Wegener's granulomatosis - Causes, Symptoms and Treatment .MF .

August 12, 2017 17:52 | Musculoskeletal System.

Wegener's granulomatosis - a disease characterized by inflammatory changes in the blood vessels of small and medium caliber with a primary lesion of the respiratory system and kidneys.

Wegener's granulomatosis are sick with the same frequency, both men and women.The disease is described in different ages, but is most common between the ages of 55-60 years.Currently the disease incidence of 3 per 100,000 population.

reasons for Wegener's granulomatosis

The exact cause of the disease has not been established.But given the frequent occurrence of the disease after undergoing respiratory infection, many researchers do not exclude the connection of Wegener's granulomatosis with pathogens.

disease is based on the formation of specific proteins, antibodies to the cells of blood neutrophils.Interacting with neutrophils, they promote adhesion of the latter to the wall of the vessel and the release of these reactive substances.These substances cause damage to the vessel wall with the formation of local inflam

mation.During inflammation specific form dense bundles - granuloma.The granulomas can reach large sizes and are characterized by a tendency to destroy the walls of blood vessels and the development of bleeding.As a rule, initially granulomas appear in the upper respiratory tract (nose, pharynx, larynx), then the lower (lungs).In the third stage kidney damage.

symptoms of Wegener's granulomatosis

disease begins with influenza-like symptoms.At this time, the external signs are nonspecific.It appears fever, weakness, thirst, loss of appetite, can be a pain in the joints, weight loss.Many people point to an infection of the respiratory tract transferred earlier and therefore do not seek treatment because they perceive these symptoms as the effects of early disease.This period lasts about 3 weeks.

Because in Wegener's granulomatosis affects the blood vessels, during the development of symptoms of the disease can be very diverse.

• A characteristic feature of the disease is a primary lesion of the upper respiratory tract.Typically, the disease starts with rhinitis (inflammation of the nasal mucosa).The nasal ulcers are formed, joined by purulent infection.Characteristically purulent or bloody discharge from the nose.Over time, the propagation process on the underlying layers, it is possible degradation of the cartilage from the nose deformation or perforation of the nasal septum.

nose deformation in Wegener's granulomatosis.

addition rhinitis can develop sinusitis (inflammation of the sinuses), or tracheitis (inflammation of the mucous membrane of the trachea).For tracheitis characterized by hoarseness, difficulty in breathing.In rare cases, serious complication develops such as stenosis of the larynx (total or partial closure of the lumen of the larynx, leading to respiratory failure).

• Another specific feature of the disease is a lung infection.At the beginning of the disease in the lungs appear foci of inflammation, which is usually detected only radiographically.Further in these areas are formed granulomas, and are formed at their destruction cavity.The cavities accession infection can accumulate pus.Formed abscesses (closed cavity with pus) of the lung.Patients complain of cough, in some cases, mixed with blood in the sputum, shortness of breath on slight exertion and at rest, pain in the chest.In some cases symptoms may be absent, the disease diagnosed by X-ray of the chest.

• The third characteristic manifestation of the disease is kidney disease.With a high degree of activity Wegener's granulomatosis has been a rapid progression of renal dysfunction.In the early stages of the disease kidney disease is diagnosed when a protein in the blood and urine.It is also characterized by the development of renal (caused by kidney disease) hypertension.With the progression of the disease is increasing renal failure symptoms - nausea, vomiting, thirst, dry mouth, loss of appetite, weight loss, decreased amount of urine and symptoms of fluid retention in the body (edema, fluid accumulation in the cavities of the body, shortness of breath).Approximately 20% of cases of Wegener's granulomatosis in patients diagnosed for the first time at the stage of terminal renal failure.

• Eye involvement is characterized by a decrease in vision.In the formation of granulomas formed behind the eye proptosis (forward displacement of the eyeball), which in most cases leads to blindness.

• When inflammation of the vessels in the skin formed purpura.Purpura is a dense precipitation of dark purple disposed over the affected vessels, usually in the lower extremities.

Purpura in Wegener's granulomatosis.

Over time, the formation of ulcers.

• The defeat of the gastrointestinal tract characterized by the violation of a chair on the type of diarrhea, pain in the umbilical region.In the presence of ulcers may cause intestinal bleeding.

diagnosis of Wegener's granulomatosis

The diagnosis is based on the definition of specific antibodies to neutrophils (ANCA), the concentration of which indicates the severity of the disease.Also in laboratory assays may cause common signs of inflammation in the body (increased white blood cell count, erythrocyte sedimentation rate, acceleration, detection of acute phase proteins).

treatment of Wegener's granulomatosis

treatment of Wegener's granulomatosis should be as early.If untreated, the disease in the first year mortality rate reaches 80%.When the disease shows the use of hormonal therapy (prednisone, dexamethasone) in combination with cytostatics (cyclophosphamide, azathioprine) and continuously in large doses.In cases of bleeding or abscess (abscess formation) surgical treatment.

In end-stage renal failure is used renal replacement therapy - dialysis.In the case of pronounced activity process in a timely beginning of the anti-inflammatory therapy may restore kidney function.But in 80% of cases are diagnosed at a stage of irreversible changes, dialysis is carried out for life.

physician therapist, nephrologist Sirotkin EV