Churg-Strauss Syndrome lies within the spectrum of Wegener’s Granulomatosis, microscopic polyangiitis, and ANCA-associated vasculitis: all diseases which result from inflammation of small-to-medium-sized vessels. The “classic triad” of Churg-Strauss syndrome is asthma, eosinophilia, and vasculitis, and although “renal involvement”…
A subset of patients with ANCA-associated vasculitis have drug-induced ANCA disease. There are a few features of drug-induced ANCA disease which set it apart from “idiopathic” ANCA disease, but overall the clinical symptoms are similar. Drug-induced ANCA almost…
We all know about the p-ANCA and the c-ANCA, which are antibodies against either myeloperoxidase (MPO) or proteinase-3 (PR3), respectively. Now get ready for a 3rd ANCA subtype, an antibody against LAMP-2! In a recent Nature Medicine article…
Polyarteritis nodosa (PAN) and Microscopic polyangiitis (MPA) are two vasculitides which were previously grouped together. However, they are currently thought of as two separate entities. PAN is a vasculitis of the medium-sized arteries, sparing the smaller vessels and…
The incidence of ANCA-positive disease is in the ballpark of 1:10,000. The incidence of anti-GBM disease is even more rare, at about 1:1,000,000. One would expect if these 2 disease-causing antibodies were entirely independent of one another, the…
Pauci-immune disease–a common cause of glomerulonephritis–refers to the histologic finding that there is an absence of glomerular immune staining (e.g., no immune complex or anti-GBM staining). Usually this is accompanied by positive ANCA titers, but not necessarily. When…
There is a patient now on the renal consult service with Churg-Strauss Syndrome. This disease falls under the category of “small vessel vasculitis” and most commonly is thought of as a pulmonary disease, as the characteristic triad consists…
Rituxan is seeing a lot of use these days. Although technically only having formal indications for rheumatoid arthritis and non-Hodgkin’s lymphoma, it is being used for a number of off-label indications, and in general the results seem to…
ANCA-positive vasculitides are a not uncommon cause of rapidly progressive glomerulonephritis. When we send off an ANCA test, what are we actually measuring? ANCAs (anti-neutrophil cytoplasmic antibodies) are a type of IgG autoantibodies that are directed against antigens…
When you are called at 5pm to start pheresis on a patient with Wegener’s granulomatosis who is already on dialysis…there is a certain part of the renal fellow which wants to say, “Come on–the dude is already on…
