Gland Involvement Research Articles

Diagnostic performance of gray-scale ultrasound and shear wave elastography in assessing salivary gland involvement in patients with primary Sjögren's syndrome.

To investigate the diagnostic performance of gray-scale ultrasound (US) and shear wave elastography (SWE) for determining salivary gland involvement primary Sjögren's syndrome (pSS). In this prospective study, the salivary glands of 72 healthy volunteers and 74 participants with pSS were examined by two blinded radiologists with consensus using gray-scale US and SWE. SWE parameters were compared between groups. The area under the curve (AUC), sensitivity, and specificity of gray-scale US and SWE was analyzed. The correlation between SWE and clinical findings was investigated. The SWE parameters of the parotid and submandibular glands were significantly higher in the pSS group, but did not differ significantly based on serologic assays, Schirmer test, minor salivary gland biopsy, and comorbidities. The AUC values for gray-scale US of the salivary glands were significantly lower than the AUC values for SWE. The elasticity modulus (kPa) of parotid gland had the highest AUC value (0.937; 95% CI, 0.901-0.973), with a sensitivity of 93.2% and a specificity of 83.3%. SWE had no correlation with age, disease duration, laboratory values, or disease activity. SWE provides excellent diagnostic performance for submandibular and parotid gland involvement in pSS and can be used to complement gray-scale US.

Distinct impact of malignancy and allergy on the clinical and immunological features of IgG4-related disease.

To clarify the clinical and immunological characteristics of IgG4-RD based on the underlying diseases. Consecutive patients with IgG4-RD treated at Keio University Hospital between 2010 and 2021 were divided according to the presence of malignancy or allergy into three groups. The clinical characteristics and 56 immune cell subsets in the peripheral blood were compared among the groups. Among 123 patients, 18 (14.6%) had malignancy including 4 with allergy (malignancy group), 57 (46.3%) had allergy alone (allergy group), and 48 (39.0%) had neither (idiopathic group). In the malignancy group, the patients were older (70.1 vs. 54.4 vs. 64.9 years, p<0.001), male-dominant (83.3 vs. 42.1 vs. 54.2%, p=0.008), and had smoking habits (77.8 vs. 42.1 vs. 43.8%, p=0.02). They also had significant involvement of the aorta/large vessels (33.3 vs. 7.0 vs. 20.8%, p=0.02), while the patients in the allergy group tended to have orbital/lacrimal gland involvement. Remission and relapse rates were not different between the groups; however, overall survival was significantly poorer in the malignancy group (p=0.02). Comprehensive immunophenotyping of the peripheral blood revealed that the increase in CXCR5+CD2-double negative T cells and the decrease in naive CD8 T cells were characteristic of the malignancy group. The clinical and immunological phenotypes of IgG4-RD differ among those with underlying diseases.

The role of virus infections in Sjögren’s syndrome

Primary Sjögren’s syndrome (pSS) is an autoimmune disease with a clinical picture of not only mainly exocrine gland involvement, with dryness symptoms, but also internal organ and systems involvement. The epithelial damage and releasing of antigens, which, in some circumstances, become autoantigens, underlay the pathogenesis of pSS. The activation of autoimmune processes in pSS leads to the hyperactivation of B cells with autoantibody production and other immunological phenomena such as hypergammaglobulinemia, production of cryoglobulins, or formation of extra-nodal lymphoid tissue. Among the risk factors for the development of this disease are viral infections, which themselves can activate autoimmune reactions and influence the host’s immune response. It is known that viruses, through various mechanisms, can influence the immune system and initiate autoimmune reactions. These mechanisms include molecular mimicry, bystander activation, production of superantigens—proteins encoded by viruses—or a programming to produce viral cytokines similar to host cytokines such as, e.g., interleukin-10. Of particular importance for pSS are viruses which not only, as expected, activate the interferon pathway but also play a particular role, directly or indirectly, in B cell activation or present tropism to organs also targeted in the course of pSS. This article is an attempt to present the current knowledge of the influence specific viruses have on the development and course of pSS.

Frontal Fibrosing Alopecia and Sunscreen Use: A Cross-sectional Study of Actinic Damage.

Patients with frontal fibrosing alopecia report higher rates of sunscreen use than control subjects. However, it is not known whether the higher use of sunscreens is a cause or a consequence of the alopecia. A greater use of sunscreens should be associated with a lower incidence of signs of actinic damage. The aim of this study is to assess the presence of actinic damage in patients with frontal fibrosing alopecia. A cross-sectional study was carried out on 101 patients with frontal fibrosing alopecia and 40 control subjects. The presence of actinic damage, in the form of solar lentigines, actinic keratoses, and basal and squamous cell carcinomas, was recorded in both groups, together with sunscreen use. Trichoscopy and skin biopsy were performed on patients. Actinic damage was present more frequently in patients with frontal fibrosing alopecia (69.3%) than in control subjects (50%) (p = 0.031). Patients used sunscreens more frequently than did control subjects (83.2% vs 62.5%, p = 0.008). However, the prevalence of trichoscopic inflammatory signs, peripheral alopecia, and inflammatory infiltrate and sebaceous gland involvement in skin biopsy, were similar in patients who used sunscreens and those who did not use them. In conclusion, patients with frontal fibrosing alopecia had greater actinic damage than did control subjects, and this is hypothesized as a reason for the higher use of sunscreens among patients. Thus, use of sunscreens may not be the trigger for frontal fibrosing alopecia that dermatologists have proposed.

Tubarial gland involvement in IgG4-related diseases

Background Tubarial glands (TGs) are a collection of unidentified salivary glands overlying the torus tubarius in the nasopharyngeal wall. Immunoglobulin G4-related disease (IgG4-RD) is a chronic fibroinflammatory state that often has multiple organ involvement. Involvement of the head and neck, especially the salivary glands, is common in IgG4-RD. Aims/objectives This study aimed to elucidate the clinical significance of TGs in IgG4-RD. Materials and methods We investigated the local findings of TGs in ten patients with IgG4-RD. Results Nasopharyngeal endoscopic examination revealed oedematous swelling of the nasopharyngeal wall surrounding the TGs, which improved after steroid treatment. Moreover, sonotubometry showed a stenotic pattern in three out of seven patients with IgG4-RD. Conclusions and significance TGs may be involved in IgG4-RD. The swollen TGs may be responsible for obstructive Eustachian tube dysfunction. Further studies are required to clarify the clinical significance and physiological roles of TGs in IgG4-RD.

Immunoglobulin G4-related endocrine diseases

Immunoglobulin G4-related disease has become the focus of interest in recent years. The disease is characterized by inflammation of the organs involved, often with a macroscopic appearance suggestive of a tumor, elevated immuno-globulin G4 levels, immunoglobulin G4-positive plasma cell infiltration on histological examination, fibrosis, oblit-erative phlebitis, and typically a rapid therapeutic response to corticosteroids. The disease can show a variety of organ manifestations, with frequent involvement of exocrine glands. Among the endocrine organs, symptoms may appear in the thyroid gland and the pituitary gland. The criteria for immunoglobulin G4-related hypophysitis were formu-lated in 2011. Until a few years ago, a condition formerly known as Riedel's thyroiditis was identified as immuno-globulin G4-related thyroiditis. Based on the criteria system for immunoglobulin G4-related thyroid diseases pub-lished in 2021, some patients with Hashimoto's thyroiditis and Graves' disease can also be classified as immunoglobulin G4-related thyroid disease. The identification of immunoglobulin G4-related endocrine diseases and the establishment of an accurate diagnosis can modify the treatment of the patient and determine the course of the disease. Other organ manifestations should be sought in patients with immunoglobulin G4-related endocrine disease and lifelong immunological follow-up is warranted.

Symptom Outcomes After Sialendoscopy-Assisted Salivary Duct Surgery: A Prospective 6-Year Study.

The objective is to evaluate the long-term impact of sialendoscopic-assisted salivary duct surgery (SASDS) on sialadenitis symptoms using the Chronic Obstructive Sialadenitis Symptoms (COSS) questionnaire. The COSS questionnaire, which scores symptoms on a 0-100 scale, was administered prospectively to adult patients pre-operatively, 1-year,and 6-years post-operatively. We examined COSS scores and categories representing complete (<10), partial (10-25), and no (>25) resolution of symptoms with attention to factors significantly associated with incomplete resolution (≥10). Approximately 6-years after SASDS, 111 patients reported scores for 128 symptomatic glands (72 with sialolithiasis, 56 without sialolithiasis). For glands with sialolithiasis, the median COSS score pre-SASDS was 27.5 (interquartile range [IQR]: 13.5-43), which was significantly reduced to 1.0 (IQR: 0-5.5) at 1-year and 1.5 (IQR 0-5) at 6-years postoperatively. Glands without sialolithiasis had a median COSS score of 40.5 (IQR: 23-52.5) preoperatively, that significantly reduced to 13.5 (IQR 5-21) at 1-year and 14 (IQR 6.5-25.5) at 6-years post-operatively. There was no significant difference in scores from 1- to 6-years. Intraoperative presence of sialolith, absence of stenosis, stenosis in the distal duct, and submandibular gland involvement were significant predictors of complete resolution of symptoms at 6-years. Approximately 6-years after SASDS for sialadenitis, the majority of patients have durable symptom improvement. The sialolithiasis group had higher rates of complete resolution compared to the non-sialolithiasis group. Presence of ductal stenosis on sialendoscopy, non-distal stenoses, and parotid gland involvement were risk factors for persistent long-term sialadenitis symptoms. Laryngoscope, 133:792-800, 2023.

Subtyping primary aldosteronism by inconclusive adrenal vein sampling: A derivation and validation study in a tertiary centre.

Indices based on aldosterone/cortisol (A/C) concentration in the successfully cannulated adrenal vein (AV) and in the inferior vena cava (IVC) (AV/IVC) appear to be possible markers to verify the subtype of primary aldosteronism (PA) in the case of inconclusive results of adrenal vein sampling (AVS). The variability of results in previous studies encouraged us to calculate AV/IVC and adrenal A/C cutoff values that could predict the aetiology of PA. This retrospective study included 96 patients who underwent AVS due to PA between 2015 and 2020. The derivation cohort ultimately consisted of 60 patients with bilaterally successful AVS and a clear diagnosis of unilateral or bilateral disease. Receiver operating characteristic analysis was used to find the optimal A/C and AV/IVC cutoff values predicting the subtype of PA. The validation cohort consisted of 11 patients with either unsuccessful cannulation or a borderline lateralization index (LI), those patients underwent adrenalectomy because their indices were suggestive of unilateral disease based on the derivation cohort data. The cutoff values of A/C ≤ 0.63 or AV/IVC ≤ 0.37 identified unaffected glands with a sensitivity of 91.2% and 97.1%, respectively, and a specificity of 90.7% and 88.4%, respectively. Unilateral ipsilateral gland involvement was characterized by A/C ≥ 3.5 or AV/IVC ≥ 3.4 with a corresponding specificity of 100%. All patients in the validation cohort achieved biochemical remission postoperatively. A/C and AV/IVC cutoff values could be a useful tool to determine the subtype of PA in patients with unilateral successful AVS as well as in patients with a borderline LI.

Adult Langerhans cell histiocytosis with thyroid gland involvement: clinical presentation, genomic analysis, and outcome.

The present study aims to evaluate the characteristics and treatment outcomes of adult Langerhans cell histiocytosis (LCH) patients with thyroid involvement. We retrospectively described the clinical, biological, and genomic characteristics of a series of 36 LCH patients with thyroid involvement in our center between January 2001 and December 2021. At the time of diagnosis, only one patient was classified as having single-system LCH, and 35 patients were classified as having multisystem (MS) LCH. Three patients had coexisting papillary thyroid carcinoma. Patients with thyroid gland involvement had higher frequencies of pituitary (88.6% vs. 53.4%, P < 0.001), liver (45.7% vs. 20.7%, P = 0.003), and lymph node (54.3% vs. 31.6%, P = 0.012) involvement and a lower frequency of bone (45.7% vs. 72.0%, P = 0.003) involvement than patients without thyroid gland involvement. Sixteen patients had abnormal thyroid function, including nine patients with primary hypothyroidism, one patient with central hypothyroidism, and six patients with subclinical hypothyroidism. BRAFV600E, BRAF N486_P490, and MAP2K1 mutations were detected in 14.3%, 57.1%, and 7.1% of patients, respectively. After a 43-month median follow-up, none of the patients died, and 15 patients experienced reactivation. The median event-free survival was 37.5months. Two of 6 patients with subclinical hypothyroidism had normal thyroid function, and 12 patients still had hypothyroidism after treatment. As the largest adult LCH cohort with thyroid gland involvement to date, we found that patients with thyroid gland involvement had different clinical characteristics, genetic profiles, and outcomes than patients without thyroid gland involvement.

MRI Evaluation of Salivary Gland Involvement by Buccal Squamous Cell Carcinoma

Introduction: The salivary gland involvement by oral squamous cell carcinoma (OSCC) and diagnostic delays may influence the recurrence and survival of OSCC cases. Aims and Objective: To estimate salivary gland involvement in buccal OSCC cases. Materials and Methods: For 91 histopathologically proven cases of buccal OSCC, the primary tumor and salivary gland were evaluated using magnetic resonance imaging (MRI) sequences as T1-T2-weighted, diffusion-weighted, short tau inversion recovery, and contrast-enhanced images. Results: Out of 91 buccal OSCC patients, 10 (10.98%) cases showed salivary gland involvement, primarily the ipsilateral submandibular gland involvement with heterogeneous enhancement and few areas of necrosis. Conclusion: The considerable proportion of buccal OSCC cases with salivary gland involvement as evaluated in MRI was demonstrated in the present study. The study findings underscore the need for careful pre-treatment evaluations toward salivary gland involvement, especially in occult cases.

TNFSF4 is a risk factor for rheumatoid arthritis but not for primary Sjögren's syndrome in the Mexican population

PurposeRheumatoid arthritis (RA) and primary Sjögren’s syndrome (pSS) are autoimmune diseases (ADs) characterized by joint damage and involvement of the salivary glands, respectively. ADs share some susceptibility loci, such as TNFSF4, which is a classical susceptibility gene associated with systemic lupus erythematosus, but its role in RA and pSS is not yet clear. Thus, the aim of this study was to determine whether three TNFSFS4 polymorphisms are associated with RA and pSS. MethodsOur case-control study included 500 controls, 459 patients with RA, and 210 patients with pSS from Mexico. TNFSF4 single nucleotide polymorphisms (SNPs) rs1234315C/T, rs2205960G/T, and rs704840T/G were genotyped using TaqMan probes and discrimination allelic assay. ResultsThe three TNFSF4 SNPs were associated with susceptibility to RA (rs1234315C/T: odds ratio [OR] 1.4, p = 0.01; rs2205960G/T: OR 1.23, p = 0.03; rs704840T/G: OR 1.24, p = 0.02). An association between TNFSF4 rs1234315C/T and pSS was also observed (OR 1.28, p = 0.04), however, after Bonferroni correction, this association was lost. ConclusionOur data suggest that TNFSF4 could be a risk factor in RA but not pSS in a Mexican population.

Thyroidectomy in laryngectomy-rationale and extent

&lt;p&gt;&lt;strong&gt;Background:&lt;/strong&gt; To study the incidence of thyroid gland invasion by laryngeal carcinoma (LC) and the risk factors that may predispose to thyroid gland involvement, aiding the surgeons to develop an evidence-based plan for the management of the thyroid gland during total laryngectomy.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Methods:&lt;/strong&gt; A retrospective analysis of patients with carcinoma larynx treated at government Royapettah hospital, Chennai between January 1, 2013, and December 31, 2020, was done. The total number of final pathology reports retrieved from our hospital system for patients who met the inclusion criteria within the specified time frame was 43.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Results:&lt;/strong&gt; The thyroid gland was found to be invaded by LC in 13 patients (30.2%). Three patients (3/13) had only microscopic involvement while 9 patients showed gross extension. Four pathological features were found to have Statistically significant association with the incidence of thyroid gland invasion by laryngeal carcinoma. Patients with primary tumor pathological stage T4a were 30/43, with 11 patients showing thyroid gland invasion. 20 patients had subglottic invasion by the LC, with 10 patients showing thyroid gland invasion. Poorly differentiated carcinoma incidence was 13, with 9 patients showing thyroid gland infiltration.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Conclusions:&lt;/strong&gt; Several risk factors are associated with higher incidence of invasion of the thyroid gland by laryngeal carcinoma. Identification of these factors can help surgeons develop a surgical strategy for the management of the thyroid gland during total laryngectomy.&lt;strong&gt;&lt;/strong&gt;&lt;/p&gt;

Can salivary gland ultrasonography replace salivary gland biopsy in the diagnosis of Sjögren's syndrome?

Ultrasound is a promising diagnostic method when it comes to assessing the involvement of major salivary glands in patients with primary Sjögren's syndrome (pSS). A matter of debate is whether ultrasound of the major salivary glands (SGUS) can replace a salivary gland biopsy in the diagnosis or classification of pSS. The intra- and inter-observer reliability of SGUS was found to be good, especially when focusing on hypoechogenic areas and homogeneity, and comparable to the reliability of histopathologic characteristics of salivary gland biopsies of pSS patients. However, replacing salivary gland biopsy by SGUS led to substantial decrease of the accuracy of the 2016 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) classification criteria with clinical diagnosis as the gold standard. When SGUS was added as an additional item to the criteria, the accuracy of the criteria remained high, offering at the same time the clinicians a wider array of tools to assess patients. Combination of SGUS and anti-SSA antibodies was shown to be highly predictive of the classification of a patient suspected of pSS, making routine salivary gland biopsy debatable.

Clinical and Pathological Features and Outcome of Patients with Salivary Gland Cancer a Single Centre Report

Introduction: Salivary gland tumors are rare tumors that account for 3 to 4% of head and neck neoplasms. These tumors may arise from 6 major and numerous minor salivary glands. This study was designed to investigate the factors affecting the prognosis of salivary gland tumors. Methods: This study is a retrospective descriptive study in which the records of patients with salivary gland tumors referred to the Radiotherapy and Oncology Center of Namazi Hospital from 2005 to 2015 were reviewed. Results: 158 patients were included in this study with a mean age of 55.37 years. 49% of the patients were men. Adenoid Cystic Carcinoma was the most common histological type (30.57%) and Adenocarcinoma with a prevalence of 1.91% had the lowest prevalence rate in this study. 65.19% of salivary gland neoplasms in this study had parotid gland involvement and sublingual gland involvement had the lowest prevalence (3.8%). The results of survival analysis clearly illustrated that surgery, pathology type and stage was statistically significant in survival. Discussion: Clinical, pathological and therapeutic factors in people with malignant salivary gland neoplasms have a significant relationship with their survival. controversies on treatment and outcome continues.

Epidemiologic, radiologic, and sialendoscopic aspects in chronic obstructive sialadenitis.

Chronic obstructive sialadenitis (COS) is a recurring inflammation of the salivary gland. To date, there are no known predisposing factors for COS. Given the advances seen in radiology and sialendoscopy, we must update our knowledge of COS, analyzing factors that can favor its development. We prospectively analyzed 333 patients who underwent sialendoscopy between 2012 and 2021. Epidemiologic, radiologic, and sialendoscopy-related factors were correlated. Suspected diagnosis was established based on the clinical and radiologic data. The final diagnosis was determined on the basis of sialendoscopic findings. The most common etiology of COS was stricture (40.8%). Lack of papilla distensibility (LPD) was also described as an etiology. COS was related to patient gender and age. Submandibular gland involvement was significantly more associated with lithiasis and LPD, while COS of the parotid gland was most frequently caused by stricture. Radioiodine sialadenitis and Sjögren's syndrome were significantly associated with stricture. MR sialography (MR-Si) showed the best overall sensitivity and specificity. In our series, stricture was the most common cause of COS. We describe LPD as a frequent cause of COS in this series; ours is the first study to report this finding. There was a significant association between the salivary gland involved, patient sex and age, and the cause of COS. MR-Si showed the greatest diagnostic yield.

Characterization of Endocrine Glands Involved in Erdheim-Chester Disease

Objective To investigate the clinical and imaging characteristics of endocrine glands involved in Erdheim-Chester disease (ECD).Methods A retrospective analysis was performed on 48 ECD cases pathologically diagnosed from January 2014 to October 2020 in Peking Union Medical College Hospital,including 22 cases of endocrine gland involvement.The clinical,imaging,and pathological characteristics were summarized. Results Pituitary was involved in 17 cases (17/48,35.4%),adrenal gland in 8 cases (8/48,16.7%),and both pituitary and adrenal gland in 3 cases (3/48,6.25%).The most common symptom in patients with pituitary involvement was central diabetes insipidus (13/17,76.5%),and the T1-weighted imaging showed posterior pituitary hypersignal disappearance,pituitary stalk thickening,and abnormally enhanced pituitary nodules.The most common symptom in patients with adrenal gland involvement was adrenal function reduction (3/8,37.5%),and the CT scanning showed diffuse thickening of adrenal glands.BRAF V600E mutation was positive in 13 (13/22,59.1%) cases with ECD involving endocrine glands.Conclusion Pituitary and adrenal glands are the most common sites of ECD involving endocrine glands.A definite diagnosis can be achieved by combining clinical,imaging,and pathological characteristics for timely treatment.

A Novel Technique for the Evaluation and Interpretation of Elastography in Salivary Gland Involvement in Primary Sjögren Syndrome.

Ultrasound (US) of major salivary glands (MSG) evaluates echogenicity, border features and vascularization, with elastography, it can detect tissue elasticity and glandular fibrosis, related to inflammation in Primary Sjögren’s syndrome (pSS). This study aimed to develop a novel technique by pixel analysis for evaluation and interpretation of elastography in MSG in pSS. A cross-sectional and observational multicenter study was conducted. The US of MSG performed in orthogonal planes in grayscale, Doppler, and shear-wave elastography. For elastography images of each gland were analyzed with the open-source program ImageJ to perform a pixel analysis. Statistical analysis was performed with the IBM-SPSS v25 program. Fifty-nine women with a mean age of 57.69 (23–83) years were recruited; pSS mean duration of 87 (5–275) months, and 12 healthy women without sicca symptoms as a control group with a mean age of 50.67 (42–60) years. Intragroup analysis showed p-values >0.05 between sicca symptoms, ocular/dryness tests, biopsy, US, and pixel analysis; correlation between Hocevar and pixel analysis was not found (rho < 0.1, p >0.5). MSG anatomical size was 41.7 ± 28.2 mm vs. 67.6 ± 8.8 mm (p ≤ 0.0001); unstimulated whole saliva flow rate was 0.80 ± 0.80 ml/5 min vs. 1.85 ± 1.27 ml/5 min (p = 0.016). The elastography values (absolute number of pixels) were 572.38 ± 99.21 vs. 539.69 ± 93.12 (p = 0.290). A cut-off point risk for pSS identified with less than 54% of red pixels in the global MSG mass [OR of 3.8 95% CI (1.01–15.00)]. Pixel analysis is a new tool that could lead to a better understanding of the MSG chronic inflammatory process in pSS.

POS0790 18F-FLUORODEOXYGLUCOSE (FDG) PET-CT IMAGING OF SALIVARY GLANDS IN PRIMARY SJÖGREN’S SYNDROME AND ITS CORRELATION WITH ULTRASONOGRAPHIC SCORES AND SALIVARY FLOW RATE COMPARED TO HEALTHY CONTROLS

BackgroundThe use of salivary gland imaging modalities in patients with primary Sjögren’s syndrome (pSS) has been increasing recently. The contribution of each imaging method in terms of diagnosis or disease activity differs from each other. Although ultrasound and MRI are the most commonly used imaging modalities, the role of PET-CT for diagnosing pSS and determining glandular and extraglandular involvement has largely been neglected.ObjectivesThis study aimed to compare the sizes and metabolic activities of the major salivary glands in patients with pSS and healthy controls (HC). Correlation of the 18F-FDG PET-CT uptake characteristics with ultrasound scores and salivary flow rates of the patients and HC was also determined.Methods22 patients with pSS and 10 age/sex-matched HC were included in the study. The sizes and FDG uptakes of the parotid and submandibular glands of pSS patients and HC were assessed by PET-CT. The maximum standardized uptake value (SUVmax) was evaluated for FDG uptakes, and each patient’s liver uptake and salivary gland uptake ratio were calculated. In addition, correlations of gland sizes and FDG uptakes in PET-CT with OMERACT and Hocevar ultrasound scores, stimulated and unstimulated SFR, ESSPRI dryness scores and disease durations of pSS patients were calculated by Spearman test.ResultsThe mean age (SD) of the patients was 58.6 years (10.5) versus 58.6 years (19.1) of HC; the mean (SD) disease duration was 8.96 (8.77) years. ANA was positive in all patients, anti-SSA positivity was present in 82.6%, and 30.4% of patients experienced ≥1 parotid swelling episode.Compared to HCs, the mean size of both submandibular glands (p=0.006 for left and p=0.032 for right) and SUVmax of the left submandibular gland (p=0.044) were significantly smaller in patients with pSS. In pSS patients, both right and left parotid sizes were smaller and SUVmax uptake was greater than in HC; these differences however did not reach statistical significance.When the PET-CT involvement characteristics of the patients were compared with the salivary gland ultrasonography scores, there was a statistically significant negative correlation between the left parotid gland size in PET-CT and the ultrasonographic inhomogeneity of Hocevar score and OMERACT score. There was a statistically significant negative correlation between right parotid gland size measured on PET-CT with ultrasonographic inhomogeneity, hyperechoic foci, parenchymal echogenicity, Hocevar total score, and OMERACT score. No statistically significant correlation was found between SUVmax scores detected by PET-CT and ultrasound scores in both parotid glands and submandibular glands.A statistically significant positive correlation was found between the total gland size measured in PET-CT and the unstimulated salivary flow rate (p=0.038, r=0.604). There was a negative correlation between total gland size and ESSPRI dryness scores and symptom duration, which did not reach statistical significance.ConclusionPET-CT SUVmax measurements do not provide sufficient information for pSS-related involvement of the major salivary glands. Secondly, size measurement of the parotid glands by PET-CT is associated with OMERACT ultrasound scores, and also the sizes of both submandibular and parotid glands are smaller than HC.Table 1.Correlations of gland sizes and ultrasonographic scoresGlandOMERACT ScoreParenchymalHomogeneityHypoechoic AreasHiperechoicVisibility of Gland BorderParenchymal InhomogenicityTotalSize (PET/CT)EchogenityFociR-Parotisr-.699*-.717*-.704*-0,598-.656*-0,368-.758**-.645*p0,0170,0130,0160,0520,0280,2660,0070,032L-Parotisr-.699*-.717*-.704*-0,598-.656*-0,368-.758**-0,482p0,0170,0130,0160,0520,0280,2660,0070,134R-Submr-0,0110.118-0,011-0,2700,203-0,0060,2470.024p0,9720.7140,9720,3970,5260,9860,4390.942L-Submr0,2450,3060,2450,0710,3270,1180,3060,435p0,4430,3330,4430,8260,3000,7140,3330,158Disclosure of InterestsNone declared

Translational Research in Cystic Fibrosis: From Bench to Beside.

Cystic fibrosis is the most common life-limiting recessive genetic disorder in Caucasian populations, characterized by the involvement of exocrine glands, causing multisystemic comorbidities. Since the first descriptions of pancreatic and pulmonary involvement in children, technological development and basic science research have allowed great advances in the diagnosis and treatment of cystic fibrosis. The great search for treatments that acted at the genetic level, despite not having found a cure for this disease, culminated in the creation of CFTR modulators, highly effective medications for certain groups of patients. However, there are still many obstacles behind the treatment of the disease to be discussed, given the wide variety of mutations and phenotypes involved and the difficulty of access that permeate these new therapies around the world.

Shear wave elastography as a potential additional diagnostic tool in primary Sjögren's syndrome: an observational study.

The primary aim of this study was to verify if shear wave elastography can be used to evaluate salivary gland involvement in primary Sjögren's syndrome (pSS). The secondary objective was to establish an accurate cut-off value for parotid and submandibular salivary gland stiffness and to verify whether there are any distinctions among pSS patients with or without subjective mouth dryness. This prospective study included 45 patients with pSS (2016 ACR/EULAR classification criteria) and 108 healthy controls. All subjects underwent bilateral shear wave elastography of the parotid and submandibular salivary glands. Clinical data of pSS patients were collected and compared to elastography results. Patients with pSS had significantly higher shear wave elastography values for the parotid and submandibular salivary glands than the controls. There were no statistical differences in SWE values between patients with or without mouth dryness. The optimal cut-off value (mean value of 4 salivary glands shear wave elastography results) to distinguish patients with or without pSS was 13.19kPa with sensitivity = 97.8% and specificity = 100.0%. It was, therefore, confirmed that shear wave elastography measurement of salivary glands has strong predictive ability in pSS detection (AUC 97.8%, 95% CI 93.4-100.0%). Shear wave elastography seems to be a promising, non-invasive and simple quantitative adjunct test to support the diagnosis of pSS with good sensitivity and specificity. More extensive prospective studies are needed to standardize a study protocol.