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p-ANCA c-ANCA causes

25975 Views 11 Replies 9 Participants Last post by  m82_ghasemi
Hi everyone..

I had a quick question in case anyone had some insight to offer... In Polyarteritis Nodosa and Churg-Strauss Syndrome, you'll typically see an elevation of p-ANCA whereas in Wegener's Granulomatosis you'll see c-ANCA - am I right about this?

Specifically, I'm confused about the PAN and p-ANCA association.. Some places say the association is present. Other places say that p-ANCA is a USMLE buzzword for PAN and yet others (like First AID) only associate p-ANCA with microscopic polyangitis (not sure how this fits into this) Yet others say there is really no p-ANCA and PAN association...

The only part is seem to really get is treat all of these with steroids or cyclophosphamide, but I'm not really getting the diagnosis part - any help would be appreciated :)

Thanks!
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p ANCA is only seen in microscopic polyangiitis not seen in polyarteritis nodosa.
Reference: Harrison's
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Perinuclear pattern of antineutrophil cytoplasmic antibodies (p-ANCA) - not associated with "classic" polyarteritis nodosa, but is present in a form of the disease affecting smaller blood vessels, known as microsopic polyangiitis or leukocytoclastic angiitis.
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Here's a list of causes of the positive ANCA

p- ANCA positive (antibodies against myeloperoxidase) in microscopic polyangiitis and Churg Strauss syndrome and Crescentic glomerulonephritis
c- ANCA positive (antibodies against proteinase 3) in Wegener granulomatosis, ulcerative colitis, and ankylosing spondylitis (the last two it's unrelated to disease activity)
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1)wegner --> cANCA
2)Microscopic plyangitis:-->P ANCA
3)Churg strauss--> P ANCA in 60% of cases & cANCA only in 10% & no ANCA in 30%
SO The ANCA-associated vasculitides include Wegener's granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome, and idiopathic pauci-immune crescentic glomerulonephritis
with no relation between PAN , ANCA
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1)wegner --> cANCA
2)Microscopic plyangitis:-->P ANCA
3)Churg strauss--> P ANCA in 60% of cases & cANCA only in 10% & no ANCA in 30%
SO The ANCA-associated vasculitides include Wegener's granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome, and idiopathic pauci-immune crescentic glomerulonephritis
with no relation between PAN , ANCA
Just remember that Polyarteritis Nodosa has a 30% association with Hepatitis B.
~First Aid
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Just remember that Polyarteritis Nodosa has a 30% association with Hepatitis B.
~First Aid
now just less than 10% due to preventive masures and early treatment
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golyan gave a menemonic for this.

P (p) polyart...-P ANCA.

C- Anca= Wegner Gran. Since it is treateded by C( C)Cyclophophomide...
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p-ANCA is also seen in some people with primary sclerosing cholangitis (a notable extra intestinal symptom in people with UC)
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With all the respect, Goljan was wrong by that time... In fact P-ANCA rules out Polyarteritis Nodosa...

References: FA / DIT / UWorld and Kaplan Qbank.
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p-ANCA and c-ANCA

Perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA) are found in up to 80% of primary sclerosing cholingitis (PSC) patients - they are atypical, according to Robbins, because they are directed against a nuclear envelope protein insted of myeloperoxidase; however, the histologic appearance is the same as other p-ANCAs.

p-ANCAs are also found in patients with Churg-Strauss syndrome (50%), microscopic polyangiitis (60%; rarely positive in polyarteritis nodosa now that PAN and MPA are classified as different diseases, but might still be associated on the boards), Kawasaki disease, glomerular basement membrane disease, rheumatoid arthritis (especially JRA), IBD (Crohn's and ulcerative colitis), and drug-induced vasculitis.
75-90% of PSC patients have IBD
-- 87% of these ulcerative colitis
-- 13% with Crohn colitis
c-ANCA is associated with Wegener's granulomatosis.
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