Wegener's granulomatosis is one of the most rare and severe ailments related to a number of vasculitis. He deals a serious blow to the respiratory, urinary and bone system of the body, which often leads to sad consequences for the patient.
The clinical course of this form of vasculitis in many ways resembles the course of other diseases, because of which it is very difficult to make a timely diagnosis.
- 1 Description and statistics
- 2 Causes and risk factors
- 3 Classification
- 4 risks and complications
- 5 Symptoms
- 6 When to see a doctor
- 7 Diagnostics
- 7.1 blood test
- 7.2 biopsy
- 8 Treatment
- 9 forecasts and prevention
Description and statistics
Wegener's granulomatosis isa systemic chronic vasculitis that is characterized by formation of granulomas in the walls of small veins, arteries and vessels , as well as surrounding tissues lekidneys, and other organs.
It was first described and isolated from a number of vasculitides into a separate disease in 1936 by the famous German physician Friedrich Wegener.
The prevalence of the disease is 3-12 cases per 1 million people, but according to some data, over the past 30 years, these figures have increased approximately 4-fold. Men are more likely to develop it than women , and the average age of patients is about 40 years. The least sick of Wegener are children under 10 years old.
Causes and Risk Factors
The exact etiology of the disease has not yet been established by .According to the research, most often it occurs in people who have previously experienced respiratory infections, but doctors do not exclude the connection of the inflammatory process with harmful microorganisms and other agents.
Also, the presence of antigens B8, HLA B7, DR2, antibodies to the cytoplasm of neutrophils and carriage of Staphylococcus aureus in the nasopharynx - presumably these factors are the catalysts for the development of the disease or its relapses. In addition, granulomas with giant multinucleated cells may be a manifestation of a delayed allergic reaction or the body's reaction to foreign bodies.
Depending on the location of the pathological process, it is common to distinguish three main forms of the disease:
- local is characterized by an isolated lesion of the upper respiratory tract, hearing or vision;
- with the restricted form, in addition to the above-mentioned organs, the lungs are involved in the process;
- generalized Wegener's granulomatosis includes multiple internal lesions with mandatory involvement of the kidneys.
In addition, its clinical course has four stages:
- I stage , or granulomatous necrotic vasculitis. It flows with manifestations of purulent-necrotic or ulcerative necrotic rhinosinusitis, nasopharyngitis and laryngitis, as well as destructive changes in the tissues of the nasal septum and orbits;
- II stage , or pulmonary vasculitis. At this stage, the inflammatory process affects the lung parenchyma;
- III stage , or generalized vasculitis. At the third stage of the disease, multiple lesions are observed in the patients, which are localized in the lower respiratory tract, gastrointestinal tract, kidneys, as well as in the vessels and arteries.
- IV, or the terminal stage .The fourth stage of the disease causes pulmonary-cardiac or renal failure, which within a year lead to the death of the patient.
Dangers and complications of
In addition to the fact that Wegener's granulomatosis carries a direct danger to human life, can cause a number of serious complications of :
- pulmonary bleeding that occurs due to vascular rupture;
- plugging of lacrimal ducts, chronic conjunctivitis, dysfunction of Eustachian tubes or even loss of hearing and vision caused by the development of numerous granulomas;
- deformation of the nose and deep scars on the skin, remaining after the characteristic ulcers of the disease;
- bronchial stenosis, laryngeal stenosis, pneumocystis pneumonia;
- myocardial infarction as a result of rupture of the coronary arteries;
- trophic ulcers, gangrene of toes;
- chronic or terminal renal insufficiency.
The early stage of the disease is characterized by typical signs of influenza or SARS:
- high fever;
- pain in muscles and joints;
- weakness and loss of appetite;
- difficulty with nasal breathing, rhinitis, sinusitis;
- otitis media and occlusion of the auditory tube.
The acute period lasts about three weeks, after which the passes to the second stage of - involvement in the process of the circulatory system with the addition of the following symptoms:
- Respiratory tract infection. The patient has a nasopharyngeal, numerous ulcers with purulent contents appear on the mucous membrane, the nasal cartilage is deformed.
- Lesion of the lung .First, the inflammation begins in the lungs, after which granulomas and cavities are formed, in which pus accumulates. Over time, they form abscesses, the patient begins to suffer from shortness of breath, chest pains and hemoptysis.
- Kidney damage .With the development of the disease, kidney dysfunction progresses, nephrogenic arterial hypertension develops and symptoms that are characteristic of kidney failure, including lack of appetite, nausea, vomiting, dry mouth, urinary problems, presence of blood in the urine. Renal damage is the most dangerous complication of Wegener's granulomatosis, which occurs in approximately 75% of patients.
- Lesion of vision organs .The visual acuity in the patient gradually decreases, the eyeball moves forward, after which there is complete blindness.
- Inflammation of the vessels .On the surface of the skin appear ulcers and eruptions, characteristic of most vasculitides: purpura( dark purple formations that appear due to the release of red blood cells from the vessels), papules, vesicle-bullous eruptions.
- Dysfunction of the digestive tract .The patient is suffering from pain in the umbilical region, stool disorders and other manifestations of gastrointestinal dysfunction.
- Joint damage .Complaints about inflammation and pain in the joints are noted in 60% of patients, which in the early stages becomes the reason for the diagnosis of "rheumatoid arthritis".
When to call a doctor
Symptoms of this form of vasculitis are quite diverse, so patients often write them off for a common cold or .Refer to a doctor should be when a person is worried about a permanent cold, which is not treated with usual nasal drops and sprays, and is accompanied by bleeding and purulent secretions.
rheumatologist deals with the treatment of the disease, but in addition, the patient will need consultation from the ophthalmologist, pulmonologist, otolaryngologist and nephrologist .It is important to remember that Wegener's disease progresses quite quickly, and the earlier the correct diagnosis is established, the more the patient's chances of recovery.
The examination of patients with suspected Wegener's granulomatosis includes:
- General and biochemical blood tests of .Studies are important for determining the inflammatory process in the body, which is characterized by an increase in ESR, leukocytosis, increased creatinine, urea, y-globulin and other markers.
In addition, very important determinants for diagnosis are the presence of antineutrophil antibodies and rheumatoid factor, antigens of the HLA class, a decrease in the level of complement.
- Urine analysis .It is carried out in order to identify proteinuria, microhematuria, as well as urinary syndrome, characteristic of glomerulonephritis.
- X-ray examination of the chest .When radiographing lungs in the tissue, multiple infiltrates, pleural exudate and decay cavities are found.
- Biopsy of the .One of the most reliable methods for diagnosing this disease: in samples of tissues that are taken from the nasal passages, kidneys, lungs or skin of the patient, signs of granulomatous necrotizing vasculitis are revealed.
Given that the disease is characterized by various symptoms and multiple lesions of internal organs , the spectrum of diseases for differential diagnosis is quite wide. First of all, it includes other forms of vasculitis( for example, nodular periarteritis in any form, Takayasu disease, Kawasaki, etc.), some malignant tumors, middle granuloma, sarcoidosis, lymphoid granulomatosis.
If a suspected Veneger's disease is suspected, the specialist will prescribe a blood test for for specific autoantibodies( proteins) - ANCA , which are detected during blood testing in more than 90% of patients with Wegener's disease. The detection of proteins is an occasion for additional tests, in order to confirm or refute the diagnosis.
Also, when analyzing blood, special attention is paid to the data of the erythrocyte sedimentation time( ESR) .With an elevated level of ESR, there is a suspicion of an inflammatory process in the body. With Wegener's disease, this indicator can be quite high.
When receiving blood test results, can be seen with anemia, characteristic of the disease - a reduced number of red blood cells.
The blood test for creatinine is performed to determine how well the patient's kidneys function. With the same purpose, a urine test is mandatory.
The most accurate and only method for determining the presence of the disease in the human body is to examine a tissue sample in the laboratory - a biopsy. For the study, the tissue sample is taken from the lungs, nasal passages, skin, kidneys.
Biopsy is carried out in two ways:
- using a special needle - the procedure is fast, the patient is almost immediately released home;
- with the operation of ( for example, in the open lung), when the patient lies in the hospital.
The tactics of treating Wegener's granulomatosis depend on the stage of the disease, the localization of the pathological process, the condition of the patient and other factors. In any case, it pursues the two main goals: achieving remission and maintaining it .Drug therapy includes the following medications:
- Corticosteroid hormones ."Prednisolone", "Methylprednisolone" can suppress the negative reaction of immunity at the earliest stage.
- Immunosuppressors of .The most effective in this case are "Cyclophosphamide", "Azathioprine" and "Methotrexate".Experimental drugs .Appointed in cases where the reception of standard drugs does not give the expected result. In particular, a drug called "Rituximab" is able to reduce the number of lymphocytes taking part in the inflammatory process.
Treatment of granulomatosis is associated with the emergence of certain risks of .The drugs prescribed against Veneger suppress immunity, which leads to a decrease in the body's ability to cope with infections. Corticosteroids can weaken the bones, especially with prolonged admission.
The attending physician may prescribe other agents to reduce the risk of complications:
- Bisphosphonate( Fosamax) - reduces the risk of osteoporosis with prolonged admission of the patient with prednisolone;
- Trimethoprim-sulfomethoxazole( Bactrim, Biseptol) - prevention of pulmonary infections;
- Folic acid( vitamin B9) - prevention of ulcers and other symptoms of B9 deficiency caused by taking methotrexate.
In particular, with serious kidney lesions, the patient often needs transplantation, and with stenosing changes in the larynx, the implantation of a reanastomosis and a tracheostomy.
Forecasts and prevention measures
In case of late diagnosis and lack of adequate treatment, the prediction of Wegener's granulomatosis is unfavorable - approximately 95% of patients die between 6 months and 2 years.
In addition, the chances for recovery largely depend on the form of the disease : local granulomatosis proceeds more benignly and practically does not carry the risks of disability or death.
Immunosuppressive therapy of significantly improves the condition of 90% of patients, and 75% cause persistent and prolonged remission, with a new exacerbation occurring in 50% of cases. Because of the unclear etiology of the disease, preventive measures have not been developed.
Wegener's granulomatosis refers to severe ailments that "mask" their symptoms under the manifestation of lighter, non-life-threatening diseases. Therefore, it is important to remember that any unpleasant symptoms that continue for quite some time require special attention of and mandatory specialist advice.