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Tuesday, July 20, 2010

Vasculitis

I hope you enjoyed morning report today, where we presented a case of young woman with disseminated necrotic skin lesions ultimately found to be pANCA+ levamisole-associated vasculitis.

Here's a case report of 2 cases from NY published just this June. Similarly, we have had a string of such cases here in Toronto. As we
mentioned, levamisole is an anti-helminthic (veterinary) drug that has been tried for
immunomodulatory/antineoplastic purposes in the past. Increasingly it is being used to
cut cocaine, which we are seeing manifest as vasculitis and as agranulocytosis in our
patient populations. The patients described in this case report were also PANCA+, and
like our patient this morning, one's biopsy more consistent with a thrombotic process.
Interestingly, the other's biopsy was more consistent with a small-vessel vasculitis,
demonstrating that levamisole-induced vasculitis can have different manifestations on
biopsy.

See the CDC morbidity & mortality weekly report
http://www.cdc.gov/mmwr/preview/mmwrhtml/mm5849a3.htm for details of a series of
agranulocytosis reports to come out of the US. Note reference 6 - it's a case series out
of Canada. Note that several of these patients came to present because of acute febrile
illnesses, and many had pharyngitis.

Lastly, here's an old but great review article on vasculitis. Figure 1 is great
for getting a sense of which vessels are involved in this inflammatory process when we
say Small, Medium, and Large-vessel vasculitis.

Table 1 gives you a great framework for:
large vessel:Giant cell and takayasu's arteritis
medium vessel: PAN, Kawasaki's and Primary CNS vasculitis
small vessel: ANCA associated (sometimes called pauci-immune for the lack of
immune-complex deposition), immune-complex mediated (infectious and noninfectious), and
paraeneoplastic (with IBD as a separate heading).

I like this article's approach to diagnosis:
1) Diagnose that there is a small-vessel vasculitis in the first place
2) Now, figure out which one it is.
The presentations can be very similar, and it is important to look for clues to help
differentiate. Purpura, nephritis, abdominal pain, peripheral neuropathy, myalgias, and
arthralgias, and constitutional symptoms can be common to many of the vasculitidies, and
looking for clues (ie which organs are involved? Is there nasal involvement? Is derm the
primary problem?). Table 3 & 4 are useful for this.

Also, make sure to send: ANCA, antinuclear antibodies, complement, cryoglobulins, fecal
blood, antibodies to hepatitis B and C, rheumatoid factor, BUN, Cr, Urine R&M (for blood,
protein, and LOOK FOR CASTS - if the lab doesn't routinely do this, ask specifically or
spin it yourself).

I won't go in to too much more detail, as it's a broad topic. The best advice I can give
you is to review it again and again, whenever you have a patient with vasculitis.

Enjoy!

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