ANCA-associated and other childhood vasculitides

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Target exams

Red flags

• A child with a pulmonary-renal syndrome of dyspnoea, haemoptysis and rapidly progressive glomerulonephritis has ANCA-associated vasculitis until proven otherwise; this is a medical emergency requiring urgent ANCA, renal review and induction therapy, because untreated pauci-immune crescentic glomerulonephritis can destroy the kidneys within days
• Subglottic stenosis and destructive sinonasal disease with a saddle-nose deformity in a child is granulomatosis with polyangiitis, not recurrent infection; a positive PR3 (c-ANCA) and granulomatous histology confirm it, and delay risks irreversible airway and renal injury
• Late-onset asthma with marked eosinophilia and new mononeuritis multiplex or cardiac failure is eosinophilic granulomatosis with polyangiitis; eosinophilic myocarditis is the leading cause of death and demands urgent echocardiography and high-dose glucocorticoids
• A child or adolescent with absent pulses, a blood-pressure discrepancy between limbs, bruits, or unexplained hypertension has Takayasu arteritis until excluded; angiographic skip lesions of the aorta and its branches are mandatory for the diagnosis
• Prolonged fever with a Kawasaki phenotype that is ANCA-positive is almost always Kawasaki disease, not ANCA-associated vasculitis; Kawasaki is a medium-vessel, ANCA-negative disease treated with intravenous immunoglobulin and aspirin to protect the coronary arteries

Life stages

Care settings

Clinical exam formats

Board mappings

• Classifies the childhood vasculitides by vessel size using the 2012 Chapel Hill nomenclature, separating large-vessel Takayasu arteritis, medium-vessel Kawasaki disease and polyarteritis nodosa, and small-vessel ANCA-associated disease
• Recognises granulomatosis with polyangiitis (PR3 or c-ANCA) by its destructive upper-airway and pulmonary-renal features, microscopic polyangiitis (MPO or p-ANCA) by necrotising glomerulonephritis without granuloma, and eosinophilic granulomatosis with polyangiitis by asthma, eosinophilia and nerve or cardiac disease
• Identifies Takayasu arteritis in the child or adolescent with absent pulses, a blood-pressure discrepancy, bruits or unexplained hypertension, and states that angiographic abnormalities are mandatory for diagnosis
• Explains the pauci-immune mechanism in which PR3 and MPO anti-neutrophil cytoplasmic antibodies activate primed neutrophils to degranulate and release NETs, producing fibrinoid necrotising small-vessel vasculitis
• Justifies remission induction with rituximab or cyclophosphamide plus glucocorticoids for organ or life-threatening ANCA-associated vasculitis, and the limited-disease methotrexate pathway, citing the SHARE recommendations
• Coordinates multidisciplinary care with nephrology for crescentic glomerulonephritis, cardiology for EGPA myocarditis and Kawasaki surveillance, and vascular or interventional services for Takayasu revascularisation
• The child with a pulmonary-renal syndrome, haemoptysis and rapidly progressive glomerulonephritis, and the urgent ANCA, renal biopsy and induction pathway
• The adolescent with absent pulses, claudication and hypertension that opens the Takayasu arteritis pathway of angiography and glucocorticoids
• The safety-netting for infection on immunosuppression, glucocorticoid toxicity, and the relapse patterns that signal re-treatment
• Bedside assessment of the child with upper-airway destruction and a saddle-nose deformity, distinguishing granulomatosis with polyangiitis from infection, malignancy and IgA vasculitis
• Examination of all four limbs for pulse deficit, a blood-pressure discrepancy and bruits in the suspected large-vessel vasculitis
• Communication of a rare, chronic, multisystem diagnosis and the commitment to long-term immunosuppression and transition to adult care
• Level 1: Classifies childhood vasculitis by vessel size and recognises the ANCA-associated vasculitides and Takayasu arteritis as rare but serious
• Level 2: Applies the Ankara 2008 childhood GPA and Takayasu criteria, the ANCA interpretation, and the remission-induction and maintenance framework
• Level 3: Leads the management of pulmonary-renal syndrome, EGPA myocarditis and Takayasu revascularisation, coordinating nephrology, cardiology and rheumatology
• The Chapel Hill 2012 vessel-size nomenclature and the distinction between ANCA-associated pauci-immune and immune-complex small-vessel vasculitis
• The EULAR/PRINTO/PRES Ankara 2008 childhood GPA three-of-six criteria and Takayasu mandatory angiography plus one-of-five criteria
• The PR3 and MPO autoantibody associations and the remission-induction regimens of rituximab or cyclophosphamide with glucocorticoids
• Recognition of destructive sinonasal disease, subglottic stenosis and the saddle-nose deformity of granulomatosis with polyangiitis at the bedside
• Examination of pulses, four-limb blood pressure and bruits for Takayasu arteritis, and urinalysis for the pulmonary-renal syndrome
• Communication of the diagnosis, the prognosis, the immunosuppression burden and the long-term surveillance to the family
• ANCA-associated vasculitis and Takayasu arteritis as rare childhood vasculitides classified by the Chapel Hill vessel-size nomenclature
• The three ANCA-associated vasculitides and their distinguishing PR3 and MPO autoantibody and clinical patterns
• Remission induction with rituximab or cyclophosphamide and glucocorticoids, and the Kawasaki cross-link of intravenous immunoglobulin and aspirin
• Recognition of the pulmonary-renal syndrome and the destructive upper-airway disease of ANCA-associated vasculitis as medical emergencies
• Application of the remission-induction and maintenance framework and the Takayasu glucocorticoid and revascularisation pathway
• Coordination of multidisciplinary care and the transition of the adolescent with chronic vasculitis to adult services
• Canadian approach to childhood vasculitis classification using the Chapel Hill 2012 nomenclature and the Ankara 2008 criteria
• The remission-induction evidence and the biologic options for refractory Takayasu and EGPA
• The management of the pulmonary-renal syndrome and the long-term renal, cardiac and vascular surveillance