Vasculitis Referral Guideline

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The primary systemic vasculitides are a varied group of rare diseases that are characterised by inflammation of blood vessels. They are often difficult to diagnose because the clinical manifestations are varied and can mimic several infectious, neoplastic, and other autoimmune conditions. The type of vasculitis is often related to a patient's age, gender, and ethnic origin.

A definitive diagnosis of systemic vasculitis should be made by a person experienced in diagnosing vasculitis, in the presence of characteristic symptoms and signs of vasculitis, and at least one of the following: histologic evidence of vasculitis; positive serology for ANCA; or specific indirect evidence of vasculitis.

History & Examination

The clinical presentations are highly varied and will depend upon the distribution and extent of organ involvement and size of the involved vessels.

  • Systemic: Fever, weight loss, fatigue, myalgia, arthralgia
  • Cutaneous: purpura, ulceration, gangrene
  • Ocular: red eye, visual loss, diplopia
  • Mucocutaneous: oral & genital ulceration
  • ENT: Hearing loss (conductive, sensorineural); sinus pain; nasal crusting, epistaxis
  • Chest: wheeze, cough, haemoptysis, dyspnoea
  • Cardiac: pericarditis, cardiomyopathy, ischaemic chest pain
  • Abdomen: Ischaemic abdominal pain, bloody diarrhoea
  • CNS: Peripheral neuropathy, mononeuritis multiplex, CN palsy, cord lesions, stroke syndromes
  • Renal: blood and protein on urine dipstick, deteriorating renal function

Helpful Investigations

  • ESR, CRP – usually elevated
  • FBC, U&E, LFTs
  • If high clinical index of suspicion check ANCA (MPO/ PR3) – this can be negative.
  • Urine dipstick – if positive for blood/protein refer to renal.
  • Further investigations are guided by system involved – in many cases additional imaging or biopsies of the affected organ(s) is required to confirm the diagnosis of vasculitis.

Most vasculitidies do not have an associated blood marker. Elevated ANCA levels are associated with the ANCA-associated, small vessel vasculitidies (e.g. Granulomatosis with Polyangiitis, Eosinophillic Granulomatosis with Polyangiitis, Microscopic Polyangiitis, Pauci-Immune Glomerulonephritis). However, in isolation, without other relevant clinical features, elevated ANCA levels are not diagnostic and can be misleading. Elevated ANCA levels can also be related to inflammatory bowel disease, primary sclerosing cholangitis, rheumatoid arthritis, SLE, autoimmune liver disease and some infectious disease.

Primary Care Management

If vasculitis is suspected then the patient should be referred urgently to the relevant specialty.

Who to Refer

  • The majority of patients seen with vasculitis are referred by secondary care once other causes have been ruled out.
  • Consider referring patients with unexplained systemic symptoms and raised ESR/CRP and/or positive ANCA (MPO/PR3) where there is no evidence of underlying infection or malignancy.
  • If vasculitis affects one system only consider direct referral to the relevant specialty:
    • Isolated cutaneous vasculitis – dermatology,
    • Evidence of renal involvement – nephrology,
    • Cerebral vasculitis alone – neurology.

Who Not to Refer

  • Those who have known sepsis or malignancy with weakly positive ANCA (MPO/PR3).
  • Diagnosis of vasculitis requires confirmation of vasculitic involvement within the symptomatic or dysfunctioning organ(s). Patients who are non-specifically unwell, who do not have localising symptoms or evidence of end-organ dysfunction are unlikely to have a vasculitis.

Last reviewed: 01 November 2020

Next review: 01 November 2022

Author(s): Dr Anna Ciechomska

Co-Author(s): Rheumatology team: Dr Robin Munro, Dr Karen Donaldson, Dr Elizabeth Murphy, Dr James Dale, Dr Sanjiv Nandwani, Dr Georgiana Young, Dr May Chee, Mr Nico Groenendijk; GP representatives: Dr Tyra Smith, Dr Mark Russell, Dr Linda Findlay, Dr Paul Deehan, Dr Lucy Munro; Medical directorate: Dr Mehrdad Malekian

Approved By: Dr Karen Donaldson

Reviewer Name(s): Dr Anna Ciechomska