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Thorax 55:870-877 doi:10.1136/thorax.55.10.870
  • Review series

Churg-Strauss syndrome

Table 2

ANCA in diseases other than systemic vasculitis

Disease ANCA
Cystic fibrosis c, p or aANCA often to BPI found in 32%26
Bronchogenic carcinoma pANCA seen in non small cell lung cancer27
Mycobacterial infection aANCA correlation uncertain28
Sarcoidosis cANCA in 5%29
HIV c or pANCA in 20%, MPO or PR3 positive30
Inflammatory bowel disease/Crohn's disease p or aANCA, ulcerative colitis 60–75% and Crohn's disease 15–25%, usually lactoferrin not MPO31
Primary biliary cirrhosis pANCA in 30–40%32 33
Autoimmune hepatitis c or pANCA in 35–72%32 34
Sclerosing cholangitis pANCA often to lactoferrin in 60–75%32-34
Amoebiasis cANCA positive 97% (PR3 positive in 75%)35
Post streptococcal glomerulonephritis p or aANCA in 9% occasionally MPO positive36
Chromomycosis cANCA in 20% not to PR-337
Malaria aANCA in 50% (anti-cathepsin-G Ab)38
Leprosy c or pANCA positive in 17%39
Myeloproliferative disorders pANCA positive in 40% (MPO positive 8%)40
Systemic lupus erythematosis pANCA positive in 42%41
Juvenile chronic arthritis c or pANCA not to PR3 or MPO42
Rheumatoid arthritis pANCA positive most frequently to lactoferrin43
Chronic graft versus host disease aANCA 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.

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