Wegener's Disease: Specialists and Information

Wegener’s granulomatosis and related vasculitides are inflammatory vascular diseases. This group of diseases is characterized by the destruction of blood vessels and their infiltration by inflammatory cells from the blood. As the disease progresses, the blood vessels become obliterated.

Wegener’s granulomatosis is additionally characterized by so-called granulomatous changes (granulomas). It has a characteristic clinical course and responds well to treatment.

The diagnosis can be further confirmed using serological testing (c- and p-ANCA tests).

Wegener’s granulomatosis can occur at any age and affects men and women with nearly equal frequency. It is not known whether there is a specific genetic predisposition. With other immunological diseases, this is often the case.

Specific triggers for Wegener’s granulomatosis are not known. We can only speculate about its cause.

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 blood vessel walls.

Wegener’s disease usually manifests throughout the entire body—anywhere blood vessels are present. Consequently, general symptoms such as

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

appear.

In addition, there are symptoms of specific organ involvement.

• Eyes: Inflammation in almost all parts of the eye, so-called “red eye,” visual disturbances (episcleritis, uveitis, exophthalmos, etc.).
• Ear, Nose, and Throat (ENT) area: Bloody chronic inflammation of the nasal mucosa with bloody nasal discharge, followed by destruction of the nasal septum. Chronic middle ear inflammation with hearing loss, sore throats, and salivary gland inflammation that do not respond to standard treatment.
• Trachea: Acute shortness of breath, gasping for air, sometimes risk of suffocation, narrowing of the trachea just below the vocal cords.
• Lungs: Often only mild symptoms, despite frequently observed, very conspicuous pulmonary opacities in the form of round pulmonary foci and pleurisy. Occasionally, pulmonary hemorrhage.
• Joints: Generalized pain in the small joints of the hands and feet without a characteristic pattern of involvement, sometimes accompanied by muscle pain.
• Kidneys:
  • Pain in the renal regions due to swelling of the kidneys within the renal capsule,
  • headaches due to high blood pressure,
  • Swelling around the eyelids due to protein loss and inflammation of the glomeruli (glomerulitis),
  • Nausea, vomiting, and
  • severe malaise upon the onset of kidney failure (uremia)
• Nervous system: Numbness with a tingling sensation in the fingertips and toes (polyneuritis), unsteady gait, weakness due to accompanying muscle inflammation (myositis)
• Skin: Punctate or diffuse discoloration and ulcerations with destruction of large areas of the skin and subcutaneous fat tissue (necrosis); if large blood vessels are affected, e.g., in the fingers and toes (gangrene).

Frequently to always, there is

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

In acute cases of Wegener’s granulomatosis, an increase in the c-ANCA titer can be detected

The underlying mechanisms are usually still unclear at the time of diagnostic testing. Therefore, the physician investigates the organ involvement that is most prominent in each case.

If, for example, a round pulmonary lesion or another type of opacity is noted, a tumor workup is performed due to suspicion of bronchial carcinoma.

Bronchoscopy often yields insufficient material: the pulmonary opacities are located too close to the pleura within the lung tissue, without involvement of the bronchi. In such cases, the only effective option is an open lung biopsy, i.e., opening the chest cavity and performing a targeted tissue sample collection.

If the kidneys are affected, the physician will perform a kidney biopsy; if the skin, muscles, nerves, etc., are involved, a sample will be taken from the respective organ.

The fields of otolaryngology and ophthalmology are of paramount importance. A histological diagnosis is achieved through targeted specialist examination and multiple tissue biopsies. The tissue samples are sent to an experienced pathologist for examination.

It is not uncommon for a considerable amount of time to pass before the final diagnosis is confirmed.

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

Alternatively, lmurek® (3 × 50 mg per day) and other immunosuppressive medications can be used. Cotrim® (1 tablet twice daily) is recommended as an antibiotic that may be particularly effective. If uremia has set in, hemodialysis can help manage kidney failure. Kidney transplants may also be performed at a later stage.

Endoxan® should be administered for several weeks to up to one year in doses ranging from 50 to 150 mg per day. This can lead to significant side effects that are visible in blood tests. These include

• a decrease in white blood cell count and
• the dreaded Endoxan cystitis (a bloody bladder infection that is difficult to treat).

lmurek®, for its part, also causes bone marrow damage with a decrease in white blood cell count. The patient requires general rest and care.

Frequent medical checkups and thorough patient education about the various courses of the disease are important. This helps to identify and treat a flare-up of Wegener’s granulomatosis in a timely manner.

Only 10 to 20 percent of patients treated with Endoxan® experience a relapse. Other immunosuppressive medications have been used. Based on experience with other immunological disorders, such as systemic lupus erythematosus,

• extreme physical exertion,
• pregnancy,
• non-life-threatening surgeries, and similar situations

be avoided. Vaccinations are possible.

With early detection, consistent treatment, and close follow-up care, Wegener’s granulomatosis can usually be managed. In many cases, a cure is even possible.