This site is intended for healthcare professionals

Go to /sign-in page

You can view 5 more pages before signing in

Go to /pro/cpd-dashboard page

This page is worth 0.05 CPD credits. CPD dashboard

Go to /account/subscription-details page

This page is worth 0.05 CPD credits. Upgrade to Pro

Microscopic polyangiitis

Last reviewed dd mmm yyyy. Last edited dd mmm yyyy

Authoring team

Microscopic polyangiitis affects the smaller arteries and is the most common form of arteritis. There may be a history of hypersensitivity to drugs or foreign proteins, and the natural history is a short duration of vasculitis in the small veins, arteries and capillaries.

  • microscopic polyangiitis is characterized by (1,2,3):
    • renal disease
    • pulmonary disease
    • ANCA directed against myeloperoxidase (p-ANCA)
  • characterised by (3):
    • necrotising vasculitis, with few or no immune deposits, predominantly affecting small vessels (i.e. capillaries, venules or arterioles)
    • necrotising arteritis involving small and medium arteries may be present
    • necrotising glomerulonephritis is very common
    • pulmonary capillaritis often occurs
    • granulomatous inflammation is absent

This disease is uncommon with an annual incidence depending on geographic location (varying from 2.7 to 11.6 cases per million)

  • most frequent in the elderly but has a wide age range with men being affected more often than women

Management

  • induction treatment for most patients with ANCA associated vasculitis (AAV) should be with cyclophosphamide or rituximab and glucocorticoids
  • AAV should be considered to be a chronic disease needing longterm immunosuppressive therapy
  • rituximab should be considered as an alternative induction agent for those at high risk of infertility and infection
  • mortality remains high, and late death is due to cardiovascular disease, infection (secondary to treatment) and malignancy

Reference:

  • Davies DJ. Small vessel vasculitis. Cardiovascular Pathology 2005; 14 (6): 335-346.
  • ARC Autumn 2012. Topical Reviews - ANCA-associated vasculitis; 1:1-12.
  • Yates A, Watts R. ANCA-associated vasculitis. Clinical Medicine 2017 Vol 17, No 1: 60–4

Related pages

Create an account to add page annotations

Annotations allow you to add information to this page that would be handy to have on hand during a consultation. E.g. a website or number. This information will always show when you visit this page.