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This study describes the clinical picture of the disease in a large cohort of GPA paediatric patients. This disease is typically associated with granulomatous inflammation, pauci-immune necrotizing glomerulonephritis, involvement of upper and lower respiratory tract, and with presence of anti-neutrophil cytoplasmic antibodies ANCA.
In children, the estimated incidence is approximately 0. Treatment usually consists of combination of corticosteroids and cyclophosphamide, and more recently rituximab to induce remission, followed by a maintenance phase with lower doses of corticosteroids combined with azathioprine or other disease modifying agents for several years.
Despite treatment relapses are common and therapy related complications of significant concern [ 4 — 9 ]. Patients with co-morbidities were excluded from the study. The study was approved by the ethics committee of the Gaslini Hospital Genoa, Italy and by the ethics committees of all remaining participating centres and informed consent was obtained from parent s as required by the national law in each participating country.
Statistical analysis The quantitative demographic, clinical and laboratory data were reported as median 1st and 3rd quartile ; the categorical data were reported as absolute frequencies and as percentage frequencies of measured subjects. The data were entered in an Access XP database and analysed with Statistica 6.
Granulomatosis with polyangiits is very rare in children and, although it appears to be the same disease observed in adults, it is not known if childhood presentations are characterized by differences in the frequency of some clinical features well described in adults.
Indeed, in other connective tissue diseases, such as systemic lupus erythematosus or juvenile dermatomyositis, the frequency of several clinical manifestations differed from those observed in adults [ 18 , 19 ]. In the present paediatric series, the disease was characterised by high frequency of constitutional symptoms, upper and lower respiratory tract, mucosa, skin and musculoskeletal involvement.
The frequencies of symptoms typically decreased in the first few months after diagnosis, presumably due to the effect of treatment although data on treatment modalities were not collected in the database. This likely reflects the non-specific nature of ENT and cutaneous features in GPA, that occur commonly in children and hence may not prompt clinicians to initially consider vasculitis as the cause. In adult patients, the data were similar with Comparing the paediatric and adult cohorts, while organ involvement, signs and symptoms were similar, there were differences in their frequencies at disease presentation.
Adults had a lower frequency of constitutional, ENT and respiratory involvement; while hearing loss was more frequent in the adult population. The current work is limited by the lack of information on treatment and disease outcomes. In addition, comparison of clinical and laboratory findings with literature data might be hampered by the lack of standardised definition and by differences in the time of evaluation in the different series.
Moreover our study was likely hampered by inconsistencies in laboratory techniques in particular in relation to ANCA testing , and by the exclusion of cases in this series who did not fulfil the new paediatric classification criteria for c-GPA.
Indeed, we recognise that patients may present with limited forms of the disease, without initially fulfilling all criteria for classification and clinicians must remain vigilant in relation to this presentation in children. Paediatric patients compared to adults have similar clinical manifestations but different frequencies of organ involvement.
Granulomatosis con poliangitis
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