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Autoimmun Rev. 2018 Feb;17(2):108-114. doi: 10.1016/j.autrev.2017.11.014. Epub 2017 Nov 24.

Childhood- versus adult-onset ANCA-associated vasculitides: A nested, matched case-control study from the French Vasculitis Study Group Registry.

Author information

1
National Referral Center for Rare Systemic Autoimmune Diseases, Department of Internal Medicine, Hôpital Cochin, Assistance Publique - Hôpitaux de Paris (APHP), Paris, France.
2
Paris-Descartes University, Imagine Institute, Pediatric Immunology-Hematology and Rheumatology Unit, Necker-Enfants Malades, APHP, Paris, France.
3
CHU Bretonneau, Tours, France.
4
Centre Hospitalier Saint-Vincent, Strasbourg, France.
5
National Referral Center for Rare Systemic Autoimmune Diseases, Department of Internal Medicine, Hôpital Cochin, Assistance Publique - Hôpitaux de Paris (APHP), Paris, France; Université Paris Descartes, Paris, France.
6
CHU La Cavale Blanche, Brest, France.
7
CHU Saint-Eloi, Montpellier, France.
8
CHU La Timone, Marseille, France.
9
National Referral Center for Rare Systemic Autoimmune Diseases, Department of Internal Medicine, Hôpital Cochin, Assistance Publique - Hôpitaux de Paris (APHP), Paris, France; Université Paris Descartes, Paris, France. Electronic address: xavier.puechal@aphp.fr.

Abstract

OBJECTIVE:

To investigate differences between childhood-onset ANCA-associated vasculitides (cAAVs) and matched adult-onset controls (aAAVs).

METHODS:

cAAV clinical pictures at onset and outcomes were compared to a randomly selected sample of aAAV patients from the French Vasculitis Study Group Registry. Cases and controls were matched for AAV (granulomatosis with polyangiitis [GPA], microscopic polyangiitis [MPA] or eosinophilic granulomatosis with polyangiitis [EGPA]), sex and year of enrollment. Medications, disease activity and damage were prospectively recorded. Kaplan-Meier curves and the log-rank test were used to analyze case-vs.-control differences for predefined outcomes.

RESULTS:

Comparing 35 cAAVs (25 GPA, 4 MPA, 6 EGPA) to 151 aAAVs (106 GPA, 17 MPA, 28 EGPA), their respective median follow-up durations were 71 and 64months (P=0.49), and, at baseline, children had less frequent myalgias (P=0.005) and peripheral neuropathy (P<0.001) but were more frequently febrile (P<0.05). Rates of renal involvement were comparable (13 [37%] cAAVs vs. 73 [48%] aAAVs; P=0.31). Initial GPA-associated ischemic abdominal pain and nasal cartilage damage were more common in cAAVs than aAAVs (P<0.05). During follow-up, the cAAV relapse rate was higher (24.5 vs. 18.7 flares per 100 patient-years; P<0.05) and, at last visit, cases had accumulated more damage, mostly ear, nose & throat sequelae (P=0.001), associated with longer maintenance therapy (P=0.03), than aAAV controls. Four (11.4%) cAAV and 13 (8.6%) aAAV patients died (P=0.53).

CONCLUSION:

cAAVs are severe diseases, characterized by a higher relapse rate, more accrued damage and longer maintenance therapy than for aAAVs.

KEYWORDS:

ANCA-associated vasculitis; Childhood vasculitis; Damage; Survival

PMID:
29180123
DOI:
10.1016/j.autrev.2017.11.014
[Indexed for MEDLINE]

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