Wegener's granulomatosis is an inflammation of the vascular walls, which has an autoimmune granulomatous character. The disease is serious because it involves the upper respiratory tract, lungs, kidneys, eyes and other organs in addition to capillaries, arteries, venules and arterioles.
Why does it occur? How do the symptoms indicate its development? How is the diagnosis carried out? What is needed for treatment? Now these and many other questions must be answered.
Characteristics of the disease
Wegener's granulomatosis is a severe, rapidly progressive disease. If you do not pay attention to the symptoms in time and do not start treatment, then death will occur within 6-12 months.
Unfortunately, the etiology has not yet been established. However, according to statistics, this disease most often affects those people who have previously suffered an infectious disease of the respiratory tract. Doctors, however, do not exclude the connectiongranulomatosis with harmful microorganisms and parasitic agents.
It is important to note that immune complexes are almost always found in the blood of patients with this diagnosis, but their role in pathogenesis has not yet been proven. In the same way as the presence of antibodies to the cytoplasm, neutrophils and antigens DR2, HLA B7 and B8. But there is an assumption that all these factors are catalysts for the development of Wegener's granulomatosis.
The reason may also be increased sensitivity of the body or weakened immunity.
Symptoms
This disease is accompanied by a pronounced clinical picture. Symptoms of Wegener's granulomatosis are:
- Fever with chills.
- Weakness and fatigue.
- Upper respiratory tract infection.
- Rhinitis with purulent nasal discharge.
- Saddle nose deformity.
- Excessive sweating.
- Ulceration of the mucous membranes, which can reach a perforation of the nasal septum.
- Unreasonable weight loss.
- Muscle pain due to cell hypertonicity (myalgia).
- Appearance of diseases of the joints (arthritis).
These symptoms of Wegener's granulomatosis occur in almost all cases. Some also have the following symptoms:
- Infiltrative changes in lung tissue.
- Cough with bloody sputum (indicating pleurisy).
- Development of glomerulonephritis.
- Acute kidney failure.
- Hemorrhagic rash.
- Development of episcleritis (manifested in inflammation of the sclera).
- Formation of exophthalmos and orbital granulomas.
- Problems with the nervous system.
Advanced cases may be accompanied by damage to the coronary arteries and myocardium.
Localized form
This type of Wegener's granulomatosis is characterized by lesions of the upper respiratory tract. Therefore, the most common symptoms are:
- Hoarse voice.
- Formation of bloody crusts in the nasal passages.
- Stubborn runny nose.
- Nosebleeds.
- Difficulty breathing.
If a patient is diagnosed with serious damage in a localized form of the disease, then he will have to undergo surgery.
Indications for surgery
Usually they are:
- Destruction of the nasal septum. In this case, plastic surgery of the back of the nose is performed.
- Stenosis with threat of suffocation. Impose a tracheostomy (artificial windpipe).
- The threat of blindness. The granuloma is resected.
- Terminal renal failure. In this situation, kidney transplantation is indicated.
It is important to clarify that with Wegener's granulomatosis, the recommendation for an operation is put forward by a doctor only if the patient's life is really in danger ordisability.
Generalized form of the disease
It is characterized by a variety of systemic manifestations. These include:
- Hemorrhages.
- A paroxysmal hacking cough, accompanied by bloody-purulent sputum.
- Fever.
- Joint-muscular pain.
- Abscess pneumonia.
- Renal and cardiopulmonary failure.
- Polymorphic rash.
Wegener's granulomatosis of the generalized form is treated using methods of extracorporeal hemocorrection. These include:
- Cryoapheresis. Pathological substances are selectively removed from the blood plasma. Useful connections remain. The difference from plasma filtration is that during cryoapheresis, the plasma is cooled.
- Plasmapheresis. This procedure involves taking blood, then cleaning it and returning it back to the bloodstream.
- Cascade plasma filtration. A high-tech semi-selective method that allows selective removal of viruses and pathogens from blood plasma. One of the most effective blood purifiers in the world.
- Extracorporeal pharmacotherapy. Hemocorrection, due to which drugs are delivered directly to the area of the pathological process. The peculiarity of the method lies in the point effect. Often used in the treatment of Wegener's granulomatosis of the nose. Using the method allows you to avoid the undesirable effects of drugs on the body as a whole.
And of coursepharmacological treatment is mandatory. What drugs are commonly prescribed for both forms of Wegener's granulomatosis is described below.
Diagnosis
It should be told about its implementation. If a person notices the symptoms of Wegener's granulomatosis (the photo of the manifestations of the disease is not presented for ethical reasons), then he needs to go to see a rheumatologist.
Diagnosis of this disease is a difficult task. It must be distinguished from sarcoidosis, malignant tumors, uveitis, sinusitis, otitis media, blood and kidney diseases, tuberculosis and other autoimmune diseases. The initial stage includes the following studies:
- Donating urine and blood for clinical analysis. The results can indicate the presence of anemia in a person, an inflammatory process, help to make sure that the density of urine changes and the content of blood and protein in it. Also, this study reveals an acceleration of the erythrocyte sedimentation rate, normochromic anemia, thrombocytosis, as well as microhematuria and proteinuria.
- Blood donation for biochemical analysis. The results will indicate signs of liver and kidney damage. It is also possible to detect an increase in γ-globulin, urea, creatinine, haptoglobin, seromucoid and fibrin.
- Ultrasound of the kidneys and abdominal organs. Ultrasound examination will help to identify lesions and reduced blood flow.
- Urological tests (scintigraphy, excretory urography). These studies will help determine the degree of decline in functionkidney.
- X-ray of the lungs. Helps to determine the presence of infiltrates in their tissue, as well as to identify pleural exudate and decay cavities.
- Bronchoscopy with biopsy of the mucous membranes of the upper respiratory tract. This helps to identify the morphological signs of the disease.
In addition to the above, as part of the diagnosis of Wegener's granulomatosis, it may be necessary to consult related specialists - a pulmonologist, an otolaryngologist, an ophthalmologist, a nephrologist, a urologist and a dermatologist. They, after examining and questioning the patient, will exclude other similar diseases.
Treatment
Therapy for Wegener's granulomatosis is prescribed by a doctor after a complete examination.
As a rule, the drug "Cyclophosphamide" is prescribed. It is a cytostatic chemotherapeutic drug that has an alkylating type of action. It has a very broad spectrum of antitumor activity. In addition, the drug has a pronounced immunosuppressive effect. Take this remedy intravenously, the daily dosage is 150 mg per day.
They combine the drug with "Prednisolone", a synthetic glucocorticoid drug, the action of which is aimed at suppressing the functions of tissue macrophages and leukocytes. It also stabilizes lysosomal membranes, disrupts the ability of macrophages to phagocytosis and prevents leukocytes from entering the inflamed area. The daily dosage of "Prednisolone" is 60 mg. As the condition improves, the amount of drug administereddecreasing.
This treatment of Wegener's granulomatosis is practiced in systemic lesions, when the disease covers the skin, joints, kidneys and eyes.
Drug therapy lasts a long time. So, for example, "Cyclophosphamide" will have to be taken for another 1 year after the doctor diagnoses the stage of complete remission of acute symptoms. Of course, the daily dose will need to be gradually reduced.
Taking immunosuppressants
Without these drugs with Wegener's granulomatosis (a photo of granulomas under a microscope is presented above). Because this disease is autoimmune in nature, and taking medication to suppress the immune defense is mandatory.
The two drugs listed above are also immunosuppressants, and the most effective. But there are alternatives:
- Methotrexate. An antitumor agent related to antimetabolites. Inhibits the synthesis of thymidylate and purine nucleotides. Particularly active against rapidly growing cells, it also has an immunosuppressive effect.
- "Fluorouracil". Its action is aimed at blocking DNA synthesis and the formation of structurally imperfect RNA. So the process of cell division is inhibited.
- "Dexamethasone". Reduces the concentration of proteolytic enzymes, reduces capillary permeability, inhibits collagen formation and fibroblast activity.
- "Azathioprine". Structural analogue of adenine, guanine and hypoxanthine, which make up nucleic acids. The drug blocks tissue proliferation and cell division, and also affectsantibody synthesis.
- Chlorbutin. It has an alkylating effect. The drug has a cytostatic property, so it also disrupts DNA replication. It has a particularly strong effect on lymphoid tissue.
The listed drugs reduce the degree of immunity activity that has increased with Wegener's granulomatosis, affecting the "raging" body. In other words, suppress his reaction.
Unfortunately, a side effect is a decrease in the overall resistance of the body to various infections and metabolic disorders, but without taking these drugs, the disease cannot be coped with.
What about doses and mode of administration? This is determined by a rheumatologist. For some patients, for example, pulse therapy is suitable - the administration of the drug in large doses 1-2 times a week.
Maintaining remission and treating relapses
When a person's condition improves, all forces are directed to maintaining his remission. To do this, you must continue to undergo immunosuppressive therapy, otherwise the disease will progress. But what drugs to take, and in what quantity - this is determined by the doctor.
Remission can be long. But the patient must be prepared for relapses. When the disease worsens, it is necessary to increase the dosage of hormone-containing drugs or return to the norm that was prescribed at the beginning of treatment.
Many people, by the way, are interested in whether there are any effective folk remedies that can help cope with thisdisease? The answer is obvious. Even if there are no pharmacological preparations, the use of which could lead to a complete recovery, then herbal treatment will not help. Infusions and decoctions can only alleviate negative symptoms. But even in this case, their intake should be discussed with your doctor beforehand.
Complications and prognosis
If the treatment of the disease was started in a timely manner, then the chance of remission is very high. With proper therapy, it occurs in almost all patients.
But the progression of the disease leads to serious consequences, including:
- Destruction of facial bones.
- Kidney failure.
- Chronic conjunctivitis.
- Respiratory failure.
- Developing necrotic granulomas in lung tissue.
- Gangrene of fingers.
- Development of Pneumocystis pneumonia.
- Deafness due to persistent otitis.
- Gangrenous and trophic changes in the foot.
- Accession of secondary infections on the background of weakened immunity.
If a person ignores the symptoms and does not turn to a specialist, then a favorable outcome can not be counted on. About 93% of patients die between 5 months and 2 years.
It is also important to note that the local form of granulomatosis is benign. When conducting high-quality therapy, in 90% of patients the condition improves significantly, and in 75% remission occurs. But, unfortunately, for 50% it does not last long,about six months, after which there is an exacerbation and all symptoms return.
Therefore, in order to avoid complications and exacerbations, it is necessary to constantly strengthen your immunity, treat infectious diseases in a timely manner, regularly visit a rheumatologist and follow all his recommendations without exception.
