Sjogren’s Syndrome with Multiple Bullae and Pulmonary Nodular Amyloidosis

17 Feb
2015

Sjogren’s Syndrome with Multiple Bullae and Pulmonary Nodular AmyloidosisIt is well known that various pulmonary diseases may accompany Sjogren’s syndrome. We report the first case of Sjogren’s syndrome complicated by multiple bullae and pulmonary nodular amyloidosis. asthma inhalers
Case Report
A 53-year-old woman was admitted to our hospital with the chief complaints of cough and exertional dyspnea, which had appeared three years earlier and had steadily progressed. Multiple linear shadows and bullous change were presented on chest roentgenograms six years ago, but at that time, she did not have any pulmonary complaint. She also complained of dryness of the conjunctiva and mouth. The physical examination disclosed only slight crepitations on chest auscultation. Laboratory examination revealed polyclonal hypergammaglobulinemia (29.8 percent) without an M-component in serum or urine. The arterial oxygen pressure was 78.1 mm Hg. Pulmonary function tests indicated obstruction and a decreased diffusing capacity (VC, 2.32 L; FEV„ 1.01 L; FEV./FVC, 45 percent; RV/TLC, 43.8 percent; and Deo, 36 percent of predicted). The results of rose bengal and Schirmer’s tests, sialography, lip biopsy, and anti-SSB antibody testing confirmed the diagnosis of Sjogren’s syndrome.

A chest roentgenogram showed multiple linear shadows and hyperlucency in the lower lung fields (Fig 1). These findings were more prominent on computed tomography (CT). Figure 2 shows multiple cystic lesions of varying size, up to 10 cm, on CT taken three years earlier already demonstrating the presence of bullae, and the size and number were increased. Lymphangiomyomatosis, eosinophilic granuloma, or nodular sclerosis were suspected as the possible cause of the roentgenologic abnormalities.
An open lung biopsy disclosed not only multiple bullae, but also several hard nodules up to 2 cm in diameter in the left lung. Histologic examination of the lung revealed marked lymphocyte and plasma cell infiltration of the bronchiolar wall, accompanied by narrowing of the airway (Fig 3). Destruction of the alveolar wall, indicative of the presence of bullae, was evident in the peripheral regions of the narrowed bronchioles. These findings suggested that stenosis of the airway acted as the check-valve mechanism, and resulted in the formation of bullae. The finding of lymphocyte and plasma cell infiltration into the alveolar wall (lymphocytic interstitial pneumonitis) was not detected.

Figure 1. Chest roentgenogram on admission. Both lung fields are hyperlucent and contain linear shadows, suggesting the presence of multiple bullae.

Figure 1. Chest roentgenogram on admission. Both lung fields are hyperlucent and contain linear shadows, suggesting the presence of multiple bullae.

Figure 2. Chest computed tomography at subcarinal level and lower lobe. Cystic lesions up to 10 cm in diameter are clearly identifiable through the lung.

Figure 2. Chest computed tomography at subcarinal level and lower lobe. Cystic lesions up to 10 cm in diameter are clearly identifiable through the lung.

Figure 3. Open lung biopsy specimen from left lower lobe. Marked inflammatory mononuclear cell infiltration to the bronchiolar wall resulted in the narrowing of the airway The alveolar walls in the distal area of the narrowed bronchiole were destroyed, which led to formation of bullae (hematoxylin eosin stain, original magnification x 33).

Figure 3. Open lung biopsy specimen from left lower lobe. Marked inflammatory mononuclear cell infiltration to the bronchiolar wall resulted in the narrowing of the airway The alveolar walls in the distal area of the narrowed bronchiole were destroyed, which led to formation of bullae (hematoxylin eosin stain, original magnification x 33).

top