Wegener's Granulomatosis Studies

[pberggren1]

I have come across some interesting studies and thought I should share.




Wegeners in a father and son (http://priory.com/med/wegener.htm)

Journal of rheumatology, Clinical and Experimental Rheumatology, Submission on line (http://www.clinexprheumatol.org/pubmed/find-pii.asp?pii=23663682)

Familial granulomatosis with polyangiitis: three cases of this rare disorder in one Indoasian family carrying an identical HLA DPB1 allele -- Tanna et al. 2012 -- BMJ Case Reports (http://casereports.bmj.com/content/2012/bcr.01.2012.5502.abstract)

I have some others as well but can't seem to find them right now.

[Alysia]

Hi Phil,
thanks for sharing :thumbup:
poor families :sad:
it is scary to think that our children are in risk of developing WG :w00t:
I think that in my case there can be some genetic basis because my parents are relatives....

[pberggren1]

Alysia, your parents are relatives? In what way?

[Alysia]

Alysia, your parents are relatives? In what way?

they have same grandfather 5 generations back.

[pberggren1]

That makes them 4th cousins. That is fine.

[Alysia]

my syster has Icthyosis which is genetic skin disorder.

[pberggren1]

Wow, that must suck.

[annekat]

Interesting stuff, Phil, thanks for sharing!

[marylz]

Thanks Phil.

[rebekah]

Thank for sharing, Phil. They are interesting articles... I can't imagine 3 people in one family having weg's.

[Barbara N]

I have come across some interesting studies and thought I should share.




Wegeners in a father and son (http://priory.com/med/wegener.htm)

Journal of rheumatology, Clinical and Experimental Rheumatology, Submission on line (http://www.clinexprheumatol.org/pubmed/find-pii.asp?pii=23663682)

Familial granulomatosis with polyangiitis: three cases of this rare disorder in one Indoasian family carrying an identical HLA DPB1 allele -- Tanna et al. 2012 -- BMJ Case Reports (http://casereports.bmj.com/content/2012/bcr.01.2012.5502.abstract)

I have some others as well but can't seem to find them right now.

Good researching Phil, keep it up and we my resolve the mystery to WG

[Linda22]

Is Wegner's as rare as what the medical profession and the researchers say it is or is it becoming a little too common? The specialist that treated my son also told me that more than likely it was genetic but could not tell me exactely

[Dirty Don]

WG is rare in comparison to cancers, etc. I've heard all kinds of stats, but most seem to be around 1 in 30,000. Unless, of course, you live near Phil...he attracts Weggies! The amount of 'newcomers' on here indicates a rise in awareness for sure, maybe in actual illness too. But, awareness, due to some on here and some national exposure, of AIs in general and WG specifically, has gone up, but it's nowhere near the heights of financial and research support of many other more common diseases. Genetics are brandished around cuz no one really has an exact idea of where WG comes from...so blame it on the family!!! LOL!

[Barry Forshaw]

According to the National Health Service website around 500 people each year are diagnosed with Wegener’s in the UK. It is estimated to affect 3 out of every 100,000 people globally. According to my rusty maths that means over 200,000 people are suffering with Wegener’s right now.

[pberggren1]

Actually, Wegs is more like 1 in 5,000 globally.

[annekat]

Actually, Wegs is more like 1 in 5,000 globally. Phil, you are the one that keeps coming up with that figure, where other people typically come up with one in 30,000. I don't discount your information, since I don't know, but am wondering why there is that discrepancy. Are people using different means of measuring this? Or are different criteria being applied somehow? Are the statistics we usually hear outdated? I would totally believe that there is one Weggie in 5000 people but would like to know where you got that info, what your source is, so I could better understand.

[drz]

From what I recall GPA is lot of other diseases in that it is not spread equally around the world or even in countries. Some other auto immune disorders like MS seem more prevalent in certain areas and less common in others. Some of difference in studies of frequency are of course greatly skewed by the lack of a good medical system to accurately diagnose the illness so it will show up as very rare in those locales.

Even in countries and and areas with good health care systems we all know quite well the difficulty in getting our GPA correctly diagnosed in timely manner. In my locale I know of four diagnosed cases in a community of 13,000 people so one in 30,000 wouldn't seem to fit here. But it is unclear whether we really have a higher incidence in our area or just had better luck finding our GPA cases.

Once I read info on a Canadian study that listed diagnosed cases in Canada and I believe the ratio of diagnosed cases to the population of the country indicated a much higher ratio than 1 in 30, 000. Maybe we can all search for such numbers and ask the experts we see what they think is the incidence of GPA. They must discuss this issue at their conferences.

[pberggren1]

I have talked with the top Wegs docs in the US and Canada and UK and they all say it is more like 1 in 5,000. In Saskatchewan there are about 200 Weggies and in Alberta there are about 700.

[drz]

Here is a link on frequency too:

Epidemiology of Granulomatosis with Polyangiitis and Microscopic Polyangiitis (http://www.gpa-mpaclinical.com/overview/epidemiology)

It says there are an estimate 30,000 cases in USA which has an estimated population of just over 313 million. I compute that to close to 1 in 10,000 people.

Another study says new cases are 10 in a million or one in 100, 000 since 90% of us survive initial treatment that should equal a lot more than 1 in 30, 000. It also suports the idea that this is not equally distributed around the world.

Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosisWegener's granulomatosis, or Granulomatosis with polyangiitis (GPA), is an incurable form of vasculitis that affects the nose, lungs, kidneys and other organs.
The incidence of GPA is 10 cases per million per year where 90% of the patients are white. Due to its end-organ damage, it is life-threatening and requires long-term immunosuppression. Although GPA affects small and medium-sized vessels, it is formally classified as one of the small vessel vasculitides in the Chapel Hill system. Initial signs are extremely variable, and diagnosis can be severely delayed due to the nonspecific nature of the symptoms. Often the kidneys and lungs are affected and the renal and lung functions are impaired. A serious complication of GPA is rapidly progressive glomerulonephritis (RPGN). This is a syndrome of the kidney that is characterized by a rapid loss of renal function; usually a 50% decline in the glomerular filtration rate (GFR) within 3 months.

[drz]

Epidemiology

The incidence and prevalence of GPA varies considerably between countries. In the US, a prevalence of 3 per 100,000 individuals has been reported, although this figure is likely to be an underestimate as the study was based on hospital discharges. [1] (http://bestpractice.bmj.com/best-practice/monograph/327/resources/references.html#ref-1)Higher rates have been reported in Scandinavia, with a prevalence of 160 per million in Sweden. (http://bestpractice.bmj.com/best-practice/monograph/327/resources/references.html#ref-2)This would equal one in 6250 (http://bestpractice.bmj.com/best-practice/monograph/327/resources/references.html#ref-2) (http://bestpractice.bmj.com/best-practice/monograph/327/resources/references.html#ref-2)
GPA can occur at any age. The mean age of onset in most series is between 40 and 60 years of age, with an approximately equal gender distribution. It is most commonly seen in white people, but can also occur in other racial and ethnic groups. Scandinavian studies have suggested a rising incidence, although this is likely due, at least in part, to improvement in diagnosis. Incidence appears to increase at northerly and southerly latitudes. [3] (http://bestpractice.bmj.com/best-practice/monograph/327/resources/references.html#ref-3)[4] (http://bestpractice.bmj.com/best-practice/monograph/327/resources/references.html#ref-4)

[annekat]

I would tend to trust the top experts you talked with, Phil, but still don't know how they arrived at that number. I do understand that it is more common in certain geographical areas. My unscientific conclusion, which I'm sure many of us have reached, is that there are a lot more Weggies than can be scientifically documented, due to the new cases every day that haven't yet been counted, and even more due to what I assume to be hundreds of thousands, and perhaps more, undiagnosed cases.

[pberggren1]

I discussed the numbers with my new doc and she agrees that it is more like 1 in 5,000, at least in North America and Europe. She has 20 cases of Weggies herself.

[Linda22]

In my opinion I think that WG is not as rare as what the statistics tell us. Yes we have to go by it because that is supposedly the ratio that has been treated, But in my son's case, where the disease moved so quickly and he died within two months of showing symptoms, how many other people have also died without been diagnosed properly and a lot of people are not as persistant as I was to know what was wrong with Brandon. Yes these AI conditions are not as common as Cancer and so on, but in its own right I think it is becoming more common than what we think. Not to mention some of the countries in Africa, that don't have modern medicines and technology to diagnose properly. I also find it so hard to believe that with all the medical technology today and no one has found the exact cause of WG. We are living in the 21st Century not the dark ages!!!!!!!!

[mishb]

I have talked with the top Wegs docs in the US and Canada and UK and they all say it is more like 1 in 5,000. In Saskatchewan there are about 200 Weggies and in Alberta there are about 700.


That does it - I'm never coming to visit Canada :thumbdn:

Oh wait ........it's too late for me, I should just fit in perfectly :razz:

[Dirty Don]

I told ya...Phil attracts Weggies!

[DJS]

Actually, this thread started out saying that some studies are showing that the banner line of the Vasculitis Foundation information on GPA is incorrect. That is that there is no evidence that it is hereditary. My three sons are more comfortable believing that their mothers condition is hers alone. I wish there was more work on finding a cause, such as it is more common in nurses who have sand blasted spoke wheels on a Model A Ford or sum such.

[pberggren1]

It's a Model T.....get it right DJS.....lol.

[Alysia]

I think the numbers are different in different countries.
when I was dx I was told the statistics is 1 to 100,000.
In my area, which includes cluster of small cities and bigger city, about 300,000 citizens together, most of the docs didnt see any weggie but me. I never met one in Israel. (Im meeting you) In searching forums of AI in israel I found about 3 weggies writing in the past 10 years.
lately I finally found ENT doc in the biggest city in the country that prob is the most experienced about WG in Israel.he is working in an area of 3,000,000 and he was proud to tell me that he met 10 weggies and some others with unclear diagnosis (I'm going to meet him next week).

[Barry Forshaw]

Great discussion! I find this fascinating. There's still so little known about it, no-one has the full picture yet.

From what I can gather, Wegener's Disease is less common in non-caucasians, but I do wonder how many cases go unreported in less developed areas of the world. It's frightening to think that people might be dying from this disease without having it diagnosed, never mind receiving any treatment. The 500 figure I quoted is the number of people diagnosed in the UK per year. I suppose it's possible that there will even be people in the UK who aren't diagnosed. The population of the UK is just over 63 million so 500 per year works out at about 1 in 125,000. If we extrapolate that out to the world population of 7 billion that would give us around 56,000 sufferers diagnosed per year.

If we used the figures you have Phil, there would be almost 1.5 million people with Wegener's worldwide. It doesn't sound like an obscure, rare disease when you think about it like that!

[pberggren1]

That's true Barry but let's look at something else. How many of us know a person with cochlear implants? That is about 1 in 5,000.

[Dirty Don]

Hmmm, 1.5 million vs 8 billion...it's rare enough!

[pberggren1]

Here is another.


Increasing incidence of Wegener's Granulomatosis | Rheumatology Update (http://www.rheumatologyupdate.com.au/news/increasing-incidence-of-wegener-s-granulomatosis)

[pberggren1]

And another..........


American Journal of Nephrology 2013, Vol. 38, No. 2 - Antineutrophil Cytoplasmic Antibody Vasculitis Associated with Influenza Vaccination - FullText - Karger Publishers (http://www.karger.com/Article/FullText/354084)

[Alysia]

And another..........


American Journal of Nephrology 2013, Vol. 38, No. 2 - Antineutrophil Cytoplasmic Antibody Vasculitis Associated with Influenza Vaccination - FullText - Karger Publishers (http://www.karger.com/Article/FullText/354084)


Hi Phil,
thanks for the references :thumbup:
It turns out that influenza vaccine can cause/ triger WG !
all those vaccines can be dangerous :scared:
I was working with a girl who got auto-immune encephalitis from unknown reasons, one of the hypothsesis was taking some vaccines for a trip abroad. she recovered after half year.
I wonder if there is anyone in the forum who "got" his wg after taking some vaccine...??

[annekat]

Interesting links, Phil, thanks for sharing. I have always managed to avoid getting flu shots even though my docs have always recommended them. I'm just not comfortable with the idea, which may be irrational, hard to say. As for the rising incidence of WG, it is pretty much what we have been suspecting, I guess.

[Linda22]

Thanks for those interesting Links Phil, it is what we have been suspecting that it is not as rare as what is thought. As for the flu vaccine I have never taken my children to get it because I never ever trusted them

[drz]

And another..........


American Journal of Nephrology 2013, Vol. 38, No. 2 - Antineutrophil Cytoplasmic Antibody Vasculitis Associated with Influenza Vaccination - FullText - Karger Publishers (http://www.karger.com/Article/FullText/354084)

Nice article to discuss the issues. I think this part sums it up well for us:

"Furthermore, a prospective study of influenza vaccination in patients with GPA with quiescent disease showed an adequate antibody response to the vaccine and no rise in disease activity following influenza immunization [13]. Therefore, the routine use of influenza vaccination in patients with autoimmune disease should be performed after careful assessment of the benefit-to-risk ratio."

It seems it might or could trigger, not cause, some vasculitis or other auto immune diseases in some people although this pretty rare event. But once we have it, it does not seem to affect relapse rate. Having the flu seemed to cause a higher relapse rate for MS than the vaccine. We think GPA is rare but how about AAV which they said is one in twenty million.

[Barbara N]

I don't take the flu vaccine and I seem to make it through the winters better than most. The last vaccine I got , I got pregnant and I don't want that to happen LOL again so I don't take them.