Accuracy of two-dimensional transverse wave elastography of salivary glands in diagnosis of Sjögren's disease: a systematic review and meta-analysis.

OBJECTIVES: Two-dimensional shear wave elastography (2D-SWE) is a non-invasive technique for the evaluation of Sjögren's disease (SjD). This study investigated the diagnostic accuracy of 2D-SWE in assessing major salivary gland involvement in SjD. DESIGN: A systematic review and meta-analysis. DATA SOURCES: Data were obtained by searching PubMed, MEDLINE, EMBASE, Scopus, Cochrane library and CNKI from 1999 to 26 September 2022, which includes randomised clinical trial of 2D-SWE for the diagnosis of SjD. ELIGIBILITY CRITERIA: (1) Patients (≥18 years old) diagnosed with SjD following the international classification in 2002 or 2016 American College of Rheumatology-European League Against Rheumatism classification criteria for SjD; (2) The purpose of this study was to evaluate the diagnostic value of 2D-SWE in SjD; (3) The evaluation parameters for the diagnosis of SjD can be extracted or indirectly obtained in this article, including sensitivity, specificity, true positive, false positive, false negative, true negative, diagnostic point (Young's modulus) and other data. DATA EXTRACTION AND SYNTHESIS: Four authors independently screened and assessed the literature and extracted the data. RevMan V.5.3 and StataMP V.18 software were used for quality assessment and meta-analysis. RESULTS: We included 8 studies with a total of 912 cases, including 509 patients with SjD. The high-risk bias in the quality evaluation focused on patient selection and index test. The pooled sensitivity, specificity and summary area under the curve of 2D-SWE were 0.75 (95% CI 0.62 to 0.84), 0.89 (95% CI 0.80 to 0.94) and 0.90 (95% CI 0.87 to 0.92), respectively. CONCLUSIONS: 2D-SWE has an acceptable diagnostic accuracy for SjD patients and is an effective tool for auxiliary diagnosis of SjD. PROSPERO REGISTRATION NUMBER: CRD42022365766.

Performance of salivary glands ultrasonography, shear wave elastography and their combined use for the diagnosis of primary and secondary Sjögren's syndrome.

BACKGROUND: Sjögren's syndrome (SS) is a common rheumatic disease for which finding the right imaging tool remains a challenge. PURPOSE: This study aimed to evaluate the performance of salivary gland ultrasonography (SGUS), shear wave elastography (SWE) and their combined use for the diagnosis of primary and secondary SS (pSS and sSS). METHODS: This retrospective study included patients with dry symptoms who underwent routine examinations between May 2019 and December 2023. Patients were categorized into the pSS (n = 41), sSS (n = 26), and control (n = 27) groups based on the American College of Rheumatology/European League Against Rheumatism classification criteria (2016). A comparison of SGUS and shear wave velocity (SWV) results was conducted among the three groups. The diagnostic capabilities of different ultrasound methods for SS were evaluated using receiver operating characteristic (ROC) curves and the area under the curve (AUC) for specificity. RESULTS: Compared to the control group, both the pSS (1.80 ± 1.03 vs. 0.67 ± 0.48, p < 0.001) and the sSS (1.85 ± 0.88 vs. 0.67 ± 0.48, p < 0.001) groups exhibited significantly elevated SGUS scores. However, there was no statistically significant difference between the pSS and sSS groups (p = 0.849). The SWV values in both the pSS and sSS groups were significantly higher than those in the control group (all p < 0.001). The AUC for diagnosing SS using only SGUS scores was 0.823 (95% confidence interval [CI]: 0.731-0.894). Combining SGUS scores and SWV values resulted in improved diagnostic accuracy (AUC: 0.883, 95% CI: 0.801-0.940). CONCLUSIONS: SGUS and SWE are pivotal in the diagnosis of Sjögren's syndrome, with their synergistic application poised to bolster diagnostic precision. This combined approach also furnishes substantial backing for the clinical assessment and management of Sjögren's syndrome.

Systematic review and meta-analysis of the diagnostic performance of lacrimal gland ultrasound elastography in primary Sjögren's syndrome.

OBJECTIVE: This research conducted a comprehensive evaluation of the effectiveness of ultrasonic elastography (USE) in detecting lacrimal gland involvement in individuals suffering from primary Sjögren's syndrome (pSS). METHODS: A comprehensive search was undertaken across multiple databases including PubMed, the Cochrane Library, EMBASE, Wanfang, Web of Science, and the Chinese National Knowledge Infrastructure, to gather relevant literature pertaining to the application of USE in diagnosing pSS from January 1, 2000, to October 1, 2023. Pooled data were used to calculate sensitivity, specificity, and diagnostic odds ratios. Several summary metrics were used to evaluate SWE's performance in detecting pSS, including the area under the receiver operating characteristic curve, diagnostic odds ratios, sensitivities, and specificities. RESULTS: Five pertinent studies included a total of 273 patients. Shear wave elastography (SWE) demonstrated a pooled sensitivity of 0.88 (95% CI 0.77-0.94) and specificity of 0.94 (95% CI 0.88-0.98), with an area under the receiver operating characteristic curve of 0.97 (95% CI 0.95-0.98). SWE exhibited a positive likelihood ratio of 15.86 (95% CI 6.99-36.00) and a negative likelihood ratio of 0.13 (95% CI 0.07-0.25). No evidence of publication bias was observed (p = 0.70). CONCLUSION: SWE demonstrates a remarkable degree of precision in detecting lacrimal gland involvement in individuals suffering from pSS.

Optical coherence tomography angiography suggests different retinal pathologies in multiple sclerosis and Sjögren's syndrome.

BACKGROUND: While retinal vessel changes are evident in the eyes of patients with relapsing-remitting multiple sclerosis (RRMS), changes in the vasculature of possible MS mimics such as primary Sjögren's syndrome (pSS) remain to be determined. We investigated the potential of retinal optical coherence tomography (OCT) angiography (OCTA) as diagnostic tool to differentiate between patients with RRMS and pSS. METHODS: This cross-sectional study included patients with RRMS (n = 36), pSS (n = 36) and healthy controls (n = 30). Participants underwent clinical examination, assessment of visual acuity, retinal OCT, OCTA, and serum markers of glial and neuronal damage. We investigated the associations between OCTA parameters, visual functions, and serum markers. Eyes with a history of optic neuritis (ON) were excluded from analysis. RESULTS: We observed a significant thinning of the combined ganglion cell and inner plexiform layer in the eyes of patients with RRMS but not with pSS, when compared to healthy controls. Retinal vessel densities of the superficial vascular complex (SVC) were reduced in both patients with RRMS and pSS. However, retinal vessel rarefication of the deep vascular complex (DVC) was only evident in patients with pSS but not RRMS. Using multivariate regression analysis, we found that DVC vessel loss in pSS patients was associated with worse visual acuity. CONCLUSIONS: Compared to patients with RRMS, rarefication of deep retinal vessels is a unique characteristic of pSS and associated with worse visual function. Assuming a disease-specific retinal vessel pathology, these data are indicative of a differential affliction of the gliovascular complex in the retina of RRMS and pSS patients.

Added value of the salivary gland ultrasonography OMERACT score in the ACR/EULAR classification criteria for Sjögren's disease.

OBJECTIVES: To assess whether addition of the salivary gland ultrasonography (SGUS) OMERACT score influences the performance of the 2016 ACR/EULAR classification criteria for Sjögren's disease (SjD) in daily clinical practice. METHODS: Patients visiting the Sjögren Expertise centre in the University Medical Center Groningen for a diagnostic trajectory because of a suspicion of SjD were included. SGUS was performed of both parotid and submandibular glands. ROC analysis was used to assess the accuracy to predict clinical diagnosis of SjD with the SGUS OMERACT score, and by adding the SGUS OMERACT score to the ACR/EULAR criteria. Furthermore, the performance of the SGUS OMERACT and total SGUS Hocevar score were compared. RESULTS: In total, 419 consecutive patients were included. ROC analysis of the highest SGUS OMERACT score out of all four salivary glands (range 0-3) showed good accuracy (AUC 0.849) to predict clinical diagnosis of SjD, comparable to the accuracy of the total SGUS OMERACT score (range 0-12; AUC 0.868) and total Hocevar score (range 0-48; AUC 0.864). When incorporating the highest SGUS OMERACT score (cut-off score of ≥2) as additional item in the ACR/EULAR criteria, accuracy remained excellent (AUC 0.974), and clinical diagnosis could be predicted with a sensitivity of 96.4% and specificity of 86.5%. CONCLUSION: The accuracy of the ACR/EULAR classification criteria for predicting the clinical diagnosis of SjD remained excellent after incorporating the SGUS OMERACT score and extends the diagnostic options in patients suspected with SjD.

In Vivo Human Lacrimal Gland Imaging Using an Ultrasound Biomicroscopy.

PURPOSE: In the present study, we introduce human lacrimal gland imaging using an ultrasound biomicroscopy (UBM) with a soft cover and show their findings. METHODS: The representative UBM findings of palpebral lobes in seven subjects (four with non-Sjögren dry eye syndrome, one with Sjögren syndrome, and two healthy subjects) were described in this study. To prolapse the palpebral lobe, the examiner pulled the temporal part of the upper eyelid in the superotemporal direction and directed the subject to look in the inferonasal direction. We scanned the palpebral lobes longitudinally and transversely using UBM. We used an Aviso UBM with a 50 MHz linear probe and ClearScan. RESULTS: In UBM of two healthy subjects, the echogenicity of the lacrimal gland was lower than that of the sclera and homogeneous. But the parenchyma of a patient with Sjögren dry eye syndrome was quite inhomogeneous compared to the healthy subjects. In two patients with dry eye syndrome, we were able to observe some lobules in the parenchyma. We could find excretory ducts running parallel at the surface of the longitudinal section in some subjects. In the longitudinal UBM scan of a subject, we observed a tubular structure at a depth of 1,500 µm that was considered a blood vessel. It ran from the superonasal to the inferotemporal direction. In a subject, we observed a large cyst beneath the conjunctiva. CONCLUSIONS: Lacrimal gland imaging using UBM has both advantages of optical coherence tomography and sonography, and could be useful for evaluating dry eye syndrome.

Can We Differentiate Between Primary Sjögren Syndrome and Idiopathic Multicentric Castleman Disease Based on the Characteristics of Pulmonary Cysts?

PURPOSE: To identify radiological characteristics that could help differentiate cystic lung diseases between primary Sjögren syndrome (pSS) and idiopathic multicentric Castleman disease (iMCD). PATIENTS AND METHODS: Patients with pSS or iMCD who had cysts were enrolled. Cyst characteristics (number, size, morphology, and distribution) and other accompanying manifestations (nodules, ground-glass opacities, calcification, and thickening of the bronchovascular bundles and interlobular septa) were compared between them. RESULTS: Eleven patients with pSS and 25 patients with iMCD were eligible for our study. Eleven patients with pSS (100.0%) and 23 patients with iMCD (92.0%) had round or oval cysts. None of the patients with pSS had irregular cysts, but 21 (84.0%) patients with iMCD had irregular cysts ( P = 0.005). Smooth-walled cysts were present in 11 patients with pSS (100.0%) and 18 patients with iMCD (72.0%). Only 1 patient with pSS (9.1%) exhibited non-smooth-walled cysts, whereas 23 patients with iMCD (92.0%) had non-smooth-walled cysts ( P = 0.003). The presence of nodules was common in both groups ( P = 1.000). However, the nodules were more likely to be larger and more numerous in patients with iMCD ( P < 0.001). Cysts with mural nodules (52.2%) and central nodules (47.8%) were only observed in iMCD ( P = 0.007). CONCLUSION: Although regular and smooth-walled cysts were common in the 2 diseases, irregular and non-smooth-walled cysts were more often associated with iMCD than pSS. Nodules in iMCD tended to be larger and more numerous, and a close positional relationship between nodules and cysts was only observed in iMCD.

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.

The value of MRI-based radiomics for evaluating early parotid gland injury in primary Sjögren's syndrome.

OBJECTIVE: This study aimed to evaluate the value of machine learning models (ML) based on MRI radiomics in diagnosing early parotid gland injury in primary Sjögren's syndrome (pSS). METHODS: A total of 164 patients (114 in the training cohort and 50 in the testing cohort) with pSS (n=82) or healthy controls (HC) (n=82) were enrolled. Itksnap software was used to perform two-dimensional segmentation of the bilateral parotid glands on T1-weighted (T1WI) and fat-suppressed T2-weighted imaging (fs-T2WI) images. A total of 1548 texture features of the parotid glands were extracted using radiomics software. A radiomics score (Radscore) was constructed and calculated. A t-test was used to compare the Radscore between the two groups. Finally, five machine learning models were trained and tested to identify early pSS parotid injury, and the performance of the machine learning models was evaluated by calculating the acceptance operating curve (ROC) and other parameters. RESULTS: The Radscores between the pSS and HC groups showed significant statistical differences (p<0.001). Among the five machine learning models, the Extra Trees Classifier (ETC) model performed high predictive efficacy in identifying early pSS parotid injury, with an AUC of 0.87 in the testing set. CONCLUSION: MRI radiomics-based machine learning models can effectively diagnose early parotid gland injury in primary Sjögren's syndrome.

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.

Does ultrasound elastography have a role as a diagnostic method for Sjögren's syndrome in the salivary glands? A systematic review.

OBJECTIVE: This systematic review was performed to examine the usefulness of salivary gland ultrasound elastography (USE) as a diagnostic tool for Sjögren's syndrome (SjS). METHODS: Electronic databases (MEDLINE, EMBASE, the Cochrane Library, and Web of Science: Science Citation Index) were searched to identify studies using USE to diagnose SjS from database inception to 15 July 2022. The primary outcome was improved diagnostic accuracy for SjS with the use of USE. Risk of bias and applicability concerns were assessed using the GRADE system, which is continuously developed by the GRADE Working Group. RESULTS: Among 4550 screened studies, 24 full-text articles describing the applications of USE to diagnose SjS were reviewed. The overall risk of bias was determined to be low for 17 of the 24 articles, medium for 5, and high for 2. Articles comparing patients with SjS and healthy subjects reported high diagnostic accuracy of USE, with most results showed statistically significant differences (parotid glands: 15 of the 16 articles, submandibular glands: 11 of the 14 articles). CONCLUSIONS: This systematic review suggests that the assessment of salivary glands using USE is a useful diagnostic tool for SjS.

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.

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.

Lung fibrosis is uncommon in primary Sjögren's disease: A retrospective analysis of computed tomography features in 77 patients.

PURPOSE: The purpose of this study was to describe lung abnormalities observed on computed tomography (CT) in patients meeting the 2016 American College of Rheumatology/European League Against Rheumatism (EULAR) classification criteria for primary Sjögren's disease (pSD). MATERIALS AND METHODS: All patients with pSD seen between January 2009 and December 2020 in the day care centre of our National Reference Center for rare systemic autoimmune diseases, who had at least one chest CT examination available for review and for whom the cumulative EULAR Sjögren's Syndrome Disease Activity Index (cumESSDAI) could be calculated were retrospectively evaluated. CT examinations were reviewed, together with clinical symptoms and pulmonary functional results. RESULTS: Seventy-seven patients (73 women, four men) with a median age of 51 years at pSD diagnosis (age range: 17-79 years), a median follow-up time of 6 years and a median cumESSDAI of 7 were included. Sixty-six patients (86%) had anti-SSA antibodies. Thirty-three patients (33/77; 43%) had respiratory symptoms, without significant alteration in pulmonary function tests. Forty patients (40/77; 52%) had abnormal lung CT findings of whom almost half of them had no respiratory symptoms. Abnormalities on chest CT were more frequently observed in patients with anti-SSA positivity and a history of lymphoma. Air cysts (28/77; 36%) and mosaic perfusion (35/77; 35%) were the predominant abnormalities, whereas lung fibrosis was observed in five patients (5/77; 6%). CONCLUSION: More than half of patients with pSD have abnormal CT findings, mainly air cysts and mosaic perfusion, indicative of small airways disease, whereas lung fibrosis is rare, observed in less than 10% of such patients.

A nomogram based on ultrasound scoring system for differentiating between immunoglobulin G4-related sialadenitis and primary Sjögren syndrome.

OBJECTIVES: Accurate distinguishing between immunoglobulin G4-related sialadenitis (IgG4-RS) and primary Sjögren syndrome (pSS) is crucial due to their different treatment approaches. This study aimed to construct and validate a nomogram based on the ultrasound (US) scoring system for the differentiation of IgG4-RS and pSS. METHODS: A total of 193 patients with a clinical diagnosis of IgG4-RS or pSS treated at our institution were enrolled in the training cohort (n = 135; IgG4-RS = 28, pSS = 107) and the validation cohort (n = 58; IgG4-RS = 15, pSS = 43). The least absolute shrinkage and selection operator regression algorithm was utilized to screen the most optimal clinical features and US scoring parameters. A model for the differential diagnosis of IgG4-RS or pSS was built using logistic regression and visualized as a nomogram. The performance levels of the nomogram model were evaluated and validated in both the training and validation cohorts. RESULTS: The nomogram incorporating clinical features and US scoring parameters showed better predictive value in differentiating IgG4-RS from pSS, with the area under the curves of 0.947 and 0.958 for the training cohort and the validation cohort, respectively. Decision curve analysis demonstrated that the nomogram was clinically useful. CONCLUSIONS: A nomogram based on the US scoring system showed favourable predictive efficacy in differentiating IgG4-RS from pSS. It has the potential to aid in clinical decision-making.

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.

Microvascular status in juvenile Sjögren's disease: the first nailfold videocapillaroscopy investigation.

INTRODUCTION: Juvenile Sjögren's disease (jSjD) is a rare autoimmune disease characterized by exocrine gland involvement and systemic manifestations, including small vessel vasculitis and Raynaud's phenomenon (RP). We aimed to investigate the microvascular status in jSjD patients by nailfold videocapillaroscopy (NVC) and the potential correlations with clinical and serological features. METHODS: Clinical data from thirteen consecutive jSjD patients (11 females and 2 males), with a mean age of 16 ± 4 years, diagnosed before 16 years of age (mean age at diagnosis 12 ± 3) according to the 2016 American College of Rheumatology/EULAR criteria for adult SjD, were collected including age- and sex-matched healthy controls (HCs). Clinical, laboratory, and instrumental data were collected, together with NVC examination. Non-specific and specific NVC parameters were investigated, such as capillary density, capillary dilations, giant capillaries, microhaemorrhages and abnormal shapes. Associations between NVC findings and clinical/serological features were explored and analysed using parametrical and non-parametrical tests. RESULTS: Capillary density reduction correlated significantly with articular involvement (arthralgias) (p = 0.024). Microhaemorrhages correlated with lower C3 levels (p = 0.034). No specific NVC pattern for jSjD was identified, whereas abnormal capillary shapes were significantly higher in jSjD patients than HCs (p = 0.005). NVC abnormalities were not associated with SjD-specific instrumental tests (biopsy, imaging, Schirmer's test). RP was present in 8% of jSjD patients. CONCLUSIONS: The reduction of capillary density, as well as microhaemorrhages at NVC analysis, are significantly associated with some clinical aspects like articular involvement and serum biomarkers (C3 reduction). The NVC is suggested as safe and further analysis in jSjD patients.

Diagnostic Value of Vessel Wall Imaging to Determine the Timing of Extracranial‒Intracranial Bypass for Moyamoya Syndrome Associated with Active Sjögren's Syndrome: A Case Report.

BACKGROUND: Sjögren's syndrome is a chronic autoimmune disorder that predominantly affects exocrine organs. It is characterized by an organ-specific infiltration of lymphocytes. The involvement of the major cerebral arteries in Sjögren's syndrome has rarely been reported. A recent study reported a case of successful extracranial-intracranial (EC-IC) bypass without complications, even in the active inflammatory state, although the optimal timing of such a bypass remains unclear. CASE DESCRIPTION: We here report the case of a 43-year-old woman presenting with acute ischemic stroke due to progressive middle cerebral artery (MCA) occlusion and signs of primary Sjögren's syndrome. During intensive immunosuppressive therapy for active Sjögren's syndrome, the patient was monitored using contrast-enhanced magnetic resonance vessel wall imaging (MR-VWI). A couple of intravenous cyclophosphamide injections combined with a methylprednisolone pulse and antiplatelet therapy resulted in clear resolution of vessel wall enhancement, which suggested remission of inflammatory vasculitis. Nevertheless, she still experienced a transient ischemic attack (TIA) due to decreased regional cerebral blood flow by MCA occlusion, as demonstrated by the conventional time-of-flight MR angiography and single-photon emission computed tomography. Considering the increased risk of further stroke, the decision was made to perform an EC-IC bypass as a treatment for medically uncontrollable hemodynamic impairment. Her postoperative course was uneventful without further repeated TIAs, and continued immunosuppressive therapy for Sjögren's syndrome provided effective management. CONCLUSIONS: Our findings emphasize the diagnostic value of contrast-enhanced MR-VWI in monitoring the effect of immunosuppressive therapy for the major cerebral artery vasculitis and in determining the timing of EC-IC bypass as a "rescue" treatment for moyamoya syndrome associated with active Sjögren's syndrome.

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.