Morbus Wegener – causes, symptoms & therapy

Wegener’s disease triggers inflammation of the small arterial blood vessels. This sign of disease affects the entire body and is also known as systemic vasculitis . Morbus Wegener is also known under the name of Wegener’s granulomatosis or granulomatosis with polyangiitis.

Gradually, this inflammation leads to narrowing or occlusion of the blood vessels in Wegener’s disease. In the course of the disease, so-called granulomas form in the body of the patient. These are small tissue knots that primarily affect the respiratory tract and kidneys in Wegener’s disease.

causes

Morbus Wegener usually occurs around the age of fifty, but it can also occur at any other age. Men get sick slightly more often than women. Overall, the disease is rather rare, about 5-7 people per 100,000 inhabitants fall ill.The causes are not exactly clear. What is certain is that the body’s immune system gets out of sync. Autoimmune antibodies, ANCA for short, trigger inflammation of the vessels in Wegener’s disease. So far, we can only speculate as to why these antibodies attack our own blood vessels. In addition to a genetic predisposition, certain pathogens and ingested allergens can also play a role.

Bacterial infections, for example by the bacterium Staphylococcus aureus, could be a cause. Inhaled allergens – i.e. allergy-causing substances – are also suspected of promoting the occurrence of Wegener’s disease. Sometimes the metabolic disease alpha-1-antitrypsin deficiency occurs together with Wegener’s disease.

symptoms and course

Typical symptoms of Morbus Wegener:

• Sniffles

• nosebleeds

• earache

• numbness

• Cough

• hoarseness

• shortness of breath

• visual impairment

• eye pain

In many cases, the symptoms of Wegener’s disease initially appear as a general feeling of illness and symptoms in the upper respiratory tract. A runny nose, nosebleeds, nasal mucosa and sinusitis are typical.

Changes in the nasal septum, earache, deafness and otitis media as well as inflammation of the throat or salivary glands can also occur. This is often followed by symptoms such as coughing, hoarseness and shortness of breath.

Muscle and joint problems and changes in the appearance of the skin also occur in the course of the disease. The eyes can also be affected in Wegener’s disease. This is manifested by blurred vision, inflammation or eye pain. In some sufferers, Wegener’s disease causes inflammation of the kidney vessels, which, if left untreated, leads to kidney failure .

Diagnose

The diagnosis of Morbus Wegener is not always easy due to the rare occurrence and the diverse clinical picture. Special diagnostic procedures are required here. As a rule, the diagnosis of Wegener’s disease is made on the basis of the existing symptoms, a blood test and a microscopic finding.

Four criteria are the focus of the diagnosis: There is inflammation in the area of ​​the airways, X-rays of the lungs show changes, blood or erythrocytes are detected in the urine and inflammatory changes in the vessel walls are visible.

The suspicion of Morbus Wegener can also be confirmed by examining tissue samples. The typical ANCA antibodies are also usually found in the blood count in Wegener’s disease.

treatment and therapy

Morbus Wegener is a chronic disease and therefore not curable. However, the symptoms can be treated with medication. If the disease is detected in the early stages, antibiotics are administered first . The drug of choice here is co-trimoxazole. In addition, in the case of Morbus Wegener, the cortisone prednisolone is often administered in low doses in order to achieve as long-lasting freedom from symptoms as possible.

About two thirds of those affected are almost symptom-free after this treatment, and in most patients at least an improvement can be achieved. In the case of more severe, sometimes life-threatening disease progression of Wegener’s disease with organ involvement, prednisolone is administered in combination with other drugs. The active ingredients methotrexate and cyclophosphamide, among others, have proven themselves here.

In individual cases, plasmapheresis – an exchange of blood plasma – is also used as a therapy for Morbus Wegener. After successful therapy, patients must continue to take medication. These include low-dose prednisolone and the drug azathioprine.

The antibiotic cotrimoxazole is also given to prevent infection with the bacterium Staphylococcus aureus. The treatment of Morbus Wegener is often supplemented by physiotherapy and suitable pain therapy .

prevention

The exact causes of Morbus Wegener are not known to medicine today. It is therefore not possible to prevent the disease. Since the disease can recur after successful therapy for Wegener’s disease, it is important to pay attention to renewed signs of the disease and, if necessary, to consult the doctor treating you. In any case, rapid treatment of Wegener’s disease is advisable in order to contain the symptoms and prevent complications.