The document discusses several types of vasculitis: - Antineutrophil cytoplasmic antibody associated vasculitis is diagnosed via biopsy and treated with high dose glucocorticoids and cyclophosphamide. It often relapses so patients need long term monitoring. - Takayasu arteritis is diagnosed via angiography showing vessel abnormalities and treated with glucocorticoids and immunosuppressants. - Giant cell arteritis/polymyalgia rheumatica is diagnosed via temporal artery biopsy or PET scan and treated with high dose prednisolone tapered over 8 weeks.

1. ANTINEUTROPHIL CYTOPLASMIC ANTIBODY ASSOCIATED
VASCULITIS
Investigation show:
• Leucocytosis
• Elevated CRP, ESR,Proteinase-3(PR3)
• Diagnosis should be confirmed by biopsy of the kidney or lesion in the
sinuses and upper airway
TREATMENT :
• High dose glucocorticoids(intravenous methylprednisolone 0.5-1g for
3 days then oral prednisolone 0.5 mg/kg )
• Intravenous cyclophosphamide0.5-1g every 2 weeks for 3 month

2. • Followed by maintenance therapy with lower dose glucocorticoid
and azathioprine , methotrexate
• AAV has a tendency to relapse and patients must be followed on a
regular and long term basis , monitoring urinalysis for blood and
protein , plasma creatinine ,ESR,CRP, lung function and PR3 or MPO
antibody titres

3. TAKAYASU ARTERITIS
• Investigation show – normocytic , normochromic anaemia
• Diagnosis is based on angiography –which revels coarctation ,
occlusion and aneurysmal dilatation
• Treatment – high dose glucocorticoids and immunosuppresants

4. KAWASAKI DISEASE
• Treatment – aspirin 5mg/kg daily for 14 days
IVIg 400 mg/kg daily for 4 days
POLYARTERITIS NODOSA :
• Diagnosis is confirmed by magnetic resonance angiography – which
show multiple aneurysms and smooth narrowing of mesenteric ,
hepatic or renal system
• Treatment – high dose glucocorticoids and immunosuppressant

5. GIANT CELL ARTERITIS / POLYMYALGIA RHEUMATICA
• Investigation show :
• Elevated ESR
• Normochromic normocytic anaemia
• CRP – may be elevated
• Abnormal liver function
3 investigation to consider:
• Temporal artery biopsy
• Ultrasound of temporal arteries
• Fluorodeoxyglucose positron emission tomography (19 FDG PET scan)

6. Biopsy finding:
• Fragmentation of the internal elastic lamina with necrosis of media
• Inflammatory cell infiltrate
• On ultrasound :temporal artery show halo sign
Treatment :
• Prednisolone ( in GCA 60-80 mg is used but in PMR 15- 20 mg ) is
used
• In both condition, glucocorticoid dose should be progressively
reduced , guided by symptom and ESR with the aim of reaching a
dose of 10-15 mg by about 8 weeks

7. BEHCET’S DISEASE
• Chronic relapsing multisystem autoimmune inflammatory disease
• Target small arteries and venules
• Oral ulcer
• Genital ulcers
• Skin lesions – erythema nodosum or acneiform lesion
• Ocular involvement - anterior or posterior uveitis or retinal vasculitis

9. • Oral ulcer managed with topical glucocorticoid preparation ( soluble
prednisolone mouthwashes , glucocorticoid pastes )
• Colchicine can be effective for erythema nodosum and arthralgia
• Glucocorticoids and immunosuppressants are indicated for uveitis
and neurological disease