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Anti-neutrophil cytoplasmic antibody-associated vasculitis

Evidence: T Cell

Names 

Anti-neutrophil cytoplasmic antibody-associated vasculitis
ANCA-associated vasculitis
Granulomatosis with polyangiitis
Eosinophilic granulomatosis with polyangiitis
Microscopic polyangiitis
Churg-Strauss syndrome
Wegener’s Granulomatosis

Description

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of diseases characterized by destruction and inflammation of small vessels. The clinical signs vary and affect several organs, such as the kidney, stomach, intestine, and lung. Skin lesions, such as purpura and urticaria, result when blood from small vessels leaks under the skin. AAV occurs when neutrophils attack small and medium vessels of the body. The underlying reason for this remains unclear.

Patient Groups

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Prevalence

US Cases: 15,000 - 60,000 [Berti 2017]

Typical Age of onset

Varies depending on type of vasculitis; estimates range from 15 to 70 years old. (No data referenced.)

Symptoms

These symptoms vary depending on the type of vasculitis:

  • Fever

  • Headache

  • Fatigue

  • Weight loss

  • General aches and pains

  • Night sweats

  • Rash

  • Numbness

  • Weakness

Source: Mayo Clinic