eprintid: 1540409 rev_number: 34 eprint_status: archive userid: 608 dir: disk0/01/54/04/09 datestamp: 2017-02-11 19:55:08 lastmod: 2021-12-19 00:09:23 status_changed: 2017-05-30 12:13:03 type: article metadata_visibility: show creators_name: Plumb, LA creators_name: Oni, L creators_name: Marks, SD creators_name: Tullus, K title: Paediatric anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis: an update on renal management ispublished: pub divisions: UCL divisions: B02 divisions: D13 keywords: ANCA vasculitis, Diagnosis, Glomerulonephritis, Management, Paediatric note: © The Author(s) 2017. This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. abstract: 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 against the cytoplasmic region of the neutrophil. Included in this definition are granulomatosis with polyangiitis (GPA, formerly known as Wegener's granulomatosis), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (formerly known as Churg-Strauss syndrome). AAV are chronic, often relapsing diseases that can be organ or life threatening. Despite immunosuppression, the morbidity and mortality remain high. Renal involvement contributes significantly to the morbidity with high numbers of patients progressing to end-stage kidney disease. Current therapies have enabled improvements in renal function in the short term, but evidence for long-term protection is lacking. In MPA, renal involvement is common at presentation (90%) and often follows a more severe course than that seen in paediatric GPA. Renal biopsy remains the 'gold standard' in diagnosing ANCA-associated glomerulonephritis. While GPA and MPA are considered separate entities, the two are managed identically. Current treatment regimens are extrapolated from adult studies, although it is encouraging to see recruitment of paediatric patients to recent vasculitis trials. Traditionally more severe disease has been managed with the 'gold standard' treatment of glucocorticoids and cyclophosphamide, with remission rates achieved of between 70 and 100%. Other agents employed in remission induction include anti-tumor necrosis factor-alpha therapy and mycophenolate mofetil. Recently, however, increasing consideration is being given to rituximab as a therapy for children in severe or relapsing disease, particularly for those at risk for glucocorticoid or cyclophosphamide toxicity. Removal of circulating ANCA through plasma exchange is a short-term measure reserved for severe or refractory disease. Maintenance therapy usually involves azathioprine. The aim of this article is to provide a comprehensive review of paediatric AAV, with a focus on renal manifestations, and to highlight the recent advances made in therapeutic management. date: 2018-01 date_type: published official_url: http://doi.org/10.1007/s00467-016-3559-2 oa_status: green full_text_type: pub language: eng primo: open primo_central: open_green article_type: review article_type_text: Review verified: verified_manual elements_id: 1201663 doi: 10.1007/s00467-016-3559-2 pii: 10.1007/s00467-016-3559-2 lyricists_name: Marks, Stephen lyricists_name: Tullus, Kjell lyricists_id: SDMAR28 lyricists_id: KTULL90 actors_name: Marks, Stephen actors_name: Laslett, David actors_id: SDMAR28 actors_id: DLASL34 actors_role: owner actors_role: impersonator full_text_status: public publication: Pediatric Nephrology volume: 33 number: 1 pagerange: 25-39 event_location: Germany issn: 1432-198X citation: Plumb, LA; Oni, L; Marks, SD; Tullus, K; (2018) Paediatric anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis: an update on renal management. [Review]. Pediatric Nephrology , 33 (1) pp. 25-39. 10.1007/s00467-016-3559-2 <https://doi.org/10.1007/s00467-016-3559-2>. Green open access document_url: https://discovery.ucl.ac.uk/id/eprint/1540409/1/Marks_art%25253A10.1007%25252Fs00467-016-3559-2.pdf