Session Information
Date: Sunday, November 8, 2015
Title: Vasculitis Poster I
Session Type: ACR Poster Session A
Session Time: 9:00AM-11:00AM
Background/Purpose:
The chronic
primary systemic vasculitides are a group of rare
conditions with affected patients subject to a significant burden of morbidity
from both the disease itself and its treatment. In adult vasculitides,
the Vasculitis Damage Index (VDI) is a validated
damage assessment tool and an integral outcome measure. The lack of such a
validated index in childhood vasculitis has contributed to the paucity of
outcome data in children with vasculitis. The aim of this study was to use a
modification of the adult VDI to assess damage accrual
in a cohort of children with primary systemic vasculitis recruited to A
Registry of Childhood Vasculitis (ARCHiVe), a
web-based registry founded in 2007 by the Canadian/US based Childhood Arthritis
and Rheumatology Research Alliance (CARRA).
Methods:
A multi-centre
cohort study of children enrolled into ARCHiVe was
conducted. Children were included if they were followed for at least 12 months
and had a PVDI completed.
Results:
A total of 74
children were included in the study.
65% were female. The mean age was 11.8 years (range 2-18). Granulomatosis with polyangiitis
(GPA) was the most frequently encountered diagnosis (Table 1). At baseline, the median Paediatric Vasculitis Activity Score (PVAS)
was 12 (range 2-31). At 12 months, 39/74 children (52.7%) had accumulated at
least one item of damage (PVDI=1 in 18 patients, PVDI=2 in 14, PVDI=3 in 4, PVDI=4 in 1 and PVDI=5 in
2). The most commonly recorded
damage items were in the “Other” section (consisting of significant striae [16.2%], chronic cough [5.4%] and vocal cord
paralysis [5.4%]), followed by “Renal” due to end stage renal failure (9.5%)
and proteinuria (9.5%) and “Pulmonary” due to
impaired lung function (9.5%) as shown in Figure 1.
Conclusion:
Within the first year of disease more than 50% of children
with systemic vasculitis have accumulated damage. The information gained from
this study provides a useful data driven basis for future development and
validation of a damage assessment tool in paediatric
vasculitis.
Table 1
|
Diagnosis |
No of Patients |
Percentage % |
|
Granulomatosis with Polyangiitis (GPA) |
43 |
58.1 |
|
Limited Granulomatosis with Polyangiitis |
8 |
10.8 |
|
Takayasu Arteritis |
7 |
9.5 |
|
Eosinophilic Granulomatosis with Polyangiitis (EGPA) |
5 |
6.8 |
|
Microscopic Polyangiitis (MPA) |
3 |
4 |
|
Isolated Renal Microscopic Polyangiitis |
3 |
4 |
|
Polyarteritis Nodosa |
3 |
4 |
|
Unclassified Primary Vasculitis |
1 |
1.4 |
|
ANCA positive pauci-immune glomerulonephritis |
1 |
1.4 |
Figure 1
|
Number of patients with systemic vasculitis showing items of damage using the provisional pediatric modification of the VDI |
To cite this abstract in AMA style:
O'Neill L, Twilt M, Benseler S, Föll D, Cabral DA, Luqmani R. Damage Develops Early and Is Common in Children with Chronic Systemic Vasculitis [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/damage-develops-early-and-is-common-in-children-with-chronic-systemic-vasculitis/. Accessed .« Back to 2015 ACR/ARHP Annual Meeting
ACR Meeting Abstracts - https://acrabstracts.org/abstract/damage-develops-early-and-is-common-in-children-with-chronic-systemic-vasculitis/