What is the name of this kidney disease?

My grandfather died of kidney disease when I was seven years old. I know it was rare, and that it was named something like wagner’s, or wegner’s, but I cannot for the life of me find it on the web. Anyone have any idea?

Could it have been Wegener’s Granulomatosis?
It is a generalized vasculitis that can cause glomerulonephritis.
The only other renal disease I could think of that starts with “W” is Wilms’ tumor, which is usually seen in children.

Wegener’s granulomatosis?

YES! Yes, that’s it, thank you! I remember the word “vasculitis” was there as well!

Vasculitis refers to inflammation of the vascular system, which includes those nice tubular thingies that take/bring blood from/to organs. Vasculitis can screw up an organ by complicating its’ blood supply. Kidneys do not, generally, respond well to problematic blood supply.

Although what you say is true in a generic sense, the kidney’s problem in most vasculitis results not from poor blood supply to it, but, rather, inflammation within the intrarenal (i.e. inside the kidney) blood vessels as well as within the glomeruli. The term glomeruli (singular = glomerulus) refers to specialized structures inside the kidney where the filtering of the blood is performed.

These glomeruli, the workunit and workhorses of the kidney, are an anatomically entwined combination of specialized blood vessels and certain kidney cells. Basically, blood flows into the kidney, then into the entrance artery of the glomeruli (the afferent arteriole)), and then out from the glomeruli (via the efferent artiole). The high pressure of blood entering the afferent arteriole squeezes the water part of the blood out of the arteriole and into a tube made of specialized kidney cells. Later on, most of the water is reabsorbed back out of the tube into the arteriole. However, the reabsorbed water, having been filtered by the miracle of the activity of the kidney’s cells, as well as the selective addition and removal of various toxins, metabolites, etc. is much “cleaner”. The 1% or so of the water that doesn’t get reabsorbed is commonly known as urine!

Factoid: Each kidney has about 1,000,000 glomeruli.

In many vasculitides (singular = vasculitis), the arterioles of the glomeruli get inflamed and that is what causes the kidney failure. This is quite different than a “problematic blood supply”. Lupus is a prototypical example of a condition causing kidney vasculitis, as is Wegener’s Granulomatosis.

Nice picture 1

Nice picture 2

Yes, by “complicating its’ blood supply” I also meant the internal blood flow.

So I imagine that vasculitis is at the heart of a number of organ problems where said organs have lots of tiny parts with microvascular structure? Not just the kidneys, but things like the pancreas? Is vasculitis a player in problems in degenerative eye conditions? Does vasculitis respond well to anti-inflammatories?

Indeed. The vasculitides can affect any organ with a circulation. Of course, this means potentially all organs are targets :slight_smile:

Clinically, the vasculitides are divided into three groups - those affecting primarily the large, medium, or small blood vessels.

Large vessel vasculitis - affects the aorta and large vessels branching off from the aorta and going to the brain from (e.g. "Takayasu’s Disease"affecting young women and causing strokes and arm problems in particular)

Medium vessel vasculitis - affects especially the vessels of the abdomen and/or brain (e.g. “periarteritis nodosa” most common in middle-aged people who have concomitant hepatitis-B infection)

Small vessel vasculitis - all the rest. A huge category really. These disorders usually involve the kidneys, nerves, lungs, and skin but essentially any organ can be affected (e.g. “Wegener’s Granulomatosis”, the vasculitis of lupus, the vasculitis associated with hepatitis-C infection …)

Interestingly, joint involvement, with arthritis, is common to most vasculitides. The mechanism for this is not simply a reflection of inflammation of the blood vessels supplying the joint. Rather, the joint fluid becomes a preferential site for the accumulation of immune complexes (i.e. antibody-antigen +/- complement complexes) that elicit a potent local inflammatory response.

This completes introductory lesson 1 to internal medicine, subsection rheumatology, subcategory vasculitides. :cool:

I forgot to answer your question about vasculitides responding to anti-inflammatories. Yes, they do, but only to the most potent anti-inflammatories (i.e. steroids and so-called immunosuppressant medications, but not to, say, drugs like aspirin or ibuprofen).