Wicks reports that 90% or more of those affected are Caucasian. 1 The comparative rarity also makes research difficult, especially when it will benefit only small numbers, which may mean funding for possible research projects is limited. Mahr et al 2(2006) give figures of between 24 and 157 cases per million people and yearly occurrence rates of 3 to 14 cases per million. Coleman ( 2006)3 suggests a figure of 30 per million. As with many other diseases and abnormalities of the immune system, it seems probable that Wegener’s Granulomatosis develops when someone who is already genetically predisposed to the condition is exposed to a particular trigger mechanism, perhaps a bacterial or viral infectionThe condition has a number of synonymous names:- Klinger’s syndrome, Klinger-Wegener syndrome, Wegener-Churg-Klinger syndrome, and Wegener-Klinger syndrome. Also because Wegener is said to have had Nazi connections there has been some inclination to re-name the condition as ANCA associated granulomatous vasculitis. Symptoms result from extreme inflammation which that can affect many different types of body tissues. These include the blood vessels when it is described as vasculitis when the immune system mistakenly attacks healthy blood vessels. The inflammation results in a reduction in blood oxygen levels accompanied by a restriction of blood flow to affected organs, which results in a destruction of normal tissue.4 ‘Incomplete’ forms exist that only attack one part of the body.5The condition can result in failure of the kidneys or lungs.6This condition is considered to the result of an abnormal functioning of the immune system producing an over a response to stimuli. , although there is at present no known cause. 7, but this is not a contagious disease, nor is there any compelling evidence of it being hereditary.
Information about Wegener’s Granulomatosis