Decramer, S

Flodrops, H

Florkin, B

Harambat, J

Quartier, P

Ranchin, B

Sacri, AS

Salomon, R

See, H

Ulinski, T

English

PubMed

AS Sacri

B Ranchin

B Florkin

H See

T Ulinski

H Flodrops

S Decramer

R Salomon

P Quartier

J Harambat

Crossref

Clinical features of anti-neutrophil cytoplasmic antibody-associated systemic vasculitis in French children

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POSTER PRESENTATION Open Access
Clinical features of anti-neutrophil cytoplasmic
antibody-associated systemic vasculitis in French
children
AS Sacri1*, B Ranchin2, B Florkin3, H See4, T Ulinski5, H Flodrops6, S Decramer7, R Salomon8, P Quartier3,
J Harambat1
From 18th Pediatric Rheumatology European Society (PReS) Congress
Bruges, Belgium. 14-18 September 2011
Background
Anti-neutrophil cytoplasmic antibody (ANCA)-asso-
ciated vasculitis is mainly reported in adults. Data in
children is scarce. The current study aimed to describe
the clinical features of the disease at diagnosis and the
long-term outcomes in pediatric patients with ANCA-
associated vasculitis.
Methods
This retrospective study was conducted in 9 French
Hospitals (mainly pediatric nephrology units) and
includes patients diagnosed during the past 25 years.
Wegener’s granulomatosis (WG) and microscopic poly-
angiitis (MPA) were included, but not Churg-Strauss
syndrome.
Results
Forty-seven children were included, 15 (32%) with WG
and 32 (68%) with MPA, 77% children were pANCA
+/MPO. Female predominated (85%), the median age at
diagnosis was 10 years (2-16), and average time to diag-
nosis was 8 months (0-58). 53% children presented with
fever, 77% with deterioration of general condition. 91%
had renal involvement. Among them 91% had protei-
nuria, 44% had hypertension, 79% had renal failure of
whom one third presented with end-stage renal disease
(ESRD), 22% had gross hematuria and 82% microscopic
hematuria. Half the patients showed pulmonary involve-
ment (alveolar hemorrhage for 77% and nodules for
18%). 28% presented with upper airways involvement,
38% with cutaneous lesions (67% purpura), and 19%
with arthritis. Few had digestive, ophthalmic or CNS
involvement. After an average follow-up of 5 years (2m-
24y), 3 patients (6%) died. 60% were considered in
remission (partial or total) after induction treatment and
77% at last follow-up. 33% of children with initial renal
involvement developed chronic kidney disease, half of
them progressed to ESRD. 43% needed dialysis and 29%
underwent a renal transplantation.
Conclusion
Clinical features and outcome in children with ANCA-
vasculitis are close to the findings in adults but they are
characterized by delayed diagnosis and female predomi-
nance. Although the cases may be selected, renal invol-
vement appears as a major prognostic factor of the
disease.
Author details
1Department of Pediatric Nephrology, CHU Pellegrin, Bordeaux, France.
2Department of Pediatric Nephrology, HFME, Lyon, France. 3Department of
Pediatric Rhumatolgy, Necker Enfants Malades, Paris, France. 4Department of
Pediatric Nephrology, CHU Robert Debré, Paris, France. 5Department of
Pediatric Nephrology, CHU Armand Trousseau, Paris, France. 6Department of
Pediatric Nephrology, CHR Réunion, France. 7Department of Pediatric
Nephrology, CHU Toulouse, France. 8Department of Pediatric Nephrology,
Necker Enfants Malades, Paris, France.
Published: 14 September 2011
doi:10.1186/1546-0096-9-S1-P88
Cite this article as: Sacri et al.: Clinical features of anti-neutrophil
cytoplasmic antibody-associated systemic vasculitis in French children.
Pediatric Rheumatology 2011 9(Suppl 1):P88.
* Correspondence: annesylvia.sacri@yahoo.fr
1Department of Pediatric Nephrology, CHU Pellegrin, Bordeaux, France
Full list of author information is available at the end of the article
Sacri et al. Pediatric Rheumatology 2011, 9(Suppl 1):P88
http://www.ped-rheum.com/content/9/S1/P88
© 2011 Sacri et al; licensee BioMed Central Ltd. This is an open access article distributed under the terms of the Creative Commons
Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in
any medium, provided the original work is properly cited.


Salomon R

Decramer S

Flodrops H

Ulinski T

See H

Florkin B

Ranchin B

Sacri AS

Quartier P

Harambat J

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Clinical features of anti-neutrophil cytoplasmic antibody-associated systemic vasculitis in French children

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