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Pathophysiology: Wegener's granulomatosis is a systemic autoimmune disease characterized by granulomatous vasculitis of the upper and lower respiratory tracts, glomerulonephritis and small vessel vasculitis. 90% of patients have pulmonary involvement. As the affected vessels are occluded, stroke developed with subsequent radiographic manifestations of ill-defined multiple bilateral opacities and delimit as myocardial is organized.
Symptoms: Signs and symptoms of granulomatosis with polyangiitis may develop suddenly or over several months. The first warning signs usually involve areas of your respiratory tract, such as your sinuses, throat or lungs. The condition of people with this disease often worsens rapidly, affecting blood vessels and the organs they supply, such as the kidneys.
Diagnosis: Whenever possible, it is important to confirm the diagnosis of Wegener’s by biopsying an involved organ and finding the pathologic features of this disease under the microscope. Because many diseases may mimic Wegener’s (and vice versa), before starting a treatment regimen it is essential to be as certain of the diagnosis as possible.
Treatment
Corticosteroids: Medications such as prednisone help suppress the immune system and treat the early signs and symptoms of Wegener's granulomatosis.
Immune suppressant medications: Most people require another immunosuppressive drug, such as cyclophosphamide, azathioprine or methotrexate, to counteract the body's immune reaction.