Vasculitis

Classification

• Small-vessel: often have palpable purpura
  • ANCA-associated vasculitis
    • Microscopic polyangiitis: anti-MPO/p-ANCA; pulmonary-renal syndrome
    • Granulomatosis with polyangiitis: anti-PR3/c-ANCA; pulmonary-renal syndrome
    • Eosinophilic granulomatosis with polyangiitis: more often anti-MPO; rhinosinusitis, asthma, eosinophilia, glomerulonephritis
  • Non-ANCA
    • Immune-complex
      • Anti-GBM disease
      • Cryoglobulinemic vasculitis: often associated with hepatitis C
    • IgA vasculitis (HSP): abdominal pain, arthritis, rash, sometimes IgA nephropathy
    • Anti-C1q vasculitis (hypocomplementemic urticarial vasculitis)
• Medium-vessel: typically cause a lacy rash
  • Polyarteritis nodosa: often associated with hepatitis B
  • Kawasaki disease: usually in children, associated with mucocutaneous lymph node syndrome
• Large-vessel
  • Takayasu arteritis: usually aortitis
  • Giant cell arteritis: usually carotids and its branches
• Variable-vessel
  • Behçet syndrome: recurrent oral and genital ulcers with skin, eye, joint, GI, or CNS involvement
  • Cogan syndrome: ocular inflammatory lesions
  • Primary CNS vasculitis
• Associated with a systemic disease
  • Lupus
  • Rheumatoid arthritis
  • Relapsing polychondritis
  • Etc.

Clinical Manifestations

History

• Fever, fatigue, weight loss, arthralgias
• Scleritis
• Nasal crusting, epistaxis, or upper airway disease: GPA
• Acute foot or wrist drop
• Limb claudication, especially unusual patient or location: Takayasu or giant cell arteritis
• Hemoptysis: ANCA-associated vasculitis
• Glomerulonephritis: ANCA-associated vasculitis or anti-GBM disease
• Drug exposure in past 6-12 months
  • Cocaine/Levamasole
  • Hydralazine
  • Propylthiouracil (PTU) more than methimazole (MMI)
  • Minocycline
  • Allopurinol, penicillamine, procainamide, thiamazole, clozapine, phenytoin, rifampicin, cefotaxime, isoniazid, indomethacin
• Hepatitis B or C
• Lupus or other rheumatologic diseases

Physical exam

• Mononeuritis multiplex: sensory or motor neuropathy, which can be subtle, and symmetric or asymmetric
• Palpable purpura: small-vessel
• Diminished peripheral pulses: large-vessel vasculitis

Investigations

• ANA: lupus and other rheumatologic diseases
• Complements: lupus and mixed cryoglobulinemia
• ANCA: for ANCA-associated vasculitis
• CXR if respiratory symptoms
• EMG if concern for mononeuritis multiplex
• Lumbar puncture if concern for primary CNS vasculitis