| Abstract [eng] |
Granulomatosis with Polyangiitis (formerly known as Wegener’s granulomatosis) is a systemic disease characterized by granulomatous inflammation of the upper and lower respiratory tract with necrotizing vasculitis, predominantly affecting small and medium-sized blood vessels. The disease has a heterogeneous presentation, and early diagnosis and treatment significantly improve patient outcomes. One of the life-threatening manifestations is airway stenosis. Although airway stenosis is relatively rare, it has a tendency to reoccur. Presented clinical case of 26 year old female who was diagnosed with granulomatosis with polyangiitis. The patient presented to the medical facility with fever, shortness of breath, cough, and hemoptysis. She had previously been treated for upper respiratory tract infection, otitis, and pneumonia. Investigations revealed elevated inflammatory markers, positive c-ANCA against PR3, and signs of granulomatous inflammation with necrosis in biopsy samples. Computed tomography revealed multiple bilateral pulmonary lesions. Based on the clinical findings and patient condition, granulomatosis with polyangiitis was confirmed, and treatment with methylprednisolone pulse-therapy was initiated. During the course of treatment, the patient experienced recurrent infectious complications and airway stenosis. Due to active infectious process, it was not possible to administer optimal immunosuppressive therapy. As the disease progressed, immunosuppressive therapy was adjusted – the patient was treated with cyclophosphamide, hydroxychloroquine, and rituximab. During the course of treatment, bronchoscopies were performed and the airways were recanalized on multiple occasions. This case illustrates the complexity of the clinical course of granulomatosis with polyangiitis, the challenges of immunosuppressive treatment, and the therapeutic importance of bronchoscopy. Objective: The aim of this thesis is to present a clinical case of granulomatosis with polyangiitis (Wegener's granulomatosis) and to review scientific literature sources regarding diagnostic and treatment options. |
