Wegener's granulomatosis
The content of the article:
- Causes and risk factors
- Forms of the disease
- Disease stages
- Symptoms of Wegener's granulomatosis
- Diagnostics of the Wegener's granulomatosis
- Treatment of Wegener's granulomatosis
- Possible complications and consequences
- Prognosis for Wegener's granulomatosis
- Prevention
Wegener's granulomatosis is a systemic granulomatous-necrotic giant cell vasculitis, which initially affects the upper and lower respiratory tract, and later - the kidneys.
There are no reliable statistics on the incidence and prevalence of Wegener's granulomatosis. The disease is more often observed in men of young and middle age, practically does not occur in childhood.
Wegener's granulomatosis - an autoimmune granulomatous inflammation of the walls of blood vessels (vasculitis)
Causes and risk factors
The exact causes of Wegener's granulomatosis are unknown. Considering that immune complexes are found in the blood of this disease, it is classified as an autoimmune pathology. Presumably, the disease develops as a result of an error of the immune system, due to which the tissues of the vascular walls are perceived by the immune system as foreign and are subject to destruction. Various inflammatory processes, including infectious ones, can lead to this.
In Wegener's granulomatosis, immune cells attack the walls of blood vessels and surrounding tissue
The immune system, defining its own tissue as foreign, produces antibodies to it. Antibodies damage the walls of blood vessels, which leads to the formation of areas of inflammation, that is, granulomas. Gradually increasing in size, the granulomas close the vessel lumen. As a result, the blood supply to tissues, the processes of tissue respiration and metabolism are disrupted.
Forms of the disease
Depending on the extent of the pathological changes, two forms of Wegener's granulomatosis are distinguished:
- localized. Mainly the upper part of the respiratory system is affected, which is manifested by hoarseness, frequent nosebleeds, the formation of bloody crusts in the nasal cavity, persistent rhinitis;
- generalized. Characterized by extensive systemic manifestations (fever, abscessing pneumonia, musculoskeletal pain, increasing signs of renal, cardiovascular and respiratory failure).
Disease stages
In the clinical picture of Wegener's granulomatosis, several stages are distinguished:
- Rhinogenous granulomatosis (granulomatous necrotic vasculitis). Necrotic ulcerative or purulent-necrotizing rhinosinusitis, nasopharyngitis, laryngitis, destructive changes in the orbit and nasal septum develop.
- Pulmonary granulomatosis. The pathological process captures lung tissue.
- Generalized granulomatosis. The lower parts of the respiratory system, the cardiovascular system, the organs of the digestive tract and the kidneys are affected.
- Terminal granulomatosis. Renal and / or pulmonary failure develops. The lethal outcome occurs within a year from the moment of the transition of the disease to the terminal stage.
Symptoms of Wegener's granulomatosis
Common symptoms of Wegener's granulomatosis include:
- increased body temperature with chills;
- severe weakness;
- hyperhidrosis (excessive sweating);
- joint and muscle pain;
- loss of body weight.
In 90% of patients, Wegener's granulomatosis is manifested by damage to the upper respiratory system. Formed chronic rhinitis, resistant to the therapy, which is accompanied by purulent-bloody discharge. Ulcerative defects form on the mucous membrane of the nasal cavity. Gradually increasing in size, they lead to perforation of the nasal septum, saddle deformity of the nose. The presence of granulomatous-necrotic changes is also detected in the paranasal sinuses, external auditory canals, trachea, larynx and oral cavity.
Symptoms of Wegener's granulomatosis
In 2/3 of patients, the disease is accompanied by the formation of destructive cavities in the lungs, persistent cough, and hemoptysis.
Involvement of the kidney vessels in the pathological process leads to the development of glomerulonephritis, and in the future - renal failure.
Skin symptoms of Wegener's granulomatosis are the appearance of a hemorrhagic rash, elements of which are prone to necrotization.
The eyes, nervous system, myocardium, coronary arteries can also be affected.
Diagnostics of the Wegener's granulomatosis
If you suspect Wegener's granulomatosis, an instrumental and laboratory examination is performed:
- general blood test (for thrombocytosis, accelerated ESR, normochromic anemia);
- general urine analysis (microhematuria, proteinuria are detected);
- biochemical blood test (for an increase in haptoglobin, seromucoid, fibrin, urea, creatinine, γ-globulin);
- X-ray of the lungs (for pleural exudate, decay cavities, infiltrates);
- immunodiagnostics (a decrease in the level of complement, the presence of antineutrophil antibodies, HLA class antigens - B8, B7, DR2 and DQW7 are established).
Radiography of the lungs - pulmonary nodes with Wegener's granulomatosis
If necessary, other methods are used in the diagnosis of Wegener's granulomatosis: for example, computed tomography or biopsy of the tissues of the upper respiratory tract with subsequent histological analysis.
Treatment of Wegener's granulomatosis
The main method of treatment for Wegener's granulomatosis is immunosuppressive therapy, which is carried out with a combination of cyclophosphamide and prednisolone. As the patient's condition improves, their dosage is gradually reduced until completely canceled. After that, the patient is prescribed methotrexate for a long course (1.5-2 years).
With Wegener's granulomatosis, Methotrexate is indicated for a long course of up to 2 years
In the treatment of the generalized form of Wegener's granulomatosis, methods of extracorporeal hemocorrection are used:
- extracorporeal pharmacotherapy;
- cascade plasma filtration;
- plasmapheresis;
- cryoapheresis.
Possible complications and consequences
The progressive course of Wegener's granulomatosis can be complicated by:
- hemoptysis;
- deafness;
- destruction of the bones of the facial skull;
- gangrene of the feet;
- the formation of areas of necrosis in the lung parenchyma;
- secondary infections during immunosuppressive therapy;
- renal failure.
Prognosis for Wegener's granulomatosis
The prognosis for Wegener's granulomatosis in the absence of adequate and timely treatment is extremely unfavorable. More than 90% of patients die in the first two years from the moment of diagnosis.
Against the background of immunosuppressive therapy, 85% of patients show a significant improvement, in 75% of patients the disease goes into remission. In more than half of cases, remission lasts about a year, after which an exacerbation occurs.
In 13% of patients, the disease is resistant to the ongoing immunosuppressive therapy and is characterized by a constantly progressive course.
Prevention
Due to the fact that the exact cause of Wegener's granulomatosis is unknown, measures for its prevention have not been developed.
Elena Minkina Doctor anesthesiologist-resuscitator About the author
Education: graduated from the Tashkent State Medical Institute, specializing in general medicine in 1991. Repeatedly passed refresher courses.
Work experience: anesthesiologist-resuscitator of the city maternity complex, resuscitator of the hemodialysis department.
The information is generalized and provided for informational purposes only. At the first sign of illness, see your doctor. Self-medication is hazardous to health!
