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Clinical features in 17 paediatric patients with Wegener granulomatosis
Vladimir M Belostotsky1, Vanita Shah, Michael J Dillon
1Great Ormond Street Hospital for Children and Institute of Child Health, London WC1N 1EH, UK.
Insights
This study details childhood Wegener granulomatosis (WG) cases, highlighting varied symptoms and higher kidney involvement in older children. Early diagnosis and treatment are crucial for managing this rare pediatric vasculitis.
Area of Science:
- Pediatric Rheumatology
- Vasculitis Research
- Autoimmune Diseases
Background:
- Wegener granulomatosis (WG), now known as Granulomatosis with Polyangiitis (GPA), is a rare autoimmune vasculitis.
- Understanding pediatric WG is crucial due to its potential for severe organ damage.
Purpose of the Study:
- To describe childhood WG cases from a single center.
- To analyze clinical manifestations and compare with existing literature.
- To identify factors influencing disease presentation and outcomes.
Main Methods:
- Retrospective review of 17 pediatric WG patients treated at Great Ormond Street Hospital (1981-1998).
- Analysis of presenting features, clinical signs, and diagnostic criteria (American College of Rheumatology).
- Evaluation of system involvement, cANCA/pANCA status, and treatment responses.
Main Results:
- Respiratory (87%) and kidney (53%) involvement were most common.
- Older children (6-14 years) showed significantly higher rates of kidney disease (78%) compared to younger ones (25%).
- cANCA positivity was associated with kidney disease (78%) and seen in 59% of patients.
Conclusions:
- Pediatric WG presents with diverse manifestations, with respiratory and kidney systems frequently affected.
- Age at onset is a significant factor for kidney involvement in childhood WG.
- cANCA positivity correlates with kidney disease, aiding diagnosis.
Abstract:
The aim of this report was to describe childhood patients with Wegener granulomatosis (WG) from one centre, to analyse the variety of clinical manifestations seen and compare the data with other published paediatric and adult series. The records of 17 patients with WG who were under the care of Great Ormond Street Hospital for Children (GOSH) from 1981 to 1998 were reviewed. We analysed presenting features before admittance to GOSH and the clinical signs observed whilst the children were under the care of the hospital. Of 17 patients, 13 were females and there was a male/female ratio of 1:3.25. Among the patients there were 2 sisters. The age of the patients at disease onset varied from 2 weeks to 14 years. The median/mean age was 6/6.3 years. American College of Rheumatology criteria for diagnosing WG were fulfilled in 11 of 17 patients. The frequency of different system involvement was: respiratory 87%, kidneys 53%, sinuses 35%, joints 53%, eyes 53%, nervous system 12%, skin 53%. cANCA was positive in 10 patients (59%), but pANCA was negative in all measured sera. Kidneys were involved in 2 of 8 patients (25%) with the disease onset from 0 to 5 years and in 7 of 9 patients (78%) with the disease onset from 6 to 14 years ( P<0.05). cANCA was positive in 7 of 9 patients with kidney disease (78%) and in 2 of 8 patients (25%) without kidney involvement ( P<0.05). Colchicine as a supplement to prednisolone and cytotoxic/immunosuppressant drugs was used effectively in 5 patients.