Alright, what I have is a mouthful to say and is currently undergoing a name change as are most vasculitis types. This is because the names don't really say anything about the disease. This can be dangerous because not many doctors know anything about these diseases/syndromes. I have actually had to explain what my disease is to medical staff before and ended up getting an antibiotic prescribed to me that would have seriously interacted with my most important drug in the cocktail I take. It was another lucky break that worked in my favor here, my mom thought I was allergic to the drug so she called my rheumy (rheumatologist) to see if there was anything else I can take.
Back to the point, though, Wegner's Granulomatosis (VEG-uh-nurz gran-u-loe-muh-TOE-sis: Wegner's refers to the discovering doctor and Granulomatosis refers to the type of tumor like growths and damage that can occur to the organs) is a type of vasculitis that effects the small and medium sized arteries providing blood to the upper airways (sinuses, nose, trachea), the lower airways (lungs) and the kidneys (if you don't know what those are...) (Mayoclinic) but it is not uncommon to see it go for the joints (causing a arthritis) and the skin (it can cause lesions and a rash called purpuria (sp?). It can also attack anywhere in the body, there's been cases where people have had it go for their brains I know of one patient where it went for his eyes and ears, it's just not as common.
I had involvement in all the common areas (I am happy to report that my kidney function has improved to around 60% over these past 6 months) and a little bit in my ears (I was near deaf in one ear for 6 weeks). Almost a year ago my sinuses scarred over completely for no apparent reason and I needed surgery to fix them (unfortunately, or maybe fortunately depending on how stinky an area is, I sometimes have no sense of smell and when I do it is not very strong), this is what started my journey down the vasculitis road.
Wegner's cannot be cured, but it can be treated. For a patient that is in an active flair up and not recieving treatment life expectancy on average is only 5 months. This is because the disease causes inflammation in the blood vessels giving blood to these organs. The granulomas themselves are a type of tissue that produces inflammation and they are normally found around the points where the blood vessels link up with the organ in question (Mayoclinic). This is why Wegner's is deadly if not treated, it can restrict blood flow to these organs to the point where the tissue dies. Most Wenger's patients that succumb to the disease do so because of kidney or lung failure.
The good news is, this disease is treatable. There is no known cure and once you have it, you have it for life, but when you are having a flair-up early treatment gives you a high probability of not only survival but also, no lasting damage. Common treatments involve a corticosteroid (such as my enemy that I literally can't live without Prednisone) and other immune suppressing drugs (most of which were hijacked from cancer treatments) I personally take methotrexate (aka Rheumatrix) but there are also cyclophosphamide (Cytoxan), azathioprine (Imuran)and more recently rituximab (Rituxan) which was finally approved just last month for use with vasculitis. There are also many other supplements that you're doctor may prescribe (I take a folic acid supplement because methotrexate prevents the body from metabolizing this nutrient).
There are many different types of vasculitis, but the one that seems most closely related to Wegner's is Microscopic Polyangitis (or MPA for short, because we all know that short is better. And I can actually pronounce the shorter version of these names!). It causes the same sort of damage, but is associated with a different ANCA (anti-neutrophil cytoplasmic autoantibodies, try saying that three times fast!) which is a protein that your immune system begins producing just for the purpose of killing you (that's a little extreme on the self-hate scale, self!). MPA is sometimes seen as the less severe of the two because it normally only goes for the upper and lower airways. My rheumy told me that they were actually thinking about combining the two disease into one group (ANCA associated vasculitis. Hey, I can remember that AND it would go a long way towards telling people what exactly this disease is attempting to do). He told me that they were thinking of doing this because they were seeing more cases like mine; my first test showed the ANCA associated with MPA, the second showed the ANCA associated with Wegner's so even the disease can't make up its mind about what it wants to be on any given day.
So, that's Wegner's in a nutshell. Or in my shell. Cause I don't think nuts can get vasculitis as they're missing an important ingredient, but they do make good butters....