Vasculitides Flashcards
Vasculitis types
Large - giant cell arteritis, Takayasu’s arteritis
Medium - Polyarteritis nodosa, Kawasaki disease
Small - microscopic polyangiitis, Wegener’s granulomatosis, Churg-Strauss syndrome, Goodpasture’s, Henoch-Schonlein purpura
Variable vessel vasculitis - Behçet’s, Cogan’s
What is GCA
Giant cell arteritis = temporal arteritis
GCA presentation
Headache (temporal artery/scalp tenderness)
Tongue/jaw claudication
Amaurosis fugax (temp blindness)
>55 yrs
GCA associations
Polymyalgia rheumatica in 50%
GCA investigations
ESR + CRP very raised, dec Hb
Vision testing, damage often irreversible
Temporal artery biopsy (biopsy sometimes negative as occasionally skip lesions)
FDG-PET
GCA management
Prednisolone 60mg/d oral immediately, IV methylpred if sight loss occurring
Typically 2yr course induces remission, reduce pred once ESR decreased (main cause of mort/morb is steroid use so balance risk)
Use PPI/bisphosphonates/calcium with colecalciferol/aspirin if needed
What is Takayasu’s arteritis
Granulomatous inflammation of aorta and major branches causing systemic effects from stenosis/thrombosis/aneurysm of aorta
Takayasu’s arteritis presentation
Rare outside of Japan
20-40yr old women typically
Symptoms depend on arteries involved, can be cerebral/eye/upper limb
Takayasu’s arteritis management
BP control
Very good prognosis, 1mg/kg/day prednisolone
Methotrexate/cyclophosphamide in resistant disease
Surgery for critical stenosis
What is polyarteritis nodosa
Necrotising vasculitis that causes aneurysms + thrombosis in medium-sized arteries, symptoms depend on infarcted arteries
Polyarteritis nodosa presentation
Rare in UK and can be associated with Hep B
Rash, punched out ulcers
Renal/cardiac/GI/GU issues
Polyarteritis nodosa investigations
ANCA -ve
Inc WCC, ESR + CRP
Renal/mesenteric angiography or renal biopsy can be diagnostic
Polyarteritis nodosa management
Control BP
Steroids for mild cases, steroid sparing if more severe
What is Kawasaki disease
Febrile vasculitis causing coronary aneurysms
Kawasaki disease presentation
Typically 18-24 mths
3 phases:
Acute febrile for 1-2 wks
Subacute lasts 2-4 wks after fever and CA aneurysms develop, thrombocytosis
Convalescent wks 6-12, resolution of signs + inflammatory markers