Wegener's granulomatosis is an inflammation of the vascular walls that has an autoimmune granulomatous nature. The disease is serious because it, in addition to capillaries, arteries, venules and arterioles, involves the upper respiratory tract, lungs, kidneys, eyes and other organs.
Why does it arise? How do symptoms indicate its development? How is the diagnosis carried out? What is needed for treatment? Now these and many other questions should be answered.
Disease characteristics
Wegener's granulomatosis is a severe, rapidly progressing illness. If you do not pay attention to the symptoms in time and do not start treatment, then within 6-12 months there will be a fatal outcome.
Unfortunately, the etiology has not yet been established. However, according to statistics, this ailment most often affects people who have previously suffered an infectious disease of the respiratory tract. Doctors, however, do not exclude the connection of granulomatosis 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 the 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 cause 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 accompanied by chills.
- Weakness and fatigue.
- Damage to the upper respiratory tract.
- Rhinitis, accompanied by purulent discharge from the nose.
- Saddle deformity of the nose.
- Increased sweating.
- Ulceration of the mucous membranes, which can reach perforation of the nasal septum.
- Unreasonable weight loss.
- Muscle pain due to hypertonicity of the cells (myalgia).
- The appearance of joint diseases (arthritis).
These symptoms of Wegener's granulomatosis occur in almost all patients. Some also have such manifestations:
- Infiltrative changes in the lung tissue.
- Cough, accompanied by bloody sputum (indicates the development of pleurisy).
- The development of glomerulonephritis.
- Acute renal failure.
- Hemorrhagic rash.
- The development of episcleritis (manifested in inflammation of the sclera).
- The formation of exophthalmos and orbital granulomas.
- Problems with the nervous system.
Launched cases may be accompanied by damage to the coronary arteries and myocardium.
Localized form
This type of Wegener's granulomatosis is characterized by damage to the upper respiratory tract. Consequently, the most common symptoms are:
- Hoarseness of voice.
- The formation of blood crusts in the nasal passages.
- Persistent runny nose.
- Nasal bleeding.
- Difficulty in breathing.
If a patient is diagnosed with serious damage with a localized form of the disease, then he will have to undergo surgery.
Indications for surgery
As a rule, they are:
- Destruction of the nasal septum. In this case, plastic surgery of the nasal bridge.
- Stenosis with the threat of suffocation. Impose a tracheostomy (artificial respiratory throat).
- The threat of blindness. A 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 the operation is put forward by the doctor only if the patient's life is really in danger or disability.
Generalized form of the disease
It is characterized by a variety of systemic manifestations. These include:
- Hemorrhages.
- Paroxysmal coughing cough, accompanied by bloody-purulent sputum.
- Fever.
- Joint and muscle pain.
- Abscessive pneumonia.
- Renal and cardiopulmonary failure.
- Polymorphic rash.
Wegener's granulomatosis of the generalized form is treated using extracorporeal hemocorrection methods . These include:
- Cryoapheresis. Pathological substances are selectively removed from blood plasma. Useful compounds remain. The difference from plasma filtration is that the plasma cools during cryoapheresis.
- Plasmapheresis This procedure involves the collection of blood, its subsequent purification and return back to the bloodstream.
- Cascade plasma filtration. High-tech semi-selective method that selectively removes viruses and pathogens from blood plasma. One of the most effective ways to cleanse the blood in the world.
- Extracorporeal pharmacotherapy. Hemocorrection, due to which drugs are delivered directly to the pathological process zone. The peculiarity of the method is the point impact. Often used in the treatment of Wegener's granulomatosis of the nose. Using the method avoids the undesirable effects of drugs on the body as a whole.
And, of course, pharmacological treatment is mandatory. What drugs are usually prescribed for both forms of Wegener's granulomatosis are described below.
Diagnostics
It should talk about its implementation. If a person noticed symptoms of Wegener's granulomatosis (a photo of the manifestations of the disease is not presented for ethical reasons), then he needs to go to an appointment with a rheumatologist.
Diagnosing 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:
- Urine and blood donation for clinical analysis. The results may indicate the presence of anemia in the person, the inflammatory process, will help to verify the change in the density of urine and in its blood and protein content. Also, this study allows us to identify acceleration of the erythrocyte sedimentation rate, normochromic anemia, thrombocytosis, as well as microhematuria and proteinuria.
- Blood donation for biochemical analysis. Results will indicate signs of liver and kidney damage. It is also possible to detect an increase in Ξ³-globulin, urea, creatinine, haptoglobin, seromucoid and fibrin.
- Ultrasound of the kidneys and abdominal organs. An ultrasound scan can help identify lesions and reduce blood flow.
- Urological tests (scintigraphy, excretory urography). These studies will help identify the degree of decline in kidney function.
- X-ray of the lungs. It 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, in the diagnosis of Wegener's granulomatosis, you may need to consult related specialists - a pulmonologist, otolaryngologist, ophthalmologist, nephrologist, urologist and dermatologist. They, having examined and questioned the patient, will exclude other similar diseases.
Treatment
Therapy for Wegener's granulomatosis is prescribed by a doctor after a full examination.
As a rule, Cyclophosphamide is prescribed. This is a cytostatic chemotherapeutic drug that has an alkylating type effect. It has a very wide spectrum of antitumor activity. In addition, the drug has a pronounced immunosuppressive effect. Take this drug intravenously, the daily dosage is 150 mg per day.
Combine the drug with Prednisolone, a synthetic glucocorticoid drug whose action 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 administered decreases.
This treatment of Wegener's granulomatosis is practiced for 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 subsidence of acute symptoms. Of course, the daily dose will need to be gradually reduced.
Reception of immunosuppressants
Wegeners cannot do without these preparations with Wegener's granulomatosis (the photo of granulomas under a microscope is presented above). Because this disease has an autoimmune nature, and medication is required to suppress immune defense.
Two of the above drugs also belong to immunosuppressants, and the most effective ones. But there are alternatives:
- "Methotrexate." Antitumor agent related to antimetabolites. Inhibits the synthesis of thymidylate and purine nucleotides. Particularly active against rapidly growing cells, 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." The structural analogue of adenine, guanine and hypoxanthine, which make up nucleic acids. The drug blocks tissue proliferation and cell division, and also affects the synthesis of antibodies.
- Chlorobutin. It has an alkylating effect. The drug has a cytostatic property, so it also disrupts DNA replication. It especially affects lymphoid tissue.
The listed drugs lower the degree of immunity activity increased by Wegener's granulomatosis, affecting the "raging" organism. In other words, they suppress his reaction.
Unfortunately, a side effect is a decrease in the body's overall resistance to various infections and metabolic disorders, however, without taking these drugs, there is no way to cope with the disease.
What about the doses and the regimen of administration? This is determined by a rheumatologist. For some patients, for example, pulse therapy is suitable - administering the drug in large doses 1-2 times a week.
Maintenance of remission and treatment of relapses
When a personβs condition improves, all forces are directed at maintaining his remission. To do this, continue to undergo immunosuppressive therapy, otherwise the disease will progress. But what drugs to take, and in what quantity - it is determined by the doctor.
Remission can be long. But the patient must be prepared for relapse. 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 this disease? 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 reception must first be discussed with your doctor.
Complications and prognosis
If the treatment of the disease was started in a timely manner, then the chance of remission is very high. When conducting competent therapy, it occurs in almost all patients.
But the progression of the disease leads to serious consequences, among which:
- Facial bone destruction.
- Renal failure.
- Chronic conjunctivitis.
- Respiratory failure.
- Developing necrotic granulomas in the lung tissue.
- Gangrene of the fingers.
- The development of pneumocystis pneumonia.
- Deafness, as a result of persistent otitis media.
- Gangrenous and trophic changes in the foot.
- Accession of secondary infections against a background of weakened immunity.
If a person ignores the symptoms and does not consult a specialist, then a favorable outcome can not be expected. 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 quality therapy, in 90% of patients, the condition improves significantly, and in 75%, remission occurs. But, unfortunately, in 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, timely treat infectious diseases, regularly visit a rheumatologist and follow all his recommendations without exception.