Anti-GBM Disease (Goodpasture's)

00:00 Next up, let's move up the list and let's talk about anti-glomerular basement membrane disease which, I'll remind you, is one of the small vessel vasculitides and to keep highlighting this as we go through. Okay, so anti-GBM antibody disease is actually one of the more rare vasculitides.

00:19 It can affect men and women of any age, the pathophysiology of anti-GBM disease involves circulating antibodies that are attacking either glomerular basement membranes or alveolar basement membranes.

00:33 Used to be called Goodpasture's disease as I mentioned before and when you think Goodpasture's you should be thinking about the kidneys and the lungs together. When you look at the kidneys, you'll find a rapidly progressive glomerulonephritis. On urologic testing, you'll simply find urinalysis with proteinuria, maybe some dysmorphic red cells and red cell cast would certainly be a clue to this diagnosis. In the lungs, you're seeing hemorrhage. Perhaps you'll just see pulmonary infiltrates that can be kind of vague, some dyspnea and cough when you're inquiring about the patient's symptoms, and you may even potentially see overt hemoptysis under extreme cases. Importantly, unlike most of the other vasculitides, anti-GBM disease for whatever reason does not typically present with systemic symptoms like fevers and weight loss as we're seeing in our patient. The diagnosis, like all the vasculitides, is usually going to require biopsy. There is 1or 2 exceptions that we hear about down the road and on biopsy shown here we have a normal glomerulus and then we have a crescentic glomerulonephritis picture which would be typical on biopsy of the kidneys and anti-GBM disease. What we can see here in this picture is in addition to crescentic glomerulonephritis, there is also linear deposition of IgG along the glomerular capillaries. Remember that anti-GBM disease is one of those immune complex small vessel vasculitides which is distinguished from the pauci-immune ANCA-associated vasculitides by virtue of the fact that you will find deposition of these immune complexes in tissues that you biopsy. And lastly, if you perform some serum testing you could get anti-GBM antibodies which would further support this diagnosis. Alright, yet again I think we've probably got to keep this one on our list until we have some more data.