Childhood- Vs. Adult-Onset ANCA-Associated Vasculitides: A Nested, Matched Case–Control Study from the French Vasculitis Study Group Registry

Michele Iudici¹, Christian Pagnoux², Pierre Quartier³, Mathias Büchler⁴, Ramiro Cevallos⁵, Pascal Cohen⁶, Claire de Moreuil⁷, Philippe Guilpain⁸, Alain Le Quellec⁸, Jacques Serratrice⁹, Benjamin Terrier⁶, Claire Le Jeunne⁶, Loïc Guillevin¹, Luc Mouthon¹⁰ and Xavier Puéchal for the French Vasculitis Study Group¹, ¹National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, France, ²Mount Sinai Hospital, Toronto, ON, Canada, ³Necker-Enfants Malades Hospital, Paris, France, ⁴Tours, Tours, France, ⁵Strasbourg, Strasbourg, France, ⁶Service de Médecine Interne, Hôpital Cochin, Centre de référence national pour les maladies systémiques autoimmunes rares d’Ile de France, DHU Authors, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France, Paris, France, ⁷CHU de Brest, Brest, France, ⁸Montpellier, Montpellier, France, ⁹Marseille, Marseille, France, ¹⁰Service de Médecine Interne, Hôpital Cochin, Centre de référence national pour les maladies systémiques autoimmunes rares d’Ile de France, DHU Authors, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France ;Université Paris Descartes Sorbonne Paris, Paris, France

Meeting: 2017 ACR/ARHP Annual Meeting

Date of first publication: September 18, 2017

Keywords: outcomes, pediatrics and vasculitis, Transition

Session Information

Date: Monday, November 6, 2017

Title: Vasculitis Poster II: ANCA-Associated Vasculitis

Session Type: ACR Poster Session B

Session Time: 9:00AM-11:00AM

Background/Purpose: ANCA-associated vasculitides (AAVs) are potentially life-threatening diseases rarely observed in childhood. Whether AAVs in children (cAAVs) differ from adult-onset AAVs (aAAVs) is still not known. This study was undertaken to investigate differences in clinical presentations and disease outcomes between cAAV cases and matched aAAV controls.

Methods: Demographic and clinical data and disease outcomes of consecutive patients (age <18 years at diagnosis) with cAAVs satisfying ACR, PRINTO/EULAR classification criteria and/or the revised Chapel Hill Nomenclature for Vasculitides were compared to a randomly selected sample of aAAV patients from the French Vasculitis Study Group (FVSG) registry. Cases and controls were matched for the following features: AAV (granulomatosis with polyangiitis [GPA], microscopic polyangiitis [MPA] or eosinophilic granulomatosis with polyangiitis [EGPA]), sex, year of enrollment and duration of follow-up after diagnosis. Prospectively collected information included medications, and disease activity and damage as assessed by the Birmingham Vasculitis Activity Score (BVAS) and the Vasculitis Damage Index (VDI), respectively. Relapses, survival rates and causes of death were analyzed. Kaplan–Meier curves and the log-rank test were used to analyze predefined-outcome differences between cases and controls. STROBE guidelines for reporting observational studies were applied.

Results: Thirty-five cAAV cases (25 GPA, 4 MPA, 6 EGPA) were compared with 151 aAAV controls (106 GPA, 17 MPA, 28 EGPA). Respective median ages (range) were 14 (2–17) vs. 53 (18–87) years, with median AAV follow-up durations of 71 and 64 months (P=0.49), respectively. At study entry, children had less frequent myalgias (P=0.005) and peripheral neuropathy (P<0.001) but were more frequently febrile (P<0.05). Their respective rates of renal involvement were comparable (13 (37%) vs. 73 (48%); P=0.31). Initial GPA-associated ischemic abdominal pain and nasal cartilage damage were more common in cAAVs than aAAVs (P<0.05). Their first remission-induction regimens were comparable (P=0.13), most frequently combining glucocorticoids and an immunosuppressant. During follow-up, cAAV patients had a higher overall relapse rate (24.5 vs. 18.7 flares per 100 patient-years; P<0.05) and, at last visit, had accumulated more damage, mostly ENT sequelae (P=0.001), associated with longer maintenance therapy (P=0.03) than for aAAV controls. Four (11.4%) cAAV and 13 (8.6%) aAAV patients died (P=0.54).

Conclusion: cAAVs are severe diseases, characterized by a higher relapse rate, more accrued damage, mostly ENT sequelae, and longer maintenance therapy than aAAVs.

Disclosure: M. Iudici, None; C. Pagnoux, None; P. Quartier, None; M. Büchler, None; R. Cevallos, None; P. Cohen, None; C. de Moreuil, None; P. Guilpain, None; A. Le Quellec, None; J. Serratrice, None; B. Terrier, None; C. Le Jeunne, None; L. Guillevin, None; L. Mouthon, None; X. Puéchal for the French Vasculitis Study Group, None.

To cite this abstract in AMA style:

Iudici M, Pagnoux C, Quartier P, Büchler M, Cevallos R, Cohen P, de Moreuil C, Guilpain P, Le Quellec A, Serratrice J, Terrier B, Le Jeunne C, Guillevin L, Mouthon L, Puéchal for the French Vasculitis Study Group X. Childhood- Vs. Adult-Onset ANCA-Associated Vasculitides: A Nested, Matched Case–Control Study from the French Vasculitis Study Group Registry [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/childhood-vs-adult-onset-anca-associated-vasculitides-a-nested-matched-case-control-study-from-the-french-vasculitis-study-group-registry/. Accessed .

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