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Morbus Wegener: Causes, Symptoms & Treatment

Morbus Wegener is a rare autoimmune disease that causes inflammation of blood vessels, primarily affecting the airways and kidneys. Early treatment is essential.

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Things worth knowing about "Morbus Wegener"

Morbus Wegener is a rare autoimmune disease that causes inflammation of blood vessels, primarily affecting the airways and kidneys. Early treatment is essential.

What is Morbus Wegener?

Morbus Wegener, now officially known as Granulomatosis with Polyangiitis (GPA), is a rare and serious autoimmune disease. The immune system mistakenly attacks the body's own blood vessels, causing inflammation known as vasculitis. Small and medium-sized blood vessels in the upper and lower respiratory tract and the kidneys are most commonly affected, although virtually any organ can be involved. The condition is named after German physician Friedrich Wegener, who first described it in the 1930s.

Causes

The exact cause of Morbus Wegener is not yet fully understood. It is classified as an autoimmune disease in which the immune system incorrectly targets the body's own tissue. Several contributing factors are being discussed:

Genetic predisposition: Certain genetic traits may increase susceptibility to the disease.
Infections: Bacterial infections, particularly with Staphylococcus aureus, may act as a trigger.
Immunological dysfunction: Overproduction of ANCA (antineutrophil cytoplasmic antibodies) plays a central role in the inflammatory response.
Environmental factors: Exposure to certain chemicals or dust is also under investigation.

Symptoms

The symptoms of Morbus Wegener are diverse and depend on which organs are affected. Common manifestations include:

Upper Respiratory Tract

Chronic runny nose, often with bloody or purulent discharge
Nosebleeds
Blockage or perforation of the nasal septum
Saddle nose deformity (collapsed nasal bridge)
Inflammation of the sinuses (sinusitis)
Middle ear inflammation and hearing loss

Lower Respiratory Tract

Cough, sometimes with coughing up blood (haemoptysis)
Shortness of breath and breathing difficulties
Pneumonia or pulmonary granulomas

Kidneys

Inflammation of the kidney's filtering units (glomerulonephritis)
Blood in the urine (haematuria)
Reduced kidney function, potentially leading to kidney failure

General Symptoms

Fever, fatigue, and general malaise
Unintentional weight loss
Joint and muscle pain
Skin changes such as purpura (skin bleeding)
Eye inflammation (episcleritis, scleritis)

Diagnosis

Diagnosing Morbus Wegener can be challenging, as its symptoms may resemble those of other conditions. The following investigations are commonly used:

Blood tests: Detection of c-ANCA (anti-proteinase-3 antibodies) is highly characteristic of the disease.
Urine analysis: Presence of blood or protein in the urine indicates possible kidney involvement.
Imaging: X-ray and CT scans of the lungs and sinuses to identify granulomas or areas of inflammation.
Biopsy: Tissue samples from affected areas (e.g., nose, lung, kidney) to confirm the diagnosis by detecting granulomas and vasculitis.
Kidney function tests: Creatinine and urea levels in the blood to assess renal function.

Treatment

While Morbus Wegener cannot be cured, it can be effectively managed with consistent medical therapy. The goals are to achieve remission and to prevent relapses.

Induction Therapy (to achieve remission)

Rituximab (a monoclonal antibody) or cyclophosphamide in combination with high-dose corticosteroids (e.g., prednisolone) are the standard treatments for inducing remission.

Maintenance Therapy (to sustain remission)

Rituximab, azathioprine, or methotrexate are used to prevent relapse.
Corticosteroid doses are gradually tapered over time.

Supportive Measures

Antibiotic prophylaxis (e.g., trimethoprim/sulfamethoxazole) to reduce the risk of infections during immunosuppressive therapy.
Regular monitoring of kidney function and ANCA levels.
In cases of severe kidney failure, plasmapheresis (plasma exchange) may be required.

Prognosis

Without treatment, Morbus Wegener can be life-threatening. However, with modern immunosuppressive therapy, most patients are able to achieve stable remission. Relapses are possible and require close medical follow-up. Long-term organ damage, particularly to the kidneys or airways, may occur despite treatment.

References

Jennette JC et al. - 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis & Rheumatism, 2013.
Ntatsaki E et al. - BSR and BHPR guideline for the management of adults with ANCA-associated vasculitis. Rheumatology, 2014.
Falk RJ, Gross WL et al. - Granulomatosis with Polyangiitis (Wegener's): An MRA Disease. New England Journal of Medicine, 2011.

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