Day 5- Systemic Vasculitis
Types of Vasculitis:
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%