Vasculitis

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Learning objectives

  • Appropriate settings to look for vasculitis
  • Supporting evidence to ensure firm diagnosis
  • Treatment options

Introduction

The inflammation of blood vessels can lead to changes in blood vessel structure and function as well as stasis and thrombosis. There are many different types of patterns of pathologies that affect blood vessels and some of these only happen with intracranial vessels. A size based approach is often used.

Cerebral VasculitisPoints
Large VesselGiant cell arteritis, Cranial arteritis, Takayasu arteritis
Medium sizedClassical Polyarteritis nodosa, Kawasaki disease
Small vessel
  • ANCA positive vasculitis (Wegener's granulomatosis, Microscopic polyarteritis, Churg–Strauss syndrome)
  • ANCA negative vasculitis (Cryoglobulinemic vasculitis, Behçet syndrome)
  • Primary Angiitis

Summaries of individual characteristics

Cerebral VasculitisPoints
Giant cell arteritisOlder patient at least > 50 with ESR > 50 and elevated CRP. Affects large vessels. Headache, temporal artery tenderness, jaw claudication, diplopia, transient or more lasting visual loss. The target antigen of the immune response is probably within the internal elastic layer of the vessel wall. This is supported by the observation that the anterior circulation is relatively spared due to lack of an internal elastic layer
Kawasaki syndrome A mucocutaneous lymph node syndrome. Cerebral involvement very rare. Classically seen in children with prolonged fever
Takayasu Disease A mucocutaneous lymph node syndrome. Cerebral involvement very rare. Classically seen in children with prolonged fever
Classic polyarteritis nodosa Medium sized vessels. Part of a systemic PAN. Skin, renal and other changes
Churg–Strauss SyndromeAsthma and eosinophilic granulomas pANCA/MPO
Microscopic polyangiitisAsthma and eosinophilic granulomas pANCA/MPO
Wegener's granulomatosisGranulomas of the upper airways and renal involvement. cANCA /PR3
Immune complex depositionSLE, RA
Cryoglobulinaemic angiitis

Clinical

Inflammatory changes lead to focal ischaemic changes and stroke, seizures, headache and encephalopathy. Coma may develop due to widespread oedema and raised ICP. A thorough examination of skin for vasculitic rashes, eyes for inflammatory changes, retina and even perineum for ulceration should ensure. Renal urine analysis to exclude a glomerulonephritis.

Investigations

  • Bloods: FBC, WCC, DWCC, CRP, ESR and abnormal LFTs, U&E, Urinalysis. Coagulation.
  • Complement levels: fall in vasculitis associated with immune complexes
  • Autoantibodies: Anticardiolipin-antibodies, lupus anticoagulant, electrophoresis, CK, LDH, haptoglobulin, ferritin, ACE, Cryoglobulins, TSH, thyroid antibodies, RF, ANA, Anti ds-DNA, anti-histone, complement, anti-Ro [SS-A] and anti-La [SS-B-], c- and pANCA/MPO [myeloperoxidase[MPO]], anti-endothelial antibodies
  • Miscellaneous: Drug screening, blood cultures, Syphilis serology, borreliosis, hepatitis B, and C, HIV
  • CSF analysis: may be useful. The CSF may (it is not always) inflammatory with raised protein and white cells but no evidence of organisms or malignant cells or TB which is important especially before considering Immunosuppression. For example in primary angiitis of the central nervous system the CSF may be normal.
  • MRI: will show the characteristic changes of infarction on DWI and ADC maps and gradient echo sequences will show changes. Some MRI sequences will allow evidence of inflammatory changes.
  • PET18-fluorodeoxyglucose positron emission tomography: scanning can also be useful in showing increased uptake and inflammatory changes in the vessel walls.

Management

Management requires the use of toxic immunosuppressive therapy so all attempts must be made to establish a reliable diagnosis and assess if the treatment is more harmful than the vasculitis. Standard therapies include steroids and pulse cyclophosphamide as induction treatment. An alternative option is the use of the anti- CD20 antibody rituximab. Methotrexate, azathioprine and mycophenolate mofetil are recommended as alternatives to cyclophosphamide once remission is achieved.

References