Minor Salivary Gland Biopsy To Detect Primary Sjogren Syndrome in Patients With Interstitial Lung Disease (original) (raw)
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Quantitative assessment of interstitial lung disease in Sjögren’s syndrome
PLOS ONE
Background Interstitial lung disease (ILD) is a frequent manifestation of Sjö gren's syndrome (SS), an autoimmune disease of salivary and lacrimal glands, and affects approximately 20% of patients. No clinical or serological features appear to be useful to predict its presence, severity or progression, and chest high-resolution computed tomography (CT) remains the gold standard for diagnosis. Semiquantitative CT (SQCT) based on visual assessment (Goh and Taouli scoring) can estimate ILD extent, although it is burdened by relevant intra-and interobserver variability. Quantitative chest CT (QCT) is a promising alternative modality to assess ILD severity. Aim To determine whether QCT assessment can identify extensive or limited lung disease in patients with SS and ILD. Methods This multi-center, cross-sectional and retrospective study enrolled patients with SS and a chest CT scan. SQCT assessment was carried out in a blinded and centralized manner to calculate both Goh and Taouli scores. An operator-independent analysis of all CT scans
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.
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.
Idiopathic interstitial lung disease with anti-SSA antibody
Rheumatology, 2011
Objectives. Consensus is lacking on the immunological tests to perform for diagnosis of interstitial lung diseases (ILDs). In particular, the value of detecting anti-SSA antibody in this context is unknown. We aimed to determine whether the detection of anti-SSA antibody in patients with ILD can identify a subgroup of patients with CTD. Methods. We compared the characteristics of patients with newly diagnosed apparently idiopathic ILD with anti-SSA antibody [anti-SSA(+) group] and without anti-SSA antibody (control group). Results. Anti-SSA(+) patients (n = 15) more often had extra-respiratory signs (xerostomia and ocular dryness), auto-immune features, a CT scan pattern of non-specific interstitial pneumonia and more severe lung function alteration than controls (n = 30). Of patients who were anti-SSA(+), 2 met the criteria for SS and 13 (86%) of 15 met the criteria for the diagnosis of undifferentiated CTD. Conclusions. Our results suggest that identification of anti-SSA antibody in patients with early ILD can reveal a specific subgroup of patients with more ground glass opacity and more severe lung function impairment than those without anti-SSA antibody.
Respiratory Medicine, 2020
Background: Significant overlap may occur between idiopathic interstitial pneumonia (IIP) and connective tissue diseases (CTDs) that do not meet the established classification criteria for any known CTDs (i.e., occult CTD). Performing minor salivary gland biopsy (MSGB) to detect occult primary Sjogren's syndrome (pSS) in IIP patients is not well studied. Methods: Consecutive IIP patients underwent MSGB to determine the prevalence of positive MSGB findings. Furthermore, we characterised the clinical, physiological and serological profiles of the MSGB-positive patients. Cox regression models were used to identify independent predictors of survival. Results: The data of 155 patients with IIP were available for analysis. Sixty patients (38.7%) had positive MSGB findings. Of them, the mean age was 63.3 years, 51.6% were women, usual interstitial pneumonia (UIP) was the predominant pattern (63.3%), and seronegative antibodies (61.6%) were likely. Patients with positive MSGB findings had significantly greater survival than those with negative MSGB findings (p = 0.041). After stratifying the MSGB cohort based on the presence of a UIP pattern, no significant difference in survival was noted between those with positive MSGB-UIP pattern and those with a negative MSGB-UIP pattern (p = 0.231). Multivariate analysis on all UIP patients showed that higher forced vital capacity (p = 0.010) and smoking status (p = 0.035) were independently associated with survival. Conclusions: A substantial number of IIP patients had underlying occult CTD, highlighting the importance of performing MSGB to identify the salivary component of pSS when evaluating patients with interstitial lung disease of undetermined aetiology.
Diagnostic Gain from Surgical Biopsy for Interstitial Lung Disease – When is it Worth the Risk?
Pneumologie, 2016
Background: History, clinical presentation, lung function testing, radiographs including HRCT and nonsurgical biopsy techniques in most cases provide sufficient information for classification of interstitial lung disease (ILD). However, in a small percentage it is not possible to establish the diagnosis so that lung biopsy may be required. We analyzed under which circumstances a reduction of invasive procedures is reasonable. Methods: Between January 1997 and December 2009 we examined 3399 specimens from 1299 patients with benign inflammatory and granulomatous diseases in whom ILD was clinically hypothesized. We compared the probability of disease according to Bayes before and after surgery which corresponds to the clinical diagnosis (a priori probability) and the final diagnosis (a posteriori probability). Additionally, procedures, operation related complications and the patientsʼ smoking habits were documented. Results: In 111 patients (8.5 %) surgical evaluation was performed (14 mediastinoscopies, 97 thoracotomies/VATS biopsies). All mediastinoscopies substantiated a epitheloid cell granulomatosis. In 30 % of all VATS procedures a prolonged air leak of more than 4 days was observed. One patient died and one had to get a new chest tube after removal. Changes of a priori/a posteriori probabilities was shown for non-smokers in Wegnerʼs granulomatosis (0.6 vs. 2.2 %) and IPF (16.7 vs. 34.8 %), for smokers in Langerhansʼ cell histiocytosis (1.4 vs. 7.8 %) and IPF (16.7 vs. 33.3 %). In the majority of cases even a reduction of probability was seen. Conclusion: Considering complications and limited diagnostic gain, lung biopsies for diagnosis of ILD should be recommended only in selected patients. Zusammenfassung ! Hintergrund: Anamnese, Klinik, bildgebende, insbesondere HRCT, und klinisch-bioptische Verfahren ermöglichen die Einordnung der Mehrzahl interstitieller Lungenerkrankungen. Am Ende der klinischen Diagnostik bleibt ein kleiner Teil dieser Erkrankungen dennoch ungeklärt und ist Anlass für eine offene Lungenbiopsie. Ziel der vorliegenden Arbeit war es zu prüfen, unter welchen Voraussetzungen eine Reduktion invasiver diagnostischer Eingriffe möglich ist. Methoden: Zwischen 01/97 und 12/09 wurden 3399 Proben von 1299 Patienten mit benignen entzündlichen und granulomatösen Erkrankungen untersucht, bei denen am Beginn der Untersuchung die klinische Verdachtsdiagnose auf das Vorliegen einer interstitiellen Erkrankung vorlag. Für alle mit diagnostischer Zielsetzung operierten Patienten wurden Art des thoraxchirurgischen Eingriffs, operationsbedingte Komplikationen und Raucheranamnese erfasst. Anschließend berechneten wir die a-priori-und in Abhängigkeit von den ergänzend erfassten Daten die a-posteriori-Wahrscheinlichkeiten für die unterschiedlichen Erkrankungen (Bayes-Theorem). Ergebnisse: Bei 111 Patienten (8,5 %) erfolgte eine operative Diagnostik (14 Mediastinoskopien, 97 Video-assistierte-Thorakoskopien,-tomien). Alle Mediastinoskopien ergaben eine epitheloidzellige Granulomatose. 30 % aller Thorakoskopien gingen mit einer Fistelproblematik > 4 d einher. Ein Patient wurde redrainiert, ein Patient verstarb. Änderungen der a-priori-/a-posteriori-Wahrscheinlichkeiten fanden sich bei den Nichtrauchern für Wegenersche Granulomatose (0,6 vs. 2,2 %) und IPF (16,7 vs. 34,8 %), bei den Rauchern für Histiozytosis X (1,4 vs. 7,8 %) und IPF (16,7 vs. 33,3 %). Bei der Mehrzahl aller anderen interstitieller Erkrankungen reduzierten sich dagegen die a-posteriori-Wahrscheinlichkeiten. This document was downloaded for personal use only. Unauthorized distribution is strictly prohibited.
Diagnostic surgical lung biopsies for suspected interstitial lung diseases: a retrospective study
The Annals of thoracic surgery, 2009
Background. Current guidelines for interstitial lung disease support a surgical biopsy for optimal diagnosis and treatment, yet only a minority of patients undergo such biopsy. Our objectives were to address the properties of a surgical lung biopsy for suspected interstitial lung disease, the diagnostic yield of the procedure, and whether it resulted in changes in diagnosis and treatment.