Wegener's disease: specialists and information

Wegener's disease is an inflammatory rheumatic vascular disease. It is also known as Wegener's granulomatosis, Wegener-Klinger granulomatosis or granulomatosis with polyangiitis.

Find out more about the symptoms and treatment here and find selected Wegener's disease specialists.

Definition: What is Wegener's disease?

Wegener's disease and related vasculitides are inflammatory vascular diseases. This group of diseases has in common the destruction of blood vessels and their penetration by inflammatory blood cells. At the end of the development, the blood vessels become obliterated.

Wegener's granulomatosis is also characterized by so-called granulomatous changes (granulomas). It has a characteristic clinical course and offers good treatment options.

With the help of serological findings (c- and p-ANCA test), the diagnosis can be further confirmed.

Causes for the development of Wegener's disease

Wegener's disease can occur at any age and affects men and women almost equally often. It is not known whether there is a particular genetic predisposition. This is often the case with other immunological diseases.

No specific triggers for Wegener's disease are known. We have to rely on conjecture as to how it develops.

In the vast majority of cases, a preceding bacterial infection can be detected. It is possible that parts of the pathogen (possibly staphylococci) trigger the subsequent immunological inflammation in the area of the vessel walls.

Symptoms of Wegener's disease

Wegener's disease usually manifests itself throughout the body: wherever there are blood vessels. General symptoms therefore occur, such as

• fatigue,
• tiredness,
• loss of appetite,
• general weakness and
• joint pain,

occur.

In addition, there are the symptoms of the particular organ infestation.

• Eyes: Inflammation in almost all parts of the eyes, so-called "red eye", visual disturbances (episcleritis, uveitis, exophthalmos, etc.).
• Ear, nose and throat: Chronic bloody inflammation of the nasal mucosa with bloody nasal secretions, later destruction of the nasal septum. Chronic suppuration of the middle ear with deafness, sore throat, inflammation of the salivary glands, which do not respond to the usual therapy.
• Trachea: Acute shortness of breath, air hunger, sometimes suffocation, narrowing of the trachea just below the vocal fold.
• Lungs: Often only minor symptoms, despite frequently observed, very conspicuous lung shadowing in the form of round lung foci, pleurisy. Sometimes pulmonary hemorrhage.
• Joints: General pain in the small joints of the hands and feet without a characteristic pattern of involvement, also in conjunction with muscle pain.
• Kidneys:
  • Pain in the kidney areas due to swelling of the kidneys in the renal capsule,
  • Headaches due to increased blood pressure,
  • Swelling in the eyelid area due to loss of protein and inflammation of the renal tubules (glomerulitis),
  • nausea, vomiting and
  • severe feeling of illness when kidney failure (uremia) occurs
• Nervous system: numbness with a furry sensation in the tips of the fingers and feet (polyneuritis), unsteady gait, weakness due to accompanying muscle inflammation (myositis)
• Skin: Punctiform or extensive discoloration and ulceration with destruction of large areas of skin and subcutaneous fatty tissue (necrosis), with involvement of large blood vessels, e.g. in the area of the fingers and toes (gangrene).

Frequently to always there is

• an increase in the erythrocyte sedimentation rate and CRP,
• a reduction in red blood cells(anemia),
• an increase in white blood cells (leukocytosis) and
• an altered protein composition in the serum.

In acute illness with Wegener's disease, an increase in the c-ANCA titer can be detected

Among other things, Wegener's disease leads to a change in the composition of blood components © sveta | AdobeStock

Diagnosis of Wegener's disease

The correlations are usually still unclear at the time of the diagnostic tests. The doctor therefore investigates the organ infestation that is in the foreground.

If, for example, a round lung focus or a different type of shadowing is found, the tumor search is carried out on suspicion of a bronchial carcinoma.

Bronchoscopy often reveals insufficient material: the lung shadows are too close to the pleura in the lung tissue without bronchus involvement. In such a case, only an open lung biopsy, i.e. opening the chest and targeted tissue removal, can help.

If the kidneys are affected, the doctor will aim for a kidney biopsy; if the skin, muscles, nerves, etc. are affected, a sample will be taken from the respective organ.

Otorhinolaryngology and ophthalmology are of particular importance. Targeted specialist examination and multiple tissue samples are used to make a histological diagnosis. The tissue samples are sent to an experienced pathologist who examines them.

It often takes a long time before the final diagnosis is made.

Treatment of Wegener's disease

Today, Wegener's granulomatosis can be treated highly effectively with Endoxan® (3 x 50 mg per day or as shock therapy, high dose) in combination with cortisone (20 to 100 mg per day).

Alternatively, lmurek® (3 x 50 mg per day) and other immunosuppressive drugs can be used. Cotrim® (2 x 1 tablet per day) is recommended as an antibiotic that may have a special effect. If urinary intoxication has occurred, renal failure can be treated by hemodialysis. Subsequent kidney transplants are also possible.

Endoxan® should be administered for several weeks to a year in doses between 50 and 150 mg per day. This can lead to considerable side effects, which are visible in the blood count. These include

• A drop in the number of white blood cells and
• the dreaded endoxancystis (a bloody bladder infection that is difficult to treat).

For its part, lmurek® also causes bone marrow damage with a drop in the white blood cell count. The affected person requires general rest and care.

It is important to have close medical check-ups and to provide patients with good information about the various courses of the disease. This helps to assess and treat a flare-up of Wegener's disease in good time.

Conclusion on Wegener's disease?

Only 10 to 20 percent of patients treated with Endoxan® suffer a relapse. Other immunosuppressive drugs have been used. Experience with other immunopathies, e.g. lupus erythematosus, has shown that

• extraordinary physical stress,
• pregnancies,
• non-vital operations and the like should be

should be avoided. Vaccinations are possible.

With early detection and consistent treatment as well as close follow-up care, Wegener's disease can usually be improved. It is often even possible to cure the disease.