Paeds Vivas · rheumatology-musculoskeletal-and-sports
ANCA-associated and other childhood vasculitides — branching viva
Branching viva on ANCA-associated and other childhood vasculitides: classifying the vasculitides by vessel size and recognising the pulmonary-renal syndrome of granulomatosis with polyangiitis, then branching to the pauci-immune mechanism and the remission-induction framework, to the absent pulse and hypertension of Takayasu arteritis, to the asthma and eosinophilia of eosinophilic granulomatosis with polyangiitis, and to the Kawasaki cross-link.
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A 14-year-old boy presents with three weeks of bloody nasal discharge, a septal ulcer, a productive cough, and cola-coloured urine. His creatinine is rising, his urinalysis shows blood and protein with red-cell casts, and his PR3 (c-ANCA) is positive. Name the diagnosis, classify the childhood vasculitides by vessel size using the Chapel Hill 2012 nomenclature, and state what makes this the pulmonary-renal syndrome. [1][7]
References8ShowHide
- [1]Ozen S, Pistorio A, Iusan SM, et al EULAR/PRINTO/PRES criteria for Henoch-Schonlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria. Ann Rheum Dis, 2010.PMID 20413568
- [3]Jennette JC, Falk RJ, Bacon PA, et al 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum, 2013.PMID 23045170
- [5]Grayson PC, Ponte C, Suppiah R, et al 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis With Polyangiitis. Arthritis Rheumatol, 2022.PMID 35106968
- [6]de Graeff N, Groot N, Brogan P, et al European consensus-based recommendations for the diagnosis and treatment of rare paediatric vasculitides - the SHARE initiative. Rheumatology (Oxford), 2019.PMID 30535249
- [7]Cabral DA, Canter DL, Muscal E, et al Comparing Presenting Clinical Features in 48 Children With Microscopic Polyangiitis to 183 Children Who Have Granulomatosis With Polyangiitis (Wegener's): An ARChiVe Cohort Study. Arthritis Rheumatol, 2016.PMID 27111558
- [8]McCrindle BW, Rowley AH, Newburger JW, et al Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association. Circulation, 2017.PMID 28356445
- [9]Brunner J, Feldman BM, Tyrrell PN, et al Takayasu arteritis in children and adolescents. Rheumatology (Oxford), 2010.PMID 20562196
- [10]Pop AA, Bot Rachisan AL, Botan E, et al Renal Involvement in Pediatric Small-Vessel Vasculitis: A Comprehensive Review of Clinical Impact, Diagnosis, and Management. Med Sci (Basel), 2026.PMID 42346872