Usual Interstitial Pneumonia Associated With Primary Sjogren's Syndrome

Kadota, Jun‐ichi; Kusano, Shiro; Kawakami, K.; Morikawa, Toru; Kohno, Shigeru

doi:10.1378/chest.108.6.1756

Cited by 24 publications

(5 citation statements)

References 12 publications

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“…In addition, the association between LIP and PSS has also been reported by other investigators [23,24,25]. Furthermore, it has been described that OP [26,27,28], usual interstitial pneumonia (UIP) and NSIP [29,30] can also be associated with PSS.…”

Section: Discussionsupporting

confidence: 64%

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Pulmonary Manifestations of Sjögren’s Syndrome

Shi

Liu²,

et al. 2009

Respiration

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Background: Primary Sjögren’s syndrome (PSS) is associated with various histological patterns of interstitial lung disease. Although chest images and lung function studies showed that lung involvement predominantly occurs in small airways, pathological findings were not consistent with the results of high-resolution CT (HRCT) and lung function tests. Objectives: To investigate the pathological characteristics of PSS-associated interstitial lung disease (PSS-ILD) and their relationship with HRCT lung function tests. Methods: Fourteen patients diagnosed as PSS who underwent surgical lung biopsy in Peking Union Medical College Hospital from October 2000 to October 2006 were reviewed. Histopathologic findings, radiologic findings and lung function tests were analyzed. Results:The study included 13 women. The median age was 46 years. Most patients presented with dyspnea and cough. CT scans revealed bilateral ground-glass, consolidative, reticular and nodular opacities and cyst lesions. The histological patterns included nonspecific interstitial pneumonia (NSIP) cellular pattern associated with organizing pneumonia (OP), NSIP mixed pattern associated with OP, noncaseating granulomas, chronic bronchiolitis, follicular bronchiolitis, constrictive bronchiolitis, lymphocytic interstitial pneumonia associated with follicular bronchiolitis, NSIP mixed pattern associated with follicular bronchiolitis, NSIP mixed pattern coexisting with chronic bronchiolitis, OP associated with chronic bronchiolitis, and noncaseating granulomas coexisting with OP. Treatment included prednisone and cyclophosphamide. During the follow-up period (median 38 months), most patients improved or remained stable. The patient with constrictive bronchiolitis died from progression of primary disease. Conclusions: The histopathologic patterns of PSS-ILD included lung interstitial involvement and small airway involvement or both. Corticosteroid therapy combined with cyclophosphamide was administered with a favorable response in the majority of patients.

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Section: Discussionsupporting

confidence: 64%

“…Surgical lung biopsies were employed in three articles [22,24,29], and TBLB was used in four studies [23,25,26,27]. Specimens were obtained by surgical lung biopsy or TBLB in one study [11], and the methods of obtaining lung biopsy were not mentioned in two articles [5,30].…”

Section: Discussionmentioning

confidence: 99%

Pulmonary Manifestations of Sjögren’s Syndrome

Shi

Liu²,

et al. 2009

Respiration

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“…This syndrome can be associated with another autoimmune disorder such as rheumatoid arthritis, systemic lupus erythematosus or systemic sclerosis, or it can exist in the absence of an underlying condition (primary SS, PSS). The bronchopulmonary manifestations reported in SS are tracheobronchial sicca or xerotrachea [3] and follicular bronchitis/bronchiolitis, sometimes associated with cystic lesions and amyloidosis [4,5,6,7,8], interstitial lung fibrosis (ILF) [4,9,10,11,12], lymphocytic interstitial pneumonia (LIP) [13,14,15] and pulmonary lymphoma [9,10,16,17,18,19]. Less common manifestations include bronchiolitis obliterans with organizing pneumonia [20,21], pulmonary hypertension [22,23] and hyperresponsiveness to metacholine [24].…”

Section: Introductionmentioning

confidence: 99%

Thin-section chest CT findings of primary Sjögren's syndrome: correlation with pulmonary function

et al. 2001

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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.

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“…Gastric involvement was reported in 55% and pulmonary involvement in 9% of patients with primary SS (5,6). Interstitial pneumonia appears to be the most prevalent pulmonary condition in Sjögren's syndrome (7). Upper respiratory tract malignant lymphoma and pulmonary non-Hodgkin's lymphoma were also reported in association with Sjögren's syndrome (8,9).…”

mentioning

confidence: 99%

Frequency and predictive value of the clinical manifestations in Sjögren’s syndrome

Al‐Hashimi

Khuder

Haghighat

et al. 2001

J Oral Pathology Medicine

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The purpose of this study was to examine the frequency and predictive value of glandular and extraglandular manifestations in S ogren's syndrome (SS). The clinical profiles of 169 SS patients were compared to those of 44 non-SS controls. The specific symptoms examined were oral, ocular, vaginal, gastric, pulmonary, skin, joint and muscle pain. Statistical analyses were performed on both individual and grouped symptoms. Chi-squared analyses showed that the frequency of all symptoms was significantly higher among patients than controls. Stepwise discriminant analysis of individual symptoms suggests that the combined symptoms of dry mouth, sore mouth, and dry eyes correctly classified 93% of SS and 97.7% of the controls. While grouped gastric, muscle, psychological, vaginal, skin, nasal, and thyroid symptoms correctly classified 64.3% of SS and 86.1% of the controls. This is the first study to examine the diagnostic value of multi-system manifestation in SS. The overall results suggest that a comprehensive questionnaire of various symptoms may assist the diagnosis of SS. The high predictive value of the combined symptoms confirms their value in the evaluation of SS.

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Usual Interstitial Pneumonia Associated With Primary Sjogren's Syndrome

Cited by 24 publications

References 12 publications

Pulmonary Manifestations of Sjögren’s Syndrome

Pulmonary Manifestations of Sjögren’s Syndrome

Thin-section chest CT findings of primary Sjögren's syndrome: correlation with pulmonary function

Frequency and predictive value of the clinical manifestations in Sjögren’s syndrome

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