Morbus Wegener (synonyms: granulomatosis with polyangiitis, granulomatous polyangiitis, Wegener’s granulomatosis and Wegener’s granulomatosis) is a chronic inflammation of the blood vessels, which is comparatively rare with an incidence of 5 to 7 per 100,000 inhabitants. Men are affected more frequently than women, with the peak age for Wegener’s disease being around 50 years of age.
What is Wegener’s disease?
Morbus Wegener is an inflammatory disease of the blood vessels, which is accompanied by necrosis and manifestations of granulomas in the lower (lung) and upper respiratory tract (nasal cavity, oropharynx, middle ear) as well as in the kidneys. See growtheology for Comprehensive Guide to Meningioma.
In the initial stage, the disease manifests itself in the form of symptoms similar to a cold or flu, such as headaches and joint pain, exhaustion, fever and weight loss. In the later course, the disease generalizes and in most cases (about 80 percent) glomerulonephritis (inflammation of the kidney corpuscles) and microaneurysms develop in the kidneys.
The chronic vasculitis (inflammation of the vessels) that is characteristic of Wegener’s disease leads to a lack of blood flow and supply to the affected organs, whereby in principle all organ systems can be affected.
Causes
The cause or etiology of Wegener’s disease is not yet known. It is assumed that the disease can be traced back to a dysregulation of the immune system, which synthesizes antibodies (c-ANCA) against the body’s own blood cells as a result of this disorder.
The specific triggers for the dysregulation have not yet been clarified. The involvement of inhalation allergens, which leads to a hypersensitivity reaction of the immune system (allergic reaction), is discussed.
Bacterial infections of the nasal mucosa with Staphylococcus aureus and a possible genetic disposition (predisposition) are also assumed to be potential trigger factors (initiating factors). In addition, in some cases, Wegener’s disease can be associated with an alpha-1-antitripsin deficiency (genetic disease of protein metabolism).
Symptoms, Ailments & Signs
Wegener’s disease can affect various organ systems and trigger a variety of symptoms. At the beginning of the disease, complaints in the ear, nose and throat area are usually in the foreground: A chronic runny nose with blood admixture, frequent nosebleeds and ulcers of the oral mucosa are characteristic.
If the granuloma formation spreads to the paranasal sinuses, the result is inflammation associated with pain in the forehead and jaw area. Involvement of the ears is noticeable through severe ear pain, occasionally vertigo attacks occur. Signs of changes in the throat can be swallowing difficulties, hoarseness and a dry, dry cough. If it spreads to the lungs, coughing up blood and massive shortness of breath with an acute risk of suffocation can occur.
Severe breathing-dependent pain in the chest area can indicate pleurisy or pericarditis. Many patients suffer from eye inflammation and visual disturbances, especially in the initial phase of the disease. Kidney involvement can trigger high blood pressure as the disease progresses. If there is blood in the urine, an inflammation of the kidney corpuscles (glomerulonephritis) should be considered.
Other possible symptoms of Wegener’s disease are painful, pressure-sensitive swellings in the joints (especially on the extremities) and paraesthesia and numbness in the toes and fingers. Bleeding of the skin and small skin ulcers also occur, and as the disease progresses, entire areas of skin can die off. Accompanying this, unspecific symptoms such as tiredness, exhaustion, loss of appetite and weight loss often occur.
Diagnosis & History
A suspicion of Wegener’s disease arises when two of the so-called four ACR criteria (oronasal inflammation, pathological chest X-ray, pathological urine sediment, granulomatous inflammation) can be clinically confirmed.
The diagnosis is confirmed by a biopsy followed by histological examination, during which necrotizing and partially granulomatous vasculitis of the smaller blood vessels can be detected in Wegener’s disease. A blood test also shows increased CRP and creatinine values (renal insufficiency) as well as leukocytosis as a marker for chronic inflammation and an accelerated blood sedimentation rate.
If glomerulonephritis is present, c-ANCA (anti-neutrophil cytoplasmic antibodies) in the serum and erythrocyturia (blood in the urine) can also be detected. The X-ray image shows shadowing in the area of the paranasal sinuses and infiltrated lung tissue, while a CT (computed tomography) scan reveals granulomas, scars and caverns (pathologically conspicuous cavities).
In the differential diagnosis, Wegener’s disease should be differentiated from bronchial carcinoma and Goodpasture’s syndrome. If left untreated, Wegener’s disease has an unfavorable (unfavorable) prognosis with a fatal outcome. On the other hand, in the majority of cases (90 percent), an improvement in symptoms can be achieved as part of therapy, even though the risk of recurrence is very high.
Complications
This disease usually results in a significant reduction in life expectancy for most patients. The symptoms of this disease can be very different and do not always have to be characteristic, so that in many cases early diagnosis and treatment is not possible. Those affected suffer primarily from coughs and runny noses and appear tired or exhausted.
There is also a severe reduction in the patient’s resilience and inflammation in the nose or ears. This inflammation can significantly reduce the quality of life. Sometimes bronchitis occurs. Patients often also suffer from inflammation of the eyes, which can be accompanied by visual problems.
Furthermore, there is fever and pain in the limbs, which leads to restricted mobility in everyday life. The quality of life is significantly reduced by Morbus Wegener. If left untreated, it can also lead to kidney failure, which if left untreated can lead to the death of the patient. Treatment can reduce most symptoms.
However, despite treatment, the life expectancy of those affected is significantly reduced and limited. The patient is also dependent on regular check-ups and can also suffer from mental illnesses due to the symptoms.
When should you go to the doctor?
An increased tendency to bleed is considered unusual and should be monitored further. If there is a frequent occurrence of nosebleeds or bleeding from the gums, the observations should be discussed with a doctor. A doctor is also required in the event of sensory disturbances.
Earaches, inflammation of the eyes, visual impairments, impaired breathing or a dry cough are causes for concern. Changes in the act of swallowing or vocalization must be presented to a doctor. If there is shortness of breath, there is an acute life-threatening condition. A rescue service is to be alerted and first aid measures are to be initiated so that the patient does not die prematurely.
Swelling of the joints, impairment of locomotion or general mobility disorders must be examined and treated. Discrepancies in blood flow, unusual sensations on the skin or a loss of muscle strength should be checked out by a doctor. High blood pressure, cardiac rhythm irregularities and unsteady gait should be examined. Rapid fatigue, a decrease in usual performance and a loss of well-being are warning signals from the body that should be discussed with a doctor. Fatigue, an unwanted decrease in body weight, loss of appetite, and a refusal to eat must be presented to a doctor. There is a risk of an undersupply of the organism, which can lead to an acute health-threatening situation.
Treatment & Therapy
Due to the unclear etiology, Wegener’s disease cannot be treated causally, but only symptomatically. The therapeutic measures are aimed at curbing the dysregulated immune system and are adapted to the stage.
For example, in an initial and locally limited stage, a drug combination therapy consisting of cotrimoxazole, which consists of the antibiotics trimethoprim and sulfamethoxazole and is also used prophylactically against oronasal colonization with Staphylococcus aureus, and a low-dose glucocorticoid such as prednisolone is recommended. If there is a generalized stage with life-threatening extrarespiratory symptoms, high-dose prednisolone and the cytostatic cyclophosphamide are usually used, with the latter being substituted by another cytostatic such as methotrexate if there is a contraindication.
Short-term shock therapy with methylprednisolone, which is administered intravenously, can also be indicated in therapy-resistant patients. If remissions are detected, the cytostatic can be replaced by immunosuppressive drugs such as mycophenolate or azathioprine, which are usually better tolerated, while the dose of prednisolone is gradually tapered to a permanent dosage.
Renal insufficiency and/or pulmonary hemorrhage requiring dialysis are treated within the framework of plasmapheresis, in which the body’s own plasma is substituted with a solution of electrolytes and hydrogen carbonate. When choosing the individual drug therapy, the risk of kidney damage, which can significantly reduce life expectancy, should be taken into account. Due to the side effects, kidney function in particular should be closely monitored in the presence of Wegener’s disease.
Outlook & Forecast
If left untreated, Wegener’s disease has an extremely poor prognosis. In this case, the inflammation spreads further and further and leads to permanent damage. Hearing, vision and kidney function are particularly affected. If the kidneys are affected, death can occur within a few months due to kidney failure. Frequent inflammations in the nasal area are also reflected in a so-called saddle nose (depression of the bridge of the nose).
Therapy, on the other hand, counteracts the spread of the inflammation. Life expectancy and quality hardly change with early diagnosis and treatment. In more than 90 percent of those affected, the therapy reduces the symptoms considerably, in 75 percent even to such an extent that a symptom-free life is possible, at least temporarily (complete remission). Nevertheless, in about 50 percent of those affected in remission there is a possibility that symptoms will recur even with successful treatment.
In addition, long-term or repeated use of immunosuppressive drugs and corticosteroids (maintenance therapy) is associated with an increased risk of infections and the development of cataracts. Regular blood count and vision checks are therefore required. In addition, steroid-related hair loss, acne, mouth ulcers and weight gain are possible.
Prevention
Since the exact trigger factors and the etiology of Wegener’s disease are unclear, the disease cannot currently be prevented.
Aftercare
In most cases, those affected with Morbus Wegener only have very limited and very few measures of direct aftercare available, so that the affected person should ideally see a doctor very early on. Early diagnosis can prevent further complications. However, since this is a hereditary disease, it cannot usually be completely cured, so that the affected person is always dependent on a doctor.
Genetic testing and counseling is advisable, especially if you wish to have children, in order to prevent a recurrence of Wegener’s disease. During the treatment itself, in most cases, patients are dependent on taking various medications that can alleviate and limit the symptoms.
The doctor’s instructions should always be followed, and the correct dosage should also be observed. Those affected are also dependent on regular checks and examinations of the internal organs, with the kidneys in particular being checked. The further course of the disease depends very much on the time of diagnosis, so that a general prediction cannot usually be made. In some cases, Wegener’s disease reduces the life expectancy of those affected.
You can do that yourself
This disease can be very stressful for those affected, especially if Wegener’s disease was not recognized until late. Although all symptoms of the disease can be treated, the patient’s quality of life is usually severely restricted.
For many patients and their relatives, it is therefore advisable to have psychotherapeutic treatment in addition to the medically required therapies in order to take the peaks out of the psychological strain. It is also helpful to have contacts with the Morbus Wegener self-help groups, which are often represented in the region and can be found on the Internet. Alternatively, those affected can also contact the Vaskulitis self-help group (www.vaskulitis-shg.de), which deals with all rare autoimmune diseases that lead to chronic vascular inflammation. The links and tips listed there can make everyday life much easier for those suffering from Morbus Wegener.
You will also benefit from any type of relaxation technique, as it also counteracts the tiredness and the feeling of exhaustion, from which patients suffer more. Yoga, progressive muscle relaxation according to Jacobson, Qigong and Tai Chi are recommended here. But the new, alternative forms of therapy such as music therapy, laughter yoga or EFT tapping therapy should also be tried out, because they have already proven to provide relief for those suffering from Morbus Wegener.
