o Granulomatous polyangiitis (GPA)
o Microscopic polyangiitis (MPA)
o Eosinophilic granulomatous polyangitis (EGPA)
• Anti-neutrophil cytoplasmic antibody (ANCA)
o Cytoplasmic ANCA ( cANCA)
• It develops against the proteinase-3 antigen.
Often associated with granulomatous polyangiitis (Wegener's granulomatosis)
o Perinuclear ANCA (pANCA)
Develops against the myeloperoxidase (MPO) enzyme
It is often associated with eosinophilic granulomatous polyangiitis (Churg-Strauss syndrome) and microscopic polyangiitis.
• They can have pulmonary and renal involvement (pulmonary-renal syndrome).
• Deposition of immune complexes in tissue biopsies is not expected in ANCA-associated vasculitides (pauci-immune).
• Rarely; ANCA positive vasculitis may develop with the use of propylthiouracil (PTU) and hydralazine.
Pulmonary-Renal syndromes
ANCA positive vasculitides
systemic lupus erythematosus SLE
Goodpasture syndrome