Wegener's disease is a granulomatous vasculitis of the small blood vessels with unknown etiology, classified as autoimmune disease.
Wegener's granulomatosis almost always sets out as locally limited disease of the respiratory system. Initially, glomerulonephritis or other systemic manifestations of the illness do not occur.
Typical manifestations of locally limited Wegener's disease include: Rhinits, frequently with chronical development over a long time and bloody mucus, deformation of the nose (saddle-nose, deviation of the septum), spreading of the disease into the paranasal sinuses and the middle ear, and scabby encrustation of the nasal cavity.
Pulmonary nodules, frequently incidental findings, can be observed as pseudo-cavities, possibly being bronchial or subglottal tracheal stenoses, with imaging.
Ulcerations of the oral and pharyngeal mucosa.
With further progression of the disease, generalized symptoms occur.
Glomerulonephritis, often progressing corresponding to the pathological pattern of a rapid progressive glomerulonephritis, is dreaded. Furthermore, the disease can extensively infest the lungs, causing hemorrhage and haemoptysis.
Other possibly affected organic systems include joints, muscles (arthralgia and myalgia), eyes (episcleritis, retinal artery occlusion), and CNS (vasculitis of the cerebral blood vessels). Generaly b-symptoms frequently develop in the systemic stage of illness.
Author: Yale Rosen, M.D., Original file: Flickr file here, Modifications: square crop, License: Creative Commons Share alike 2.0
