Wegener's granulomatosis is an autoimmune disease that refers to severe and rapidly progressive diseases. Systemic vasculitis and Wegener's granulomatosis are closely related pathologies, because antibodies (antineutrophil cytoplasmic) are formed in granulomatosis, which is characteristic of ANCA-associated vasculitis.
Causes of Wegener's granulomatosis
Wegener's granulomatosis refers to autoimmune, so there may be a genetic factor. In fact, granulomatosis is an inadequate immune response. So, the markers of the disease are antigens - HLA 〖B〗 _7, B_8, 〖DR〗 _2, 〖DQ〗 _w7.
The role of pathogens is also played by antineutrophil cytoplasmic antibodies reacting with proteinase-3.
Wegener's granulomatosis - symptoms
Symptoms of granulomatosis most often occur at the age of 40 years, while the gender factor does not matter.
Granulomatosis - an inflammation of the walls of small and medium vessels: venules, capillaries, arteries and arterioles. In the defeat process, the upper respiratory tract, kidneys, eyes, lungs and other organs are involved.
The symptoms are as follows:
- general - weight loss, weakness, myalgia, arthralgia, fever, in rare cases, arthritis;
- nervous system - asymmetric polyneuropathy;
- eyes - ischemia of the optic nerve, blindness, episcleritis with orbital granuloma and exophthalmos;
- skin - rashes with necrotic elements;
- kidneys - glomerulonephritis , which can take a fast course;
- lungs - lesions develop in 75% of patients in the form of infiltrates, which form cavities during decay; with this can be observed pleurisy, cough.
- respiratory tracts - are affected in 90% of patients in the form of rhinitis with purulent-haemorrhagic secretion and ulceration; perforation of the nasal septum leads to saddle deformation; Also, the lesion affects the larynx, ears, trachea, paranasal sinuses, in which a necrotic process is observed.
Wegener's granulomatosis also has two forms:
- localized - it affects mainly the upper respiratory tract;
- generalized - with her many organs are affected, and this is accompanied by fever, joint-muscle pains, coughing and heart failure.
Diagnosis of Wegener's granulomatosis
This diagnosis is made by a rheumatologist based on several data:
- blood tests - biochemical and general, which can not confirm granulomatosis, but help to rule out other diseases;
- urine analysis - it is able to confirm or exclude kidney damage (the form of the disease is determined);
- X-ray of the paranasal sinus and thorax (computed tomography gives more detailed information than the one obtained by X-ray);
- biopsy - the only way that definitively confirms or denies the presence of granulomatosis; doctors take tissue from the nose or throat, and in some cases, the kidneys and lungs.
Treatment of Wegener's granulomatosis
Treatment of the disease is carried out, mainly, with the participation of corticosteroids and cytostatics, which reduce the activity
With severe kidney damage, in some cases, the patient needs an organ transplant.
Wegener's granulomatosis - prognosis
If treatment has not been started in a timely manner, then the unfavorable prognosis will come true within 6-12 months, and the average life expectancy does not exceed 5 months.
In the case of treatment, remission lasts about 4 years, in some cases 10 years. A complete cure at the present stage of the development of medicine is impossible.