High-resolution CT imaging of the lung for patients with primary Sjögren’s syndrome

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Abstract

Purpose: To assess pulmonary abnormalities in patients with primary Sjögren’s syndrome (PSS) using high-resolution computed tomography (HRCT). Material and methods: The HRCT scans of 24 patients with the diagnosis PSS were retrospectively reviewed regarding the presence, extension and distribution of 16 pathological findings. Results: Nineteen patients (79.2%) showed pathological findings and in five patients (21.8%) the HRCT scan was judged to be normal. A predominance of abnormalities in the lower lobes and subpleural areas was detected. The following pathologies were found: bronchiectasis, thin-walled cysts and small pulmonary nodules (46.2%), ground-glass attenuation and emphysema (37.8%), interlobular-septal thickening (29.4%), honeycombing (25.2%), bronchial wall thickening, tree-in-bud pattern (21.0%), mosaic perfusion (16.8%), architectural distortion (12.6%). Airspace consolidation, air trapping, large nodules (10–30 mm) and masses (>30 mm), mediastinal lymph node enlargement (>15 mm) and free pleural fluid were seen each in 4.2%. In 7 of the 11 patients with thin-walled cysts areas of ground-glass attenuation were detected. Conclusion: HRCT seems is contributive to the characterization of the wide variety of lung abnormalities in PSS. Airway disease alone or in association with the presence of varying degrees of interstitial disease represents the main findings in accordance with earlier reports. Unexpectedly, almost half of the patients had thin-walled cysts on the HRCT scans, which etiology is unclear but could be associated with areas of ground-glass attenuation indicating LIP.

Introduction

Sjögren’s syndrome, known as the triad of dry eyes, dry mouth, and arthritis [1], is a chronic inflammatory autoimmune disorder characterized by infiltration of the exocrine glands by immunglobulin-producing lymphocytes [2]. Affecting mainly women, the prevalence ranges similar to that of rheumatoid arthritis between 1 and 3% [3], [4]. The term primary Sjögren’s syndrome (PSS) indicates the involvement of the lachrymal and salivary glands (sicca syndrome) without any underlying autoimmune condition. If associated with rheumatoid arthritis, systemic lupus erythematosus, scleroderma, or dermatomyositis it is classified as secondary, which comprises one-half to two-thirds of patients with sicca syndrome [5], [6]. Additionally an increased risk of lymphoma in various organs has been reported. PSS has shown a wide spectrum of bronchopulmonary manifestations mainly evaluated on the basis of lung function tests and conventional chest radiography [7], [8], [9], [10]. To our knowledge, a few studies exist assessing lung findings in PSS in a larger population group using high-resolution computed tomography (HRCT) [11], [12], [13], [14], [15], [16], which has shown to be an accurate method for evaluating pulmonary abnormalities in connective tissue disorders [17], [18], [19], [20]. The purpose of this study was to describe the HRCT findings of pulmonary pathologies in PSS.

Section snippets

Study population

The study comprised HRCT examinations of the chest from 24 patients with PSS referred to the Department of Diagnostic Radiology between 1993 and 2003 for further work-up. There were 21 women and 3 men (age range 44–83 years, mean age 63.5 years). All patients fulfilled the European criteria for the diagnosis of PSS [21]. Patients with other associated autoimmune diseases, a history of smoking and with bronchopulmonary abnormalities not necessarily related to PSS were excluded from the study.

CT scanning

Results

Among the 24 patients evaluated 19 showed positive findings, and in five patients the HRCT scan was judged to be normal. In seven patients, up to three findings were present, in six patients there were between four to six abnormalities, and in six patients seven to nine different pathologies were detected. The patient group with up to three presented pathologies did not show a preference to one finding. There was a predominance of abnormalities in the lower lobes and subpleural areas. None of

Discussion

Pulmonary involvement in systemic autoimmune diseases is common, especially in rheumatoid arthritis and systemic sclerosis [19], [23]. In PSS, the pathophysiology of pulmonary pathology is unknown, as is the etiology of the underlying syndrome. The syndrome is characterized by a lymphocytic infiltration, atrophy, and destruction of exocrine glands. Therefore, the lung is a primary target with its abundant mucosal glands, although glandular structures throughout the entire airway system may be

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