What's on this Page
Antineutrophil Cytoplasmic Antibody Associated Vasculitis
What are the primary ANCA associated vasculitides (AAV), and why are they classified in the current manner?
Following the discovery of ANCA in 1985, the International Chapel Hill Consensus Conference reclassified vasculitic syndromes to conform to a better understanding of their pathophysiology. Over time, this classification has been further refined as follows:
• GPA (formerly known as Wegener’s granulomatosis).
• MPA.
• EGPA (Churg–Strauss syndrome).
• Renal-limited vasculitis (RLV) with pauci-immune necrotizing/crescentic glomerulonephritis (GN).
6 Interesting Facts of Antineutrophil Cytoplasmic Antibody Associated Vasculitis
1. Granulomatosis with polyangiitis (GPA) predominantly affects the upper and lower respiratory tracts and kidneys and is typically associated with proteinase 3 antineutrophil cytoplasmic antibody (PR3-ANCA).
2. Microscopic polyangiitis (MPA) should be considered in all patients presenting with a pulmonary-renal syndrome and is typically associated with myeloperoxidase (MPO)-ANCA.
3. PR3-ANCA is associated with disease relapse; the presence of this antibody, more than the clinical designation of GPA or MPA, is associated with a relapsing disease course.
4. Eosinophilic granulomatosis with polyangiitis (EGPA) presents as pulmonary infiltrates and eosinophilia in a patient with adult-onset asthma. The diagnosis is unlikely in the absence of severe asthma and marked eosinophilia.
5. Rituximab is an effective first-line agent for induction and maintenance therapy in patients with GPA and MPA.
6. Mepolizumab, a monoclonal antibody to interleukin (IL)-5, is the first medication approved by the Food and Drug Administration (FDA) for the treatment of EGPA.
