Weggies Statistics

[Minneapolismark]

As a former biomedical engineer I have tried to put together a few Wegener's facts and stats. Of course we all know that Wegener's facts are scarcer than hen's teeth, but still it was a way to kill a couple of evenings. The first thing that I wanted to know after being diagnosed was "what are my odds." It turns out that even morbidity statistics are bizarre, poorly documented, inconsistent and contradictory. Here, to the best of my ability, are the stats as far as I can find them. I haven't been diligent enough to document my sources but may go back and do so if I feel like it, which I may not. So caveat emptor, let the reader beware!

There are approximately 2,500 Wegener's sufferers in the U.S. at any one time. This equates to one in 100,000.

If untreated or improperly treated, mortality is 95% at five months after the start of a flare-up.

Under treatment the mortality rates are 25% at five years and 75% at ten years.

About 75% of Weggies achieve some level of remission. I say "some level" because there is no consistency as to what remission means. Does it mean SED and ANCA at normal levels? Does it mean no symptoms? Does it mean no symptoms without medication?

About 80% to 90% of those in remission will again experience a flare-up.

Under treatment the most popular modes of death seem to be pneumonia, kidney failure and medication toxicity.

Please feel free to correct or add to the list. I think it would be great if we began gathering our facts since we probably represent one of the largest and most active groups of weggies around.

[Minneapolismark]

Oh, there are a number of other interesting stats. I was reading in the Journal of Rheumatology that those with Limited Wegeners (no kidney involvement) seem to have a reduction in quality of life equal to or greater than those with "full" Wegener's. This is counter-intuitive and no one has the slightest idea why. So, even though my kidneys are not involved I seem to have a very broad and severe range of symptoms, leading to reduced capacity and quality of life.

[Sangye]

Mark, I think you should run those stats by a Wegs specialist-- like when you go to Mayo for treatment (hint, hint). Many of the stats I've found in otherwise good sources (eg Merck Manual) are inaccurate or outdated and Dr Seo (JHU) sets me straight. I particularly question the "75% mortality rate at 10 yrs" stat. That's definitely not true.

[andrew]

Hmmmm....2500 WG sufferers in the US? Is that really correct? It just seems staggeringly small given a total population of approx 310 million :) Using the same calculation then that would mean there are approximately 161 people with WG in Australia. I just can't see that figure being correct. I have absolutely no fact to back that up though :tongue1:

[Minneapolismark]

According to the Journal of Rheumatology https://www.jrheum.com/subscribers/06/10/1923.html (https://www.jrheum.com/subscribers/06/10/1923.html)the incidence rates are slightly increasing and currently as follows:

Norway of 12.0 per million
U.K. 10.3 per million
Sweden 11.9 per million

[andrew]

Pffffttt...what do they know? :biggrin1: I had no idea the numbers were that low. I feel kinda special now! Thanks for the stats, keep up the good work.

[Minneapolismark]

Here is a 2006 table of survival statistics by therapeutic modality. It is a little out of date but noteworthy. It is from the Journal of Rheumatology.

http://www.jrheum.com/subscribers/06/07/images/2006-397.table.1.gif

Here is a 2006 table of relapse statistics by level of Wegener's involvement. It is also from the Journal of Rheumatology.

http://www.jrheum.com/subscribers/06/07/images/2006-397.table.2.gif

Now don't get all freaked out, OK. These are just numbers and it is four years after the journal article. And some of the contributing studies are nearly ten years old.

[Minneapolismark]

The interesting thing is that the incidence of Wegener's is increasingly fairly rapidly. Even factoring out improved diagnostic criteria and tests, the rate of incidence is on a significant upswing.

What is REALLY interesting is that the increase is most notable in "clean" countries like Sweden and Norway, where they have stringent controls on Radio Frequency hazards, environmental hazards, socialized medicine and so on. So it isn't easy to pin this on things that seem like natural causes like cell phones, food additives and pesticides.

[Sangye]

Dr Seo once told me that with proper treatment, Weggies are much more likely to die from infection than from Wegs.

[Sangye]

What is REALLY interesting is that the increase is most notable in "clean" countries like Sweden and Norway, where they have stringent controls on Radio Frequency hazards, environmental hazards, socialized medicine and so on. So it isn't easy to pin this on things that seem like natural causes like cell phones, food additives and pesticides.
That's very interesting. Someone told me recently that Norway's fishing waters are heavily polluted--among the worst in the world. I didn't check it out, though, so I have no idea if it's actually true.

[Minneapolismark]

I would think that Wegener's would be worth a great deal of study because it seems to be a "snowflake disease" (my term for a disease that manifests in each person very differently).

-- Since there is a relatively small population involved, it would be possible to study the ENTIRE POPULATION and not just a representative sample.

-- By studying common symptoms and modes, it would be possible to group and compare people within the small study population.

-- This could provide great insight in to how the immune system works generally, and may provide assistance in understanding more prevalent diseases.

-- At the very least they should be saving our genetic material for future research based on which ones of us survive and which ones do not.

-- That this affects Caucasians much more frequently than non-Caucasians probably indicates a significant genetic factor, no?

[Psyborg]

I would think that Wegener's would be worth a great deal of study because it seems to be a "snowflake disease" (my term for a disease that manifests in each person very differently).

-- Since there is a relatively small population involved, it would be possible to study the ENTIRE POPULATION and not just a representative sample.

-- By studying common symptoms and modes, it would be possible to group and compare people within the small study population.

-- This could provide great insight in to how the immune system works generally, and may provide assistance in understanding more prevalent diseases.

-- At the very least they should be saving our genetic material for future research based on which ones of us survive and which ones do not.

-- That this affects Caucasians much more frequently than non-Caucasians probably indicates a significant genetic factor, no?

I would think that figuring out the genetic link would be an advantage to possibly helping people at risk avoid potential triggers. So if toxins or certain vaccines are a trigger you might advise a person with genetic risk to avoid them.

Also I believe they have determined that the disease is not actually more common in Caucasians, but is more likely caught more frequently due to socio-economic factors. I could be wrong on that, but I believe I had heard that somewhere.

[Thakator]

Thanks, Mark, for taking the time and making the effort to share your research findings with us. It's not just diagnosing, treatment and drugs that we need - - ours is a marathon and to run it successfully we need to be informed as well. So thank you for your part. Ron

[Sangye]

Mark, those are interesting ideas. The one about us being easier to study because we're a small population only works if everyone is being treated at a small number of centers. Weggies are spread out all over the world, being treated by individual docs of varying specialties-- no way to organize it.

It's incredibly difficult to study larger groups of Weggies. They have highly variable disease presentation and histories of treatment, and they tend to have many complications which might exclude them from a study. It's very different than studying a population of people with a rare type of cancer, for example. The RAVE study was considered a huge study and had only 200 patients. Many studies actually combine patients with various vasculitides to get enough data.

Regarding genetic studies....
This one is recruiting participants. When you get to Mayo, you'll be able to participate (hint, hint)!
One-Time DNA Study for Vasculitis - Full Text View - ClinicalTrials.gov (http://www.clinicaltrials.gov/ct2/show/NCT01241305?term=wegener%27s+granulomatosis&rank=31)

This one is ongoing and anyone can participate (ie, you don't have to be treated a vasculitis center) Contact info is in the link.
Identification of Novel Wegener?s Granulomatosis Susceptibility Genes | Vasculitis Foundation (http://www.vasculitisfoundation.org/wegenersgranulomatosissusceptibilitygenes)

[Minneapolismark]

I would think that figuring out the genetic link would be an advantage to possibly helping people at risk avoid potential triggers. So if toxins or certain vaccines are a trigger you might advise a person with genetic risk to avoid them.

Also I believe they have determined that the disease is not actually more common in Caucasians, but is more likely caught more frequently due to socio-economic factors. I could be wrong on that, but I believe I had heard that somewhere.

Your observation about genetic risk factors is good too know. It will give me something to look up on one of my good days! :thumbsup:

The biggest problem I think we face is that there are so very few Weggies in the world. If someone has a research dollar they will probably spend it on a disease that impacts more people. I imagine that we only get the crumbs from the table. "Hmmm. This works for RA, I wonder if it would also work for... hmmm... what's that other disease with the weird name?" So in order to drive more research it seems we would have to show that we are a good test case for understanding diseases with much larger effected populations.

So our argument would do well to include using us as a reference or experimental comparison for Auto-Immune diseases. Since a good strategy turns weakness into strength, our greatest experimental strength may be that we are so very rare. They can study the whole population, we appear to be equally distributed across geographic, socio-economic and racial strata. (I just picked that out of the air. Those aren't researched facts, just my own observations thus far).

So perhaps helping us is ultimately advancing the greater good. Perhaps we are the key and not simply the poor second cousins.

[Minneapolismark]

Mark, those are interesting ideas. The one about us being easier to study because we're a small population only works if everyone is being treated at a small number of centers. Weggies are spread out all over the world, being treated by individual docs of varying specialties-- no way to organize it.

It's incredibly difficult to study larger groups of Weggies. They have highly variable disease presentation and histories of treatment, and they tend to have many complications which might exclude them from a study. It's very different than studying a population of people with a rare type of cancer, for example. The RAVE study was considered a huge study and had only 200 patients. Many studies actually combine patients with various vasculitides to get enough data.

Regarding genetic studies....
This one is recruiting participants. When you get to Mayo, you'll be able to participate (hint, hint)!
One-Time DNA Study for Vasculitis - Full Text View - ClinicalTrials.gov (http://www.clinicaltrials.gov/ct2/show/NCT01241305?term=wegener%27s+granulomatosis&rank=31)

This one is ongoing and anyone can participate (ie, you don't have to be treated a vasculitis center) Contact info is in the link.
Identification of Novel Wegener?s Granulomatosis Susceptibility Genes | Vasculitis Foundation (http://www.vasculitisfoundation.org/wegenersgranulomatosissusceptibilitygenes)

Good points of course. On the other hand, the best way to defeat an obstacle is to turn it into an asset. The advantage of having us so evenly distributed is that we are a relatively unbiased statistical sample. Perhaps a study would encourage more consistency and rigor in our treatment, even though it is conducted by a wide variety of providers?

[Sangye]

The problem is that you can't glean much useful info from studying a few people--which is basically what Weggies are. This is why all of our drugs come from treatment protocols for other AI diseases that are more prevalent (eg RA, lupus). Yes, it's a "crumbs from the table" approach, but it's also a fast and efficient way of making more drugs available to us. And, by the time a drug gets to us they know a lot about its short- and long-term side effects. I went on rtx last year with minimal fear. If I'd been one of the first people to use rtx, I would have been terrified.

The autoimmune process is basically the same regardless of which disease is studied. It makes more sense to study huge populations (eg RA) and extrapolate the results to small populations (eg Wegs), than the opposite. My guess is that the triggers are likely the same for all or most AI diseases since the number of people dx'ed with them is rapidly increasing.

[Sangye]

The advantage of having us so evenly distributed is that we are a relatively unbiased statistical sample.
Can you explain what you mean?

[Minneapolismark]

Can you explain what you mean?

Wegener's is surprisingly uniformly distributed by population density, without regard to geography, socio-economic factors or (apparently) race. It runs about 10 people per million regardless of other factors.

We are a small population.

If you compared us you would find that we are short and tall, fat and thin, healthy and not-so-healthy otherwise. A genetic study might reveal the one thing we have in common - the markers that serve as a vulnerability to Wegener's. Isolate those markers and then compare them to the larger AI population, and you might have a key to understanding the genetics of AI disorders as a whole.

We are valuable because we are a small population that has only one apparent thing in common -- our disease.

[drz]

When I was diagnosed I was told it is uncommon, about one case per 20-30,000 people. I questioned even this number since i know of three cases in our small town. I found these stats on emedicine:

Frequency

United States

The prevalence of WG in the United States is estimated at 3 cases per 100,000 population.
International

The prevalence of WG in Europe is estimated at 5 cases per 100,000 population. Higher incidences have been reported in northern compared to southern Europe.
Race

While all racial groups are affected, WG is a disease that predominantly affects whites. African Americans account for only 2-3% of patients with WG.3
Sex

Both sexes are affected equally.
Age

The mean age at diagnosis is 40 years, although patients may present at nearly any age. The age range is 8-99 years.1