- This a systemic necrotising vasculitis of medium-sized arteries without the involvement of smaller vessels.
- It may be associated with hepatitis virus infection.
- Only about 20% have any cerebral involvement.
- Patients have typical systemic illness, myalgia, weight loss, fever, arthritis
- Peripheral ischaemic changes. They can have a polyneuropathy.
- Ischaemic and haemorrhagic strokes as well as an encephalopathy.
|PAN may be diagnosed with three of these ten criteria (82% sensitivity, 86% specificity), if other vasculitides are excluded|
|1||Loss of weight >4 kg|
|5||Mononeuritis or polyneuritis|
|6||Blood pressure elevation >90 mmHg|
|7||Creatinine >1,5 mg/dl|
|8||Hepatitis B or C virus antibodies|
|9||Pathologic arteriography (aneurysm, occlusions)|
|10||Typical histology finding|
- FBC, U&E, ESR, CRP, Vasculitis screen
- Brain imaging CT/MRI/MRA
- Biopsies classically show necrotising granulomatous inflammation.
- Induction therapy is usually with Steroids and Cyclophosphamide.
- Plasma exchange has been used.
- If there is positive viral serology then appropriate antivirals against Hepatitis B or C may be given.
- Diagnosis and treatment of cerebral vasculitis, Peter Berlit. Ther Adv Neurol Disord (2010) 3(1) 2942