage item present), and relapse rates at 12 months. Results: In total, 105 children with AAV were included in the study. The median age at diagnosis was 13.8 years (interquartile range 10.9–15.8 years). Among the study cohort, 42% of patients achieved remission at 12 months, 49% had inactive disease at postinduction (4–6 months), and 61% had inactive disease at 12 months. The majority of patients improved, even if they did not achieve inactive disease. An improvement in the PVAS score of at least 50% from time of diagnosis to postinduction was seen in 92% of patients. Minor relapses occurred in 12 (24%) of 51 patients after inactive disease had been achieved postinduction. The median PVDI damage score at 12 months was 1 (range 0–6), and 63% of patients had ≥1 PVDI damage item scored as present at 12 months. Conclusion: This is the largest study to date to assess disease outcomes in pediatric AAV. Although the study showed that a significant proportion of patients did not achieve remission, the majority of patients responded to treatment. Unfortunately, more than one-half of this patient cohort experienced damage to various organ systems early in their disease course.

Original languageEnglish (US)
Pages (from-to)1470-1479
Number of pages10
JournalArthritis and Rheumatology
Volume69
Issue number7
DOIs
StatePublished - Jul 1 2017

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Vasculitis
Pediatrics
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
Antineutrophil Cytoplasmic Antibodies
Granulomatosis with Polyangiitis
Adrenal Cortex Hormones
Microscopic Polyangiitis
Outcome Assessment (Health Care)
Recurrence
Glomerulonephritis
Cohort Studies

All Science Journal Classification (ASJC) codes

  • Immunology and Allergy
  • Rheumatology
  • Immunology

Cite this

for the ARChiVe Investigators Network within the PedVas Initiative. / Early Outcomes in Children With Antineutrophil Cytoplasmic Antibody–Associated Vasculitis. In: Arthritis and Rheumatology. 2017 ; Vol. 69, No. 7. pp. 1470-1479.
@article{fdb79898f5d642efbcf38708e9c13ce7,
title = "Early Outcomes in Children With Antineutrophil Cytoplasmic Antibody–Associated Vasculitis",
abstract = "Objective: To characterize the early disease course in childhood-onset antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV) and the 12-month outcomes in children with AAV. Methods: Eligible subjects were children entered into the Pediatric Vasculitis Initiative study who were diagnosed before their eighteenth birthday as having granulomatosis with polyangiitis (Wegener's), microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis (Churg-Strauss), or ANCA-positive pauci-immune glomerulonephritis. The primary outcome measure was achievement of disease remission (Pediatric Vasculitis Activity Score [PVAS] of 0) at 12 months with a corticosteroid dosage of <0.2 mg/kg/day. Secondary outcome measures included the rates of inactive disease (PVAS of 0, with any corticosteroid dosage) and rates of improvement at postinduction (4–6 months after diagnosis) and at 12 months, presence of damage at 12 months (measured by a modified Pediatric Vasculitis Damage Index [PVDI]; score 0 = no damage, score 1 = one damage item present), and relapse rates at 12 months. Results: In total, 105 children with AAV were included in the study. The median age at diagnosis was 13.8 years (interquartile range 10.9–15.8 years). Among the study cohort, 42{\%} of patients achieved remission at 12 months, 49{\%} had inactive disease at postinduction (4–6 months), and 61{\%} had inactive disease at 12 months. The majority of patients improved, even if they did not achieve inactive disease. An improvement in the PVAS score of at least 50{\%} from time of diagnosis to postinduction was seen in 92{\%} of patients. Minor relapses occurred in 12 (24{\%}) of 51 patients after inactive disease had been achieved postinduction. The median PVDI damage score at 12 months was 1 (range 0–6), and 63{\%} of patients had ≥1 PVDI damage item scored as present at 12 months. Conclusion: This is the largest study to date to assess disease outcomes in pediatric AAV. Although the study showed that a significant proportion of patients did not achieve remission, the majority of patients responded to treatment. Unfortunately, more than one-half of this patient cohort experienced damage to various organ systems early in their disease course.",
author = "{for the ARChiVe Investigators Network within the PedVas Initiative} and Morishita, {Kimberly A.} and Lakshmi Moorthy and Lubieniecka, {Joanna M.} and Marinka Twilt and Yeung, {Rae S.M.} and Toth, {Mary B.} and Susan Shenoi and Goran Ristic and Nielsen, {Susan M.} and Luqmani, {Raashid A.} and Li, {Suzanne C.} and Tzielan Lee and Lawson, {Erica F.} and Kostik, {Mikhail M.} and Marisa Klein-Gitelman and Huber, {Adam M.} and Hersh, {Aimee O.} and Dirk Foell and Elder, {Melissa E.} and Eberhard, {Barbara A.} and Paul Dancey and Sirirat Charuvanij and Benseler, {Susanne M.} and Cabral, {David A.}",
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for the ARChiVe Investigators Network within the PedVas Initiative 2017, 'Early Outcomes in Children With Antineutrophil Cytoplasmic Antibody–Associated Vasculitis', Arthritis and Rheumatology, vol. 69, no. 7, pp. 1470-1479. https://doi.org/10.1002/art.40112

Early Outcomes in Children With Antineutrophil Cytoplasmic Antibody–Associated Vasculitis. / for the ARChiVe Investigators Network within the PedVas Initiative.

In: Arthritis and Rheumatology, Vol. 69, No. 7, 01.07.2017, p. 1470-1479.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Early Outcomes in Children With Antineutrophil Cytoplasmic Antibody–Associated Vasculitis

AU - for the ARChiVe Investigators Network within the PedVas Initiative

AU - Morishita, Kimberly A.

AU - Moorthy, Lakshmi

AU - Lubieniecka, Joanna M.

AU - Twilt, Marinka

AU - Yeung, Rae S.M.

AU - Toth, Mary B.

AU - Shenoi, Susan

AU - Ristic, Goran

AU - Nielsen, Susan M.

AU - Luqmani, Raashid A.

AU - Li, Suzanne C.

AU - Lee, Tzielan

AU - Lawson, Erica F.

AU - Kostik, Mikhail M.

AU - Klein-Gitelman, Marisa

AU - Huber, Adam M.

AU - Hersh, Aimee O.

AU - Foell, Dirk

AU - Elder, Melissa E.

AU - Eberhard, Barbara A.

AU - Dancey, Paul

AU - Charuvanij, Sirirat

AU - Benseler, Susanne M.

AU - Cabral, David A.

PY - 2017/7/1

Y1 - 2017/7/1

N2 - Objective: To characterize the early disease course in childhood-onset antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV) and the 12-month outcomes in children with AAV. Methods: Eligible subjects were children entered into the Pediatric Vasculitis Initiative study who were diagnosed before their eighteenth birthday as having granulomatosis with polyangiitis (Wegener's), microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis (Churg-Strauss), or ANCA-positive pauci-immune glomerulonephritis. The primary outcome measure was achievement of disease remission (Pediatric Vasculitis Activity Score [PVAS] of 0) at 12 months with a corticosteroid dosage of <0.2 mg/kg/day. Secondary outcome measures included the rates of inactive disease (PVAS of 0, with any corticosteroid dosage) and rates of improvement at postinduction (4–6 months after diagnosis) and at 12 months, presence of damage at 12 months (measured by a modified Pediatric Vasculitis Damage Index [PVDI]; score 0 = no damage, score 1 = one damage item present), and relapse rates at 12 months. Results: In total, 105 children with AAV were included in the study. The median age at diagnosis was 13.8 years (interquartile range 10.9–15.8 years). Among the study cohort, 42% of patients achieved remission at 12 months, 49% had inactive disease at postinduction (4–6 months), and 61% had inactive disease at 12 months. The majority of patients improved, even if they did not achieve inactive disease. An improvement in the PVAS score of at least 50% from time of diagnosis to postinduction was seen in 92% of patients. Minor relapses occurred in 12 (24%) of 51 patients after inactive disease had been achieved postinduction. The median PVDI damage score at 12 months was 1 (range 0–6), and 63% of patients had ≥1 PVDI damage item scored as present at 12 months. Conclusion: This is the largest study to date to assess disease outcomes in pediatric AAV. Although the study showed that a significant proportion of patients did not achieve remission, the majority of patients responded to treatment. Unfortunately, more than one-half of this patient cohort experienced damage to various organ systems early in their disease course.

AB - Objective: To characterize the early disease course in childhood-onset antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV) and the 12-month outcomes in children with AAV. Methods: Eligible subjects were children entered into the Pediatric Vasculitis Initiative study who were diagnosed before their eighteenth birthday as having granulomatosis with polyangiitis (Wegener's), microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis (Churg-Strauss), or ANCA-positive pauci-immune glomerulonephritis. The primary outcome measure was achievement of disease remission (Pediatric Vasculitis Activity Score [PVAS] of 0) at 12 months with a corticosteroid dosage of <0.2 mg/kg/day. Secondary outcome measures included the rates of inactive disease (PVAS of 0, with any corticosteroid dosage) and rates of improvement at postinduction (4–6 months after diagnosis) and at 12 months, presence of damage at 12 months (measured by a modified Pediatric Vasculitis Damage Index [PVDI]; score 0 = no damage, score 1 = one damage item present), and relapse rates at 12 months. Results: In total, 105 children with AAV were included in the study. The median age at diagnosis was 13.8 years (interquartile range 10.9–15.8 years). Among the study cohort, 42% of patients achieved remission at 12 months, 49% had inactive disease at postinduction (4–6 months), and 61% had inactive disease at 12 months. The majority of patients improved, even if they did not achieve inactive disease. An improvement in the PVAS score of at least 50% from time of diagnosis to postinduction was seen in 92% of patients. Minor relapses occurred in 12 (24%) of 51 patients after inactive disease had been achieved postinduction. The median PVDI damage score at 12 months was 1 (range 0–6), and 63% of patients had ≥1 PVDI damage item scored as present at 12 months. Conclusion: This is the largest study to date to assess disease outcomes in pediatric AAV. Although the study showed that a significant proportion of patients did not achieve remission, the majority of patients responded to treatment. Unfortunately, more than one-half of this patient cohort experienced damage to various organ systems early in their disease course.

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for the ARChiVe Investigators Network within the PedVas Initiative. Early Outcomes in Children With Antineutrophil Cytoplasmic Antibody–Associated Vasculitis. Arthritis and Rheumatology. 2017 Jul 1;69(7):1470-1479. https://doi.org/10.1002/art.40112