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An Unusual Presentation of Wegener’s Granulomatosis

University of Florida, Shands Hospital, Gainesville, FL

INTRODUCTION: Wegener’s Granulomatosis (WG) is a necrotizing small vessel vasculitis predominantly affecting the sinuses, lungs and kidneys. Although the diagnosis is suggested by chronic upper airway infections, WG is a protean disease and can affect any organ in the body (1). This case is an example of WG masquerading as a metastatic lung carcinoma.

CASE PRESENTATION: A 38-year-old male smoker presented with four months of severe right ear pain, right eye ptosis, right facial droop, dysphagia, cough, and hoarseness, with an associated forty pound weight loss. He also noted migratory, episodic swelling of his elbows, wrist, knees and ankles. Symptoms did not improve after a prolonged course of oral antibiotics and low dose corticosteroids. Physical examination: Right facial droop and ptosis, right lateral gaze paralysis. Purulent material in the right external auditory canal. The lungs were clear. Laboratory/Radiographic Findings: Serum sodium of 132. CBC: mild, normocytic anemia. Computed tomography (CT ) of the sinuses and head revealed an infiltrating mass in the right post-styloid parapharyngeal space compressing the facial nerve. CT of the chest: 4.3 x 3.2 cm cavitary lesion in the right lung apex, 1 cm nodule in the left lung apex, enlarged azygos lymph nodes, and lytic and sclerotic changes of the right clavicle. The right adrenal gland was enlarged at 3.5 cm. A diagnostic bronchoscopy revealed normal airways without endobronchial lesions. Histopathology from transbronchial biopsies: peri-vascular caseating granulomas consistent with Wegener’s Granulomatosis. Cytoplasmic-anti-neutrophil cytoplasmic (C-ANCA) and anti-protease 3 (anti- PR 3 Ab) antibodies were positive. The patient was admitted to the hospital and begun on high dose systemic corticosteroids and intravenous cyclophosphamide. He was continued on prednisone and oral cyclophosphamide. Over the next two months, he had resolution of the cranial neuropathies and systemic symptoms.

DISCUSSIONS: Wegener’s Granulomatosis (WG) is a small vessel vasculitis that most commonly affects the airway, upper and lower, and kidneys. It is uncommon, with a reported incidence of three per 100,000 (1). The most common areas of involvement at presentation are the sinuses, lungs, and kidneys. Neurologic symptoms are less common, but have been reported in approximately one third of patients, usually as a sensorineural hearing loss (2). Otolaryngologic symptoms, the most common presenting complaint, are manifested as chronic otitis media (OM) or sinus disease (3). WG manifesting as a skull-based mass is rare, however has been reported (2,4,5). It is presumed a result of extension of disease from the Eustachian tube into the temporal bone (5). WG has rarely been reported to mimic metastatic bronchogenic carcinoma, as in this case (6,7). Diagnosis may be delayed by problematic tissue sampling which relates directly to the organ sampled (1).

CONCLUSION: Wegener’s Granulomatosis may present with misleading radiographic findings. An appropriate index of suspicion and expeditious evaluation can prevent a critical delay in therapy.

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REFERENCES

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