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Objectives: Autoimmune activation by COVID-19 infection/vaccination has been postulated to be responsible for initiating or reactivating multiple types of oral mucosal immune disorders. These include: oral lichen planus; oral pemphigoid; either bullous pemphigoid or mucous membrane pemphigoid with oral involvement; pemphigus vulgaris with oral involvement; and Sjögren disease. In addition, chronic conditions such as oral burning, xerostomia, or changes in taste and/or smell have also been linked to COVID-19 infection/vaccination. Data sources: Part 1 (mucosal conditions): an English-language literature review of Pubmed, Web of Science, Scopus, and Embase was performed searching cases of oral lichen planus, oral bullous pemphigoid, mucous membrane pemphigoid, pemphigus vulgaris, and COVID-19 infection/vaccination, with additional cases from the authors’ clinical practice presented. Part 2 (nonmucosal conditions): Cases of initiated or flared Sjögren disease, chronic oral burning, or xerostomia after COVID-19 infection/vaccination from the authors’ clinical practice were aggregated. Results: The literature review discovered 29 cases of oral lichen planus following COVID-19 infection/vaccination. For bullous pemphigoid, 10 cases were identified after infection/vaccination. The number of pemphigus vulgaris cases following infection/vaccination was 28. The majority of mucosal cases were reported after vaccination. Most reported initial disease, but a substantial amount included recurrences of existing diseases. Nonmucosal disease: Sjögren disease, chronic oral burning, or xerostomia after COVID-19 infection/vaccination cases totaled 12 cases identified from the authors’ clinical practice, with the majority occurring after infection. Conclusions: Chronic conditions after infection with COVID-19 or vaccination remain relatively rare and self-limited, yet reinforce the importance of comprehensive history taking involving COVID-19 to differentiate potential etiologic factors for these conditions. [ABSTRACT FROM AUTHOR]

ObjectivePrimary Sjögren's syndrome (pSS) is the second most common chronic autoimmune connective tissue disease. Autoantibodies, immunoglobulin (IgG) anti-SSA/Ro, in serum is a key diagnostic feature of pSS. Since pSS is a disease of the salivary gland, we investigated anti-SSA/Ro52 in saliva.MethodsUsing a novel electrochemical detection platform, Electric Field-Induced Release and Measurement, we measured IgG/M/A, IgG, IgA, IgA isotypes (IgA1 and IgA2) and IgA1 subclasses (polymeric and monomeric IgA1) to anti-SSA/Ro52 in saliva supernatant of 34 pSS, 35 dry eyes and dry mouth (patients with Sicca) and 41 health controls.ResultsSaliva IgG/M/A, IgG, IgA, IgA isotypes and IgA1 subclasses to anti-SSA/Ro52 differed significantly between pSS, non-pSS Sicca and healthy subjects. Elevated monomeric IgA1 was observed in patients with non-pSS Sicca while elevated polymeric IgA1 (pIgA1) was observed in patients with pSS. Salivary polymeric but not monomeric IgA1 (mIgA1) isoform correlated with focus score (r2=0.467, p=0.001) CONCLUSIONS: Salivary anti-Ro52 polymeric IgA1 isoform is associated with glandular inflammation in pSS, while salivary monomeric IgA1 is associated with Sicca. Whether IgA1 isotope switching plays a role in the progression of the Sicca to pSS warrants further investigation.

Objectives: To investigate the prevalence and recurrence rates of spontaneous pneumothorax (SP) in patients with diffuse cystic lung diseases (DCLDs). Methods: We retrospectively identified and analyzed medical records of patients with DCLDs encountered at the First Affiliated Hospital of University of Science and Technology of China from Jan 1, 2017 to December 31, 2023. Results: A total of 289 patients were identified with DCLDs; 212 females and 77 males, with a median age of 48 years (range, 18–81 years). Among them, 89 (31%) patients had experienced SP; 59% among 115 patients with Birt-Hogg-Dubé (BHD), 34% of 41 patients with lymphangioleiomyomatosis (LAM, all women), 36% of 11 patients with pulmonary Langerhans cell histiocytosis (PLCH), none of 57 patients with Sjögren's syndrome-associated diffuse cystic lung disease (SS-DCLD), and 5% of 65 patients with no identifiable underlying disease (χ² = 90.585, P < 0.001). The overall recurrence rate of SP was higher with observation or chest tube placement strategy compared to surgical intervention, 59% vs. 11% (P < 0.001, 95% CI [0.1, -0.4]), respectively. The recurrence rate after surgical management was significantly lower compared to conservative management in patients with BHD (10% vs. 69%, P < 0.001, 95% CI [0.1, 0.3]) and LAM (20% vs. 57%, P = 0.322, 95% CI [0.1, 2.1]). Among patients with BHD, LAM, and PLCH, those who had pneumothorax as the initial presentation were diagnosed of their underlying disease at a significantly younger age (42.2 ± 13.0 years) compared to those without pneumothorax (48.1 ± 11.8 years) (P = 0.032, 95% CI [-8.24, -0.36]). Notably, eight of LAM patients who were treated with sirolimus after the initial SP did not experience recurrence of SP. Conclusion: The risk of SP secondary to DCLDs was highest in patients with BHD, followed by those with PLCH and LAM. It was extremely low in SS-DCLD. Pneumothorax as the initial presentation often facilitated diagnosis of the underlying disease. Surgical treatment was associated with a lower recurrence rate of SP compared to nonsurgical management. In addition, sirolimus therapy may reduce the risk of pneumothorax recurrence in patients with LAM. [ABSTRACT FROM AUTHOR]

Sjögren's syndrome (SS) is a chronic autoimmune disorder that primarily affects the exocrine glands. Due to the intricate nature of the disease progression, the exact mechanisms underlying SS are not completely understood. Recent research has highlighted the complex interplay between immune dysregulation and metabolic abnormalities in inflammatory diseases. Notably, lipid metabolism has emerged as a crucial factor in the modulation of immune function and the progression of autoimmune diseases, including SS. This review explores the prevalence of dyslipidemia in SS, emphasizing its role in the onset, progression, and prognosis of the disease. We specifically described the impact of altered lipid metabolism in exocrine glands and its association with disease-specific features, including inflammation and glandular dysfunction. Additionally, we discussed the potential clinical implications of lipid metabolism regulation, including the role of polyunsaturated fatty acids (PUFAs) and their deficits in SS pathogenesis. By identifying lipid metabolism as a promising therapeutic target, this review highlights the need for further research into lipid-based interventions for the management of SS. [ABSTRACT FROM AUTHOR]

Background: Sjögren's syndrome (SS) is a systemic autoimmune disease, with major symptoms including dry mouth and dry eyes, for which there is no effective treatment. Recent studies have demonstrated that mesenchymal stem cells (MSCs) are effective in the treatment of SS, but the efficacy of allogeneic MSCs is affected by variability among different cell donors, and they are easily cleared by the immune system of the recipient. Autologous MSCs are one of the ideal options for the treatment of SS; however, their function decreases with age. Regenerative fibroblast (rFib) is a type of new MSC obtained through chemical reprogramming technology from skin fibroblasts. In this study, we report the safety and efficacy of intravenous infusion of autologous rFib in a volunteer with SS. Case report: In March 2021, the volunteer was diagnosed with SS due to positive anti-SSB antibodies, lymphocyte infiltration in the lip gland, dry eyes, and a large area of purpura in both lower limbs. From May 2021 to November 2022, she received allogeneic Umbilical cord mesenchymal stem cells (UCMSC) therapy (5.0 × 107 UCMSCs per time, totaling 10 infusions), but her condition did not improve. In May 2023, the rFib for the volunteer was prepared, meeting the quality standard of T/CSCB0003-2021 Human Mesenchymal Stem Cells. Between October 2023 and June 2024, the volunteer received a total of 12 intravenous transfusions of autologous rFib. After the treatments, the volunteer experienced no recurrence of purpura in both lower limbs. Symptoms of dry mouth, dry eyes, and fatigue were relieved. ESR, B lymphocytes, rheumatoid factor IgM, and IgA declined, while the proportion of NK cells increased, and most of the cytokines returned to normal levels. In vitro experiments showed that rFib could significantly inhibit the proliferation of T lymphocytes after PHA stimulation. No adverse effects were associated with the use of rFib in the volunteer during the clinical trial. Summary: The results of this clinical trial indicate that intravenous injections of autologous rFib are both safe and effective for treating SS. Autologous rFib may be more suitable for treating autoimmune diseases than allogeneic MSCs. [ABSTRACT FROM AUTHOR]

Introduction: The neutrophil cytosolic factor 1 (NCF1) rs201802880 polymorphism is a missense mutation resulting in an amino acid substitution from arginine to histidine at position 90, which impairs the function of NADPH oxidase. This casual variant confers an increased risk for multiple autoimmune disorders, including primary Sjögren's syndrome and systemic lupus erythematosus. Given the high prevalence of this autoimmune disease risk variant in East Asia, we hypothesized that it may confer an evolutionary advantage by providing protection against infectious diseases. Methods: To test this hypothesis, we investigated whether the NCF1 rs201802880 variant offers a protective effect against tuberculosis (TB), a historically significant and deadly infectious disease. Our study included 490 healthy controls and 492 TB patients who were genotyped for the NCF1 rs201802880 polymorphism. Results: Our results showed that the NCF1 rs201802880 AA genotype was associated with a reduced risk of TB in women (OR= 0.25, 95% CI: 0.09-0.68, p =0.0023). Additionally, healthy individuals with the NCF1 rs201802880 AA genotype had significantly lower circulating white blood cell (5.56 ± 1.78 vs 6.43 ± 1.59, p =0.003) and neutrophil (3.23 ± 1.20 vs 3.74 ± 1.23, p = 0.02) counts compared to those with the GG or GA genotypes, with this difference being more pronounced in women than in men. Conclusion: This study demonstrates that the autoimmune disease-causal NCF1 variant is associated with a protective effect against TB infection. [ABSTRACT FROM AUTHOR]

This multicenter, retrospective longitudinal study identified primary Sjögren's syndrome (pSS) patients with clinically significant renal involvement, and analyzed factors associated with predisposition. To investigate clinical features and long-term prognosis of renal involvement, we compared the clinical outcomes for the entire cohort based on the presence or absence of renal involvement. Among 1306 pSS patients (mean age, 51 ± 12 years; 98% female), 36 (2.8%) had renal involvement; 17 patients had tubular involvement, 15 had glomerular involvement, one had both, and 3 were unclassified. The presence of anti-La antibodies was associated with renal involvement. Over the median 5-year follow-up period, the renal function did not change significantly; however, 44% of patients with renal involvement showed impaired renal function at the last visit. Impaired renal function at the last visit was inversely associated with baseline renal function and hemoglobin levels. Among the entire cohort, the prevalence of lymphoproliferative disease (LPD) was significantly higher in pSS patients with renal involvement than those without. Renal involvement is a rare manifestation of pSS; however, it is associated with impaired renal function and LPD. Therefore, screening for renal involvement is important for preserving renal function and early detection of LPD. [ABSTRACT FROM AUTHOR]

Sjögren disease (SD) is a chronic, autoimmune disease of unknown aetiology with significant impact on quality of life. Although dryness (sicca) of the eyes and mouth are the classically described features, dryness of other mucosal surfaces and systemic manifestations are common. The key management aim should be to empower the individual to manage their condition—conserving, replacing and stimulating secretions; and preventing damage and suppressing systemic disease activity. This guideline builds on and widens the recommendations developed for the first guideline published in 2017. We have included advice on the management of children and adolescents where appropriate to provide a comprehensive guideline for UK-based rheumatology teams. [ABSTRACT FROM AUTHOR]

Primary Sjögren's Syndrome (PSS) and psoriasis are frequently observed to co-occur in clinical settings. However, the causal associations and underlying mechanisms between PSS and psoriasis remain poorly defined. In this study, we conducted bidirectional MR analysis to explore the causal relationship between PSS and psoriasis using four MR methods: inversevariance weighted, MR-Egger regression, weighted median, and weighted mode. Sensitivity analyses were carried out, employing different models and testing methods for comparison to assess the influence of heterogeneity and pleiotropy on our findings and to confirm the robustness of these results. We primarily employed the Inverse Variance Weighting (IVW) method for our analysis. A p-value of less than 0.05 indicates a significant causal relationship, while a p-value greater than 0.05 suggests the absence of such a relationship. The IVW analysis confirmed a causal relationship between psoriasis and primary Sjögren's syndrome (PSS) (OR: 3.149E-10, 95% CI 1.114E-18-0.089, P = 0.028), with the weighted median yielding similar results. Conversely, there was no causal association found between PSS and the risk of developing psoriasis (OR: 1.000, 95% CI 0.999–1.000, P = 0.328). This study reveals a causal relationship between primary Sjögren's syndrome (PSS) and psoriasis, demonstrating that psoriasis increases the risk of developing PSS, while the reverse is not true. This potential causal link offers new insights into the etiology of both PSS and psoriasis. [ABSTRACT FROM AUTHOR]

Objective: As few cerebral venous thrombosis (CVT) patients with primary Sjögren's syndrome (pSS) have been reported, little is known about the characteristics of this rare complication. This study is aimed at describing the clinical features, treatment, and outcome of CVT combined with pSS. Materials and methods: We reported five patients of CVT and pSS admitted to our hospital and searched the relevant case reports in PubMed for literature review. Results: We reviewed a total of twelve patients with pSS and CVT. Among them, five patients were from our report in the present paper, and seven other patients were from the case reports searched in PubMed. In total twelve patients, eleven patients were female. The twelve patients had an average age of 43.7 ± 8.3 years (age range, 26–57 years). The symptoms of pSS included multiple caries (50%), dry mouth (41.7%), dry eyes (33.3%), arthritis symptoms (16.7%), and parotid gland swelling (8.3%). Headache was the most common neurological symptom in all patients. Four patients (33.3%) had no clinical symptoms associated with pSS. Anti-SSA antibodies were positive in all patients. Antiphospholipid antibodies (aPLs) were positive in 33.3% of patients. Unilateral transverse sinus (75.0%) was the most commonly involved venous sinus. All patients received anticoagulant therapy. Hydroxychloroquine was also administered to the patients. Seven patients were treated with glucocorticoids. All patients recovered completely with no clinical or radiological recurrence. Conclusion: pSS combined with CVT is a rare condition. Middle-aged women with pSS should be alert to the presence of CVT. It is of great importance to screen for autoimmune diseases during the clinical course of CVT, especially in patients with unilateral transverse sinus thrombosis. Effective treatment strategies require further study. Key Points • Patients combined with CVT are very rare. • Screening for autoimmune diseases in CVT patients is great important. • Patients may have good prognosis when effective treatment is administrated. [ABSTRACT FROM AUTHOR]

Vasculitic neuropathies are a diverse group of inflammatory polyneuropathies that result from systemic vasculitis (e.g., polyarteritis nodosa, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, microscopic polyangiitis), vasculitis resulting from rheumatological disorders (e.g., rheumatoid arthritis and Sjögren's syndrome), paraneoplastic conditions, viruses, and medications. Occasionally, vasculitis is restricted to the peripheral nerves and termed nonsystemic vasculitic neuropathy. Presenting with an acute or subacute onset of painful sensory and motor deficits, ischemia to individual peripheral nerves results in the classic "mononeuritis multiplex" pattern. Over time, overlapping mononeuropathies will result in a symmetrical or asymmetrical sensorimotor axonal polyneuropathy. The diagnosis of vasculitic neuropathies relies on extensive laboratory testing, electrodiagnostic testing, and nerve and/or other tissue biopsy. Treatment consists primarily of immunosuppressant medications such as corticosteroids, cyclophosphamide, rituximab, methotrexate, or azathioprine, in addition to neuropathic pain treatments. Frequently, other specialists such as rheumatologists, pulmonologists, and nephrologists will comanage these complex patients with systemic vasculitis. Prompt recognition of these conditions is imperative, as delays in treatment may result in permanent deficits and even death. [ABSTRACT FROM AUTHOR]

Sjogren’s disease (SjD) is a chronic and disabling autoimmune disease, predominantly characterized by dryness of the mouth and eyes, resulting from lymphocytic infiltration of exocrine glands. While these are the most prominent symptoms, extra-glandular manifestations are also common. Studies suggest that up to 70% of SjD patients experience neurological symptoms, which interestingly often precede the hallmark dryness. Although every structure of the nervous system can be affected, disorders of peripheral nervous system (PNS) are more common than central nervous system (CNS) involvement. The CNS manifestations can range from subtle to severe, with some patients experiencing a rapid progression of symptoms. The literature documents cases where patients initially diagnosed with Creutzfeldt-Jakob disease, neurosarcoidosis, temporary hemiplegia, neuromyelitis optica, or epilepsy were ultimately diagnosed as having SjD. Here, we present five SjD patient cases, each with a different manifestation of CNS involvement, along with an overview of the current understanding of CNS disorders in the course of the disease. In four cases, the neurological manifestations appeared before the onset of sicca symptoms. In one patient, diagnosis was delayed by 15 years due to an atypical presentation. After receiving immunosuppression, all patients experienced notable alleviation of their symptoms. [ABSTRACT FROM AUTHOR]

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Objective: Challenging differential diagnosis Background: Meckel's diverticulum is a congenital diverticulum that contains all normal layers of the gastrointestinal wall. In adults, Meckel's diverticulum can present with bowel obstruction, the most common presentation, in 35.6% of cases. In addition, complicated Meckel's diverticulum can present with small bowel obstruction. Sjögren syndrome is a rare systemic autoimmune disorder. The theory behind Sjögren syndrome is that lymphocyterich mononuclear cell foci infiltrate exocrine glandular tissue, and humoral factors, such as antibodies and cytokines, cause dysfunction to the exocrine glands, leading to diminished saliva and tear production and skin, tracheobronchial, and vaginal dryness. Sjögren syndrome can present with intestinal pseudo-obstruction as a rare complication. Case Report: A 25-year-old woman with a known case of Sjögren syndrome presented to the Emergency Department with abdominal pain. Laboratory investigations were sent, and all results were within reference values. Multiple imaging studies were done, and findings indicated the presence of ileus, rather than bowel, obstruction. Consequently, she was admitted under general surgery, and laparoscopic exploration was done, which showed Meckel's diverticulum. The patient was discharged home in a stable state. Two weeks later, a colonoscopy was done, showing a normal study. Conclusions: It is important to consider Meckel's diverticulum as a possible cause of sudden abdominal pain in adults, as it can have catastrophic consequences. Furthermore, studies have shown that Meckel's diverticulum and Sjögren syndrome are distinct entities. Yet, both conditions can present with symptoms of intestinal obstruction. [ABSTRACT FROM AUTHOR]

Background: Despite the well-documented immune dysregulation in both psoriasis and Sjögren's syndrome (SS), the specific link between these two autoimmune diseases has not been extensively explored. The present study aims to investigate the impact of psoriasis on the risk of SS. Methods: A retrospective cohort study using TriNetX data compared SS development in patients with psoriasis and controls using propensity score matching, Kaplan–Meier curves, and Cox models. Transcriptome data were analyzed to identify shared differentially expressed genes and pathways between the two diseases. Results: A total of 293,905 patients with psoriasis and an equal number of individuals without psoriasis were included. After propensity score matching, the baseline characteristics of both groups were balanced. During the follow-up period, 3339 patients with psoriasis and 1937 individuals without psoriasis developed SS. The Kaplan–Meier curves indicated a significantly higher risk of developing SS in the psoriasis group compared to the non-psoriasis group. Upon adjustment for multiple confounding factors, the risk of developing SS in the psoriasis group was 50% higher in the psoriasis group than the non-psoriasis group (hazard ratio [HR] 1.50, 95% confidence interval [CI] 1.42–1.58). Subgroup analyses confirmed the elevated risk of SS associated with psoriasis. Patients with psoriatic arthritis (PsA) and those treated with biological agents had an even higher risk of developing SS. Transcriptomic analysis revealed potential shared pathogenesis of psoriasis and SS involving cellular proliferation, immune cell recruitment, cytokine secretion, and the interferon response to viral infections. Conclusions: Psoriasis might increase the risk of developing SS, which is augmented by PsA. The overlapping immunological mechanisms may underlie the co-occurrence of psoriasis and SS. [ABSTRACT FROM AUTHOR]

The editorial in "Frontiers in Immunology" discusses recent advances in biomarkers for inflammatory rheumatic diseases (IRDs) and cell-based therapies. The second volume includes 3 review articles and 7 original articles focusing on biomarkers in IRDs, such as neopterin, DNA methylation, and serum proteome profiling. Studies also explore biomarkers for specific diseases like axial spondyloarthritis, psoriatic arthritis, and lupus nephritis, offering insights into diagnosis, disease activity, and treatment response. The use of laboratory analysis, statistical tools, and machine learning is highlighted as valuable in predicting and managing IRDs. [Extracted from the article]

Rheumatoid arthritis (RA) is an immune-mediated disease characterized by polyarthritis that affects the small joints of the bilateral upper and lower extremities. RA shares several common clinical symptoms with Sjögren's syndrome (SS), another rheumatic disease caused by the lymphocytic infiltration of exocrine glands, with dry eye and dry mouth being the two most common symptoms. Anti-Ro/SS-A antibodies, a diagnostic biomarker of SS, are positive in patients with RA at a certain rate. The coexistence of SS and/or positivity for anti-Ro/SS-A antibodies in patients with RA influences disease activity and the effectiveness of several classes of disease-modifying antirheumatic drugs (DMARDs). Furthermore, RA, SS, and certain DMARDs, including methotrexate, are associated with the onset of lymphoproliferative disorders (LPD). In contrast, several biological DMARDs, such as tocilizumab and rituximab, are plausible options without the risk of LPD relapse. Considering the results of the studies introduced in this article, RA with SS and/or positivity for anti-Ro/SS-A antibodies could be considered a phenotype different from isolated RA from the perspective of refractoriness to DMARD therapy and LPD risk. Hence, rheumatologists should observe caution when choosing DMARDs. Further studies are needed to establish the appropriate treatment for patients with RA, SS, and/or the presence of anti-Ro/SS-A antibodies. [ABSTRACT FROM AUTHOR]

Background: Sjogren syndrome (SS) is a chronic systemic autoimmune disease and its pathogenesis often involves the participation of numerous immune cells and inflammatory factors. Despite increased researches and studies recently focusing on this area, it remains to be fully elucidated. We decide to incorporate genetic insight into investigation of the causal link between various immune cells, inflammatory factors and pathogenesis of Sjogren syndrome (SS). Methods: Our study leveraged the genetic variants of multi-omics statistics extracted from genome-wide association study (GWAS), the University of Bristol and the FinnGen study. We performed a bidirectional Mendelian randomization and mediation study based on randomly allocated instrumental variables to infer causality, followed by external validation with UK Biobank data and Bayesian colocalization. Results: We demonstrated that an elevated level of CD27 on IgD + CD24 + B cell, a subset of B cells expressing both IgD and CD24, was associated with a higher risk of SS (OR = 1.119, 95% CI: 1.061–1.179, P < 0.001), while CD3 on CD45RA + CD4 + Treg was a protective factor (OR = 0.917, 95%CI: 0.877–0.959, P < 0.001). Results of meta-analysis and colocalization further supported the significant results identified in the primary analysis. A total of 4 inflammatory cytokines and 7 circulating proteins exhibited potential causal relationships with SS despite no significant result achieved after FDR correction. Finally, results of mediation analysis indicated that CD40L receptor levels had significant mediating effects (β = 0.0314, 95% CI: 0.0004–0.0624, P = 0.0471) at a mediation proportion of 28% (95% CI: 0.364%-55.6%) in causal relationship between CD27 on IgD + CD24 + B cell and SS. Conclusions: By providing a novel genetic insight into unveiling the roles of autoimmunity and inflammation in Sjogren syndrome, our findings may potentially lead to identifying new clinical biomarkers for disease monitoring and therapeutic targets that offer more effective alternatives for treating this condition. Therefore, our study may provide valuable evidence for future clinical intervention and targeted immunotherapy. [ABSTRACT FROM AUTHOR]

Background: In secondary Sjogren syndrome (sSS), the presence and extent of hearing and vestibular abnormalities are controversial. Hence, to ascertain if individuals with Rheumatoid arthritis (RA) associated secondary Sjogren syndrome (sSS) had subclinical audio-vestibular impairment as well as to investigate whether serum anticardiolipin and muscarinic receptors type 3 antibodies had a role in its pathogenesis, this cross-sectional study was conducted on 50 RA patients with secondary Sjogren syndrome manifestations and 50 RA patients without such manifestations were included, along with 50 apparently healthy volunteers who were aged, and sex-matched as controls. Full history taking, Otologic examination, Immunologic evaluation, and Audio-vestibular assessment were done for all subjects. Results: Twenty-five percent of our patients experienced subjective audio-vestibular symptoms. In the audiogram, 30 (60%) of the 50 RA patients with sSS and 5 (10%) of RA patients without sSS had abnormal hearing loss compared to only 4 (8%) of the controls (p ≤ 0.001) with a predominance of high-frequency sensorineural hearing loss. Also, 20% of our patients experienced a higher frequency of head-shaking nystagmus than they did (p ≤ 0.001). 30% had a statistically significant increase in the frequency of abnormal caloric test results as compared to either controls (0%) or RA patients without sSS (0%); p ≤ 0.001 Finally, all audio-vestibular symptoms and tests exhibit a strong positive correlation with muscarinic receptors type 3 and anticardiolipin antibodies. Conclusion: The use of muscarinic receptors type 3 and anticardiolipin antibodies are beneficial for early diagnosis of audio-vestibular dysfunction in RA-associated secondary Sjogren syndrome patients. Therefore, this study is important since it justifies rheumatologists' screening for inner ear damage in their RA patients especially if associated with secondary Sjogren. [ABSTRACT FROM AUTHOR]

Purpose: We sought to compare the effect of cyclosporine 0.1% after various pretreatments in patients with dry eye disease. Methods: Two hundred seventy‐four eyes of 137 patients diagnosed with dry eye disease were retrospectively enrolled. Thirty patients (Group 1, 60 eyes) were not pretreated, while 68 patients (Group 2, 136 eyes) were pretreated with fluorometholone 0.1%, and 39 patients (Group 3, 78 eyes) were pretreated with cyclosporine 0.05% before treatment with cyclosporine 0.1%. The Ocular Surface Disease Index Questionnaire (OSDI) score, Schirmer I test result, noninvasive tear film break‐up time (NItBUT), corneal staining score, matrix metalloproteinase‐9 (MMP‐9) grade, meibography result, meibum quality and expressibility scores, and tear meniscus height were examined before treatment and at 1, 2, and 3 months after treatment. Results: All dry eye signs and symptoms of all Groups at 1, 2, and 3 months were significantly improved compared to those before treatment with cyclosporine 0.1% (p < 0.05). Notably, the OSDI score, Schirmer I test result, NItBUT, corneal and conjunctival fluorescein score, and MMP‐9 grade in Group 3 were significantly improved compared to those in Groups 1 and 2 at 1, 2, and 3 months after treatment with cyclosporine 0.1% (p < 0.05). The percentages of cases with treatment discontinuation in Groups 1, 2, and 3 were 20.0%, 7.4%, and 10.0%, respectively. Conclusion: Pretreatment with cyclosporine 0.05% can augment the anti‐inflammatory effect of cyclosporine 0.1%. Pretreatment with a steroid or a lower concentration of cyclosporine can increase compliance in patients using a cyclosporine 0.1% eye drop. [ABSTRACT FROM AUTHOR]

Primary Sjögren syndrome (SS) is an autoimmune disease affecting exocrine glands, with predisposition to development of lymphoma (lymphomagenesis). We report a case of Sjogren's syndrome and discuss the role of FDG PET/CT in the primary diagnosis of lymphoma transformation in SS. Furthermore, we reviewed the literature regarding the utility of FDG PET/CT to assess systemic disease activity and also its role in the SS associated lymphoma with light into the new PET tracers that can be explored for these indications in the future. Published data suggest promising role of FDG PET/CT in SS associated lymphomas, and demands larger studies for its establishment. [ABSTRACT FROM AUTHOR]

Background: Antibodies against Ku have been described in patients with various connective tissue diseases. The objective of this study was to describe the clinical, functional, and imaging characteristics of interstitial lung disease in patients with anti-Ku antibodies. Methods: This single-center, retrospective observational study was conducted at a tertiary referral institution. Patients with positive anti-Ku antibodies and interstitial lung disease identified between 2007 and 2022 were included. Clinical, immunological, functional, and imaging data were systematically reviewed. Results: Nineteen patients (ten females) with a mean age of 59 ± 12.6 years were included. The most frequent associated diagnosis was systemic sclerosis (42%), followed by rheumatoid arthritis (26%), Sjögren syndrome, undifferentiated connective tissue disease, and overlap between systemic sclerosis and idiopathic inflammatory myopathy (scleromyositis). Imaging revealed frequent septal and intralobular reticulations and ground-glass opacities, with nonspecific interstitial pneumonia as the predominant pattern (53%). The mean forced vital capacity was 82% ± 26 of the predicted value, and the mean diffusing capacity for carbon monoxide was 55% ± 21. Over the first year of follow-up, the mean annual forced vital capacity decline was 140 mL/year (range: 0–1610 mL/year). The overall survival rate was 82% at 5 years and 67% at 10 years. Conclusions: Most patients with interstitial lung disease and anti-Ku antibodies presented with dyspnea, a mild-to-moderate restrictive ventilatory pattern, and reduced diffusing capacity for carbon monoxide. The CT pattern was heterogeneous but was consistent with nonspecific interstitial pneumonia in half of the patients. [ABSTRACT FROM AUTHOR]

Sjögren's syndrome (SS) is a slowly progressive, chronic autoimmune inflammatory condition characterized by the affliction of the exocrine glands, with issues that derive from it markedly decreasing the quality of life of these patients. Salivary gland involvement can be identified through imaging methods. Among them, salivary gland ultrasonography (SGUS) is used as a diagnostic and prognostic tool in pSS. The aim of the present study was to assess the salivary flow rate and correlations between it and SGUS findings and markers of pSS activity. A total of 112 patients with pSS and 56 healthy subjects were included in this study. All patients underwent investigations including the measurement of serum autoantibodies, salivary flow rate determination, and ultrasonographic evaluation. SGUS modifications had a strong inverse correlation with salivary flow (r = −68.002, p < 0.0001) and a positive, strong correlation with IL-6 and Beta-2-microglobulin (r = −0.78 and r = −0.84, respectively, p < 0.001 in both cases). The SGUS findings were also strongly and positively correlated with the ESSDAI (r = −0.88, p < 0.0001) and Focus scores (r = −0.82, p < 0.0001). SGUS represents a non-invasive means of assessing the state of the salivary glands and, implicitly, the salivary flow of patients, offering valuable insights into disease progression and steps that can be taken in order to improve patients' quality of life. [ABSTRACT FROM AUTHOR]

Background: Primary Sjögren's syndrome (pSS) is a chronic systemic autoimmune disease characterized by hyposalivation. Currently, there is limited evidence for the prognosis of dental implant treatment in Sjögren's syndrome. Aim/Hypothesis: We hypothesized comparable clinical outcomes of implant‐supported restorations in pSS‐patients and control subjects, and improvement in oral health‐related quality of life 5 years after restoration. Material and Methods: Patients with pSS and matched (age, gender, and tooth region) control group were recruited between June 2016 and March 2020. The clinical and radiological examination were performed, and patient‐reported oral health impact profile (OHIP‐49) questionnaire was used 2 months (baseline), 1, 3, and 5 years after prosthetic treatment. Results: We included 23 patients with pSS and 24 matched control subjects (all women, mean age: 57.1 years). The overall DMFT (decayed‐missed‐filled‐tooth) was significantly higher (p = 0.008), symptoms of dry mouth were more severe (p = 0.001), and unstimulated and chewing‐stimulated saliva flow rates were significantly lower (p < 0.001) in pSS than in control group. All implants survived with no implant mobility. At implant sites, the plaque index and probing depths did not differ (p = 0.301 and 0.446, respectively), but the gingival index was significantly higher (p = 0.003) in pSS than control group. The mean marginal bone loss, prosthetic complications, and clinician‐reported aesthetic outcomes were similar in both groups after 5 years. The OHIP scores were significantly higher in the pSS than control group (p < 0.001) but reduced significantly in both groups (p = 0.026). Conclusion: Replacement of missing single teeth with dental implants was successful in patients with pSS 5 years after restoration. [ABSTRACT FROM AUTHOR]

Background: Spondyloenchondrodysplasia is classified as an interferonopathy resulting from recessive mutations in the ACP5 gene and manifests with various clinical features, including distinctive skeletal dysplasia, neurological abnormalities, immune dysfunction resembling systemic lupus erythematosus (SLE) and Sjogren's syndrome. While SLE is typically considered multifactorial and more prevalent in adulthood, a subset of approximately 10%–25% of childhood cases arise from monogenic form. Among these, spondyloenchondrodysplasia accounts for only a rare fraction of monogenic lupus cases, with only 22 reported instances in the literature. Case Report: This paper presents a new case of spondyloenchondrodysplasia-immune dysregulation (SPENCDI) in an adult patient born to nonconsanguineous parents. The patient was diagnosed with SPENCDI and exhibited immune neutropenia, anti-dsDNA positivity, platyspondyly, immune deficiency, and a homozygous variant (c.155 A > C, p. Lys52Thr) in the ACP5 gene, previously classified as pathogenic. Notably, the patient achieved successful clinical management through the initiation of baricitinib treatment, a Janus kinase inhibitor. Conclusion: SPENCDI represents an uncommon cause of SLE in adulthood. Clinicians should be vigilant of underlying Mendelian inheritance when encountering patients with associated features. While treatments for both Mendelian and non-Mendelian SLE are similar, Janus kinase inhibitors like baricitinib show potential for managing interferon-signature monogenic SLE cases. [ABSTRACT FROM AUTHOR]

Objective: Patients with primary Sjögren's syndrome (pSS) have an increased risk of lymphoma, especially mucosa-associated lymphoid tissue (MALT) lymphoma of the salivary glands. Risk factors for lymphoma are well known, but there are no studies on screening by imaging. Therefore, we aimed to assess the usefulness and adverse effects of ultrasound of the major salivary glands and neck as lymphoma screening. Method: A retrospective, single-centre, analysis of imaging studies in pSS patients was conducted. Imaging studies were classified as either screening examinations (asymptomatic patients) or occasion-related (imaging due to signs of lymphoma or at least moderate systemic activity). Results were categorized as: not suspicious; requiring control; triggering tissue sampling with exclusion of lymphoma; or triggering tissue sampling with diagnosis of lymphoma. Results: The study included 134 patients and covered 1031 patient-years. Lymphoma was diagnosed in 15 patients (11.2%), all of whom had clinical signs of lymphoma at the time of diagnosis. During this period, 569 screening examinations and 179 occasion-related examinations were conducted. None of the screening examinations detected lymphoma, but follow-up imaging was recommended in 17.1% (95% CI 14.2–20.4%) and invasive exclusion of lymphoma was performed in 0.5% (95% CI 0.1–1.5%). In contrast, lymphoma was detected in 6.1% (95% CI 3.5–10.6%) of occasion-related examinations. Conclusion: pSS patients with neither signs of lymphoma nor increased systemic disease activity did not benefit from screening. In contrast, patients with symptoms of lymphoma or at least moderate systemic activity can benefit from imaging of the neck and major salivary glands. [ABSTRACT FROM AUTHOR]

Objective: Systemic sclerosis (SSc) is a severe rheumatic disease causing fibrotic tissue rearrangement. Aberrant toll‐like receptor (TLR) 8 transcripts in plasmacytoid dendritic cells (pDCs) were recently linked to SSc pathogenesis, which is at least partially mediated by increased type I interferon (IFN‐I) responses. Here, we addressed the functional role of TLR8 signaling in different immune cell subsets of patients with SSc. Methods: Monocytes, conventional dendritic cells (cDCs), and pDCs from the blood and skin of patients with SSc were analyzed for TLR8 protein expression. To assess TLR function, cytokine responses upon TLR7 and TLR8 stimulation were studied. To identify relevant alterations specific for patients with SSc (n = 16), patients with primary Sjögren disease (pSS; n = 10) and healthy controls (HCs; n = 13) were included into the study. Results: In all individuals, TLR8 was expressed in monocytes and cDCs but not in pDCs. The TLR8 expression levels were overall similar in patients with SSc and pSS and HCs. Additionally, in all study participants, TLR8 stimulation of pDCs did not induce IFN‐I expression. In contrast, monocytes from patients with SSc revealed increased interleukin (IL)‐10 responses upon TLR8 (patients with SSc vs HCs, P = 0.0126) and TLR7/8 stimulation (patients with SSc vs HCs, P = 0.0170). Conclusion: TLR8 protein is not expressed in pDCs of patients with SSc. Accordingly, they do not respond to TLR8 stimulation. In contrast, monocytes of patients with SSc respond to TLR8 stimulation with increased IL‐10 responses. Therefore, TLR8 signaling in monocytes participates in SSc pathogenesis by conferring aberrant IL‐10 expression. [ABSTRACT FROM AUTHOR]

The article discusses the efficacy and safety of in-office transnasal oesophagoscopy and balloon dilatation for patients with high dysphagia due to non-malignant obstructive pathologies. The study found that the procedure is safe and effective, especially for patients with cricopharyngeal hypertrophy or upper oesophageal web. Patients with head and neck cancer showed varying responses to the procedure, with some requiring repeat dilatations. The study highlights the importance of considering the patient's anatomy and comorbidities when assessing swallow function. [Extracted from the article]

Air pollution is strongly associated with autoimmune diseases (ADs), however, the genetic causality between them remains poorly understood. Therefore, the aim of this study is to determine the relationship between common air pollutants and ADs through Mendelian randomization (MR) analysis. We conducted a MR study using aggregated data from publicly available genome-wide association studies (GWAS). Single nucleotide polymorphisms (SNPs) associated with 5 common air pollutants are used as instrumental variables. Random-effects inverse variance weighted (IVW) is used as the primary method to assess causal relationships, with results reported in terms of odds ratios (OR). In addition, we used a two-step MR to assess the mediating role of common risk factors for ADs in the effects of air pollution on ADs. Our analysis revealed causal associations between selected air pollutants and specific ADs. Exposure to nitrogen oxides was positively associated with the risk of rheumatoid arthritis (RA) (OR = 1.47,95% CI 1.01–2.14, P = 0.043), Sjogren's syndrome (SS) (OR = 2.29,95% CI 1.08–4.89, P = 0.032), and systemic lupus erythematosus (OR = 7.26,95% CI 2.25–23.40, P = 9.10E−04). Genetically predicted PM2.5 and PM10 were risk factors for ulcerative colitis (OR = 1.68,95% CI 1.05–2.68, P = 0.032) and psoriasis (OR = 1.34,95% CI 1.02–1.76, P = 0.037), respectively. Our results also suggest a negative causal relationship between PM2.5–10 and SS (OR = 0.29, 95% CI 0.10–0.90, P = 0.032). In risk factor-related mediation analyses, BMI and smoking mediated 6% (95% CI 1–10%) and 9% (95% CI 2–17%) of the effect of nitrogen oxides on RA, respectively. This study provides evidence of a causal relationship between air pollutants and specific ADs risks, suggesting that improving air pollution may be important in preventing ADs. [ABSTRACT FROM AUTHOR]