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- W2947778880 abstract "Background: The anti-neutrophil cytoplasmic antibody (ANCA)- associated vasculitides (AAV) are a group of disorders characterized by necrotizing inflammation of the small to medium vessels in association with autoantibodies. Childhood ANCA vasculitides are rare but can cause organ or even life-threatening systemic vasculitis. Children most frequently present with rapidly evolving, severe disease. Objectives: To describe the clinical spectrum of ANCA associated vasculitis, the treatment given and follow up Methods: A single-centre retrospective analysis of ANCA associated vasculitis over a period of 10 years from 2008 to 2018 Results: Six children (2 boys; 4 girls) were diagnosed to have AAV during this period. Median age at diagnosis was 11.25 years (range 8-18 years). Median delay in diagnosis was 1.5 months (range 1-8 months). Presenting clinical features included lung disease- 4 children; arthritis- 3 (knee, right ankle-left 3rd MCP, left elbow-knee) hemoptysis- 2 children; ear discharge- 1; redness of eye-1; oliguria, gross hematuria-2; anasarca-2; 2 children had concomitant neurological symptoms (left foot drop, quadriparesis with seizures). All children had fever at presentation. Laboratory investigations showed elevated erthrocyte sedimentation rate(ESR) and C-reactive protein(CRP). ANCA testing was positive in 6 children (3 c-ANCA, +PR3, 3 p-ANCA, +MPO). Deranged renal function tests, proteinuria, microscopic hematuria) were seen in 4 out of 6 children. Renal biopsy showed pauci immune glomerulonephritis in 4 cases who presented with nephritis. All patients were treated with 5 pulses of intravenous methylprednisolone(30 mg/kg/day) with tapering doses of oral steroids. Intravenous cyclophosphamide pulses was given in 5 children while 1 received additional plasmapheresis (PLEX)- 8 cycles. Mintainence therapy included azathioprine(given in 5 children), low-dose prednisolone. Thrombocytopenia was noted in 1 child while on azathioprine; this was subsequently substituted with mycophenolate mofetil. Two children have been lost to follow-up. Median duration of follow-up is 35.5 months (range 8-124 months). All 4 children have attained remission with good compliance and there are no relapses. Conclusion: While an evidence base treatment of AAV in children is limited, our data suggests that prolonged remission can be achieved with aggressive immnosupressive therapy, however prognosis of childhood AAV remains guarded. Disclosure of Interests: None declared" @default.
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- W2947778880 date "2019-06-01" @default.
- W2947778880 modified "2023-09-27" @default.
- W2947778880 title "AB0589 ANCA ASSOCIATED VASCULITIS: OUR EXPERIENCE FROM A TERITIARY CARE CENTER OVER 10 YEARS" @default.
- W2947778880 doi "https://doi.org/10.1136/annrheumdis-2019-eular.8244" @default.
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