A Case of Sjögren's Syndrome with Wegener's Granulomatosis-like Pulmonary Involvement

Abstract

Background: We report a case of Sjögren's syndrome with pulmonary involvement resembling that seen in Wegener's granulomatosis. The patient was a 69-year-old woman who presented with a three-week history of persistent cough and fever. The chest radiograph and CT scans revealed multiple consolidations in the middle and lower lung fields bilaterally.
Methods & Results: Lung biopsy specimens obtained by video-assisted thoracoscopy showed infiltration of the alveoli by lymphocytes with scattered neutrophils, and microscopic vasculitis of the small arteries. The patient tested positive for antineutrophil- cytoplasmic antibodies (ANCA) against myeloperoxidase (MPO-ANCA) and for anti SS-A & SS-B antibodies; however, the test for ANCA against proteinase 3 (PR3-ANCA) was negative.
Conclusions: Based on the presence of the clinical features of the sicca syndrome and positive findings on Schirmer's test and sialoscintigraphy, the patient was given a diagnosis of Sjögren's syndrome with Wegener's granulomatosis-like involvement of the lungs. Neither upper airway nor renal involvement was detected.