Vasculitides

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This article deals with the vasculitides (singular vasculitis).

Overview

Most common:[1]

  • Polyarteritis nodosa (PAN).
  • Microscopic polyangiitis.
  • Wegener's granulomatosis.
  • Predominantly cutaneous vasculitis.
  • Giant cell arteritis (GCA).

Grouping by size

Small vessel vasculitides:

  • Predominantly cutaneous vasculitis.
  • Henoch-Schoenlein purpura.
  • Essential cryoglobulinemic vasculitis.
  • ANCA-associated:
    • Wegener's granulomatosis (c-ANCA > p-ANCA).
    • Churg-Strauss syndrome (50% ANCA +ve).
    • Microscopic polyangiitis (usually p-ANCA)

Large vessel vasculitides:[2]

  • Giant cell arteritis (AKA temporal arteritis).
  • Takayasu's arteritis.

Giant cell arteritis

  • AKA temporal arteritis.

Clinical

Features:

  • Classic finding: jaw claudication, in a patient older than 50 years.
  • Other findings: headache, vision loss or diplopia, scalp tenderness, polymyalgia, weight loss, chills, fever.

Work-up:

  • CRP, ESR, temporal artery biopsy.

Treatment:

  • Treat right away with high dose steroids.

Takayasu's arteritis

General:[3]

  • Disease of medium/large arteries.
    • Classically involves the aortic arch (leading to decreased pulses in the upper limbs).
  • Typically in patients <40 yrs old.
  • Usually asian.

Microscopic

Features:[4]

  • Adventitial mononuclear infiltrate with perivascular cuffing of the vasa vasorum.
  • Mononuclear inflammation in media.
  • Granulomas, giant cells.
  • +/-Patchy necrosis of media.

References

  1. TN05 RH3.
  2. TN05 RH20.
  3. PBoD P.538.
  4. PBoD P.538.