Causes, manifestations, diagnosis, treatment of Wegener's granulomatosis.
What is Wegener's Granulomatosis?
Wegener's granulomatosis is a chronic autoimmune vascular disease, based on necrotic-ulcerative (granulomatous) inflammation of the walls of the blood vessels of small and medium caliber. The disease mainly affects the respiratory organs, ENT organs, eyes and kidneys. The disease develops more often in men after 40 years. Children are rarely ill.
Causes of Wegener's granulomatosis
The causes of the development of this disease are not yet fully understood.
The main risk factors are:
- Bacterial and viral infections (streptococci, cytomegalovirus, etc.),
- Immunity violation
- Propensity to allergic reactions.
Mechanism of development of Wegener's granulomatosis
It is believed that under the influence of these factors in the body begin to produce autoantibodies, gradually deposited on the walls of vessels of small and medium caliber (arteries, venules, capillaries) and provoke the development of granules (clustering of specific cells) and the appearance of ulcerative-necrotic processes in them.
Symptoms of Wegener's granulomatosis
Depending on the extent of the disease, the disease may occur in the form of a local (restricted) or generalized form and begins with general or local symptoms.
Common symptoms of Wegener's granulomatosis:
- Fever (fever)
- Loss of appetite
- Pain in the muscles and joints,
- Development of anemia.
Defeats of respiratory organs and ENT organs are manifested:
- Ulcers in the cavity of the mouth and nose with purulent discharge (non-vital) with the admixture of blood, nasal congestion, violation of the sense of smell and perforation of the nasal septum, as well as the development of sinusitis (sinusitis, frontis);
- The development of tracheitis, bronchitis, pleurisy, pneumonia with prolonged cough and haemoptysis, formation of lung cavities (caverns), voiced voids, severe chest pain, shortness of breath, etc.;
- Violation of the patency of the Eustachian tubes (channels connecting the cavity of the middle ear with the throat), the development of eustacheitis and chronic otitis media of the middle ear.
Eye damage is most often associated with inflammation of the iris and sclera leading to episcleritis, uveitis and iridocyclitis and may end with irrigation and blindness.
Defeat of the skin manifests itself in the form of rashes, nodules and ulcers.
The defeat of the kidneys is accompanied by the development of glomerulonephritis with the advent of blood and protein in the urine. Also, progressive renal insufficiency and arterial hypertension can develop.
Forecast for Wegener's granulomatosis
The forecast is tnosically favorable. With timely and adequate treatment in 2/3 of cases it is possible to achieve a stable remission (for the next 5 years, the disease subsides). Without timely initiated treatment, a high probability of a fatal outcome within 6-12 months.
Diagnosis of Wegener's granulomatosis
It usually does not cause difficulties and is based on the analysis of clinical manifestations, as well as data from clinical and immunological studies of blood.
In the event of complications, the necessary additional research is carried out:
- Endoscopic examination
- Radioisotope renography
- X-ray studies
- Puncture biopsy
Conducted also consulting specialists.
Treatment of Wegener's granulomatosis
Conducted only in the hospital and includes:
- Strict adherence to bed rest;
- Immunosuppressive therapy with subsequent selection of supportive doses of drugs for a long (for several years) application;
- Extracorporal hemocorrection of the prophylaxis of the development of complications, including the introduction of heparin, the conduct of plasmonphyrosis, etc.;
- Symptomatic therapy.