Wegener's granulomatosis - pulmonary vasculitis

Wegener's granulomatosis - pulmonary vasculitis

    * Introduction
    * Treatment
Graulomatoza Wegener is a rare type of inflammation of small arteries and veins (arterioles, Venu) (vasculitis) that classically affects the arteries that supply oxygenated blood to the lung tissue, kidney and nasal sinuses. The disease can also manifest "incomplete" in that it affects only one of these regions. If you are involved in both lungs, and kidneys, we speak of systemic Wegener's granulomatosis. If pathology is restricted to the lungs, is called limited Wegener's granulomatosis. In addition to blood vessel inflammation, Wegener's granulomatosis is characterized by producing a type of tissue inflammation called granulomas, which are formed around blood vessels and have them potentially destructive. Wegener's granulomatosis usually affects young adults of age or average. Although it is rare in children, it can actually occur at any age.
Etiology Wegener's granulomatosis cause is unknown, but it knows there is no infection, no cancer. It seems that the disease develops as a result of an initial inflammatory process that causes an abnormal immune reaction. This combination of factors leads to inflammation and constriction of blood vessels and the formation of inflammatory structures called granulomas. Researchers assume that inflammation triggers the initial should ptuea be an infection, a toxin from the environment, genetic predisposition or a combination of these factors.
Clinical Signs and symptoms of Wegener granulomaoza can occur suddenly or gradually. The first symptoms usually involve the respiratory tract, but the disease may progress rapidly, affecting blood vessels and organs they supply the oxige. Signs and symptoms most commonly encountered include: - Constant runny nose; - Epistaxis; - Sinusitis; - Ear infections; - Cough; - Hemoptysis (coughing up blood); - Shortness of breath; - Malaise; - Loss of appetite; - Swelling and joint pain; - Redness, burning sensation and eye pain; - Diplopia (double vision) or decrease in visual acuity; - Fever; - Fatigue, possibly in combination with anemia;
In some cases, the disease is not limited to the respiratory tract and affects the kidneys. Renal involvement does not cause symptoms in the initial phase of the disease, therefore it can be established only through blood and urine tests. In advanced stages of renal disease can occur and anemia. We recommend consulting your doctor in case of persistent rhinorrhoea which does not respond to anti-common cold, especially if it is accompanied by epistaxis (nasal bleeding), hemoptysis (coughing up blood) or other symptoms of Wegener's granulomatosis.
Risk Factors Granulomtoza Wegenenr can occur at any age but mainly affects people between 30 and 50 years. White people are more likely to develop disease.
Complications Besides the upper and lower airways, Wegener's granulomatosis can affect other organs such as skin, eyes, ears, kidneys, spinal cord or heart. Among the complications are: - Loss of hearing: inflammation of the middle ear granulation tissue can lead to hearing loss; - Scarring skin; - Myocardial infarction: Wegener's granulomatosis can affect the arteries, the risk of myocardial infarction or angina pectoris; - Impaired renal function: As it progresses, Wegener's granulomatosis affects the kidneys, leading to glomerulonephritis, kidney disease characterized by the inability of the body to eliminate unnecessary substances and excess fluids, leading to accumulation of urate in the bloodstream (uremia). The main cause of death in Wegener's granulomatosis an constiutuie renal failure.
Diagnosis Besides a detailed history, complete clinical examination, the physician may request the following investigations: Blood tests: for anti-cytoplasmic antibodies neutrophilia (ANCA). They appear in the blood of 80-95% of people with Wegener's granulomatosis. The blood of these antibodies may be suggestive of the diagnosis of Wegener's granulomatosis, but not enough to confirm it. Analysis can detect blood and erythrocyte sedimentation rate (ESR). Measuring the speed of red blood cells fall to the bottom of a tube in the course of an hour, it actually tests the level of inflammation in the body. Usually, erythrocyte sedimentation rate (blood red) is faster in inflammation. Additionally you can get tested for anemia, which occurs frequently in this disease. Creatinine test is performed to check if your kidneys perform properly filter in a blood byproducts of catabolism. Urine tests: renal function estimates to determine if the affected kidney disease; Chest radiography: may indicate cavities in the lung, but can not be used for differential diagnosis of Wegener's granulomatosis and other diseases. Affected tissue biopsy: A diagnosis of Wegener's granulomatosis certainty in place based on microscopic examination of tissue samples taken from the affected organ (biopsy). Tissue sample taken from the upper airways, lungs, skin or kidney and for investigating exluderea obesrvarea or vasculitis or granulomas. Outpatient biopsy may be performed under local anesthesia or, if a lung biopsy in the hospital.