Wegener's Granulomatosis Studies

[Barbara N]

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




Wegeners in a father and son

Journal of rheumatology, Clinical and Experimental Rheumatology, Submission on line

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

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 Are the statistics we usually hear outdated?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

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 granulomatosis

Wegener'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] Higher rates have been reported in Scandinavia, with a prevalence of 160 per million in Sweden.This would equal one in 6250
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] [4]