Vasculitis - July 20, 2012

Thank you to team 6 for bringing an interesting case and to Dr. Ho Ping Kong (“As brilliant as House, but nicer.”) for hosting.

Among other things (malignant hyperthermia can be diagnosed with muscle biopsy, nasopharyngeal cancer is EBV associated, etc …), we discussed a case of a woman with likely non-acute kidney disease, rash, and p-ANCA positivity.  This is suspicious for a vasculitic process.  We talked about that imaging (in addition to blood rheumatological markers) is an important part of a vasculitic work-up.  Of course, biopsy can be very helpful as well.

A commonly used classification scheme of systemic vasculitis is the “Chapel Hill” classification, named after the Internal Consensus Conference in 1994 where it was convened for this matter.  It is published in:  Jennette JC, Falk RJ,Andrassy K, Bacon PA, Churg J, Gross WL, Hagen EC, Hoffman GS, Hunder GG,Kallenberg CG, et al. Nomenclature of systemic vasculitides. Proposal of aninternational consensus conference. Arthritis Rheum. 1994 Feb;37(2):187-92.

We will briefly summarize the classification here, but you can get the full details from the actual article:

- Large vessel:  Giant cell (temporal) arteritis, takayasu arteritis

- Medium vessel:  Kawasaki disease, Polyarteritis nodosa

- Small vessel:  Churg-Straus syndrome, Wegener’s granulomatosis, microscopic polyangiitis, cutaneous leukocytoclastic angiitis, essential cryoglobulinemic vasculitis, Henoch-Schonlein purpura.

Churg-Straus, Wegener’s, and microscopic polyangiitis have strong associations with ANCA.

Have a fabulous Friday!