Definition
Granulomatosis with polyangiitis (GPA), also known as Wegener's disease, is a rare autoimmune disease in which the immune system attacks healthy tissue, especially small blood vessels. The pathogenesis is complex and involves an autoimmune reaction against certain proteins, in particular the proteinase 3 antigen (PR3), which leads to inflammation and the formation of granulomas.
Symptoms
The symptoms of GPA are varied and often affect the respiratory tract, lungs, kidneys, joints, muscles and other organs. Patients may suffer from persistent rhinitis, coughing, breathing difficulties, nosebleeds, joint pain and other symptoms. The symptoms vary from person to person and require a differentiated approach.
Diagnosis
The diagnosis of GPA requires a comprehensive examination. Blood tests for specific antibodies (ANCA), as well as imaging procedures such as X-ray, CT or MRI play a decisive role. Confirmation is often provided by a biopsy from the nasal mucosa or affected organs.
Treatment
The therapy focuses on suppressing inflammation and regulating the immune system. Glucocorticoids (steroids) are used in combination with immunosuppressants such as cyclophosphamide or rituximab. New therapeutic approaches and biologics are being intensively researched in order to optimize efficacy and tolerability. The individual choice of medication depends on the severity of the disease and requires precise monitoring.
Monitoring and long-term support
Continuous monitoring of GPA patients is essential in order to detect relapses at an early stage and adapt therapy accordingly. Close interdisciplinary cooperation between rheumatology, nephrology, pneumology and other specialties ensures comprehensive care.
Overall, granulomatosis with polyangiitis requires a multidisciplinary approach. The dialog between physicians and patients is of central importance in order to jointly find the optimal approach in dealing with this complex autoimmune disease.
