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Paeds Vivasrheumatology-musculoskeletal-and-sports

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.

branching clinical structured oral1 min readVerification in progress

Target exams

RACP DCEMRCPCH ClinicalRCPSC Pediatrics
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Study tools

Target exams

RACP DCEMRCPCH ClinicalRCPSC Pediatrics
Prompt
Emergency department: a 14-year-old boy with three weeks of bloody nasal discharge, a septal ulcer, a productive cough, and cola-coloured urine, a rising creatinine, and a positive PR3 (c-ANCA). The examiner asks: name the diagnosis and classify the vasculitides by vessel size, then branches to the pauci-immune mechanism and the remission-induction and maintenance framework, to the same boy re-presenting with an absent radial pulse and hypertension that opens the Takayasu arteritis pathway, to the asthma and eosinophilia and mononeuritis multiplex that opens the eosinophilic granulomatosis with polyangiitis and its cardiac danger, and finally to the Kawasaki cross-link of the medium-vessel, ANCA-negative disease treated with intravenous immunoglobulin and aspirin.

Opening question

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]

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References8ShowHide
  1. [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
  2. [3]Jennette JC, Falk RJ, Bacon PA, et al 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum, 2013.PMID 23045170
  3. [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
  4. [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
  5. [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
  6. [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
  7. [9]Brunner J, Feldman BM, Tyrrell PN, et al Takayasu arteritis in children and adolescents. Rheumatology (Oxford), 2010.PMID 20562196
  8. [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
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