Axial CT with contrast Coronal CT with contrast

Diagnosis: Wegener granulomatosis

Wegener granulomatosis is a systemic necrotizing granulomatous disorder with a destructive angiitis that is most likely autoimmune mediated. It typically presents after the age of 40, more commonly in men. The pulmonary involvement includes alveolar infiltrates and nodular lung masses which may cavitate. Pleural effusion may be seen in 1/4 and adenopathy is extremely rare. The upper respiratory tract may also be involved with destruction of bone and cartilage as well as mucus membrane ulceration. Over 80% of patients have renal disease consisting of a focal glomerulonephritis. Other associations with Wegener granulomatosis include a migratory polyarthropathy, skin lesions, and pericarditis. Lacrimal gland involvement in Wegener granulomatosis is extremely rare. CNS manifestations occur in 1/3 of cases and include CNS vasculitis, and granulomatous lesions of the meninges, brain parenchyma, nasal or paranasal sinuses. Sarcoidosis, Sjogrens syndrome, and lymphoma are all potential causes of bilateral lacrimal gland enlargement. Acute viral lacrimal adenitis more commonly occurs in younger patients. Metastases may cause lacrimal gland enlargement but bilateral involvement is very rare. Adenoid cystic carcinoma and benign mixed tumor of the lacrimal gland may occur; however, these will cause unilateral lacrimal gland enlargement. Biopsy of the lacrimal gland showed findings compatible with Wegener granulomatosis. Related Cases

Graves ophthalmopathy Wegener granulomatosis