Granulomatosis with Polyangiitis. A Clinical Review of the Pulmonary Manifestations of GPA
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Abstract
Clinical review
Granulomatosis with Polyangiitis( GPA) formerly Wegener’s Granulomatosis is a rare multisystem autoimmune disease-causing inflammation of blood cells in the lungs, the sinuses, throat, kidneys, and the nervous system. This review focuses on the pulmonary manifestations of GPA.
People most affected are Caucasians, with age of occurrence 40 to 60ysrs.
Among genetically susceptible individuals, Bacterial and viral infections initiate autoantibody production that interacts with immune cells, adhering to epithelial membranes and causing destruction.
The disease involves the upper respiratory tract in over 90% of cases but the inflammatory process can also involve the rest of the pulmonary system, the orbit, the heart, renal system, and adrenal system.
Patients can present to a variety of clinical specialties.
Management is usually immunosuppression and supportive care