Author: Raashid Luqmani
Consider vasculitis in the patient with systemic symptoms (e.g. fever, weight loss, malaise) and headache or evidence of organ dysfunction.
- The diagnosis of a specific vasculitis (Appendix 99.1) is based on the pattern of organ injury, characteristic features on imaging, the size of involved blood vessels and histological findings on biopsy.
- The commonest vasculitis in adults is giant-cell arteritis (GCA), usually presenting with new-onset headache in older people, associated with constitutional upset; it carries a significant risk (20–30%) of visual loss from ischaemic optic neuropathy, and so needs urgent assessment and treatment (Chapter 19).
- The main focus in the immediate management of suspected new-onset vasculitis is to contact a specialist in vasculitis for advice, while at the same time exploring the broad differential diagnosis (Tables 99.1, 99.2, 99.3). Clinical assessment of the patient and investigation are summarized in Tables 99.4 and 99.5.
- With the exception of suspected giant-cell arteritis, early intervention with high-dose corticosteroid therapy is rarely needed, and should not be given unless advised by a specialist in vasculitis.
Suspected giant-cell arteritis (GCA) (Figure 99.1; see also Chapter 19)Suspected giant-cell arteritis (GCA) (Figure 99.1; see also Chapter 19)- Consider in any patient over 50 with new-onset headache (which will usually be of days or a few weeks in duration) or visual symptoms (amaurosis fugax, diplopia and partial or complete loss of vision (Chapter 19)).
- Associated symptoms include malaise, weight loss, jaw or tongue claudication and scalp tenderness. The temporal artery may be thickened, tender or non-pulsatile, and bruits may be heard over arteries of the head and neck.
- Check full blood count, C-reactive protein, ESR, biochemical profile and blood glucose. Obtain a chest X-ray (and ultrasonography of the temporal artery, if available).
- If the clinical picture is suggestive of GCA, and the C-reactive protein is raised, start prednisolone 1 mg/kg per day (max 60 mg per day) with gastric and bone protection.
- Arrange for a temporal artery biopsy or ultrasound to be done within seven days of starting prednisolone.
- Seek advice from a rheumatologist or vasculitis specialist on management and follow-up.
New presentation of suspected systemic vasculitis (Figure 99.2)New presentation of suspected systemic vasculitis (Figure 99.2)Principles of Management
- Assess for other causes of the clinical presentation (e.g. infection; see Table 99.3).
- Look for evidence of organ-threatening or life-threatening disease, which may require admission to ICU or HDU for supportive care. Close observation and repeated re-examination of the patient with suspected systemic vasculitis is very important, as the clinical features may rapidly evolve.
- Contact a specialist in vasculitis for advice on management.
- Do not start corticosteroid or other anti-inflammatory treatment without advice from a specialist in vasculitis, as this may reduce the diagnostic yield of biopsy, and prevent the patient's inclusion in clinical trials of therapy.
- Biopsy of the affected organ is the most important test to organize; imaging may be an adequate substitute for some diseases (e.g. large-vessel vasculitis).