Phys · rheumatological
The Systemic Vasculitides
Also known as systemic vasculitis · vasculitis · ANCA-associated vasculitis · giant cell arteritis · temporal arteritis · polymyalgia rheumatica · Takayasu arteritis · polyarteritis nodosa · granulomatosis with polyangiitis · Wegener granulomatosis · microscopic polyangiitis · eosinophilic granulomatosis with polyangiitis · Churg-Strauss syndrome · IgA vasculitis · Henoch-Schonlein purpura · cryoglobulinaemic vasculitis · Behcet disease
Consultant-physician-depth guide to the systemic vasculitides for FRACP DWE and DCE — the Chapel Hill 2012 vessel-size classification, large vessel disease (giant cell arteritis and polymyalgia rheumatica with urgent steroids and tocilizumab, Takayasu arteritis in young Asian women), medium vessel disease (polyarteritis nodosa with microaneurysms, HBV association and mononeuritis multiplex, Kawasaki disease), ANCA-associated small vessel vasculitis (granulomatosis with polyangiitis with c-ANCA/PR3 and ENT-lung-kidney disease, microscopic polyangiitis with p-ANCA/MPO and renal-pulmonary disease, eosinophilic granulomatosis with polyangiitis with asthma and eosinophilia), immune complex small vessel vasculitis (cryoglobulinaemic vasculitis with HCV and low complement, IgA vasculitis in children with palpable purpura and glomerulonephritis), variable vessel vasculitis (Behcet disease), the diagnostic approach using biopsy, angiography, ANCA pattern and complement, and the treatment framework of steroids first-line, cyclophosphamide or rituximab for organ-threatening disease, plasma exchange for anti-GBM overlap and severe RPGN, and maintenance therapy with azathioprine, mycophenolate or methotrexate.
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Red flags
- New headache with jaw claudication or visual disturbance in a patient over 50 — giant cell arteritis until proven otherwise; start high-dose glucocorticoids immediately, do not wait for biopsy
- Sudden painless visual loss in a patient with polymyalgia rheumatica — anterior ischaemic optic neuropathy from GCA; this is an ophthalmic emergency requiring same-day high-dose steroids
- Rapidly progressive glomerulonephritis with haemoptysis or c-ANCA positivity — pulmonary-renal syndrome from GPA or MPA; start immunosuppression within hours, not days
- Palpable purpura on the lower limbs with abdominal pain and haematuria in a child — IgA vasculitis; monitor renal function closely as glomerulonephritis can evolve over weeks
- Mononeuritis multiplex (wrist drop, foot drop, numbness in multiple nerve territories) — a vasculitic neuropathy until proven otherwise; this is a classic medium-vessel or small-vessel finding demanding urgent diagnosis
- A young woman with absent pulses and blood pressure discrepancy between arms — Takayasu arteritis; check for bruits and image the aorta and its branches
The Systemic Vasculitides
The answer first
The systemic vasculitides are a group of inflammatory diseases of blood vessel walls classified by the predominant size of vessel involved. That single organising principle — large, medium or small — is how you must approach every vasculitis question, because vessel size predicts the clinical syndrome, the organ at risk, the investigation strategy and the treatment [1].
The 2012 revised International Chapel Hill Consensus Conference (CHCC) nomenclature is the examinable framework [1]. It renamed the eponyms: Wegener granulomatosis became granulomatosis with polyangiitis (GPA), Churg-Strauss syndrome became eosinophilic granulomatosis with polyangiitis (EGPA), and Henoch-Schonlein purpura became IgA vasculitis. The eponyms persist in clinical practice and exams, so learn both names.
Three rules that change outcome: [1]
- Giant cell arteritis with visual symptoms is an emergency. Start high-dose glucocorticoids immediately — do not wait for biopsy or a rheumatology review. Anterior ischaemic optic neuropathy causes irreversible blindness within hours. Tocilizumab is the evidence-based steroid-sparing agent for relapsing or refractory disease [2].
- A pulmonary-renal syndrome is a medical emergency. Haemoptysis with rapidly progressive glomerulonephritis is GPA or microscopic polyangiitis (MPA) until proven otherwise. Start high-dose glucocorticoids plus rituximab or cyclophosphamide within hours. Plasma exchange is reserved for anti-GBM overlap or dialysis-dependent disease [3][5][6].
- Rituximab has replaced cyclophosphamide as first-line induction for most ANCA-associated vasculitis. The RAVE and RITUXVAS trials established rituximab as non-inferior to cyclophosphamide for remission induction, with the advantage of being preferred in relapsing disease and in patients wishing to preserve fertility [3][4].
The clinical reasoning moves in three steps at the bedside. First, recognise the syndrome — is this a large-vessel problem (headache, jaw claudication, absent pulses), a medium-vessel problem (mononeuritis multiplex, mesenteric ischaemia, nodules), or a small-vessel problem (palpable purpura, glomerulonephritis, alveolar haemorrhage)? Second, confirm the vessel and the mechanism — biopsy the affected tissue, check ANCA pattern, complement levels, cryoglobulins and infection screen. Third, treat by severity — glucocorticoids for everything, but add rituximab or cyclophosphamide for organ- or life-threatening disease. [1]
References11ShowHide
- [1]Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides Arthritis Rheum, 2013.PMID 23045170
- [2]Stone JH, Tuckwell K, Dimonaco S, et al. Trial of Tocilizumab in Giant-Cell Arteritis N Engl J Med, 2017.PMID 28745999
- [3]Stone JH, Merkel PA, Spiera R, et al. Rituximab versus cyclophosphamide for ANCA-associated vasculitis N Engl J Med, 2010.PMID 20647199
- [4]Jones RB, Tervaert JW, Hauser T, et al. Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis N Engl J Med, 2010.PMID 20647198
- [5]Jayne DR, Gaskin G, Rasmussen N, et al. Randomized trial of plasma exchange or high-dosage methylprednisolone as adjunctive therapy for severe renal vasculitis J Am Soc Nephrol, 2007.PMID 17582159
- [6]Walsh M, Merkel PA, Peh CA, et al. Plasma Exchange and Glucocorticoids in Severe ANCA-Associated Vasculitis N Engl J Med, 2020.PMID 32053298
- [7]Hellmich B, Sanchez-Alamo B, Schirmer JH, et al. EULAR recommendations for the management of ANCA-associated vasculitis: 2022 update Ann Rheum Dis, 2024.PMID 36927642
- [8]Ponte C, Grayson PC, Robson JC, et al. 2022 American College of Rheumatology/EULAR Classification Criteria for Giant Cell Arteritis Arthritis Rheumatol, 2022.PMID 36350123
- [9]Robson JC, Grayson PC, Ponte C, et al. 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis with Polyangiitis Ann Rheum Dis, 2022.PMID 35110334
- [10]Suppiah R, Robson JC, Grayson PC, et al. 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for microscopic polyangiitis Ann Rheum Dis, 2022.PMID 35110332
- [11]Hellmich B, Agueda A, Monti S, et al. 2018 Update of the EULAR recommendations for the management of large vessel vasculitis Ann Rheum Dis, 2020.PMID 31270110