00:00
So, what are cryoglobulins? So cryoglobulins are immunoglobulins just like any other immunoglobulin
made by a B-cell that just happen to precipitate in a glass tube under cold conditions.
00:15
Cryoglobulinemic vasculitis is a small vessel vasculitis that's due to mixed cryoglobulinemia. The
cause of this cryoglobulin phenomenon is in some ways idiopathic but we know that it's due to
the excess production of both IgG immunoglobulins and IgM immunoglobulins typically due to a
polyclonal gammopathy most often in the setting of hepatitis C though as I've listed here
there's a variety of other infections, inflammatory and auto-immune etiologies that can also
bring about this polyclonal gammopathy. For the purposes of the boards, when you hear
cryoglobulinemic vasculitis, you should be thinking about hepatitis C. Cryoglobulinemic vasculitis
tends to also run a fairly indolent course. Rarely, it can present with a somewhat acute
presentation. The symptoms that you're looking for are kind of vague, no weakness, fatigue,
you may certainly find palpable purpura as we're seeing in our patients images here on the
right and there's oftentimes renal involvement. The renal involvement classically is going to be
a membranoproliferative glomerulonephritis which can have diffuse IgM deposition in capillary
loops on a renal biopsy. Remember, cryoglobulinemic vasculitis is one of the immune complex
mediated small vessel vasculitides. So you're going to find immune complexes when you go
looking for them. In addition, like many vasculitides, you may find a peripheral neuropathy,
you may find arthralgias. The diagnosis of a cryoglobulinemic vasculitis is going to be based on
serum testing and then on biopsy. Your serum testing is just a serum cryoglobulin level which,
while it wouldn’t tell you for sure that that's what's causing the person's symptoms, it would
at least tell you whether the patient does in fact have cryoglobulins or not. And then you're
deciding between a skin biopsy and a renal biopsy, skin biopsy is certainly a lot easier and it
would show a leukocytoclastic vasculitis. If you're lucky, that biopsy might also show associated
cryoglobulins in the vessel lumen. Remember that cryoglobulinemic vasculitis is an immune
complex-mediated small vessel vasculitis, which means you should find immune complexes deposited
within the biopsy tissue. Lastly, you're going to treat with, again, corticosteroids. You might
also add on rituximab or cyclophosphamide depending upon certain factors we won't go into.
02:46
Plasmapheresis is also a consideration if there's a very high burden of cryoglobulins. And of
course if the person is hep C positive, you should go ahead and treat the hepatitis C particularly
with our robust ___ of treatment options today. In any event, I'm thinking that our
14-year-old girl is pretty unlikely to have contracted hepatitis C, I hope and we don't really
have much else going for any other cryoglobulinemic vasculitis causes right now like lupus
either so let's go back to our list.