Therapy
At the beginning of Wegener’s granulomatosis the antibiotic Clotrimazol (broad-spectrum antibiotics with the ingredients: Trimethroprim and Sulfamethoxazole), e.g. available as Cotrim®, which leads to an improvement, although the mode of action is still completely unclear. In the further course of the disease, treatment is usually with cortisone (trade names e.g. Prednisolon®, Prednihexal®, Decortin®). This can be combined with immunosuppressive drugs such as methotrexate (trade names: Lantarel®, Metex®, Neotrexat®) and cyclophosphamide (trade names: Endoxan®, Cytoxan®, Procytox®, Neosyn®).
However, these belong to the group of chemotherapeutic drugs and have corresponding side effects. For some time now, monoclonal antibodies have also been available, which also attenuate the immune system. Infliximab (available e.g. as Remicade®) is such an antibody, it blocks a receptor (TNF-alpha-blocker).
Since the production of such antibodies is very expensive and treatment with such antibodies can lead to severe hypersensitivity reactions, it is only used in patients who do not react to methotrexate. As a reserve drug, there is the possibility to give mycophenolate mofetil (trade name CellCept®) to patients in whom Wegener’s granulomatosis does not improve by administration of cortisone in combination with methotrexate. Plasmapheresis is the treatment of choice for very severe cases of dialysis-related kidney failure or life-threatening pulmonary bleeding.Other drugs used less frequently for therapy are As a maintenance therapy, after improvement of the acute condition, cortisone is prescribed in decreasing doses and an immunosuppressive drug such as Azathioprin® (e.g. Collisan®, Imurek®, Zytrim®).
In addition, antibiotics such as Cotrim® continue to be given to prevent the nasopharyngeal cavity from being colonized and infected by Staphylococcus aureus.
- Etanercept (e.g. Enbrel®) a genetically engineered protein, acts immunosuppressive
- Ciclospoprine A, an immunosuppressive drug
- Leuflunomide (e.g. Arava®) an immunosuppressive drug
- Rituximab (e.g. MabThera®) Monoclonal antibody similar to Infliximab
Prognosis
Without therapy, Wegener’s granulomatosis almost always leads to kidney failure as a result of kidney inflammation within 6 months and thus to death. With targeted therapy, the condition improves significantly in more than 90% of cases. At 3⁄4 of all patients even a temporary freedom from symptoms can be achieved. About half of the patients may experience recurrence of symptoms, which then have to be treated again.