Anti-Neutrophil Cytoplasmic Antibody (ANCA)
History
- First described in early 1980’s
Physiology
- Anti-Neutrophil Cytoplasmic Antibodies (ANCA): antibodies directed against neutrophil microbiocidal components, which are used in host defense
- ANCA antibodies activate neutrophils, monocytes, and endothelial cells
- Role of Infection in ANCA Development
- ANCA has been found to be directed against various viral/bacterial/fungal/protozoal antgens, as well as endocarditis and cystic fibrosis
- Chronic or recurent infection can result in exacerbation of pumonary vasculitis or relapse
- Hypothesis: infections may give rise to ANCA through molecular mimicry
Types of ANCA
- c-ANCA (cytoplasmic staining): typically directed against proteinase 3 (PR3)
- Sensitivity: approximately 90-95% for systemic Wegener’s Granulomatosis, but only 65-85% in organ-limited Wegener’s Granulomatosis
- p-ANCA (perinuclear staining): typically directed against myeloperoxidase (MPO)
- Sensitivity/Specificity: generally considered poor sensitivity and specificity (as they can be found in multiple diseases)
- x-ANCA: typically found in inflammatory bowel disease
Measurement of ANCA
- ELISA
- Indirect Immunofluorescence on ETOH-Fixed Neutrophils
Etiology of Elevated ANCA
- Wegener’s Granulomatosis (see [[Wegeners Granulomatosis]]): c-ANCA
- Churg-Strauss Syndrome (see [[Churg-Strauss Syndrome]]): p-ANCA
- Microscopic Polyangiitis (see [[Microscopic Polyangiitis]]): p-ANCA
- Pauci-Immune Glomerulonephritis (see [[Pauci-Immune Glomerulonephritis]]): p-ANCA
- Isolated Pulmonary Capillaritis (see [[Isolated Pulmonary Capillaritis]]): p-ANCA
- Polyarteritis Nodosa (see [[Polyarteritis Nodosa]]): p-ANCA
- Rheumatoid Arthritis (see [[Rheumatoid Arthritis]]):: p-ANCA
- Goodpasture’s Syndrome (see [[Goodpastures Syndrome]]): p-ANCA
- Drugs
- All-Trans Retinoic Acid (see [[All-Trans Retinoic Acid]])
- Phenytoin (see [[Phenytoin]])
- Propylthiouracil (see [[Propylthiouracil]])
- Mitomycin (see [[Mitomycin]])
Relationship of ANCA to Disease Activity
- ANCA titers do not correlate with disease activity or predict relapse