ANCA-Associated Vasculitis

Publication Date: July 7, 2021

Key Points

Key Points

  • Common clinical manifestations of granulomatosis with polyangiitis (GPA) include destructive sino-nasal lesions, pulmonary nodules, and pauci-immune glomerulonephritis.
    • GPA is characterized histologically by necrotizing, granulomatous inflammation in addition to vasculitis.
  • Common clinical manifestations of microscopic polyangiitis (MPA) include rapidly progressive pauci-immune glomerulonephritis and alveolar hemorrhage.
    • MPA is characterized histologically by vasculitis without granulomatous inflammation.
  • Common clinical manifestations of eosinophilic granulomatosis with polyangiitis (EGPA) include asthma, peripheral eosinophilia, and peripheral neuropathy.
    • EGPA is characterized histologically by eosinophilic tissue infiltration in addition to vasculitis.

Definitions and Treatments

...tions and Treatments...

...finitions for GPA, MPA, EGPAHaving tro...


...ality of Evidence and Strength of R...


Recommendations

...commendation...

...ble 3. Treatments: GPA, MPA, EGPAHaving tro...


...with polyangiitis (GPA) or Microscopic p...

...sion Induction for Active, Seve...

...th active, severe GPA/MPA, we condit...

...s with GPA/MPA with active glomerulonephr...

...active, severe GPA/MPA with alveolar hemo...

...ion Statement: For patients with active, seve...

...patients with active, severe GPA/MPA, we...

...ction for Active, Nonsevere granulomatosis with po...

...patients with active, nonsevere granulomatosis wi...

...th active, nonsevere granulomatosis with polyang...

...r patients with active, nonsevere...

...h active, nonsevere granulomatosis wi...

...ion Maintenance in granulomatosis with pol...

...ith severe GPA/MPA whose disease has entered...

...nts with GPA/MPA who are receiving rituximab for...

For patients with severe GPA/MPA wh...

For patients with severe GPA/MPA wh...

...r patients with GPA whose disease has entered re...

...ients with GPA whose disease has e...

...patients with GPA/MPA on remission maintenance the...

...Position Statement: The duration of non-GC remissi...

...ngraded Position Statement: The duration of GC th...

...ent of Relapse

...r patients with granulomatosis with polyangiiti...

...ients with granulomatosis with polyangiit...

...reatment of Refractory Disease...

...atients with severe granulomatosis w...

For patients with granulomatosis w...

...ent of Sinonasal, Airway, and Mass Les...

...ion Statement: For patients with sinonasal inv...

...tients with granulomatosis with polya...

...ts with granulomatosis with polyangiitis (...

...h granulomatosis with polyangiitis (GPA) and mass...

...ther Considerations

...atients with granulomatosis with polyangiitis (G...

...th granulomatosis with polyangiitis (G...

...ts with granulomatosis with polyangiitis (GPA)/ mi...

...nts with active granulomatosis with pol...

...ed Position Statement: The optimal duration of...


...ey Recommendations for the Treatment...


...nulomatosis with polyangiitis (EGPA)...

...mission Induction for Active, Severe...

...d Position Statement: For patients...

...n Statement: For patients with active, sever...

...patients with active, severe eosinophilic gran...

Remission Induction for Active, Nonsevere Disea...

...or patients with active, nonsevere eosino...

...tients with active, nonsevere eosinophi...

...r patients with active, nonsevere eos...

...ion Maintenance...

...h severe eosinophilic granulomatosis w...

...or patients with severe eosinophilic gr...

...ed Position Statement: The duration of GC therapy...

...tment of Relapse

...with eosinophilic granulomatosis with polya...

...ients with eosinophilic granulomatosis with p...

...tients with eosinophilic granulomatosis wit...

...h eosinophilic granulomatosis with polyangii...

...with eosinophilic granulomatosis with polyangii...

...er Considerations

...ients with newly diagnosed eosinophili...

...ition Statement: Use of leukotriene inhibitors...

...ts with eosinophilic granulomatosis with poly...

...n Statement: In patients with sinonasal...

...r patients with eosinophilic granulomatosis wit...


...ecommendations for the Treatment of Eosinophilic G...