Wegener's granulomatosis: a rare presentation.

Wegener's granulomatosis (WG) is a necrotizing granulomatous vasculitis involving the nose, paranasal sinuses, lungs, and kidneys. There are two types of WG-systemic, which is characterized by focal segmental necrotizing glomerulonephritis and limited in which the kidneys are spared. Without proper immunosuppression, WG can be aggressive and often fatal. There are very few reports on WG presenting as parotitis and lacrimal gland involvement. We report a lady who presented recurrent parotitis, focal segmental glomerulosclerosis, and orbital cellulitis, in whom the final diagnosis was revealed after an open lung biopsy.