Ultrasonography Of Major Salivary Glands Research Articles

Reliability Exercise of Ultrasound Salivary Glands in Sjögren's Disease: An International Web Training Initiative.

Major salivary gland ultrasonography (SGUS) demonstrated its good metric properties as an outcome measure for diagnosing primary Sjögren's disease (SD). The objective was to assess SGUS reliability among sonographers with different levels of experience, using web training. Sonographers from expert centers participated in the reliability exercise. Before exercises, training was done by videoconferencing. Reliability of the two most experienced sonographers (MES) was assessed and then compared to other sonographers. Intra-reader and inter-reader reliability of SGUS items were assessed by computing Cohen's κ coefficients. All sets were read twice by all 14 sonographers within a 4-month interval. Intra-reader reliability of MES was almost perfect for homogeneity, substantial for Outcome Measures in Rheumatology (OMERACT) scoring system (OMERACTss). Among LES (less experienced sonographers), reliability was moderate to almost perfect for homogeneity, fair to moderate for OMERACTss, and fair to almost perfect for binary OMERACTss. Inter-reader reliability between MES was almost perfect for homogeneity, substantial for diagnosis, moderate for OMERACTss, and substantial for binary OMERACTss. Compared to MES, reliabilities of LES were moderate to almost perfect for both homogeneity and diagnosis, only fair to moderate for OMERACTss, but increased in binary OMERACTss. Videoconferencing training sessions in an international reliability exercise could be an excellent tool to train experienced and less-experienced sonographers. SGUS homogeneity items is useful to distinguish normal from abnormal salivary glands parenchyma independently of diagnosis. Structural damage evaluations by OMERACT scoring system is a new comprehensive score to diagnose patients with SD and could be easily used by sonographers in a binary method.

The Role of Major Salivary Gland Ultrasound in the Diagnostic Workup of Sicca Syndrome: A Large Single-Centre Study.

(1) Objective: To determine the diagnostic accuracy of major salivary gland ultrasonography (SGUS) in primary Sjogren's syndrome (SS), we used the Outcome Measures in Rheumatology Clinical Trials (OMERACT) scoring system on a large single-centre cohort of patients with sicca syndrome. (2) Method: We retrospectively collected the clinical, imaging and serological data of all the patients referred with a suspicion of SS who underwent SGUS and minor salivary glands biopsy. (3) Results: A total of 132 patients were included. The SGUS scores were correlated between the two sides (p < 0.001). The diagnostic cut-off for SS (AUROC: 0.7408) was 6 for the SGUS-global sum (sensitivity: 32.43%; specificity: 96.84%). The cut-off with the highest specificity for SS diagnosis was 7. In the patients with a final diagnosis of SS, the mean SGUS score was significantly higher (p < 0.001) than that of the non-SS patients (3.73 vs. 1.32 for the SGUS-global sum). A significant correlation was demonstrated between the SGUS scores and final SS diagnosis (p < 0.001), biopsy positivity (p < 0.001), ANA positivity (p = 0.016), Ro-SSA positivity (p = 0.01), and gland fibrosis (p = 0.02). (4) Conclusions: SGUS, using the OMERACT scoring system, has moderate sensitivity and high specificity for the diagnosis of SS. The scoring showed a strong and direct correlation with all the clinical hallmarks of SS diagnosis, such as the positivity of a labial salivary gland biopsy, ANA and Ro-SSA statuses, and salivary gland fibrosis. Because of its high specificity, a SGUS-global score > 6 could be therefore employed for the diagnosis of SS in the case of ANA negativity or the unavailability of a biopsy.

Diagnostic accuracy of ultrasonography in relation to salivary gland biopsy in Sjögren's syndrome: a systematic review with meta-analysis.

To evaluate the accuracy of major salivary gland ultrasonography (SGUS) in relation to minor salivary gland biopsy (mSGB) in the diagnosis of Sjögren's syndrome (SS). A systematic review and meta-analysis were performed. Ten databases were searched to identify studies that compared the accuracy of SGUS and mSGB. The risk of bias was assessed, data were extracted, and univariate and bivariate random-effects meta-analyses were done. A total of 5000 records were identified; 13 studies were included in the qualitative synthesis and 10 in the quantitative synthesis. The first meta-analysis found a sensitivity of 0.86 (95% CI: 0.74-0.92) and specificity of 0.87 (95% CI: 0.81-0.92) for the predictive value of SGUS scoring in relation to the result of mSGB. In the second meta-analysis, mSGB showed higher sensitivity and specificity than SGUS. Sensitivity was 0.80 (95% CI: 0.74-0.85) for mSGB and 0.71 (95% CI: 0.58-0.81) for SGUS, and specificity was 0.94 (95% CI: 0.87-0.97) for mSGB and 0.89 (95% CI: 0.82-0.94) for SGUS. The diagnostic accuracy of SGUS was similar to that of mSGB. SGUS is an effective diagnostic test that shows good sensitivity and high specificity, in addition to being a good tool for prognosis and for avoiding unnecessary biopsies. More studies using similar methodologies are needed to assess the accuracy of SGUS in predicting the result of mSGB. Our results will contribute to decision-making for the implementation of SGUS as a diagnostic tool for SS, considering the advantages of this method.

UHFUS: A Valuable Tool in Evaluating Exocrine Gland Abnormalities in Sjögren's Disease.

Sjögren's Disease (SjD) is a chronic autoimmune disorder that affects the salivary and lacrimal glands, leading to xerostomia and xerophthalmia. Ultrasonography of Major Salivary Glands (SGUS) is a well-established tool for the identification of the salivary glands' abnormalities in SjD. Recently, a growing interest has arisen in the assessment of the other exocrine glands with ultrasonography: lacrimal glands (LGUS) and labial salivary glands (LSGUS). The objective of this study is to explore the practical applications of ultra-high frequency ultrasound (UHFUS) in the assessment of lacrimal glands and labial salivary glands. Indeed, UHFUS, with its improved spatial resolution compared to conventional ultrasonography, allows for the evaluation of microscopic structures and has been successfully applied in various medical fields. In lacrimal glands, conventional high-frequency ultrasound (HFUS) can detect characteristic inflammatory changes, atrophic alterations, blood flow patterns, and neoplastic lesions associated with SjD. However, sometimes it is challenging to identify lacrimal glands characteristics, thus making UHFUS a promising tool. Regarding labial salivary glands, limited research is available with conventional HFUS, but UHFUS proves to be a good tool to evaluate glandular inhomogeneity and to guide labial salivary glands biopsy. The comprehensive understanding of organ involvement facilitated by UHFUS may significantly improve the management of SjD patients.

Non-invasive diagnosis of primary Sjögren's syndrome using ultrasonography and transcriptome biomarkers

Diagnosing primary Sjögren's syndrome (pSS) is difficult due to clinical heterogeneity and the absence of non-invasive specific biomarkers. To develop non-invasive pSS diagnosis methods that integrate classic clinical indexes, major salivary gland ultrasonography (SGUS), and gene expression profiles shared by labial gland and peripheral blood, we conducted a study on a cohort of 358 subjects. We identified differentially expressed genes (DEGs) in glands and blood that were enriched in defense response to virus and type I interferon production pathways. Four upregulated DEGs common in glands and blood were identified as hub genes based on the protein-protein interaction networks. A random forest model was trained using features, including SGUS, anti-SSA/Ro60, keratoconjunctivitis sicca tests, and gene expression levels of MX1 and RSAD2. The model achieved comparable pSS diagnosis accuracy to the golden standard method based on labial gland biopsy. Our findings implicate this novel model as a promising diagnosis technique of pSS.

Ultrasonography in the diagnosis of suspected primary Sjögren's syndrome and concordance with salivary gland biopsy: a Spanish single-center study.

The study aims to evaluate the utility of major salivary gland ultrasonography for diagnosis of primary Sjögren's syndrome (pSS) and to assess its concordance with minor salivary gland biopsy (MSGB). A cross-sectional study of 72 patients with suspected pSS was performed. Demographic, clinical, and serological data were collected. MSGB was performed, as was ultrasonography. The ultrasound technician was blind to clinical, serological, and histological data. The validity of ultrasonography compared with MSGB, the American-European Consensus Group (AECG), and American College of Rheumatology (ACR)/European Alliance of Associations for Rheumatology (EULAR) criteria was assessed by calculating the percentage of agreement, sensitivity, specificity, positive and negative predictive values, and area under the curve (AUC). Based on MSGB as the gold standard, the percentage of agreement between both tests was 78% (AUC 0.75). Based on the ACR/EULAR criteria, the percentage of agreement was 83% (AUC 0.78) for ultrasonography and 81% (AUC 0.83) for biopsy. Sensitivity and specificity were 90% and 67%, respectively, for ultrasonography and 76% and 90% for biopsy. The results were similar with the AECG criteria. The intra- and inter-observer variability was good (κ > 0.7). Significant differences were observed for positive anti-Ro52 values and hypergammaglobulinemia in pathological ultrasound scans. Diagnostic ultrasonography is as useful as MSGB in pSS. Therefore, it could be included in the classification criteria. In this cohort, it proved more sensitive than MSGB and could be used as an initial test for patients suspected of having pSS. MSGB could be used in cases where clinical and serological results are inconclusive. Key Points • Major salivary gland ultrasonography adds diagnostic value similar to that of MSGB, thus potentially enabling this invasive procedure to be avoided. • Ultrasonography could be included in the classification criteria for primary Sjögren's syndrome. • Given that ultrasonography is more sensitive and less specific than MSGB, it could be used as an initial diagnostic test in patients with suspected Sjögren's syndrome. • Biopsy should be performed in those cases where ultrasonography, clinical, and serological data are inconclusive.

Diagnostic value of ultrasound evaluation of major salivary glands for Sjögren’s syndrome based on the novel OMERACT scoring system

ObjectiveThe novel Outcome Measures in Rheumatology Clinical Trials (OMERACT) scoring system was used to evaluate the diagnostic effectiveness of major salivary gland ultrasonography (SGUS) for Sjögren's syndrome (SS). Materials and methodsSGUS was performed on the parotid glands (PGs) and submandibular glands of 242 patients (145 patients with SS and 97 patients without SS) using the OMERACT scoring system (grade 0–3). We also evaluated the association of SGUS scores with unstimulated whole salivary flow rate (UWSF), stimulated whole salivary flow rate (SWSF), and labial salivary gland biopsy (LSGB) results. ResultsThe SGUS scores were significantly higher for the SS group than for the non-SS group (p < 0.001). Using a cutoff value of 8 for the total score yielded the highest sensitivity (76%), specificity (90%), and area under the receiver operating characteristic curve (AUC, 0.828). The correlation between SGUS scores and salivary gland function was moderate to good. A cutoff value of 10 for the total score was more effective in predicting SWSF outcomes than UWSF outcomes (sensitivity: 73% > 58%, specificity: 98% > 87%, and AUC: 0.856 > 0.723). The association of OMERACT scores with LSGB results was fair to moderate. Among 61 anti-SSA-negative patients, 17 had positive PG scores (including ten SS and seven non-SS patients), and 44 had negative PG scores (including 37 non-SS and seven SS patients). ConclusionThe OMERACT scoring system had good sensitivity and excellent specificity, demonstrating excellent diagnostic potential for SS and efficacy in assessing salivary gland function. Negative SGUS results may help reduce unnecessary biopsies in anti-SSA-negative patients.

AB0574 PREDICTOR FACTORS IN EMPOWERMENT OF PATIENTS WITH PRIMARY SJOGREN SYNDROME IN THE FRAME OF VALUE BASED HEALTH CARE

BackgroundPatient empowerment is a key component in developing awareness of the disease in patient-centred care in the setting of chronic disease management.ObjectivesThis study aimed to evaluate predictive factors in the empowerment of patients with Sjögren’s Syndrome (pSS), while examining patients reported outcome measures (PROMs) in the setting of value-based health care.MethodsIn this cross-sectional study, 169 patients with pSS (F/M: 162/7; mean age: 52.94±12.20 years) were included. Data was collected from the clinical examination, major salivary gland ultrasonography (SGUS) and a structured questionnaire regarding utilisation of health services and PROMs regarding Oral Health Impact Profile-14 (OHIP-14), Hospital Anxiety and Depression Scale (HADS; HADS-Anxiety and HADS-Depression) and Self-reported General Health Status (100-m VAS; 0: very poor vs 10: very good). Both stimulated (S-SFRs) and unstimulated salivary flow rates (U-SFRs) of patients were measured as ml/min. Patients with hyposalivation were grouped as according to U-SFR (≤0.1 ml/min). Furthermore, Outcome Measures from Rheumatology ultrasonography (OMERACT US) scores (from 0: normal to 3 points: severe inhomogeneity) were collected to evaluate glandular involvement of patients. After initial statistical analyses was carried out, mediation analysis was performed to evaluate the associations.ResultsThe rate of patients with hyposalivation was found as 45% and most (85.7%) had elevated SGUS scores (≥2 points). In addition, self-reported oral health problems were common in the study group (from 23.8% to 53.4%). Among this cohort, the patients suffered from Burning oral sensation (27 %) had poor scores in OHIP-14 (29.81±14.48 vs 20.22±12.43), HADS-Depression (10.07±4.49 vs 6.65±4.20) and Self-reported general health status (45.43±17.95 vs 55.56±22.43) compared to those without Burning oral sensation (p=0.006, p=0.019, p=0.018, respectively). Furthermore, significant correlations were observed between OHIP-14 score and HADS-A score (r: 0.4 p: 0.004) and HADS-D scores (r: 0.58 p=0.000). Utilising mediation analysis, the HADS-Depression score was directly mediated by the presence of Burning oral sensation (p=0.0005) and indirectly mediated by OHIP-14 score (p=0.0360). In this group, the interval from the last dental visit was mean: 19 months. Interestingly, 60% preferred to relate their oral discomfort during visits (3 times a year), to rheumatologists.ConclusionBetter health outcomes could be achieved by reducing oral discomfort, increasing dental visits, improvement of oral health related quality of life and managing depression by a multidisciplinary team with dentists and psychiatrists in patient-centred care. Since these factors have a significant effect on patients’ daily life, treatment plans are needed to provide patient empowerment by using suitable strategies in the frame of value-based health care.Disclosure of InterestsNone declared

Salivary gland ultrasonography in primary Sj\xf6gren\u2019s syndrome from diagnosis to clinical stratification: a multicentre study

BackgroundTo determine the diagnostic accuracy of major salivary gland ultrasonography (SGUS) in primary Sjögren’s syndrome (pSS) using the novel Outcome Measures in Rheumatology Clinical Trials (OMERACT) scoring system in a large-scale multicentre study.MethodsSGUS was conducted for 246 pSS patients, 140 control subjects with conditions other than SS and 27 healthy control subjects. The echostructure features from the parotid and submandibular glands on both sides were graded using the novel OMERACT scoring system. Receiver operating characteristic curves were used to describe the diagnostic accuracy of the scoring system for pSS. The associations between the SGUS and disease characteristics were analysed to evaluate the clinical value of SGUS for pSS.ResultsThe US scores in the pSS group were significantly higher than those in the non-pSS group (p < 0.001). The level of diagnostic accuracy was comparable with the scores of all four glands (AUC=0.908) when only the parotid and submandibular glands on either side were scored (AUC=0.910, 0.904, respectively). The optimal cut-off value for the left (right) parotid gland and the left (right) submandibular gland was 4, with maximal sensitivity (75.6% and 77.2%, respectively) and specificity (91.6% and 92.2%, respectively). The pSS patients with positive SGUS results presented a longer disease duration, parotid enlargement, dental loss and higher levels of serological markers, such as anti-SSA, anti-SSB, positive RF, IgG and γ-globulin%.ConclusionsSGUS with the OMERACT scoring system yields high sensitivity and specificity, demonstrating high diagnostic feasibility for pSS. The SGUS may have implications for deciding disease severity and treatment efficacy.

Performance Evaluation of Multiple Ultrasonographical Methods for the Detection of Primary Sjögren's Syndrome.

Major salivary gland ultrasonography (SGUS) is increasingly being recognized as having critical roles in differentiating primary Sjögren’s syndrome (pSS) from other connective tissue disorders. Contrast-enhanced ultrasonography (CEUS) has been reported to evaluate microvascularity of lesions in different tissues with objective angiographic index, eliminating the observer-dependent defect of ultrasonography. However, there are few relevant studies concentrating on the application of CEUS in the diagnosis and assessment for pSS, and their clinical utility prospect remains uncertain. In this study, a total of 227 eligible patients were enrolled, including 161 pSS and 66 non-pSS patients with comprehensive ultrasonographic evaluation of the parotid and submandibular glands, including grayscale ultrasonography, color Doppler sonography (CDS), and CEUS. Compared with non-pSS, pSS patients had significantly higher grayscale ultrasound (US) scores and CDS blood grades in the parotid gland and significantly higher grayscale US and CEUS scores in the submandibular glands. Diagnostic model combining ultrasonographic signatures, anti-SSA/Ro60, and keratoconjunctivitis sicca (KCS) tests showed a remarkable discrimination [mean area under the curve (AUC)0.963 in submandibular glands and 0.934 in parotid glands] for pSS, and the nomogram provided excellent prediction accuracy and good calibration in individualized prediction of pSS. A combination of multiple ultrasonographical examinations of the major salivary glands (SGs) is a promising technique that may be used as a practical alternative to minor SG biopsy in the detection of pSS.

Sonographic findings of immunoglobulin G4-related sialadenitis and differences from Sjögren’s syndrome

Objective To evaluate ultrasonic features of the major salivary glands in patients with immunoglobulin G4-related sialadenitis (IgG4-RS) and to explore the differences between IgG4-RS and Sjögren’s syndrome (SS). Method We conducted the study in 150 patients with IgG4-RS and 100 patients with SS. Ultrasonographic variables of the static images of major salivary glands were analysed. An experienced radiologist scored the confidence rating regarding the presence of the characteristic imaging findings using a five-grade rating system. Ultrasonography scores between IgG4-RS and SS were compared. Results The major salivary glands were significantly larger in patients with IgG4-RS than in the SS group. The main features of ultrasonography of the salivary glands in IgG4-RS were various hypoechoic lesions and increased colour Doppler signalling. In contrast, the major salivary glands in SS exhibited hyperechoic lines and/or spots and obscuration of the gland configuration. The scores of the summarized sonographic characteristics also showed statistically significant differences between the IgG4-RS and SS groups. Conclusion This study revealed different ultrasonic features of the major salivary glands in patients with IgG4-RS and SS. The scored sonographic features were helpful in differentiating IgG4-RS from SS. Consequently, we suggest that ultrasonography of major salivary glands could be a useful imaging procedure in the evaluation of patients suspected of having IgG4-RS.

Rheumatological evaluation of patients with interstitial lung disease

Objective: Interstitial lung disease (ILD) is a common feature of connective tissue disease (CTD). The diagnosis of CTD-ILD can be challenging and is important for therapeutic decisions. In this study, we aimed to determine whether a systematic rheumatological assessment could help pulmonologists in the diagnosis and care of ILD patients. Method: We conducted an observational single-centre study of patients with ILD. All patients underwent standardized pulmonary and rheumatological evaluations, including clinical evaluation (pulmonary symptoms and musculoskeletal signs), immunological screening, chest high-resolution computed tomography, pulmonary function tests, and ultrasonography (US) of joints and major salivary glands. Results: We included 100 consecutive ILD patients (47% women, mean ± sd age 67 ± 14 years); 15 patients already had CTD. The main extrapulmonary symptoms were joint pain (n = 52), joint swelling (n = 26), and sicca syndrome (n = 33). US of joints revealed synovitis, bone erosion, and tenosynovitis in 37, 17, and 13 patients, respectively. US of major salivary glands detected features associated with Sjögren’s syndrome in 13 patients. After rheumatological evaluation, CTD-ILD was confidently diagnosed in 39 patients; diseases were mainly rheumatoid arthritis (n = 20), primary Sjögren’s syndrome (n = 17), and inflammatory myopathies (n = 7). The diagnosis of CTD-ILD was associated with the presence of musculoskeletal symptoms and immunological and US abnormalities. The CTD diagnosis led to a therapeutic change in 21 patients. Conclusion: Our findings suggest that musculoskeletal symptoms are frequent in ILD patients, which supports multidisciplinary management, involving the rheumatologist, for evaluating patients with ILD.

Effectiveness of ultrasonography and shear wave sonoelastography in Sjögren syndrome with salivary gland involvement.

PurposeThis study investigated the effectiveness of major salivary gland ultrasonography (MSGUS) using a new grading system and shear wave elastography (SWE) in evaluating the major salivary glands of patients with Sjögren syndrome (SjS).MethodsThis prospectively-designed cross-sectional study included 49 SjS patients and 49 healthy controls. Major salivary glands were examined with gray-scale ultrasonography and SWE. A new grading system for salivary glands was developed using MSGUS findings. The sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of MSGUS and shear wave velocity (SWV) values were investigated. The MSGUS grading system and SWV values were evaluated together by logistic regression analysis. A cutoff value of SWE for salivary glands was determined.ResultsThe sensitivity, specificity, PPV, and NPV of MSGUS were 69.4%, 73.5%, 72.3%, and 70.6% for the submandibular gland and 69.4%, 65.3%, 66.7%, and 68.1% for the parotid gland, respectively. The mean SWV values of the parotid and submandibular glands were significantly higher in SjS patients than in controls (P<0.05). The sensitivity, specificity, PPV, and NPV for the submandibular gland (cutoff, 1.95 m/s) and the parotid gland (cutoff, 2.39 m/s) were 69.4%, 52%, 59.1%, and 63% and 82.7%, 83.7%, 83.5%, and 82.8%, respectively. Adding SWE to the parotid gland grading system increased the sensitivity and specificity (sensitivity, 82.7%; specificity, 83.7%).ConclusionEvaluating the salivary glands using MSGUS with a new grading system and SWE may contribute to the diagnosis of SjS. The combination of MSGUS and SWE may be a promising tool for diagnosing SjS.

Salivary Glands and Periodontal Changes in a Population of Sjögren's and Sicca Syndrome Treated by Pilocarpine: A Pilot Study.

IntroductionOral administration of pilocarpine enhances salivary flow in sicca patients but its effect upstream on ultrasound (US) of salivary glands (SG) and downstream on periodontium remain unknown. MethodsSicca patients were prospectively included. Echostructural and vascularization of SG were assessed using B mode and pulsed Doppler (USPD). Vascularization of SG was measured using resistive index (RI) before and after stimulation by lemon juice. Echostructure (measure of glandular length in cm2, evaluation of parotid and submandibular glands parenchymal abnormalities) was assessed at baseline (M0) and after 3 months (M3) of treatment with pilocarpine. A dental consultation was performed at M0 and M3 to evaluate changes in unstimulated salivary flow (USSF), stimulated salivary flow (SSF), and periodontal parameters such as modified gingival index (Lobene), plaque index (Silness), bleeding index, pocket depth, and pH. ResultsNineteen patients were included but only 11 received pilocarpine treatment for 3 months, as six stopped pilocarpine due to side effects and two were excluded for other causes. Among the 11 patients who completed the 3-month follow-up, five had primary Sjögren’s syndrome according to the American-European’s classification criteria. As expected, statistical differences were found concerning SSF (p = 0.018) and USSF (p = 0.027) between M0 and M3 while no statistical change in both SG echostructure and vascularization or periodontal evaluation was shown.ConclusionsPilocarpine improved SSF and USSF measurements in sicca syndrome but no ultrasonography of major salivary glands (SGUS) structural and vascular changes were detected as well as periodontal evaluation.Supplementary InformationThe online version contains supplementary material available at 10.1007/s40744-020-00263-y.

FRI0153 ULTRA HIGH-RESOLUTION ULTRASOUND (UHFUS) OF LABIAL SALIVARY GLANDS: POTENTIAL APPLICATIONS IN PRIMARY SJÖGREN’S SYNDROME

Background:Major salivary gland ultrasonography has an established role in diagnosis and assessment of pSS. Nowadays, however, interest is also growing in last-generation ultra high resolution ultrasound (UHFUS) transducers, which can produce frequencies up to 70 MHz and achieve tissue resolution up to 30 μm, opening up new possibilities for the study labial salivary glands (LSG).Objectives:To investigate the usefulness of UHFUS in LSG ultrasound-guided biopsy and preoperative planning.Methods:Consecutive patients undergoing a LSG for clinically suspected pSS were included in this study from January 2018 to December 2019. UHFUS of LSG was performed by using VEVO MD, equipped with a 70 MHz probe, scanning first the central compartment of the inferior lip, and then both peripheral compartments. The following parameters were evaluated: distribution of the glands, parenchymal inhomogeneity (score 0-3, from normal to evident), and fibrosis. UHFUS imaging was used to help locate the LSG for the US-guided biopsy. The same expert pathologist calculated the surface area of gland sections examined, the LSG focus score (FS), the number of foci and evaluated the presence of ectopic germinal centers (GCs). Consecutive patients that had undergone a traditional LSG biopsy from December 2016 to December 2017 were included as controls.Results:We included a total of 249 patients with suspected pSS: 137 undergoing the UHFUS-guided LSGs and 112 the traditional LSG biopsy procedure. No demographic differences were observed between the two groups. No differences were also observed in the distribution of the final diagnosis. A diagnosis of pSS according the ACR 2016 criteria was made in 60/137 (43.8%) and 36/112 (32.1%) patients, respectively whereas a diagnosis of no-SS sicca was made in 44/137 (32.1%) and in 43/112 (38.4%) patients; the remaining diagnosis included secondary SS (4/137, 3% and 9/112, 8%) and undifferentiated connective tissue disease (UCTD) (29/137, 21.2%, and 24/112, 21.4%). With respect to no-SS sicca controls and UCTD patients, pSS patients presented higher UHFUS inhomogeneity scores in both central and peripheral labial compartments (p=0.001). There were no complications from the HUFUS-guided LSG biopsy. The mean glandular surface area obtained was significantly higher than the area obtained by traditional LSG biopsy procedure (7.4 ±4.0 mm2vs 6.3±3.7 mm2, p=0.02) thus facilitating the assessment of the FS. Interestingly, the latter showed a good correlation with the UHFUS inhomogeneity (r=0.509**, p=0.000).Conclusion:UHFUS of LSG appeared feasible and sensitive in pSS, potentially offering unique advantages in LSG ultrasound-guided biopsy.Disclosure of Interests:None declared

AB0414 ESSPRI COMPONENTS AND SALIVARY FLOW RATE ARE RELATED TO DAILY ACTIVITY IMPAIRMENT IN PATIENTS WITH PRIMARY SJÖGREN’S SYNDROME

Background:Sjögren’s syndrome (SjS) is a chronic systemic autoimmune disease targets primarily the salivary and lacrimal glands, the severe dryness of the mouth and eyes are common manifestations in patients. Therefore, daily life could be affected by these manifestations in patients with SjS.Objectives:The aim of the study was to assess associations among daily activity impairment and scores of EULAR Sjögren’s Syndrome Patient Reported Index (ESSPRI) and major salivary gland ultrasonography (SGUS) in primary SjS.Methods:In this cross-sectional study, 41 patients with primary SjS (F/M:39/2; mean age: 52,1±10,5) were included. The mean disease duration was 9.5±6,6 years in the group.Data were collected by clinical examinations and a questionnaire regarding two patients reported outcome measures (PROMs). Firstly, Work Productivity and Activity Impairment (WPAI) questionnaire assessed paid and unpaid work during the last seven days. Scores of WPAI subgroups as absenteeism, presenteeism, overall work impairment as well as daily activity impairment were calculated by using 6 items. Secondly, dryness, fatigue and pain in ESSPRI scale were evaluated by visual analogue scale (VAS; 0-10 points) in SjS. High scores in both PROMs indicates that disease manifestations affect patient ‘life poorly.In addition, structural damage of parotid and submandibular salivary glands were examined by using Milic and Hocevar USG scoring methods. Unstimulated whole salivary flow rate (U-WSFR; as ml/min) were also used to interpret the functional status of major salivary glands. High SGUS score and low U-WSFR reflects that disease activity affects major glands poorly.Results:Daily activity impairment was calculated as 63,9±31,1 in patients with primary SjS. High scores in ESSPRI-dryness, ESSPRI-fatigue and ESSPRI-pain were also observed in the group (7,5±2,4; 6,4±2,8 and 6,1±3,1, respectively). Daily activity impairment was correlated with scores of ESSPRI-dryness (r:0,55 p=0.000), ESSPRI-fatigue (r:0,38 p=0.014) and ESSPRI-pain (r:0,56 p=0.000) as well as parenchymal inhomogeneity USG scores of right and left parotid glands (r:0,49 p=0.032; r:0,51 p=0.025).U-WSFR (0.20±0.20 ml/min) was moderately correlated with parenchymal inhomogeneity USG scores of major salivary glands (p&lt;0.05). ESSPRI-dryness score was significantly higher in patients with low U-WSFRs (≤ 0.1 ml/min) than the others (87,5±16,3 vs 68,3±25,1, respectively)(p=0.021).Conclusion:Firstly, subgroup scores of ESSPRI and low U-WSFR associated to daily activity impairment in patients with primary SjS. Secondly, parenchymal inhomogeneity scores of both parotid glands could give an important clue to clinicians for the disease-related damage. Finally, WPAI with 6-item could be thought as an useful tool to understand the effect of the disease manifestations on patients’ daily life.Disclosure of Interests: :None declared

Juvenile Sjögren's Syndrome: Clinical Characteristics With Focus on Salivary Gland Ultrasonography.

ObjectiveJuvenile Sjögren's syndrome (SS) is a rare, poorly defined, and possibly underdiagnosed condition affecting children and adolescents. The aim of this study was to characterize symptoms and clinical findings of juvenile SS and to explore the clinical application of major salivary gland ultrasonography (SGUS) in patients with juvenile SS.MethodsA cross‐sectional multicenter study recruited patients with disease onset until age 18 years (n = 67). Disease characteristics were recorded, and unstimulated whole sialometry and SGUS examination of the parotid and submandibular salivary glands were performed.ResultsThe female:male ratio was 58:9. The mean age at first symptom was 10.2 years and 12.1 years at diagnosis. Ocular and oral symptoms were noted in 42 of 67 patients (63%) and 53 of 66 patients (80%), respectively. The American‐European Consensus Group or American College of Rheumatology/European League Against Rheumatism classification criteria for primary SS were fulfilled by 42 of 67 patients (63%). Pathologic SGUS findings were observed in 41 of 67 patients (61%); 26 of 41 SGUS+ patients (63%) fulfilled primary SS criteria. Salivary gland enlargements/parotitis were noted in 37 of 58 patients and were nonsignificantly associated with SGUS+ status (P = 0.066). The mean levels of saliva were 5.6 ml/15 minutes in SGUS– patients compared to 3.3 ml/15 minutes in the SGUS+ patients (P = 0.049). A total of 36 of 41 SGUS+ patients (88%) were anti‐Ro/La+ compared to 14 of 26 SGUS– patients (54%) (P = 0.001). In addition, 24 of 39 SGUS+ patients (62%) were positive for rheumatoid factor (RF), whereas only 5 of 25 SGUS– patients (20%) were RF+ (P = 0.001).ConclusionJuvenile SS is characterized by a large spectrum of clinical symptoms and findings. Several glandular and extraglandular parameters such as hyposalivation, swollen salivary glands, and autoantibodies are associated with pathologic SGUS findings.

Diagnostic value of major salivary gland ultrasonography in primary Sjögren's syndrome: the role of grey-scale and colour/power Doppler sonography.

Primary Sjögren's syndrome (pSS) is an autoimmune connective tissue disease characterized primarily by chronic inflammatory involvement of the exocrine glands, particularly the salivary glands. The use of ultrasound in the study of salivary glands (SGUS) has expanded considerably in recent years. The ultrasound can document structural alterations that can be visualized as hyperechogenic and hypoechogenic areas, or as areas with non-homogeneous echogenicity. To date, several systems of SGUS scoring systems of abnormalities during pSS are available. From the studies published in recent decades, it has been possible to document the high sensitivity and specificity of the pathological findings that can be documented by SGUS. SGUS can also provide added value in identifying patients at risk for developing disease complications such as lymphoma. The Doppler technique can also supply information about glandular tissue vascularization, which is very useful for diagnostic and differential purposes. In this review we will present the state of the art of SGUS, with a prevailing focus on diagnostic use and possible future developments.

Salivary gland ultrasonography findings are associated with clinical, histological, and serologic features of Sjögren’s syndrome

Objective: Salivary gland ultrasonography (SGUS) has been applied in the diagnosis of Sjögren’s syndrome (SS). The aim of this study is to investigate the association of SGUS findings with clinical, histological, and serologic features of SS.Methods: A total of 104 patients with suspected SS underwent SGUS for evaluation of salivary gland involvement. Patients with primary SS were determined according to the classification criteria for SS. The parenchymal inhomogeneity of bilateral parotid and submandibular glands was graded from 0 (homogeneity) to 4 (gross inhomogeneity). Receiver operating characteristic curve analysis was performed to compare the diagnostic performance of different SGUS scoring methods. Clinical and serologic features were compared between groups classified by SGUS score. The association between SGUS and these features of SS was explored by multivariable linear regression analysis.Results: Study participants were predominantly women (96.2%) and had a mean age of 54.1 years. Eighty-seven patients and 88 patients with primary SS were identified based on AECG criteria and ACR/EULAR classification criteria for SS, respectively. Among the different scoring methods, the sum of the grades of four salivary glands (range 0–16) had the best diagnostic performance, with sensitivity of 77.3% and specificity of 87.5% (cutoff value, 7) for distinguishing primary SS from sicca non-SS. SGUS score was associated with focus score in labial salivary gland biopsy (β = 0.240, p = 0.033) and anti-Ro/SSA serology (β = 0.283, p = 0.016) and inversely associated with unstimulated whole salivary flow (β = −0.298, p = 0.011).Conclusion: Ultrasonography of major salivary glands is associated with histopathology of minor salivary glands, serology of SS, and salivary gland function.

Ultrasonography of major salivary glands compared with parotid and labial gland biopsy and classification criteria in patients with clinically suspected primary Sjögren’s syndrome

ObjectiveTo assess the validity of ultrasound of major salivary glands (sUS) compared with parotid and labial gland biopsies, sialometry, anti-SSA/Ro antibody status and classification criteria in patients clinically suspected with...