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Wegener's granulomatosis (WG) is a rare systemic disease characterized by necrotizing granulomatous inflammation of the upper and lower respiratory tract, glomerulonephritis and vasculitis. It occurs as a limited form or generalized form and usually presents with nonspecific symptoms in its early stages, making the diagnosis of this disease more elusive. Strawberry gingivitis is the most common oral manifestation and is characteristic. Prompt recognition of this early manifestation is of utmost importance for the institution of early treatment, thereby avoiding serious complications. The present paper selectively reviews the literature regarding the current status of WG with respect to diagnosis, laboratory features, and treatment.