A 24-year-old woman with Nasal septum erosion

Granulomatosis with polyangiitis (GPA), formerly known as Wegener granulomatosis, is a rare multisystem autoimmune disease of unknown etiology. Classic GPA, as detailed by Godman and Churg in 1954 ,includes the triad of necrotizing granuloma of upper and lower respiratory system, systemic vasculitis, and necrotizing glomerulonephritis. GPA can affect virtually any site in the body, but it has a predisposition for certain organs. The classic organs involved in Granulomatosis with Polyangiitis are the upper respiratory tract (sinuses, nose, ears, and trachea, the lungs, and the kidneys. It is the combination of symptoms, results of physical examinations, laboratory tests, X-rays, and sometimes a biopsy (sample) of affected tissue that together prove the diagnosis of GPA. The sex distribution (male: female) of WG is 3:2 and the peak incidence occurs in patients aged between 50 and 60 years. Treatment includes immunosuppressant therapy, most commonly systemic steroids and cyclophosphamide. Remission rates are approximately 90%, but relapses may occur. Resolution of imaging findings of Wegener s granulomatosis may lag behind clinical improvement.

کلیدواژه ها:

Wegener’s disease ، bronchoalveolar lavage ، cavitation in chest X-ray