Wegener granulomatosis (WG) is a rare disorder of unknown etiology, characterized by necrotizing granulomatous vasculitis. The condition begins in the mucus linings of the nose, throat, or lungs in the form of an inflamed mass. In its most severe form, the necrotizing granulomas affect the blood vessels in the respiratory tract and kidney, causing them to swell up. Other organs that may be affected include eyes, skin, and the joints. Initial symptoms include rhinorrhea, cough, hemoptysis, bloody sputum, saddle-nose deformity due to perforations in the cartilage of the nose, extreme cold sensitivity of the fingers and toes, fatigue, night sweats, malaise, anorexia, weight loss, wheezing, chest pain, and ocular symptoms, including tearing due to nasolacrimal duct obstruction, orbital inflammation, peripheral ulcerative keratitis, scleritis, uveitis, retinal vasculitis, and/or proptosis. Retinal complications appear later, and include retinal phlebitis, vascular occlusions, hemorrhages, acute stenosis, macular edema, and/or retinal pigmentary changes.
WG is much more commonly seen in Caucasians, and is twice as common in men as women. Although this condition can affect people at any age, it is most commonly seen to occur between the ages of 40 and 50. Diagnosis relies on results of blood and urine analysis. Urinalysis detects presence of blood and/or protein in the urine. The blood analysis aims to detect anemia, and more importantly, the presence of anti-neutrophil cytoplasmic antibodies in a cytoplasmic pattern (c-ANCA), which is highly specific of this condition. In addition, biopsy of the affected tissue also aids in the diagnosis. Differential diagnoses to be considered include polyarteritis nodosa, lupus, lymphoma, and kidney disease. Three decades ago, WG used to be considered a fatal disease. However, with recent advances in medication, this disease can be managed by immunosuppressive drugs, and corticosteroids. In severe cases of the disease where the kidney is compromised, transplantation may be required.