Table 2

ANCA in diseases other than systemic vasculitis

Disease ANCA
Cystic fibrosisc, p or aANCA often to BPI found in 32%26
Bronchogenic carcinomapANCA seen in non small cell lung cancer27
Mycobacterial infectionaANCA correlation uncertain28
SarcoidosiscANCA in 5%29
HIVc or pANCA in 20%, MPO or PR3 positive30
Inflammatory bowel disease/Crohn's diseasep or aANCA, ulcerative colitis 60–75% and Crohn's disease 15–25%, usually lactoferrin not MPO31
Primary biliary cirrhosispANCA in 30–40%32 33
Autoimmune hepatitisc or pANCA in 35–72%32 34
Sclerosing cholangitispANCA often to lactoferrin in 60–75%32-34
AmoebiasiscANCA positive 97% (PR3 positive in 75%)35
Post streptococcal glomerulonephritisp or aANCA in 9% occasionally MPO positive36
ChromomycosiscANCA in 20% not to PR-337
MalariaaANCA in 50% (anti-cathepsin-G Ab)38
Leprosyc or pANCA positive in 17%39
Myeloproliferative disorderspANCA positive in 40% (MPO positive 8%)40
Systemic lupus erythematosispANCA positive in 42%41
Juvenile chronic arthritisc or pANCA not to PR3 or MPO42
Rheumatoid arthritispANCA positive most frequently to lactoferrin43
Chronic graft versus host diseaseaANCA in 17%, no antigenic specificity44
  • ANCA = antineutrophil cytoplasmic antibody; cANCA = cytoplasmic ANCA; pANCA = perinuclear ANCA; aANCA = atypical ANCA; MPO = myeloperoxidase, PR-3 = proteinase 3; BPI = bacterial permeability increasing protein.