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Indian Journal of Pediatrics
Published in: Indian Journal of Pediatrics 10/2017

01-10-2017 | Editorial Commentary

Spectrum of ANCA-Associated Vasculitis

Published in: Indian Journal of Pediatrics | Issue 10/2017

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Excerpt

Anti-neutrophil cytoplasmic antibodies (ANCA) associated vasculitides (AAV) are severe multisystem diseases characterized by necrotizing inflammation of small blood vessels, few or no immune deposits, and circulating ANCA with specificity chiefly against myeloperoxidase or proteinase-3 [1]. These autoantibodies are believed to be generated following antigenic mimicry by diverse triggers, including viruses (Coxsackie B3, parvovirus B19), silica and drugs (propylthiouracil, allopurinol, hydralazine). ANCA are detected by indirect immunofluorescence microscopy, using patient sera admixed with healthy neutrophils followed by fluorescent staining. The presence of antibodies is confirmed by immunoassay, most often ELISA. Based on histology, type of autoantibodies and organ involvement, three chief forms are recognized: microscopic polyangiitis, granulomatosis with polyangiitis (GPA; Wegener’s granulomatosis) and eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) [1]. …
Literature
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Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised international Chapel Hill consensus conference nomenclature of vasculitides. Arthritis Rheum. 2013;65:1–11.CrossRefPubMed
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Sacri AS, Chambaraud T, Ranchin B, et al. Clinical characteristics and outcomes of childhood-onset ANCA-associated vasculitis: a French nationwide study. Nephrol Dial Transplant. 2015;30:i104–12.PubMed
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Pilania RK, Dhawan SR, Mathew JL, Singh S, Sodhi KS, Singh M. ANCA-associated vasculitis presenting as severe pulmonary hypertension and right heart failure. Indian J Pediatr. 2017; doi:10.​1007/​s12098-017-2379-0.
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Watts R, Lane S, Hanslik T, et al. Development and validation of a consensus methodology for the classification of the ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies. Ann Rheum Dis. 2007;66:222–7.CrossRefPubMed
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Launay D, Souza R, Guillevin L, et al. Pulmonary arterial hypertension in ANCA-associated vasculitis. Sarcoidosis Vasc Diffuse Lung Dis. 2006;23:223–8.PubMed
Metadata
Title
Spectrum of ANCA-Associated Vasculitis
Publication date
01-10-2017
Published in
Indian Journal of Pediatrics / Issue 10/2017
Print ISSN: 0019-5456
Electronic ISSN: 0973-7693
DOI
https://doi.org/10.1007/s12098-017-2450-x

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