Background/Purpose: Granulomatosis with polyangiitis (GPA) is a rare disease in childhood. Treatment strategies and clinical approach are still mostly derived from adult GPA studies. The present study was aimed to compare onset, disease course, therapeutic approach and clinical outcome of two cohorts of children and adults affected by GPA.

Methods: The study included children and adult patients selected on the basis of complete data set and follow up of at least one year, followed at a pediatric and adult rheumatology center, over a period of 20 years (1993-2012). GPA was diagnosed according to the ACR and EULAR/PRES criteria. Clinical features, instrumental findings, laboratory parameters and therapeutic regimens of both cohorts were analyzed at diagnosis, six months later (T6) and at the last follow-up visit. Disease activity was assessed by the Birmingham Vasculitis Activity Score (BVAS) modified for GPA.

Results: Ten children with mean age at disease onset of 10.3 years (range 3-15) entered the study, 7/10 were female. Mean follow-up time was 8.4 years (range 2.5-18). Of the 23 adults 65.3% were female, mean age at onset 53 years (range 37-71) and mean follow-up 2.2 years (range 1.9-3.5). At disease onset, BVAS, clinical features and laboratory parameters of the two cohorts were not significantly different. BVAS decreased more slowly in children (p 0.002). As for internal organs involvement, renal disease was significantly higher at T6 and persisted over time in children (p 0.003). Similarly, pulmonary involvement remained elevated at T6 in children while rapidly decreased over 50% in adults (p 0.01). At the last F/U visit, eye involvement was present in 44% of children while no adult showed signs of ocular disease (p 0.004). As for the treatment strategies, immunosuppressive drugs were more widely used in children at diagnosis (p 0.06) and biological agents were used at an earlier disease stage than adults. Despite the longer follow up, all children were still on treatment at the last follow-up visit while 17% of adults were off therapy.

Conclusion:

Adults and children with GPA had similar disease activity at onset. However, childhood GPA had a more severe-course due to persistent renal, pulmonary and eye involvement. Lower disease activity was obtained with a more aggressive treatment approach although no pediatric patients reached a drug-free remission.

References

Ozen et al. EULAR/PRINTO/PRES criteria for Henoch–Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis. Ann Rheum Dis 2010;69:798–806.

Demirkaya et al. Performing of Birmingham Vasculitis Activity Score and disease extent index in childhood vasculitides. Clin Exp Rheumatol.2012; 30(1 Suppl 70):S162-8.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/granulomatosis-with-polyangiitis-in-children-is-more-severe-than-in-adults-a-long-term-single-center-follow-up-study/