Quantitative assessment of interstitial lung disease in Sjögren’s syndrome (original) (raw)
Related papers
Chest, 2009
Purpose: To describe a cohort of patients who presented with interstitial lung disease (ILD) of unknown cause, features of primary Sjö gren syndrome (pSS), and a positive minor salivary gland biopsy (MSGB). Methods: Thirty-eight patients with ILD evaluated at our center underwent an MSGB to confirm a diagnosis of pSS. All of the samples were reviewed by pathologists experienced in the evaluation of salivary gland histology. We defined a positive MSGB finding as a lymphocyte focus score of >1. Results: At presentation, all patients had ILD, and symptoms of cough and dyspnea. None had a definable connective tissue disease (CTD) or known cause for their ILD. Thirteen patients (34%) had positive MSGB findings. Of these, the median age was 61 years (age range, 33 to 75 years); 7 patients (54%) were women; 8 patients (62%) had a smoking history; and 10 patients (77%) had sicca symptoms.
Thin-section chest CT findings of primary Sjögren's syndrome: correlation with pulmonary function
European Radiology, 2002
The purpose of this study was to describe thin-section CT findings of lung involvement in patients with primary Sjögren's syndrome (PSS), and to correlate them with pulmonary function tests (PFT). The chest thin-section CT examinations of 35 patients with proven diagnosis of PSS and respiratory symptoms were retrospectively assessed by two observers, in a first step independently with interobserver evaluation, and in a second step in consensus. The extent of the most frequent CT findings was scored. Correlation was made with PFT in 31 of these patients. Three main CT patterns were identified with good interobserver agreement (kappa coefficient 0.71): 19 of 35 (54%) large and/or small airways disease; 7 of 35 (20%) interstitial lung fibrosis (ILF); and 5 of 35 (14%) suggestive of lymphocytic interstitial pneumonia (LIP). The CT scans were normal in 2 patients (6%) and showed only dilatation of pulmonary vessels due to pulmonary arterial hypertension in two others (6%). Airway disease patients had predominantly obstructive profiles (mean FEV(1)/FVC ratio 69.7+/-12.7%, mean MEF(25) 50.1+/-22.9%), whereas patients with ILF and LIP had predominantly restrictive profiles and/or a decreased diffusing lung capacity (mean TLC 87.0+/-26.0 and 64.6+/-18.6%, mean DL(CO) 57.4+/-21.2 and 52.0+/-8.0%). Significant correlation ( p<0.01) was found between the scores of ground-glass attenuation and TLC ( r=-0.84) and DL(CO) ( r=-0.70) and between the score of air trapping and FEV1 ( r=-1.0). In patients with PSS and respiratory symptoms, thin-section CT may provide characterization of lung involvement which correlates with pulmonary function.
Clinical and Experimental Rheumatology
Pulmonary manifestations, including airway involvement and interstitial lung disease, are the most frequent extra-glandular complications of primary Sjögren's syndrome (pSS). Chest high-resolution computed tomography (HRCT) is a cornerstone of pulmonary diagnostic imaging, aiming to detect, characterise, and quantify such conditions. In patients with pSS-related lung abnormalities, HRCT proved helpful in various clinical scenarios, including baseline and follow-up evaluation, assessment of superimposed infections, suspected progressive interstitial lung diseases, and acute exacerbation. This review aims to provide a primer for rheumatologists on chest HRCT, illustrating the up-to-date technique, imaging findings, and clinical indications in pSS and highlighting the importance of rheumatologist-radiologist constructive collaboration in the clinical management of such patients. Chest HRCT examination State-of-art technique Radiologists should perform chest HRCT according to updated recommendations to make the most of its potential, as highlighted in Table I (8, 19-23). Anatomo-radiologic correlation Chest HRCT interpretation should rely on a basic understanding of pulmonary anatomy (19). In this regard, the sec
Rheumatology, 1998
Eighteen non-smoking women suffering from primary Sjö gren's syndrome (pSS) with previously documented alveolitis were re-examined, clinically and by pulmonary function tests (PFT ), bronchoalveolar lavage (BAL), chest X-ray and high-resolution computed tomography (HRCT ) after a 2 yr follow-up period. Longitudinal evaluation revealed unchanged PFT. The final BAL study showed a normal differential count in six of 14 patients with initial lymphocyte alveolitis, and a persistent alveolar lymphocytosis in the remaining eight patients, associated with an increased percentage of neutrophils in one of them. In four patients with initial mixed alveolitis, the BAL cell profile was unchanged 2 yr later. Five of 18 patients (28%) had abnormal HRCT, represented by isolated septal/subpleural lines in three patients, ground-glass opacities with irregular pleural margins in one patient, and ground-glass opacities associated with septal/subpleural lines in another. All these patients had abnormal BAL results with an increased proportion of both neutrophils and lymphocytes. The presence of alveolar neutrophils was associated with a significantly (P = 0.005) greater mean rate of reduction of carbon monoxide diffusing capacity (DLCO)more than four times the normal rate of loss of DLCO. Chest X-ray, repeated at the end of the 2 yr follow-up period, showed parenchymal abnormalities in only one patient who had evidence of fibrosis on HRCT. This study provides evidence that lung involvement is not an uncommon extraglandular manifestation of pSS and that a BAL neutrophilia may play an important role in the pathogenesis of pulmonary disease in this autoimmune disorder.
Pulmonary Manifestations of Primary Sjögren's Syndrome
American Journal of Respiratory and Critical Care Medicine, 2005
Rationale: Clinicopathologic pulmonary manifestations associated with primary Sjögren's syndrome have yet to be reviewed in a large series since the recognition of nonspecific interstitial pneumonia (NSIP) as a distinct histologic pattern. Objectives: To determine clinical presentations, high-resolution computed tomographic (HRCT) and histologic findings of the lung disease associated with primary Sjögren's syndrome in the light of NSIP, and to analyze prognosis of the disease. Methods: On the basis of 33 cases (31 surgical lung biopsies and 2 autopsies) collected consecutively from multiple centers, we have retrospectively evaluated clinical, radiologic, and pathologic manifestations of the disease. Prognostic factors were identified by univariate and multivariate analysis. Measurements and main results: We found that NSIP was the most frequently seen histologic pattern (20 of 33 cases [61%], 19 fibrosing and 1 cellular). Bronchiolar diseases and amyloid and malignant lymphoma were seen less frequently. HRCT-pathologic correlation resulted in a 94% positive predictive value of CT-NSIP pattern for pathologic diagnosis of NSIP, whereas the diagnostic value of HRCT was low (15%) with an HRCT pattern other than NSIP, data that may influence the decision to biopsy. The 5-year survival rate was 84% overall and 83% in patients with NSIP. Multivariate analysis on all patients showed that low Pa O 2 (p ϭ 0.02) and presence of microscopic honeycombing (p ϭ 0.04) were independently associated with survival. Patients with NSIP showed lower vital capacity (mean Ϯ SD: 68.5 Ϯ 16.6%pred) than patients without NSIP (92.5 Ϯ 18.6%pred; p Ͻ 0.001). Conclusion: Among a diversity of pulmonary lesions in primary Sjögren's syndrome, NSIP was the commonest histologic pattern and had a favorable prognosis.
Respiratory investigation, 2014
The different characteristics of usual interstitial pneumonia in patients with primary Sjögren׳s syndrome (UIP/pSS) compared with idiopathic pulmonary fibrosis (UIP/IPF) are not fully understood. This study aimed to compare characteristics, prognosis, and treatment responses in these patients. Among 129 consecutive patients who underwent surgical lung biopsy to diagnose diffuse lung diseases at Kanagawa Cardiovascular and Respiratory Center between 1998 and 2002, we identified 10 and 19 patients with UIP/pSS and UIP/IPF, respectively. Baseline characteristics, chest high-resolution computed tomography (HRCT) and pathological findings, and the clinical course were compared between the two groups. Responses to immunosuppressive therapy were analyzed by comparing pulmonary function and clinical status before and one year after treatment initiation. More patients in the UIP/pSS group tended to be female and older than those in the UIP/IPF group (mean age, 68 years vs. 62 years). In addi...
Lung involvement in primary Sjogren's syndrome is mainly related to the small airway disease
Annals of the Rheumatic Diseases, 1999
Objective-To evaluate lung involvement in patients with primary Sjögren's syndrome. Methods-Sixty one consecutive, nonsmoking patients, 58 women and three men, were evaluated clinically, physiologically, and radiologically. A bronchial and/or transbronchial biopsy was performed on 13 of the patients. Physiological data were compared with that of a control group of 53 healthy non-smoking subjects matched for age and sex. Results-In 41% of the patients the main symptom was dry cough. Physiological studies revealed that the patients presented significantly lower expiratory flow values (% pred) when compared with those of the control group: the forced expiratory volume in one second (FEV 1) (mean (SD)) was 96% (16) v 111% (13) (p<0.0001), the maximal expiratory flow at the 50% of the vital capacity (MEF 50) was 72% (24) v 103% (17) (p<0.0001), and the maximal expiratory flow at the 25% of the vital capacity (MEF 25) was 49% (25) v 98 % (20) (p<0.0001). No significant diVerence was noted for the carbon monoxide diVusion value (% pred), between patients and controls. Blood gases were evaluated in 44 patients: mild hypoxemia was observed, and the alveolo-arterial oxygen diVerence (P(A-a)O 2) correlated significantly with MEF 50 (r=0.35, p<0.01) and MEF 25 (r=0.33, p<0.01) values. Chest radiography showed mild, interstitial-like changes in 27 patients while slightly increased markings were present in 21. High resolution computed tomography of the lungs was performed in 32 patients (four with a normal chest radiograph, six with suspected interstitial pattern, 19 with apparent interstitial pattern, and three with hyperinflation) and revealed predominantly wall thickening at the segmental bronchi. All positive findings by computed tomography derived from the patients with abnormal chest radiographs. Transbronchial and/or endobronchial biopsy specimens in 10 of the 11 suYcient tissue samples revealed peri-bronchial and/or peribronchiolar mononuclear inflammation, while interstitial inflammation coexisted in two patients. Conclusion-The airway epithelia seem to be the main target of the inflammatory lesion of the lung in patients with primary Sjögren's syndrome. It seems to be common, subclinically leading to obstructive small airway physiological abnormalities.
Diffuse Cystic Lung Disease as the Presenting Manifestation of Sjögren's Syndrome
Annals of the American Thoracic Society, 2016
Interstitial lung diseases, especially lymphoproliferative disorders such as follicular bronchiolitis and lymphoid interstitial pneumonia, are commonly seen in association with Sjögren's syndrome. While the predominant computed tomographic (CT) findings in patients with lymphoid interstitial pneumonia/follicular bronchiolitis include poorly defined centrilobular nodules and ground glass attenuation, cystic changes can be seen in approximately two-thirds of these patients. The objective of this study was to define the clinical, radiological, and histopathological features of cyst-predominant lymphoid interstitial pneumonia/follicular bronchiolitis in patients with Sjögren's syndrome. We present 4 patients who were referred to our institution with diffuse cystic changes on chest CT imaging. All 4 had a presumptive diagnosis of lymphangioleiomyomatosis, but were subsequently found to have Sjögren's syndrome. The was established based on the clinical symptoms of xerostomia a...