🔑 Key Learning

  • Vasculitis is classified by vessel size: Large (e.g. GCA, Takayasu), Medium (e.g. PAN, Kawasaki), Small (e.g. GPA, EGPA, HSP)
  • Giant cell arteritis → headache + jaw claudication + visual loss in >50s
    • Management: High dose corticosteroids
  • Takayasu’s → young Asian female + absent pulses + uneven BPs + claudication
  • Kawasaki’s → child + fever + strawberry tongue + desquamating rash → coronary aneurysms
    • Management: IVIG and aspirin, echocardiogram
  • Granulomatosis with polyangiitis (GPA) → URT + LRT + renal + cANCA
  • EGPA → 'asthma' + eosinophilia + neuropathy + pANCA
  • HSP → palpable purpura on knees/buttocks + abdominal pain + IgA nephropathy in children
  • Goodpasture’s → haemoptysis + nephritic syndrome + anti-GBM antibodies
  • Behçet’s → recurrent oral/genital ulcers + uveitis + thrombophlebitis

🏥 Classification of Common Vasculitides

Vessel SizeExamples
Large vesselTemporal arteritis, Takayasu’s arteritis
Medium vesselPolyarteritis nodosa (PAN), Kawasaki, Buerger’s disease
Small vesselANCA-associated (GPA, EGPA, MPA)
Immune complex (HSP, Goodpasture’s, SLE)

      

🧠 Large Vessel Vasculitis

⏱️ Temporal Arteritis (Giant Cell Arteritis)

  • Age >50
  • 50% associated with PMR - history of proximal muscle weakness/stiffness/fatigue
  • Clinical feaures:
    • Temporal headache 
    • Jaw claudication (chewing)
    • Superficial tenderness (e.g. while showering/combing hair)
    • Visual disturbance - due to anterior ischaemic optic neuropathy
      • Diplopia
      • Loss of vision
      • Changes to colour vision
  • Bloods: Raised ESR/CRP
  • Diagnosis: Temporal artery biopsy (skip lesions possible)
    • Histology: granulomatous inflammation with giant cells
  • Prednisolone 60mg OD + urgent ophthalmology review if eye symptoms

🧓 Polymyalgia Rheumatica

  • Clinical features
    • Age > 60yrs
    • Pain and stiffness in the shoulders, hips, and neck which develops acutely over a few days to weeks. 
    • Stiffness is worse in the mornings 
    • Low mood/ depression
    • Night sweats
    • Polyarthralgia
  • Investigations
    • ESR > 40 (CK, EMG normal)
  • Management:
    • Prednisolone 15-20mg OD for 4 weeks, followed by gradual tapering dose over 12-18 months
    • Co-prescribe PPI
    • Most patients require bone protection - bisphosphonates, adcal D3 etc

🚫 Takayasu’s Arteritis

  • Young Asian female
  • History of intermittent claudication, angina
  • OE: Unequal BP in arms, difference >10 mmHg
  • Carotid bruit, AR murmur
  • 💊 Treated with steroids

🧲 Medium Vessel Vasculitis

🧬 Polyarteritis Nodosa (PAN)

  • Middle-aged man + Hep B
  • Mononeuritis multiplex, hypertension, livedo reticularis
  • Renal: haematuria, failure
  • 🔍 Angiography: microaneurysms
  • Antibodies: pANCA ±
  • 💊 Steroids + immunosuppression

👶 Kawasaki Disease

  • Child <5, fever ≥5 days
  • Red eyes/lips/tongue, lymphadenopathy, palm/sole desquamation
  • Risk of coronary aneurysms → do echo
  • 💊 IVIG + high-dose aspirin

🚬 Buerger’s Disease (Thromboangiitis Obliterans)

  • Smoker with limb ischaemia
  • Raynaud’s + superficial thrombophlebitis
  • 💡 Strongly linked to tobacco use

🧪 Small Vessel Vasculitis — ANCA-Associated

🫁 Granulomatosis with Polyangiitis (GPA)

  • URT: nasal crusting, epistaxis, saddle nose
    • Helps differentiate from Goodpasture's in MCQs!
  • LRT: dyspnoea, haemoptysis
  • Renal: rapidly progressive GN
  • Antibody: cANCA / PR3
  • CXR: cavitating nodules
  • 💊 Steroids + cyclophosphamide ± plasma exchange

🧼 Eosinophilic GPA (Churg-Strauss)

  • Asthma-like (wheezy), eosinophilia, sinusitis
  • Mononeuritis multiplex
  • Worsens with montelukast
  • Antibody: pANCA / MPO
  • 💊 Steroids ± DMARDs

🧬 Microscopic Polyangiitis (MPA)

  • Palpable purpura, fever, weight loss
  • Renal impairment, mononeuritis multiplex
  • Antibody: pANCA > cANCA
  • 💊 Steroids + immunosuppression

🧬 Small Vessel Vasculitis — Immune Complex

🎨 Henoch-Schönlein Purpura (HSP)

  • IgA mediated small vessel vasculitis - Most common vasculitis in children
  • Palpable purpuric rash (legs/buttocks), arthralgia, abdo pain
  • Nephritis due to IgA nephropathy  - AKI, haematuria, proteinuria
  • Triggered by URTI - history of recent sore throat is exam clue
  • 🧪 IgA deposition in skin/renal biopsy
  • 🩺 Supportive treatment
HSP. Madhero88, CC BY-SA 3.0 via Wikimedia Commons

   

🫁 Goodpasture’s Syndrome

  • Pulmonary haemorrhage + rapidly progressive GN
  • Haematuria, oedema, HTN
  • Antibody: anti-GBM (vs type IV collagen)
  • Biopsy: linear IgG along BM
  • 💊 Plasma exchange + steroids + cyclophosphamide

🌍 Behçet’s Disease

  • Middle Eastern origin
  • Triad: Recurrent oral/genital ulcers, uveitis
  • Also: DVT, arthropathy, thrombophlebitis
  • Pathergy test may be positive
  • Immunosuppressants (e.g. colchicine, azathioprine)

📝 Exam Clues & Clinchers

  • Visual loss, jaw claudication → Temporal arteritis
  • Proximal stiffness, ESR > 40, >60 yrs → PMR
  • Young Asian female + absent pulses → Takayasu
  • Child + fever + strawberry tongue + peeling skin → Kawasaki
  • Middle-aged man + Hep B + mononeuritis multiplex → PAN
  • Saddle nose + haematuria + cavitating lung lesion → GPA
  • Asthma + eosinophilia + peripheral neuropathy → EGPA
  • Palpable purpura + abdo pain + child → HSP
  • Haemoptysis + nephritic picture + anti-GBM → Goodpasture’s
  • Oral + genital ulcers + uveitis → Behçet’s