Non-invasive imaging for predicting labial salivary gland biopsy outcomes in patients with suspected primary Sjögren syndrome.

To identify the value of salivary gland ultrasound (SGUS) combined with magnetic resonance imaging (MRI) and magnetic resonance sialography (MRS) in predicting the results of labial salivary gland biopsy (LSGB) in patients with suspected primary Sjögren syndrome (pSS), and construct a nomogram model to predict LSGB results. A total of 181 patients who were admitted with suspected pSS from December 2018 to April 2023 were examined and divided into a training set (n = 120) and a validation set (n = 61). Baseline data of the two groups were examined, and the value of SGUS, MRI, and MRS in predicting LSGB was analyzed. Multivariate logistic analysis was used to screen for risk factors, and nomogram prediction models were constructed using these results. In the training set, the SGUS, MRI, and MRS scores of patients in the LSGB + group were higher than those in the LSGB - group (all P < 0.001). The positive prediction value (PPV) was 91% for an SGUS score of 3, and 82% for MRI and MRS scores of 2 or more. We developed a nomogram prediction model based on SGUS, MRI, and MRS data, and it had a concordance index (C-index) of 0.94. The Hosmer-Lemeshow test (χ2 = 3.17, P = 0.92) also indicated the nomogram prediction model had good accuracy and calibration for prediction of LSGB results. A nomogram model based on SGUS, MRI, and MRS results can help rheumatologists decide whether LSGB should be performed in patients with suspected pSS.

Anti-centromere antibody positivity is an independent variable associated with salivary gland ultrasonography score in Sjögren's syndrome.

Sjögren's syndrome (SS) is an autoimmune disease characterized by periductal lymphocytic infiltration of the salivary and lacrimal glands. SS also exhibits extra-glandular manifestations and specific autoantibodies. Salivary gland ultrasonography (SGUS) is a common procedure used to assess the severity of glandular involvement. However, the association between SGUS and extra-glandular lesions remains poorly understood. This study aimed to identify clinical indices, including disease activity, associated with glandular involvement using SGUS in patients with SS. We included 115 patients with SS and 90 without SS. Patients with SS had significantly higher ultrasonography (US) score than patients without SS. Multivariate analysis revealed focus score, Saxon test positivity, and anti-centromere antibody (ACA) positivity as independent variables associated with the US score in patients with SS. In addition, these results were similar to those obtained in patients with primary SS. Patients with SS and ACA positivity had higher US score and an increased prevalence of hyperechoic bands in the parotid glands and submandibular glands. In conclusion, this study indicated that ACA positivity is associated with the US score in patients with SS. These results suggest that US findings in patients with ACA positivity might show specific changes in the salivary glands, especially fibrosis.

Clinical characteristics of patients with Parkinson's disease with reduced 123I-metaiodobenzylguanidine uptake in the major salivary glands and heart.

BACKGROUND: Parkinson's disease (PD) shows cardiac sympathetic denervation (SD) in 123I-metaiodobezylguanidine (MIBG) scintigraphy. Recently, SD in the major salivary glands (MSG-SD) was introduced as a possible radiological feature of PD. OBJECTIVE: To identify the clinical characteristics of patients with PD with reduced MSG and cardiac MIBG uptake (dual-SD) compared with those with reduced MSG or cardiac MIBG uptake only (single-SD). METHODS: We recruited 90 patients with PD and 30 controls and evaluated their non-motor (e.g., hyposmia, autonomic dysfunction) and motor (e.g., Movement Disorder Society-Unified Parkinson's Disease Rating Scale) features. We also assessed MIBG uptake in the MSG and heart using a quantitative semi-automatic method, and compared MIBG uptakes between PD and controls. We set cut-off values for optimal sensitivity and specificity, and compared the clinical characteristics of patients with PD between dual- and single-SD groups. RESULTS: MSG and cardiac MIBG uptakes were significantly reduced in PD. Sixty-one patients had dual-SD, 25 had single-SD, and four had non-SD. In patients with PD with normal cardiac SD, 76.5% (13/17) of whom showed abnormalities only in MSG-SD. When clinical characteristics were compared between the dual-SD and single-/non-SD groups, patients in the dual-SD group were older and had more severe hyposmia and autonomic dysfunction, except motor features. Multiple logistic regression analysis identified age as an important confounder. CONCLUSIONS: Patients with PD with dual-SD have more severe non-motor features than other patients. Autonomic dysfunction might progress independently from dopaminergic degeneration. Furthermore, our findings indicate that aging is a crucial factor in PD progression.

Características operativas de la ecografía en el diagnóstico del Síndrome de Sjögren / Operational characteristics of ultrasound in the diagnosis of Sjögren's syndrome

Antecedentes y objetivo: Determinar las características operativas de la ecografía de glándula salival (EGS) en el diagnóstico del síndrome de Sjögren (SS) en una población de pacientes colombianos con síntomas secos. Materiales y métodos: Estudio de pruebas diagnósticas en pacientes con síntomas secos que asistieron a la consulta de reumatología (2018-2020). Se obtuvieron datos sociodemográficos y clínicos a través de una encuesta, pruebas paraclínicas, oftalmológicas, biopsia de glándula salival menor, flujo salival no estimulado y EGS (puntuación 0-6 basada en De Vita). Se calcularon la sensibilidad, la especificidad y los valores predictivos positivo (VPP) y negativo (VPN) (Stata 15®). Se desarrolló la curva de características operativas del receptor (COR). Resultados: Se incluyó a 102 pacientes (34 con SS y 68 sin SS), edad media ± desviación estándar de 55,69 ± 11,93 años, 94% mujeres. La ecografía positiva (puntuación de 2 o más) fue más frecuente en el grupo de SS, (70,6% vs. 22,1%, p < 0,0001). La sensibilidad fue igual para el grado 2 y 3 (70,59%), con una especificidad mayor (89,71%) para el grado 3 (VPP 77,42% VPN 85,92). La curva COR a partir de la sumatoria de las glándulas por medio de ecografía, fue mejor que las de las glándulas independientes. La curva COR de la ecografía presentó una mayor área bajo la curva (0,72 [0,61-0,82]) que la del análisis histológico (puntuación por focos) (0,68 [0,59-0,78]), p = 0,0252. Conclusión: La EGS es un método útil y confiable para la clasificación del SS. Se podría plantear su uso futuro dentro de los criterios clasificatorios del SS.(AU)

Background and objective: To determine the operational characteristics of salivary gland ultrasound (SGU) in the diagnosis of Sjögren's syndrome (SS) in a population of colombian patients with dry symptoms. Materials and methods: Study of diagnostic tests in patients with dry symptoms who consecutively attended the rheumatology consultation (2018-2020). Sociodemographic and clinical data were obtained through a survey, paraclinical and ophthalmological tests, minor salivary gland biopsy, unstimulated salivary flow and SGU (score 0-6 based on De Vita) were done. Sensitivity, specificity, positive (PPV) and negative (NPV) predictive values (Stata 15®) were calculated. The receiver operating characteristics (ROC) curve was developed. Results: 102 patients were included (34 SS and 68 non-SS), mean age 55.69 (± 11.93) years, 94% women. Positive ultrasound (score of 2 or more) was more frequent in the SS group, (70.6% vs. 22.1%, P<.0001). The sensitivity was the same for grade 2 and 3 (70.59%), with a higher specificity (89.71%) for grade 3 (PPV 77.42% NPV 85.92). The ROC curve from the sum of the glands by means of ultrasound was better than those of the independent glands. The ROC curve of the ultrasound presented a greater area under the curve (0.72 [0.61-0.82]) than that of the histological analysis (focus score) (0.68 [0.59-0.78]), P=.0252. Conclusion: Salivary gland ultrasound is a useful and reliable method for the classification of SS. Its use could be considered in the future within the SS classification criteria.

Operational characteristics of ultrasound in the diagnosis of Sjögren's syndrome.

BACKGROUND AND OBJECTIVE: To determine the operational characteristics of salivary gland ultrasound (SGU) in the diagnosis of Sjögren's syndrome (SS) in a population of colombian patients with dry symptoms. MATERIALS AND METHODS: Study of diagnostic tests in patients with dry symptoms who consecutively attended the rheumatology consultation (2018-2020). Sociodemographic and clinical data were obtained through a survey, paraclinical and ophthalmological tests, minor salivary gland biopsy, unstimulated salivary flow and SGU (score 0-6 based on De Vita) were done. Sensitivity, specificity, positive (PPV) and negative (NPV) predictive values (Stata 15®) were calculated. The receiver operating characteristics (ROC) curve was developed. RESULTS: 102 patients were included (34 SS and 68 non-SS), mean age 55.69 (±11.93) years, 94% women. Positive ultrasound (score of 2 or more) was more frequent in the SS group, (70.6% vs. 22.1%, P<0.0001). The sensitivity was the same for grade 2 and 3 (70.59%), with a higher specificity (89.71%) for grade 3 (PPV 77.42% NPV 85.92). The ROC curve from the sum of the glands by means of ultrasound was better than those of the independent glands. The ROC curve of the ultrasound presented a greater area under the curve (0.72 [0.61-0.82]) than that of the histological analysis (focus score) (0.68 [0.59-0.78]), P=0.0252. CONCLUSION: Salivary gland ultrasound is a useful and reliable method for the classification of SS. Its use could be considered in the future within the SS classification criteria.

Evaluation of major salivary glands with ultrasonography in multiple sclerosis patients.

BACKGROUND: This study aimed to investigate the homogeneity of the major salivary glands in multiple sclerosis (MS) patients using ultrasonography (USG), assess DMFT indices, measure resting salivary flow rates, and compare these values with healthy individuals. METHODS: In this study, 20 individuals diagnosed with Relapsing-Remitting Multiple Sclerosis (RRMS) (mean age 36.15 ± 8.51) and 20 systemically healthy individuals (mean age 35.7 ± 9.22) were included. Oral and radiologic examinations were performed in all individuals. The parotid and submandibular salivary glands were examined using USG, and their homogeneity was assessed based on the scoring system recommended by the Outcome Measures in Rheumatology Clinical Trials (OMERACT) study group. Resting salivary flow rates and DMFT indices were measured, and the obtained data were subjected to statistical analysis. RESULTS: The parotid glands exhibited more heterogeneity on USG within the MS patient group than the control group, with a statistically significant difference between the two groups (p = 0.015). A statistically significant correlation was observed in total homogeneity values between the patient and control groups (p = 0.021). Furthermore, the MS patient group exhibited higher DMFT indices and lower salivary flow rates than the control group. The difference between the DMFT indices and salivary flow rate between the patient groups was statistically significant (p = 0.004 and p = 0.002 respectively). CONCLUSIONS: The parenchyma of the major salivary glands in MS patients exhibited decreased homogeneity than the healthy controls. Additionally, the MS group showed a decrease in salivary flow rate and an increase in the DMFT index. Autonomic dysfunction and medications used for MS are thought to cause salivary gland hypofunction and dry mouth. It can be interpreted that hyposalivation and motor skill losses in MS patients lead to an increase in DMFT index.

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

OBJECTIVES: 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). METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

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.

The Novel OMERACT Ultrasound Scoring System for Salivary Gland Changes in Patients With Sjögren Syndrome Is Associated With MRI and Salivary Flow Rates.

OBJECTIVE: To assess the construct validity of the novel Outcome Measures in Rheumatology (OMERACT) ultrasound (US) semiquantitative scoring system for morphological lesions in major salivary glands by comparing it with magnetic resonance imaging (MRI) and unstimulated whole salivary flow rates (U-WSFRs) in patients with primary Sjögren syndrome (pSS). METHODS: Nine sonographers applied the OMERACT 0-3 grayscale scoring system for parotid (PGs) and submandibular glands (SMGs) in 11 patients with pSS who also had MRIs performed. These were evaluated by 2 radiologists using a semiquantitative 0-3 scoring system for morphological lesions. The agreement between US and MRI and the association between U-WSFRs and imaging structural lesions was determined. A score ≥ 2 for both US and MRI was defined as gland pathology. RESULTS: The prevalence of US morphological lesions in 11 patients with a score ≥ 2 was 58% for PGs and 76% for SMGs, and 46% and 41% for PGs and SMGs, respectively, for MRI. The agreement between OMERACT US scores and MRI scores was 73-91% (median 82%) in the right PG and 73-91% (median 91%) in the left PG, 55-91% (median 55%) in the right SMG and 55-82% (median 55%) in the left SMG. When relations between the presence of hyposalivation and an US score ≥ 2 were examined, agreement was 91-100% (median 83%) in both PGs and 55-91% (median 67%) in both SMGs. CONCLUSION: There is moderate to strong agreement between the OMERACT US and MRI scores for major salivary glands in patients with pSS. Similar agreement ratios were observed between the higher OMERACT US scores and presence of hyposalivation.

The diagnostic performance of salivary gland ultrasound elastography in Sjögren's syndrome and sicca symptoms: a systematic review and meta-analysis.

OBJECTIVE: To systematically evaluate the diagnostic performance of ultrasound elastography (USE) in distinguishing primary Sjögren's syndrome (pSS) from healthy/disease controls. METHODS: We searched the PubMed, Embase, Web of Science, and Cochrane Library databases for published literature on USE for diagnosing pSS. Bivariate random effects models were used to calculate the pooled sensitivity and specificity of USE. To determine the factors influencing heterogeneity, meta-regression and subgroup analyses were performed to assess country, diagnostic criteria, imaging mechanisms, shear wave elastography techniques, measurement location, control group category, and patient age. Publication bias was assessed using the asymmetry of the Deeks funnel plot. RESULTS: Fifteen articles covering 816 patients and 735 control participants were included. USE showed a pooled sensitivity of 0.80 (95% CI: 0.71-0.87) and specificity of 0.87 (95% CI: 0.78-0.92). Meta-regression and subgroup analyses revealed that shear wave elastography techniques, measurement location, and patient age were significant factors that affected study heterogeneity (p < 0.05). Elastography performs better in diagnosing patients aged ≤ 51 years compared to patients aged > 51 years. There was no significant publication bias. CONCLUSION: USE demonstrates high accuracy in differentiating between pSS and healthy/disease control groups. CLINICAL RELEVANCE STATEMENT: Ultrasound elastography, as a non-invasive and cost-effective technique, can be used to distinguish primary Sjögren's syndrome from disease/healthy control groups by measuring the stiffness of salivary glands. KEY POINTS: • Ultrasound elastography is an acceptable technique for the diagnosis of primary Sjögren's syndrome. • The pooled sensitivity and specificity of ultrasound elastography for diagnosing primary Sjögren's syndrome were 0.80 and 0.87, respectively. • In patients aged ≤ 51 years with primary Sjögren's syndrome, ultrasound elastography showed good diagnostic performance.

Value of Quantitative Salivary Gland SPECT/CT for the Detection of Saliva Leakage.

ABSTRACT: A 47-year-old woman presented to our emergency department with a 10-day history of pain, halitosis, and swelling below the left jaw. The patient was diagnosed with left sialadenitis and left submandibular abscess by tissue biopsy. An otolaryngologist performed transcervical incision and drainage of the abscess 1 day after admission. Postoperatively, the patient complained of a sensation of fluid leakage from the mouth, and a continuous purulent discharge was observed. One month postoperatively, a salivary gland scan and SPECT/CT were performed to investigate the sialorrhea and the cause of the discharge. Salivary gland SPECT/CT images localized the saliva leakage site.

INFLUENCE OF THE PATIENT'S SEX AND AGE, VOLUMES OF THE SALIVARY GLAND AND PLEOMORPHIC ADENOMA ON THE TUMOR HISTOLOGICAL VARIANTS.

OBJECTIVE: Aim: The purpose of the study was to identify the influence of the patient's sex and age, the volumes of the salivary gland and pleomorphic adenoma on the histological variants of the tumor. PATIENTS AND METHODS: Materials and Methods: The study included 21 women and 9 men with pleomorphic adenomas of the salivary gland. In 15 cases, a mesenchymal variant of pleomorphic adenoma was identified, in 5 cases - an epithelial variant, in 10 cases - a mixed variant. The average age of the patients was 39.7±2.9 years. Patients with pleomorphic adenomas underwent magnetic resonance imaging using a Siemens MAGNETOM Aera 1.5T device (Germany), during which three projections of the tumor and salivary gland were measured (antero-posterior, lateral, vertical) with subsequent calculation of their volumes, and then the ratios of these volumes. Classification trees were used to determine whether patients belonged to one of three variants of pleomorphic adenoma. The CART (Classification And Regression Tree) algorithm was used as a branching option. Stopping branching was carried out using the FACT (Fact-style direct stopping) method until each terminal (final) node of the tree does not contain incorrectly classified observations or when their number becomes less than a given proportion of the total group size (less than 5%). Determination of the structure and relationships between the tumor variants and such indicators as gender, age and radiological indicators (volume of the tumor, volume of the salivary gland, ratio of tumor volume to salivary gland volume) was carried out using correspondence analysis. RESULTS: Results: Epithelial variant of pleomorphic adenoma is more typical for women, and the mixed and mesenchymal variant is more common for patients of both sexes. The epithelial variant can develop in patients of any age, while the mixed variant occurs mainly in patients older than 41 years, and the mesenchymal variant - mainly in patients younger than 41 years. The ratio of the volume of the tumor to the volume of the salivary gland will be predominantly >0.17 in the epithelial variant of pleomorphic adenoma, predominantly ≤0.17 in the mesenchymal variant, and can take any value in the mixed variant. CONCLUSION: Conclusions: The authors conducted a study in order to identify the influence of sex, age of the patient, and the ratio of the volume of pleomorphic adenoma to the volume of the salivary gland on the histological variant of the tumor. The revealed data will be useful in the treatment and diagnostic process in patients with pleomorphic adenoma of the salivary gland.

Salivary gland 18F-FDG-PET/CT uptake patterns in Sjögren's syndrome and giant cell arteritis patients.

OBJECTIVES: Wide variety in salivary gland 18F-FDG-uptake is observed in the general population. A general consensus about the usefulness of 18F-FDG-PET/CT to detect salivary gland inflammatory conditions, such as in primary Sjögren's syndrome (pSS), is not yet clear. This study aimed to investigate whether there are differences in uptake of 18F-FDG in salivary glands among two autoimmune groups [pSS, giant cell arteritis (GCA)] and a non-autoimmune group (lung cancer). METHODS: PSS patients aged ≥50 years who underwent 18F-FDG-PET/CT were included and age-matched with GCA patients and a non-autoimmune control group (lung cancer patients). Scans were visually evaluated and quantitative analysis was performed by measuring standardised uptake values (SUV) within salivary glands and lacrimal glands. For GCA patients, arteries in the vicinity of the parotid and submandibular gland were assessed for positivity. RESULTS: PSS patients did not show increased 18F-FDG-uptake in the parotid or submandibular gland, compared to the other two groups. For the tubarial gland, significantly higher SUVmax was found in the pSS patient group. Interestingly, GCA patients had significantly higher SUVmax in the submandibular gland than the other two groups. Visual 18F-FDG-positivity of cranial arteries related to the parotid and submandibular glands was associated with significantly higher SUVmax in salivary glands of GCA patients. CONCLUSIONS: Although 18F-FDG-uptake was not increased in parotid and submandibular glands of pSS patients, increased 18F-FDG-uptake in tubarial glands of pSS patients might indicate a role for these glands in pSS. Furthermore, parotid and submandibular glands may be affected by local vasculitis in GCA.

Can ultrasound of the major salivary glands differentiate Sjögren's disease from its major mimics?

OBJECTIVES: Ultrasound of the major salivary glands (SGUS) is widely used to assess the major salivary glands in Sjögren's disease (SjD). Little is known, however, regarding the diagnostic accuracy of SGUS to differentiate SjD from its mimics. This study aims to investigate the diagnostic accuracy of SGUS in differentiating SjD from other diseases with salivary gland involvement. METHODS: SGUS was performed in 20 consecutive patients with SjD and 20 consecutive patients with well-established systemic disease, i.e., with either sarcoidosis, amyloidosis, HIV infection or chronic HCV infection. Images were scored independently by two blinded observers using the Hocevar scoring system. Diagnostic accuracy to discriminate between the patient (sub-)groups was explored. RESULTS: The accuracy of SGUS to differentiate SjD from other systemic diseases was excellent (area under ROC curve of 0.91). The optimal cut-off value to define positive or negative ultrasound for SS was 15. Sensitivity, specificity, positive predictive value and negative predictive value were high, varying from 85-90%, and diagnostic odds ratio was 51. SGUS was positive in the vast majority of SjD patients (n=18), but also in 2 patients with HIV infection and one patient with sarcoidosis. SGUS score differed significantly between patients with SjD and other systemic diseases (median 27 vs. 10, p<0.001) as well as between SjD patients and patients with either sarcoidosis, amyloidosis, HIV or HCV infection (all p<0.05). CONCLUSIONS: This study indicates that SGUS has a potentially high diagnostic accuracy to discriminate SjD from systemic diseases which can also cause salivary gland involvement.

Longitudinal evaluation of major salivary gland functioning in Sjögren's disease patients in a prospective standard-of-care cohort.

OBJECTIVES: To evaluate changes in major salivary gland functioning over time using salivary gland ultrasonography (SGUS), salivary flow measurements (sialometry), and patient-reported outcome measures (PROMs) in patients diagnosed with primary Sjögren's disease (SjD). METHODS: Consecutive outpatients from the ongoing prospective REgistry of Sjögren Syndrome LongiTudinal (RESULT) cohort, all fulfilling the ACR-EULAR classification criteria for SjD, were included. SGUS images assessed with the Hocevar and OMERACT scoring system, unstimulated and stimulated whole saliva (UWS/SWS), unstimulated and stimulated submandibular/sublingual saliva (uSMSLS/sSMSLS) and parotid saliva, EULAR Sjögren's Syndrome Patient Reported Index (ESSPRI) general dryness, oral dryness, and Xerostomia Inventory were assessed at baseline (BL), 2-year (Y2) and 5-year (Y5) follow-up. RESULTS: In total, BL and Y2 data were available for 253 patients and 75 patients had already reached Y5. At group level, SGUS Hocevar (i.e., mean±SD: 22±10 at BL, 22±10 at Y2 and 23±10 at Y5), OMERACT scores, UWS, SWS and PROMs remained stable over time (all p>0.05). Slightly decreased uSMSLS (p=0.025) and sSMSLS (p=0.004) were observed at Y5. At individual patient level, a similar proportion showed an increase or decrease of ≥25% for Hocevar, UWS and SWS. At baseline, poor associations were observed between SGUS and PROMs and fair associations between sialometry and PROMs. Over time, changes in objective assessments did not correlate with changes in PROMs. CONCLUSIONS: Overall, major salivary gland functioning assessed with SGUS, sialometry and PROMs did not change significantly up to 5 years of follow-up in a standard-of-care cohort of SjD patients from daily clinical practice.

Ultrasonographic Evaluation of Salivary Glands for Sjogren's Syndrome: Diagnostic and Monitoring Insights.

Sjogren's syndrome (SS) is a chronic autoimmune condition commonly affecting the exocrine glands, causing oral or ocular dryness and extraglandular manifestations including arthralgia, cytopenia, and lymphoma. The presence of autoantibodies against SSA/Ro, labial salivary gland biopsy, ocular staining, Schirmer's test, or salivary flow assessment are included in the current classification criteria of SS. However, the availability and invasiveness of these procedures limit their widespread use in clinical settings. Salivary gland ultrasonography (SGUS) is a non-invasive imaging modality for the evaluation of the salivary gland parenchyma and is increasingly utilized to aid diagnosis and monitoring in SS. This article presents the protocol of SGUS for image acquisition at the parotid and submandibular glands. The objective is to present a standardized, reproducible, and practical approach to diagnostic SGUS for SS in daily clinical settings. Major salivary glands are scanned in a stepwise approach, beginning at the angle of the mandible for the superficial lobe of the parotid gland, followed by the deep lobe below the ramus of the mandible. Submandibular areas are then scanned for the submandibular glands. The steps in obtaining salivary gland images at each anatomical site are explained in the accompanying video. The echogenicity and echotexture at the thyroid gland are taken as a reference. The homogeneity, the presence and distribution of hypoechoic areas within the glands, and the border of the salivary glands are examined. The sizes and features of intra-/peri-glandular lymph nodes are recorded. The most distinctive sonographic feature in SS is glandular heterogeneity with the presence of hypoechoic/hyperechoic areas within the glands. In summary, while SGUS cannot diagnose SS on its own, it can supplement the current classification criteria of SS and guide the clinical decision for salivary gland biopsy to support the diagnosis of SS in patients with sicca syndrome or suspicious systemic features, combined with autoantibody testing.

[Salivary gland ultrasound or biopsy? : Comparison of methods based on case examples]. / Speicheldrüsenultraschall oder Biopsie? : Methoden im Vergleich anhand von Fallbeispielen.

BACKGROUND: Ultrasound examination of the salivary glands (SG) is a quick and noninvasive method to detect and semiquantitatively estimate typical changes in the large SG in Sjögren's syndrome (SS). The differential diagnosis of SS is difficult because several diseases and adverse effects of treatment have a similar clinical picture as SS with sicca syndrome and can even induce alterations in the SG (mimic diseases). Hence, for a long time an SG biopsy was regarded as the diagnostic procedure of choice, especially in SS­A negative patients, whereas the significance of SD sonography is still controversially discussed. OBJECTIVE: Comparison of typical and atypical changes for SS in the salivary glands in ultrasound and associated histological sections. MATERIAL AND METHODS: This article describes six patient cases with antibody positive or negative SS with and without typical SS ultrasound patterns, SS-associated lymphoma, sarcoidosis and IgG4-associated disease. The findings of the sonographic examination of the parotid glands and the associated histology of the SD are explained and put into context. RESULTS: The SSA antibody positive patients with SS show a typical sonographic pattern with hypoechoic foci, especially if the disease has been present for a long time. This pattern can help support the diagnosis of SS. The ultrasound patterns of the mimic diseases sometimes differ significantly from the typical patterns of pSS. The histological examination of the SG helps to corroborate the diagnosis but low histological focus scores, in particular, require a critical synopsis of the clinical, serological and imaging findings. CONCLUSION: Both salivary gland ultrasound and the histological examination of SG biopsies are justified in the diagnostics and differential diagnosis of SS and sicca syndrome.

A single-center study of clinical features of pediatric Sjögren's syndrome.

OBJECTIVE: Sjögren's syndrome (SS) is a rare disease with unclear diagnostic criteria among the children and adolescents. The purpose of this study is to describe the clinical features of pediatric Sjögren's syndrome and validate with Japanese diagnostic guidelines criteria of 2018. METHODS: We conducted a retrospective analysis of the clinical data of a cohort of 54 patients with pediatric Sjögren's syndrome admitted to our hospital over a total of 10 years from September 2013 to September 2022. RESULTS: The ratio of females to males was 49:5 among the 54 children (34 cases of primary SS and 20 cases of secondary SS), the average age of onset of symptoms for the first time was 9.9 years, and the average age at diagnosis was 10.2 years. In terms of subjective symptoms, 7 cases (13.0%) presented with dry mouth and 5 cases (9.3%) reported dry eyes. The positive rates were 9.3% for Schirmer I test, 70.4% for salivary gland function test, and 55.6% for salivary gland ultrasonography. The positive rates were 94.4% for Anti-Ro/SSA antibodies, 66.7% for Anti-La/SSB antibodies, 88.9% for ANA, 59.3% for RF, and the elevation rate of IgG was 63.0%. Among the EULAR Sjögren's syndrome disease activity index (ESSDAI) domains, the biological, constitutional, glandular, cutaneous, and lymphadenopathy domains were most involved. Treatment consisted of glucocorticoids in 88.9% of the patients in our study and hydroxychloroquine in 92.6%. As per the Japanese version of the clinical practice guidance for Sjögren's Syndrome in pediatric patients (2018), 5 cases were identified as Definite SS, 35 cases as Probable SS, and 14 cases as Possible SS. With respect to primary and secondary SS, there was essentially no significant difference between the groups in any of the above aspects. CONCLUSIONS: Patients with pediatric SS presented with a wide spectrum of clinical features, a low prevalence of reported symptoms of dry mouth and dry eyes, and various clinical manifestations with multi-system involvement. These are similar to other pediatric study cohorts in terms of epidemiology, auxiliary investigation results, disease activity scores, and treatment. The coincidence between our study and the Japanese version of the clinical practice guidance for Sjögren's Syndrome in pediatric patients (2018) is good for the diagnosis of pediatric SS.

Ultrasound abnormalities of the major salivary glands in Egyptian patients with systemic sclerosis.

INTRODUCTION/OBJECTIVES: systemic sclerosis (SSc) is an autoimmune disorder with multiple organs destruction. This study aimed to identify the ultrasonographic changes of major salivary glands in Egyptian scleroderma patients and to detect their association to different disease manifestations. METHODS: Forty-seven SSc patients and 43 apparent healthy volunteers were enrolled. Demographics, inflammatory markers, and autoimmune status were recorded. Ultrasound evaluation of salivary glands was performed. Salivary gland changes' associations were statistically examined with SSc susceptibility and disease manifestations. RESULTS: Thirty-one SSc patients exhibited glandular pathology (p < 0.0001), compared to controls. Of these abnormalities, SSc patients showed a total parotid gray scale of 2, total submandibular gray scale of 2, total glandular gray scale of 4, and total glandular Doppler signal of 1 at p < 0.0001, compared to the control group. Patients with SSc and glandular pathology had a higher prevalence of arthritis (p = 0.029) and ESR (p = 0.002) than those with normal glandular ultrasound. Significant associations were reported between gray scale ultrasound (GSUS) of total parotid (odds ratio "OR" = 0.4), total submandibular (OR = 0.36), and total glandular (OR = 0.53) with susceptibility to SSc at p < 0.0001. Total glandular GSUS (p = 0.039) and total submandibular power Doppler (p = 0.044) correlated with the SSc duration. Total parotid GSUS (p = 0.008) and total glandular GSUS (p < 0.0001) correlated with Schirmer's test. CONCLUSIONS: Major salivary glands are affected in SSc. Hence, scanning these glands with ultrasound is an additive tool besides the current practice. Key Points • Major salivary gland changes, observed by ultrasonography, are new findings in Egyptian SSc patients. • Ultrasound changes of major salivary glands are associated with inflammatory markers and clinical manifestations of SSc. • Scleroderma ultrasonography scans of the main salivary glands could be added to the routine work.

Long-term efficacy of sialendoscopy in treating childhood Sjögren's disease with chronological monitoring by salivary gland ultrasonography: A novel approach.

BACKGROUND: Childhood Sjögren's Disease (cSjD) is an underdiagnosed phenomenon with clinical and pathophysiological nuances in contrast to Sjögren's Disease (SjD) in the adult population. While adults typically experience sicca symptoms, children with cSjD often present with recurrent parotitis, diverse autoantibody profiles, and renal and neurological manifestations. Diagnosis and classification in pediatric rheumatology remain controversial due to the reliance on adult-focused diagnostic criteria and the lack of standardized treatment and understanding of outcomes. The purpose of the paper is to propose a multimodal treatment plan and demonstrate the effectiveness of sialendoscopy in the management of cSjD. CASE PRESENTATION: We present the case of a twelve-year-old female diagnosed with cSjD using the 2016 American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR) diagnostic criteria for SjD. In addition to medical management, she underwent sialendoscopy with triamcinolone irrigation under sedation and was monitored for progress via salivary gland ultrasonography (SGUS). Over the course of one year, she demonstrated significant improvement in symptoms, with serial SGUS scores gradually decreasing by five points. CONCLUSIONS: This paper proposes a multimodal treatment plan involving sialendoscopy and medical management as a non-invasive and potentially more effective approach for cSjD. Standardized monitoring through SGUS scoring allows objective and quantifiable measurement of treatment progress, enabling better assessment of glandular tissue status. Recurrence is possible, and each cSjD patient may present differently. Nevertheless, our year-long observation of a patient with cSjD demonstrates that sialendoscopy, as seen in adults, can promote remission of recurrent parotitis in children as well.