Wegeners granulomatosis is a form of vasculitis that affects the lungs, kidneys and other organs. Due to its end-organ damage, it can be a serious disease that requires long-term immunosuppression.[1] It is named after Dr. Friedrich Wegener, who described the disease in 1936.[2] Wegener’s granulomatosis is part of a larger group of vasculitic syndromes, all of which feature the presence of an abnormal type of circulating antibody termed ANCAs (antineutrophil cytoplasmic antibodies) and affect small and medium-size blood vessels. Apart from Wegener’s, this category includes Churg-Strauss syndrome and microscopic polyangiitis.[1] Although Wegener’s granulomatosis affects small and medium-sized vessels,[3] it is formally classified as one of the small vessel vasculitides in the Chapel Hill system Initial signs are extremely variable, and diagnosis can be severely delayed due to the nonspecific nature of the symptoms. Rhinitis is generally the first sign in most patients.[1] Upper airway, eye and ear disease: Nose: pain, stuffiness, nosebleeds, rhinitis, crusting, saddle-nose deformity due to a perforated septum Ears: conductive hearing loss due to auditory tube dysfunction, sensorineural hearing loss (unclear mechanism) Oral cavity: strawberry gingivitis, underlying bone destruction with loosening of teeth, non-specific ulcerations throughout oral mucosa Eyes: pseudotumours, scleritis, conjunctivitis, uveitis, episcleritis Trachea: subglottal stenosis Lungs: pulmonary … Video Rating: 3 / 5