Day 5- Systemic Vasculitis

Types of Vasculitis:

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Large Vessel: Giant cell arteritis, takayasu

Medium Vessel: Polyarteritis Nodosa (PAN), Kawasaki

Small Vessel:

  • ANCA associated- MPA, eosinophilic granulomatosis with polyangitis, granulomatosis with polyangitis

  • Immune complex associated- HSP, cryoglobulinemic vasculitis

  • Others- lupus, drug associated

We have selected 2 vasculitic entities to describe here, GCA and granulomatosis with polyangitis!

Giant Cell Arteritis

Clinical Manifestations

  • scalp pain

  • headache

  • unilateral tenderness over the temporal artery

  • jaw claudication

  • malaise, weight loss, low grade fever

Investigations

  • ESR (typically great that 50mm/h)

  • temporal artery biopsy

  • CT angiography

Management

  • treatment should NEVER be deferred

    • rapidly effective, prevents blindness. Biopsy specimens remain interpretable for at least 2 weeks after treatment initiation

  • Oral prednisone 1mg/kg/day (in patients with visual loss, IV pulse glucocorticoids- methylprednisolone 1g/d for 3 days)

  • prednisone treatment duration 6-18months, ASA 81mg co-administration further reduces risk of blindness

Granulomatosis with Polyangitis

Clinical Manifestations

  • Upper respiratory tract (including sinus pain/stuffiness, rhinitis or epistaxis, saddle nose deformity, hearing loss)

  • lungs- nodules, infiltrates, capillaritis (resulting in alveolar hemorrhage and hemoptysis)

  • kidneys- rapidly progressive crescentic glomerulonephritis

Investigations

  • c-ANCA/anti-PR3

  • tissue diagnosis (often from lung or kidneys)

Management

  • Induction of remission: high dose glucocorticoids + cyclophosphamide/ritixumab followed by maintenance therapy with MTX, azathioprine, mycophenolate mofetil or rituximab for at least 24 months

  • Using these approaches, mortality has declines from 90% to 10%

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