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Updated: May 31, 2026
Vessel-sparing Excision and Primary Anastomosis
Published on: January 7, 2019
Long- term outcome of paediatric patients with ANCA vasculitis
Nishkantha Arulkumaran1, Susan Jawad, Stuart W Smith
1Renal Section, Division of Medicine, Imperial College London, Hammersmith Hospital, Ducane Road, London W12 0NN, UK. a.salama@medsch.ucl.ac.uk.
Insights
Long-term follow-up of childhood ANCA vasculitis is crucial. Patients face significant treatment and disease-related risks, including relapses and high morbidity rates, necessitating ongoing monitoring and research into novel therapies.
Area of Science:
- Pediatric Rheumatology
- Immunology
- Internal Medicine
Background:
- Childhood-onset primary systemic vasculitis is rare but serious.
- Long-term outcomes for pediatric patients transitioning to adult care require definition.
- Focus on disease and treatment-related morbidity and mortality is essential.
Purpose of the Study:
- To describe the long-term clinical course of pediatric patients diagnosed with ANCA vasculitis.
- To evaluate outcomes including disease activity, organ function, and treatment complications.
- To inform future management strategies for this patient cohort.
Main Methods:
- Retrospective review of adult patients with childhood-onset ANCA vasculitis and >10 years follow-up.
- Literature review of published clinical outcomes for pediatric ANCA vasculitis.
- Analysis of disease course, treatment regimens, and long-term morbidities.
Main Results:
- Eight adult patients with childhood ANCA vasculitis (7 Wegener's, 1 MPA) were analyzed.
- Median follow-up was 11-30 years; all patients experienced relapses (median 4 episodes).
- High treatment-related morbidity observed: infections (7), infertility (4), skeletal issues (2), malignancy (1); kidney function generally preserved, one patient reached ESRD, one died.
Conclusions:
- Close long-term follow-up is imperative for pediatric ANCA vasculitis patients due to potential for significant morbidities.
- Prospective studies with novel therapies are crucial for optimizing management.
- Late cardiovascular morbidity is a potential long-term concern for survivors.
Background:
Primary 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.
Methods:
The 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.
Results:
We 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.
Conclusion:
Close 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.
