Information about Wegener's Granulomatosis - Term Paper Example

 Wegener's Granulomatosis Abstract This essay considers various aspects of the relatively rare auto immune condition, Wegener's Granulomatosis. It looks at the possible causes, the symptoms exhibited, the epidemiology of the condition, its diagnosis, possible treatment and prognosis. Introduction This uncommon systemic condition, first described by Doctor Friedrich Wegener, a pathologist from Germany, in 1936, affects only one person in between 20, 000 to 30,000 people according to most estimates, and it is possible that many health care staff will never come across a case during their whole career, especially if their patients are mostly non-Caucasian and comparatively young.. 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 probably 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 infection The condition has a number of synonymous names :- Klinger's syndrome, Klinger-Wegener syndrome, Wegener-Churg-Klinger syndromeand 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 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.6 Epidemiology This condition is considered to the result of an abnormal functioning of the immune system producing an over 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. There must presumably be some genetic component as the disease is much more prevalent among Caucasians than other racial groups. Mahr et al ( 2006) reported an increase in cases over the past 15 years, but concluded that this may well be due to better reporting. They also point to the possibility of alpha1-antitrypsin deficiency being an active factor. Environmental factors are also discussed, with exposure to crystalline silica in a work environment being considered to have a relationship with this condition. This would mean greater susceptibility for glass workers. It is systemic, meaning it affects any part of the body. The respiratory system in its entirety may be affected or only the upper (sinuses and nose), or lower (lungs) parts. According to the Patient UK report ( 2009) all patients have some respiratory conditions. In 75% the renal system, kidneys, the eyes, ears, throat and skin may be affected as may other organs. Blood vessels in affected areas become inflamed and clusters of granulomatous cells may appear. In between 25 and 50% the joints are affected. 8The condition can occur at any age, although most often middle aged people are affected. WG is an uncommon disease, which can occur at any age. It most often occurs in the 4th and 5th decade of life. As many males as females can be affected. Caruthers et al (1996) reported that it is more likely to appear in colder weather9 and also in more northerly regions. Symptoms At its onset Wegener's Granulomatosis may produce few symptoms. In other cases though there will an onset that is both severe and rapid. By far the majority of patients will have what seem to be the symptoms of a cold, a runny nose or sinusitis which fails to respond to the usual treatments. This occurs in more than 90% of cases according to Langford ( 2006) . These symptoms will persist for much longer than most infections of the upper respiratory tract. A rash may be present. The nasal membranes may become ulcerated and crusting. The nose develops a saddle-nose deformity and inflammation of the ear may be accompanied by hearing problems. The eyes become inflamed and this is accompanied by sight problems. There could be coughing, with or without blood. Pleurisy may occur and there may be a rash or skin sores. The patient may complain of fever, with its accompanying lassitude, weakness and fatigue. Appetites may be poor so weight loss occurs. Arthritic joint pain may occur as may night sweats, and the presence of blood in the urine, occult or obvious. Not all sufferers will experience all symptoms and the severity of the condition will also vary. Because of the variations in symptoms experienced and requiring treatment a number of disciplines are involved from rheumatologists, nephrologists, ophthalmologists and pathologists. This in turn means that what reports there are cover a variety of facets. Diagnosis Diagnosis can be established using procedures such as the ANCA blood test,10 other blood and urine tests such as looking fro creatinine levels, and if required also x-rays, scans and tissue biopsies. In Wegener's granulomatosis there will be usually be a positive correlation between the ANCA level and the level of disease in most cases, although Coleman ( 2006)11 reports that insome 20% of cases no such correlation is seen. Because at first there may be no obvious renal or chest symptoms, despite the presence of the disease, if the condiiton is suspected these systems should be tested, other wise underreporting will result and the patient will not receive the correct treatment. In 1990 the American College of Rheumatology issued criteria for the classification of the condition. 12These included painless purulent or bloody nasal discharge and mouth ulcers. On chest x-ray the nodules, cavities and fixed infiltrates will be seen. Computed tomography scans (CT) and magnetic resonance imaging (MRI) may also be used where these are available. Urine will have a slight sediment of red cell casts (Microhematuria i.e. where the presence of red blood cells is in such small numbers the urinary color is not noticeably affected) to be seen upon microscopic examination. This is caused by glomerulonephritis, that is a type of nephritis which is marked by inflamed glomeruli in the kidneys.On biopsy histological changes are revealed with granulomatous inflammation within the walls of arteries or in the arterioles. For classification purposes, a patient can be said to have Wegener's granulomatosis if they have 2 or more of these 4 criteria. In 2007 Peshin and O’Gradaigh wrote about Mononeuritis multiplex, a rare neuropathy which causes patchy movement and also asymmetrical abnormalities of sensory perception, as the presenting symptom in a case of Wegener's Granulomatosis.13 Treatment This will have two purposes – an initial attempt to induce remission, and secondly the maintainance of such a period of remission. Long term immuno suppression treatment is essential. Without such treatment death will ensue in only a few months from onset. Treatment will of course depend upon the symptoms experienced and which organs are known to be involved. This also means that not one , but several specialist physicians may be involved, each making their own contribution acording to the particular organs or systems involved. Those with renal involvement, and those who are experiencing more severe symptoms, are often prescribed a combination of Cytoxan (cyclophosphamide) and oral corticosteroids such as prednisone as an initial treatment. The prednisalone will in the first instance be given in high dosage and then this will gradually be tailed of. The patients must be informed of how important it is to follow dosage instructions carefully or problems may ensue and should carry a steroid card with them so that if they see another physician he will be aware of treatment prescribed. The steroids are used to treat a wide range of conditions caused by allergic reactions. Cytoxan14 use will usually be limited to a three to six month period to be replaced, according to kidney function, by methotrexate 15, another medication with possible severe side effects or azathioprine. This will then continue for as long as 2 years. Again there may be side effects such as nausea and vomiting and in some cases more severe effects such as chest pain and unusual bleeding. The risk of side effects increase because of the length of treatment required. Both of these drugs affect the immune system and may have a positive effect by reducing over reactions. Cytoxan is a drug frequently used in cases of malignancy. It is not recommended in cases of renal problems and is associated with some cases or severe side effects and even fatalities. It is an alkylating medication which prevents both cell growth and cell multiplication and , in sufficient dosage may cause cell death. Bactrim, ( sulfamethoxazole and trimethoprim) is an antibiotic often used for ear infections, 16 is said to be useful as a way of helping to prevent a relapse once the condition is under some control. Once again there are possible side effects, in this case jn the form of loss of appetite; diarrhea and nausea and vomiting. Occsionally there are much more serious side effects , including swelling of the mouth, difficulty in respiration, blood in the stools and also such things as depression and hallucionation. 17Treatment may also result in such symptoms as dizzy spells and weakness of the limbs. Hicks18 states that in some severe cases cases plasma exchange is carried out . Prognosis There is a high mortality rate of 28% over a 5 year period 19 and after 24 years the survival rate is 80%. There is also a high rate of recurrence. Relapses can either resemble or differ from those which occurred in the first instance. Any new symptoms should therefore be reported to the relevant physician so that any new treatment can begin as soon as possible. Complications Obvious complications will be those that affect the nose with perforation of the nasal septum and the formation of a characteristic saddle nose deformity because of the collapse of the bridge of the nose. 20Hearing loss may also be present as may sinusitis and renal complications. Allergic reactions may damage the conjunctivas which will become inflamed. There may be organ damage, in particular to the renal and respiratory systems. 75% of patients have some renal involvement.21 A large percentage, 66%, are also affected by joint swellings, but these do not tend to cause permanent deformities if treated. The eyes may be affected in a number of ways apart from simple conjunctivitis. Scleritis, an inflammation of the scleral layer, may present as may episcleritis, which affects only the outer surface of the scleral layer. There may also be an orbital mass lesion. The onset of double vision or a noticeable decrease in visual ability must be considered as being serious symptoms which require immediate attention. Wrong Diagnosis The list of conditions which have been suggested for patients suffering from Wegener's Granulomatosis is a long one. It includes breast and/or lung cancer , chronic fatigue syndrome, hay fever, nose bleeds, rhinitis and exopthalmos as well as rarer conditions such as :- Churg-Strauss Syndrome - a form of vasculitis in which white cells collect together and create inflammatory nodules. Goodpasture syndrome in which the body’s immune system creates antibodies that attack the respiratory and renal systems.22 Microscopic Polyangitis , a form of vasculitis which affects the smaller blood vessels. 23 Polyarteritis nodosa 24the first form of vasculitis to be described, which happened as long ago as 1866. It is a necrotising form of vasculitis which attacks medium sized blood vessels in various parts of the body. For some reason the respiratory sytem usually escapes. Conclusion Because it shares so many symptoms with other conditions it is obvious that this is a difficult condition to diagnose. Also because the factors which cause it are largely unknown it is also difficult to treat, as the drugs required carry with them the risk of very serious, even fatal results. It therefore follows that the patients require expert attention and careful and frequent interventions. It is also obvious that much still needs to be learned about this distressing disease. 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