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DR Medhat MRCP

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ANCA<br />

o There are two main types of anti-neutrophil cytoplasmic antibodies (ANCA) :<br />

1- Cytoplasmic (cANCA)<br />

2- Perinuclear (pANCA<br />

For the exam, remember:<br />

•cANCA : Wegener's Granulomatosis<br />

•pANCA : Churg-Strauss syndrome + others (see below)<br />

cANCA:<br />

o Most common target serine proteinase 3 (PR3)<br />

o Some correlation between cANCA levels and disease activity<br />

o Wegener's granulomatosis, positive in > 90%<br />

o Microscopic polyangiitis, positive in 40%<br />

pANCA:<br />

o Most common target is myeloperoxidase (MPO)<br />

o Cannot use level of pANCA to monitor disease activity<br />

o Associated with<br />

1. Immune crescentic glomerulonephritis (positive in c. 80% of patients)<br />

2. Microscopic polyangiitis, positive in 50-75%<br />

3. Churg-Strauss syndrome, positive in 60%<br />

4. Wegener's granulomatosis, positive in 25%<br />

Other causes of positive ANCA (usually pANCA)<br />

5. Inflammatory bowel disease (Ulcerative colitis > Crohn's)<br />

6. Connective tissue disorders: RA, SLE, Sjogren's<br />

7. Autoimmune hepatitis.<br />

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