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

Publication date: Available online 24 November 2017
Source:Autoimmunity Reviews
Author(s): Michele Iudici, Christian Pagnoux, Pierre Quartier, Matthias Büchler, Ramiro Cevallos, Pascal Cohen, Claire de Moreuil, Philippe Guilpain, Alain Le Quellec, Jacques Serratrice, Benjamin Terrier, Loïc Guillevin, Luc Mouthon, Xavier Puéchal
ObjectiveTo investigate differences between childhood-onset ANCA-associated vasculitides (cAAVs) and matched adult-onset controls (aAAVs).MethodscAAV 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.ResultsComparing 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).ConclusioncAAVs are severe diseases, characterized by a higher relapse rate, more accrued damage and longer maintenance therapy than for aAAVs.



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