Abstract
BackgroundPrimary systemic vasculitis presenting in childhood is an uncommon but serious condition. As these patients transfer to adult clinics for continuing care, defining long term outcomes with emphasis on disease and treatment- related morbidity and mortality is important. The aim of this study is to describe the long- term clinical course of paediatric patients with ANCA vasculitis.MethodsThe adult patients in our vasculitis clinics who had presented in childhood, with a follow up time of greater than 10 years were included. We also reviewed the literature for articles describing the clinical outcome of paediatric patients with ANCA vasculitis.ResultsWe describe the clinical course of 8 adults who presented in childhood with ANCA vasculitis. 7 patients had Wegener's granulomatosis and 1 had microscopic polyangiitis. The median age at presentation was 11.5 years, and follow up time ranged form 11 to 30 years. Induction therapy for all patients was steroids and/or cyclophosphamide. Maintenance therapy was with azathioprine or mycophenolate mofetil. Biological agents were used in 3 patients for relapsed disease in adulthood only.Seven patients achieved complete remission. All patients experienced disease relapse, with a median of 4 episodes. Kidney function was generally well preserved, with median eGFR 76 ml/min. Only one patient developed end-stage renal failure and one patient died after 25 years of disease. Treatment-related morbidity rates were high; 7 suffered from infections, 4 were infertile, 2 had skeletal complications, and 1 developed malignancy.ConclusionClose long- term follow up of paediatric patients with ANCA vasculitis is imperative, as this patient cohort is likely to live long enough to develop significant treatment and disease- related morbidities. Prospective cohort studies with novel therapies including paediatric patients are crucial to help us determine the best approach to managing this complex group of patients. In addition, although not yet observed in our series, late cardiovascular morbidity remains a major longer-term potential concern for adult survivors of paediatric vasculitis.
Highlights
Primary systemic vasculitis presenting in childhood is an uncommon but serious condition
Demographics There have been a total of 48 paediatric patients diagnosed with Anti- neutrophil cytoplasmic antibody (ANCA) vasculitis between the years of 1996 and 2010 at the Great Ormond Street Hospital. 5 of these patients were under adult follow up at the Hammersmith Hospital, London, with more than 10 years from initial diagnosis (Table 1)
The University Hospital of Birmingham has a total of 220 patients with vasculitis under active follow up. 7 of these patients presented with vasculitis in childhood, 3 of whom had ANCA associated vasculitis and were under adult follow up with at least 10 years from initial diagnosis
Summary
Primary systemic vasculitis presenting in childhood is an uncommon but serious condition. With the exception of Henoch Schönlein purpura (HSP) and Kawasaki disease (KD), are diseases that predominantly affect people later in adulthood, typically in their sixth and seventh decades [1] Vasculitides such as Wegener’s granulomatosis (WG) and polyarteritis nodosa (PAN) have different aetiological, clinical, and prognostic features in children [2,3]. The estimated incidence of childhood WG is less than 1 per 2 million per year [6], and MPA incidence has not been determined in paediatric patients In this retrospective study, we reviewed the clinical features of 8 children diagnosed with ANCA vasculitis and the progression of their disease into adulthood. Diagnoses were made based on clinical criteria, laboratory testing, and histology, as well as the EULAR/PReS proposed consensus criteria for the classification of childhood vasculitides [7]
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