Wegener's disease • Symptoms & therapy

Wegener's disease usually begins with ENT complaints. The autoimmune disease can have very serious consequences and should be diagnosed and treated as early as possible by a specialist doctor. With consistent therapy, the disease improves in most patients.

Wegener's disease begins with inflammation in the nasopharynx and later spreads to the whole body. If the lungs and kidneys are affected, the disease can be fatal.
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Wegener's disease or Wegener's granulomatosis is an inflammatory rheumatic vascular disease that affects the small and medium-sized blood vessels. Granulomatosis is when nodules (granulomas) form in the tissue as a result of the disease. Wegener's disease typically begins in middle age. The autoimmune disease manifests itself in very different symptoms, which is why it is not always recognized immediately.

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Causes: Immune cells cause nodule formation

The causes of the disease in Wegener's disease are not yet fully understood. In addition to disturbed T cell regulation, autoreactive B cells – which produce certain antibodies, so-called c-ANCA (antineutrophilic cytoplasmic antibodies) – and other immune cells play a central role.

These "weapons" of the immune system promote the formation of inflammation-related granulomas on the walls of the blood vessels. These nodular changes can, for example, constrict or even block blood vessels.

In the event of a vascular blockage or infarction, the tissue supplied by the vessel is no longer supplied with sufficient blood and it dies. This has serious consequences in some cases.

Symptoms of Wegener's disease

Depending on which vessels are affected, the symptoms of the disease are very different. Wegener's granulomatosis often causes the inflammatory changes primarily in the upper respiratory tract at the beginning (initial phase).

Typical signs of Wegener's disease include ulcers in the area of ​​the oral mucosa, inflammation in the nasopharynx with purulent or bloody nasal discharge, and sinus and middle ear infections. These can persist for months to years, are sometimes difficult to treat and are often not recognized as a sign of Wegener's granulomatosis. In about half of those affected, the eyes are also affected, for example in the form of conjunctivitis with redness on one or both sides.

Diagnosis of Wegener's disease often later

As the disease progresses, it spreads to the whole body (generalization phase) and is often only then diagnosed as such.

In addition to a considerable general feeling of illness with fever and weight loss, severe purulent secretions from the mucous membranes in the airways, some of which are mixed with blood, occur.

Another disadvantage for the further course of Wegener's disease is the involvement of the kidneys, which occurs frequently and can lead to functional restrictions and even complete failure. The lungs, skin, and nervous system can also be affected. This can lead to death if there is no lack of therapy.

Concomitant joint complaints and muscle pain occur, but are usually not the focus of the disease process.

Laboratory tests provide evidence of Wegener's disease

When diagnosing Wegener's disease, the patient's symptoms play an important role. In addition, the detection of typical antibodies (c-ANCA) represents a major step forward. They are very characteristic of the disease and occur in more than 95 percent of patients in the generalization phase. Since the antibodies are absent in many patients in the first phase of the disease, a negative result at this point in time does not reliably rule out the disease.

Inflammatory values ​​in the blood, such as an increased rate of sedimentation and increased values ​​of the so-called C-reactive protein (CRP), provide unspecific information about the inflammatory reactions.

With the help of sampling (biopsy) in affected areas such as the mucous membranes of the upper respiratory tract and from the kidney, typical tissue changes can also be detected under the microscope. In addition, imaging procedures such as x-rays can make changes in the lungs visible.

Treatment of Wegener's disease

The treatment of Wegener's granulomatosis with a combination of glucocorticoids and cytostatics has been improved significantly in recent years. If possible, it should be done by a specialist and is divided into an initial phase and a maintenance phase.

In the initial phase of the treatment of Wegener's disease, the focus is usually on suppressing the immune system with high-dose glucocorticoids and cyclophosphamide. Infusions with immunoglobulins can also be used. This is often the best way to control the symptoms. If there is an improvement, the glucocorticoids are first gradually reduced.

After six to twelve months of therapy, the maintenance phase mainly involves a reduction in the cyclophosphamide dose, which should be tailored to the individual clinical picture. The high doses required at the beginning can be associated with serious problems such as inflammation of the urinary bladder lining (cystitis) or – especially with long-term use – an increased risk of bladder cancer. In addition to lowering the total dose, switching to immunosuppressive drugs that are better tolerated in the long term is also a therapeutic option.

In less severe cases, these immunosuppressants are now used instead of cyclophosphamide in the initial therapy. In end-stage renal dysfunction, a kidney transplant can further improve the patient's prognosis. Further treatment options for Wegener's disease are currently being tested.