Granulomatosis with polyangiitis (Wegener’s disease) is a relatively rare disease. As a result of the disease, the blood vessels located in the nose, paranasal sinuses, throat, lungs, and kidneys become inflamed. Inflammation may be neglected for a long time, because there are no clinical manifestations. It is possible to determine the disease with the help of complex diagnostics including laboratory and instrumental methods. Treatment for granulomatosis depends on its state, the patient’s age, and other factors.
Definition of Granulomatosis with Polyangiitis (Wegener’s Disease)
Few are aware what Wegener’s disease is, and without timely diagnosis it has a disastrous impact. The disorder is associated with the vascular system and is a type of vasculitis. In pathology, an inflammatory process occurs, as a result of which granulomas are formed, provoking damage to organs and tissues. Signs of the disease are equally evident in men and women. However, most often Wegener’s disease is diagnosed at the age of 40 and older.
Causes of the disease
So far, the scientists have not been able to determine the exact causes of granulomatosis. Signs of the disease may appear as a result of a chronic infectious process existing on the mucous membrane of the respiratory tract. There are factors of Wegener’s disease development:
- delayed-type hypersensitivity, progressing under the influence of bacterial pathogens;
- uncontrolled intake of drugs.
Any other inflammation can provoke a pathology in the body, as a result of which the normal functioning of the immune system is disrupted. With pathology, a spasm occurs in the vessels, granulomas form, healthy tissue is destroyed and the volume of circulating blood is reduced.
Symptoms of Wegener’s Granulomatosis
Granulomatosis with polyangiitis may appear suddenly or gradually progress over several months, accompanied by mild symptoms. The first signs of the development of the disease are damage to the respiratory tract, in particular, the throat, nose, and lungs. The condition of patients with such disorders often rapidly worsens. In addition, there are such symptoms:
- difficult breathing, constant bleeding;
- cough that may produce blood-tinged sputum
- shortness of breath, wheezing during physical activity or in a calm state;
- fever;
- numbness of the legs, hands;
- weight loss;
- blood impurities in urine
- skin ulcers or bruising;
- eye redness, burning sensation, pain;
- ear infections.
In some patients with the disease, damage extends only to the lungs. In a more severe course, kidney damage is possible, while pathological signs may be absent. It is possible to determine the problem only with the help of a laboratory study of urine and blood. In case of untimely treatment of granulomatosis, renal failure and anemia often progress.
Diagnosis and Course of the Disease
In each patient, the symptoms of granulomatosis with polyangiitis can be observed with different intensity, depending on the degree of damage. There are 4 states of the disease:
- granulomatous-necrotizing vasculitis, leading to the formation of ulcers in the nasal septum, eye sockets;
- pulmonary form with involvement in the pathological process of the lung parenchyma;
- generalized lesions extending to the lower respiratory tract, gastrointestinal tract, kidneys;
- terminal phase, characterized by renal failure and often fatal (without treatment).
When the first signs of the disease appear, it is required to consult a doctor who will conduct a visual examination and prescribe a comprehensive diagnosis. It is possible to determine the pathology using a blood test, urine, X-rays, and a biopsy of the vascular walls.
Treatment of Wegener’s Granulomatosis
Treatment for a limited and generalized form of granulomatosis involves taking hormonal drugs. With early treatment, it is possible to cope with pathology in a few months. It is important to continue therapy after the symptoms have been eliminated in order to prevent relapse. Additionally, given the degree of pathology, the following drugs can be prescribed:
- immunosuppressive agents;
- glucocorticoids.
With a more neglected course, drug treatment can be supplemented with physiotherapy procedures. The patient is prescribed plasmapheresis with the collection of damaged plasma and the introduction of fresh from a donor. If the kidney disease is severe, a transplant may be required.
Consequences
An untimely diagnosed disease and delay in treatment are dangerous due to a number of complications:
- partial or complete hearing loss;
- scars on the skin;
- kidney damage;
- a violation of the shape of the nose, which is associated with a weakening of the cartilage;
- thrombus formation in deep veins.
In severe cases of pathology, patients often have renal or heart failure. Such complications without treatment lead to a rapid deterioration in the general condition and death.
Possible Relapse
According to statistics, with a local form of the disease and timely therapy, it is possible to achieve stable remission in 75% of cases. This period lasts an average of about a year, after which half of the patients have an exacerbation. Relapse with active progression occurs in 13% of patients who have undergone immunosuppressive therapy.
Granulomatosis with polyangiitis (Wegener’s Disease) and Pregnancy
During pregnancy, special attention is paid to the health of the woman, since during this period, many diseases, including granulomatosis can be exacerbated. If necessary, treatment of Wegener’s granulomatosis is prescribed to prevent complications that are dangerous to the health and life of the mother and the unborn child. The selection of drugs, dosages is carried out individually, taking into account the trimester, the woman’s condition, age and other factors.