Vasculitides associated with IgG antineutrophil cytoplasmic autoantibodies in childhood

Vanoni, Federica; Bettinelli, Alberto; Keller, Franco; Bianchetti, Mario G; Simonetti, Giacomo D (2010). Vasculitides associated with IgG antineutrophil cytoplasmic autoantibodies in childhood. Pediatric nephrology, 25(2), pp. 205-12. Berlin: Springer-Verlag 10.1007/s00467-009-1253-3

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Immunoglobulin (Ig)G antineutrophil cytoplasmic autoantibodies are causally associated with necrotizing vasculitides that are characterized immunopathologically by little or no deposition of immunoreactants, such as Wegener granulomatosis, microscopic polyangiitis, Churg-Strauss angiitis, "renal-limited" vasculitis and a number of drug-induced vasculitides. Clinical routine testing targets the antigens myeloperoxidase and proteinase 3. However, in all of the conditions mentioned, the renal histopathologic findings are indistinguishable. Churg-Strauss angiitis (characterized by necrotizing vasculitis, granulomatous inflammation and tissue eosinophilia), Wegener granulomatosis (characterized by necrotizing vasculitis and granulomatous inflammation) and microscopic polyangiitis (characterized by necrotizing vasculitis) often present with fever, weight loss and a multisystem involvement (ear, nose, throat, lung, eyes, peripheral nerve and heart). Fifty years ago these conditions were very often fatal within 6 months of diagnosis. The introduction of corticosteroids and cyclophosphamide has resulted in a dramatic clinical benefit. Patients who develop treatment-related morbidity can be switched from cyclophosphamide to azathioprine after achieving remission. In patients with less severe disease, methotrexate achieves remission with a success rate similar to that of cyclophosphamide. Plasma exchange, in association with immunosuppression, is likely to be a beneficial therapy for patients with severe kidney disease or pulmonary hemorrhage.

Item Type:

Journal Article (Further Contribution)

Division/Institute:

04 Faculty of Medicine > Department of Gynaecology, Paediatrics and Endocrinology (DFKE) > Clinic of Paediatric Medicine

UniBE Contributor:

Bianchetti, Mario Giovanni and Simonetti, Giacomo

ISSN:

0931-041X

Publisher:

Springer-Verlag

Language:

English

Submitter:

Factscience Import

Date Deposited:

04 Oct 2013 14:13

Last Modified:

23 Oct 2019 23:34

Publisher DOI:

10.1007/s00467-009-1253-3

PubMed ID:

19641940

Web of Science ID:

000272912200004

BORIS DOI:

10.7892/boris.3000

URI:

https://boris.unibe.ch/id/eprint/3000 (FactScience: 206108)

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