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Necrotising myopathy with pipestem capillaries is a distinct form of inflammatory myopathy exhibiting only sparse inflammation on biopsy, with clinical presentation and histopathological profile entirely different from dermatomyositis, polymyositis or inclusion body myositis. A 51-year-old non-diabetic man presents with progressively worsening shortness of breath and myalgias with only mild proximal muscle weakness and elevated serum creatine kinase. Autoimmune workup, ordered after ruling out infectious and cardiac aetiologies, returned positive for Sjögren’s syndrome antibody (SSA/Ro-52). Lung imaging and biopsy were suggestive of cryptogenic organising pneumonia and muscle biopsy showed myositis with pipestem capillaries and abnormal deposition of membrane attack complex with only sparse inflammation. The patient received high-dose steroids, mycophenolate mofetil, intravenous immunoglobulin and rituximab with improvement in muscle symptoms. However, his pulmonary findings progressed, requiring evaluation for a lung transplant. This case emphasises the need for further research to better understand this disease entity and improve mortality and morbidity in these patients.

We read with interest the correspondence related to the article ‘Interleukin 6 receptor inhibition in primary Sjogren syndrome: a multicentre double-blind randomised placebo-controlled trial’ in which possible explanations for negative findings in recent phase III randomised controlled trials (RCTs) for primary Sjogren’s syndrome (pSS) are discussed, including the RCT with tocilizumab.1–3 This is an important issue to raise, since there is still an unmet need for effective treatment in pSS. As discussed in these letters,2 3 most recent RCTs used the European League Against Rheumatism (EULAR) Sjogren’s Syndrome Disease Activity Index (ESSDAI) as primary endpoint. The ESSDAI is widely applied and seems suitable to assess systemic disease activity in clinical practice and in cohort studies in a standardised way. However, there are some limitations to the ESSDAI as primary endpoint in trials.4 In addition to the points made by our colleagues, it is important to note that in several RCTs, including the tocilizumab trial, large response rates for the ESSDAI minimal clinically important improvement (≥3 points decrease) were seen in both the active treatment …

Electrocardiogram (EKG) abnormalities are predictive of subsequent cardiovascular events. Cardiac involvement is common in systemic lupus erythematosus (SLE). We aimed to determine the prevalence of EKG abnormalities in SLE patients and to examine the factors associated with EKG abnormalities with machine learning approaches.Consecutive SLE patients' records were retrieved from the database of the hospital for the cross-sectional study. Abnormal EKGs with clinical significance were grouped by the presence of tachyarrhythmias, atrioventricular block, nonspecific ST segment changes, T wave abnormalities, ventricular hypertrophy, axis deviation, bundle branch block, and QT interval prolongation. Associated factors of the most common EKG abnormalities were assessed by comparing logistic regression and 4 other machine learning approaches.In the present study, 299 patients were enrolled, with 128 showing clinically significant abnormalities on EKG. T wave changes (52.3%), nonspecific ST segment-T wave (ST-T) changes (26.6%), and prolonged QT interval (8.6%) were the most prevalent abnormalities among patients with abnormal findings on EKG. Random forests models had the best performance in the discovery of associated factors. Age, disease duration, antinuclear antibody titer, disease activity (as measured by the Systemic Lupus Erythematosus Disease Activity Index 2000) were associated with nonspecific ST-T changes, prolonged QT interval, and T wave changes. Hypertension, positivity for anti-SSA antibodies, and secondary Sjögren's syndrome were influential factors for nonspecific ST-T changes, prolonged QT interval, and T wave changes, specifically.ST-T and T wave changes were the most common abnormalities seen on EKGs of SLE patients. Our finding suggests that age, longer disease duration, higher disease activity, hypertension, anti-SSA antibody positivity, and secondary Sjögren's syndrome are important and influential factors in these EKG abnormalities.

Objectives Type I IFN (IFN-I) activation is a prominent feature of primary SS (pSS), SLE and SSc. Ultrasensitive single-molecule array (Simoa) technology has facilitated the measurement of subfemtomolar concentrations of IFNs. Here we aimed to measure IFN-α2 in serum from pSS, SLE and SSc using a Simoa immunoassay and correlate these levels to blood IFN-stimulated gene (ISG) expression and disease activity. Methods Serum IFN-α2 was measured in patients with pSS (n = 85 and n = 110), SLE (n = 24) and SSc (n = 23) and healthy controls (HCs; n = 68) using an IFN-α Simoa assay on an HD-X analyser. IFN-I pathway activation was additionally determined from serum by an IFN-I reporter assay and paired samples of whole blood ISG expression of IFI44, IFI44L, IFIT1, IFIT3 and MxA by RT-PCR or myxovirus resistance protein 1 (MxA) protein ELISA. Results Serum IFN-α2 levels were elevated in pSS (median 61.3 fg/ml) compared with HCs (median ≤5 fg/ml, P Conclusion Simoa serum IFN-α2 reflects blood ISG expression in pSS, SLE and SSc. In light of IFN-targeting treatments, Simoa could potentially be applied for patient stratification or retrospective analysis of historical cohorts.

Patients with Sjögren syndrome (SS) experience difficulties in wearing conventional dentures. After removal of all teeth, the oral rehabilitation is challenging and time consuming using conventional treatment protocols. Although implant-retained overdentures are beneficial for this specific patient group, the average total oral rehabilitation time (TORT) usually takes at least 9 months and needs to be reduced to increase patients' quality of life (QoL). In this paper, we report on a new treatment concept for immediate implant-based oral rehabilitation in a 77-year-old patient with partial edentulous SS. Because of persistent pain, discomfort, and retention problems with the conventional prosthetic devices, full clearance of the remaining mandibular dentition and immediate oral rehabilitation with an implant-retained overdenture were suggested. The treatment protocol included virtual surgical planning (VSP), combining a guided bone reduction of the mandibular alveolar process, immediate dental implant placement, and restoration using a prefabricated bar and placement of the overdenture. This method demands the use of ionizing 3D imaging optionally combined with an optical dental scan or a conventional impression. Furthermore, one needs to gain experience using VSP software. This novel treatment concept for immediate implant-based oral rehabilitation using VSP proved to be feasible and safe in a patient with SS, resulting in a significantly reduced TORT and improved QoL. Further research is needed to what extent this treatment concept could be beneficial to other patient groups, such as patients with head and neck cancer.

Amyloidosis is a rare disease characterized by the deposition of abnormal proteins in extracellular tissues. We herein report a case with instructive radiologic features of nodular pulmonary amyloidosis associated with Sjögren's syndrome. A 67-year-old woman was referred to our department because of an abnormal chest radiograph. Chest computed tomography revealed multiple round cysts accompanied by calcified nodules. The patient was clinically diagnosed with primary Sjögren's syndrome and pathologically diagnosed with nodular pulmonary amyloidosis (light chain, kappa). Although multiple lung cysts have many etiologies, the presence of calcified nodules associated with multiple lung cysts is useful for narrowing down the differential diagnosis.

Background:Primary Sjögren Syndrome (pSS) is a chronic autoimmune disease characterized by epithelial atrophy, mononuclear infiltration in exocrine glands resulting in the defective function of these glands. In pSS, atrophy of the epithelium is caused by an increased amount of apoptosis.Objective:The main aim of this study is to investigate the role of the apoptosis-related factors by studying Bcl-2, Fas and FasL expression in relation to the extent of inflammation as well as the effect of therapy on the expression of these mediators.Methods:In pSS patients (n=62) documented for their serological and clinical features, Fas, FasL and Bcl-2 plasma levels were assessed using enzyme-linked immunosorbent assays. In the same context, we investigated their expression by immunohistochemistry analysis in the labial salivary glands samples in association with the extent of inflammation.Results:Interestingly, our results indicated that in pSS patients, the plasmatic Bcl-2, Fas and FasL levels, which appeared to be associated with the severity of inflammation and were significantly elevated in comparison to the healthy controls. Moreover, a significant decrease in all these factors was observed in patients after combined corticosteroids-hydroxychloroquine therapy. Importantly, we report a strong positive correlation between Bcl-2 and NO levels. The immunohistochemical staining reveals a strong Bcl-2 expression in infiltrating mononuclear cells and a total absence in the acinar cells. The Bcl-2 level varies according to the severity of pathology. However, the expression of Fas and FasL was less important and predominantly localized in infiltrating mononuclear cells.Conclusion:Our current study highlights the involvement of Bcl-2, Fas and FasL in pSS glands injury. These factors may act as useful predictor markers of a clinical course in pSS, suggesting a novel approach in the pSS patients monitoring.

An 84-year-old woman visited our hospital for dyspnea due to right pleural effusion, with lymphocytic dominance and a high adenosine deaminase (ADA) level, that had been noted 1 month earlier. She was suspected of having tuberculosis pleurisy; however, anti-tuberculosis treatment yielded no improvements. She was diagnosed with pleural effusion due to primary Sjögren's syndrome (SjS) based on her dry eyes and mouth, positivity for anti-Sjögren's-syndrome-related antigen A/B, and histopathologic findings of a lip biopsy and thoracoscopic pleural biopsy. Her symptoms improved after starting steroid therapy. Cases of pleural effusion due to SjS with a high ADA level may be misdiagnosed as tuberculosis pleurisy.

The epidemiology of Sjögren syndrome (SS) has been extensively studied in America, Europe, and Asia.To summarize available data on the epidemiology of SS in Africa.MEDLINE, EMBASE, and African Journals Online were searched from inception up to May 17, 2020, to identify relevant articles. Data gleaned from these reports have been summarized narratively in this review.Twenty-one hospital-based studies were included. These studies reported 744 cases of SS. The mean age at diagnosis varied between 28 and 73.6 years, and the female proportion ranged from 83.3% to 100%. There was no population-based incidence or prevalence. Among people with autoimmune and other rheumatic conditions, the frequency of primary SS was in the range 1.9% to 47.6%, whereas that of rheumatoid arthritis-associated secondary SS was in the range 4.3% to 100%. Sicca symptoms were the commonest features, with most frequently involved organs being joints, lungs, and neurological structures. Main autoantibodies were anti-Ro/SS antigen A, anti-La/SS antigen B, and antinuclear antibodies.The epidemiology of SS is poorly characterized in Africa. Available data are broadly consistent with those from other populations. Extensive and high-quality research is urgently needed.

Uveitis is the most common ophthalmological disorder in the field of rheumatology, accounting for a significant proportion of visual morbidity, both locally and internationally. Causative factors can be divided into infectious and noninfectious etiologies. The diagnosis of uveitis is a major challenge due to heterogeneity in presentation. The disease course may be acute monophasic, recurrent, or chronic relapsing. Complications include posterior synechiae, secondary cataract, ocular hypertension or glaucoma, macular edema, retinal vascular occlusion, epiretinal membrane, and so on, and ultimately visual loss. Antimicrobial therapy is indicated for infection, whereas noninfectious uveitis warrants a combination of steroids, immunosuppressives, and anti-inflammatory agents. With the advancement of biologics, treatment strategies in chronic, noninfectious uveitis have had multiple breakthroughs, particularly in treatment-resistant cases. This article provides a review of the diagnostic approach to uveitis based on symptomatology and ophthalmological findings, and discussion of relevant treatment modalities and strategies.

Objective To observe the ultrasonographic features of arthritis in patients with primary Sjogren's syndrome (pSS) and to analyze its correlation with clinical manifestations and disease activity. Methods Ultrasound (US) examinations were performed in a total of 1200 joints of 40 patients with pSS. A semi-quantitative grading method (0–3) for scoring synovial hyperplasia, PD synovitis, bone erosion, tenosynovitis was used. The clinical and laboratory data were collected, disease activity was assessed. The correlation between US lesions and disease activity assessment and clinical manifestations was analyzed. Results US findings of musculoskeletal in patients with pSS mainly involved the small joints of the hands and wrists and the lesions were mild. The semi-quantitative score of musculoskeletal US was positively correlated with ESSPRI. The occurrence of musculoskeletal US lesions is associated with immunological abnormalities and inflammatory markers, and patients with high IgG, RF, and inflammatory markers are prone to abnormal US findings. Conclusion The incidence of arthritis in patients with pSS is high, and musculoskeletal US has its characteristics. The musculoskeletal US semi-quantitative method can effectively evaluate arthritis in patients with pSS, and the US score of arthritis has a certain correlation with the overall disease activity. US can provide a reference for the diagnosis of arthritis and disease activity assessment in patients with pSS.

Background B cells contribute significantly to the pathogenesis of primary Sjogren's syndrome (pSS). Free light chains (FLCs) are generated during the production of immunoglobulins (Igs) and are surrogates of B cell activity. We hypothesized that salivary FLCs and salivary Igs could represent salivary gland inflammation and therefore, serve as biomarkers in pSS. Methods Patients >18 years old fulfilling the American College of Rheumatology / European League Against Rheumatism (EULAR) 2016 criteria for pSS and age-matched healthy and disease controls (sicca non-pSS, rheumatoid arthritis, systemic lupus erythematosus) were recruited for this cross-sectional study. FLCs in saliva and serum were measured by immunoturbidimetry. Serum and salivary Igs were measured by nephelometry and enzyme-linked immunosorbent assay, respectively. Area under the receiver operator characteristic curve was determined. The factors influencing the serum and salivary FLCs in pSS were determined using backward linear regression. Results A total of 78 patients with pSS, 76 healthy controls and 62 disease controls were recruited. Median EULAR SS disease activity index (interquartile range) was 1 (3.75). Serum FLCκ and FLCλ, salivary FLCλ, serum and salivary IgG, salivary IgM was significantly higher in the pSS group compared to the controls. Areas under the curve for salivary FLCλ, serum FLCκ, serum and salivary IgG were 0.75, 0.72, 0.78 and 0.77, respectively. Regression analysis showed that salivary FLCκ, salivary FLCλ and salivary IgG were associated with positive salivary gland histopathology. Use of immunosuppressants and glucocorticoids was associated with lower values of salivary parameters. Conclusion Salivary FLCλ and salivary IgG were significantly different between pSS and control groups and could be potential non-invasive biomarkers in pSS. These findings should be confirmed in larger longitudinal studies.

Nephrocalcinosis (NC) is the augmented calcium content within the renal parenchyma. Its pathogenesis mainly involves hypercalciuria. The presence of medullary NC provides a window to the clinician for the diagnosis of many important diseases. In this case series, we highlight three diseases that could be diagnosed with a high index of suspicion and detailed evaluation after their presentation as medullary NC.

Sjögren disease increasingly is recognized in pediatric patients. Clinical features, primarily parotitis and sicca symptoms, and results of diagnostic tests may be different from those in adult disease. Adult criteria fail to capture most pediatric patients. Pediatric-specific criteria are urgently needed to define the natural history of the disease, identify risk and prognostic factors, and evaluate the impact of therapeutics and other interventions on disease course in young patients.

Background/Aims: This study was performed to clarify inf luences of anticentromere antibody (ACA) on clinical phenotypes of primary Sjögren’s syndrome (pSS) patients in Korea. Methods: We assessed 318 patients who met the 2016 American College of Rheumatology/ European League Against Rheumatism classification criteria for pSS. All patients were selected from the Korean Initiative of primary Sjögren’s Syndrome (KISS), a prospective cohort. Among them, 53 patients were positive for ACA, while another 265 patients were not. We compared various clinical data including demographic features, extra-glandular manifestations (EGMs), clinical indices, and laboratory values available from the KISS database between the two groups. Results: Patients in the ACA-positive pSS group were older (p = 0.042), and had higher xerostomia inventory scores (p = 0.040), whereas glandular dysfunction represented with Schirmer I test was more severe in the ACA-negative group. More frequent Raynaud’s phenomenon and liver involvement (both p < 0.001) and less articular involvement (p = 0.037) were observed among the EGMs in the ACA-positive group. Less frequency of leukopenia (p = 0.021), rheumatoid factor (p < 0.001), anti-Ro/SSA antibody positivity (p < 0.001), and hypergammaglobulinemia (p = 0.006), as well as higher positivity rates of anti-nuclear antibody and anti- topoisomerase antibody (p < 0.001 and p = 0.006, respectively) were found in the laboratory data in the ACA-positive pSS group. Conclusions: Considering distinct phenotypes in hematological and serological features and EGMs, we should monitor the occurrence of these clinical features among pSS patients with ACA in caution.

Aim To evaluate subclinical left ventricular (LV) regional dysfunction in patients with primary Sjogren's syndrome (pSS) using feature tracking cardiac magnetic resonance (FT-CMR) imaging and to identify pSS characteristics independently associated with LV regional dysfunction. Method Fifty patients with pSS and 20 controls without cardiovascular disease underwent non-contrast CMR imaging. Labial gland biopsy was performed in 42 patients (84%). Disease activity was assessed using the European League Against Rheumatism Sjogren's syndrome disease activity index (ESSDAI). LV global longitudinal strain (GLS), global circumferential strain (GCS), and global radial strain (GRS) were measured using FT-CMR. Results No significant differences in cardiovascular risk factors were found between the pSS group and controls. The pSS group had significantly lower GLS (P = .015) and GCS (P = .008) than the control group. Multiple linear regression analysis indicated that GCS was significantly associated with Raynaud's phenomenon (P = .015), focus score ≥2 (P = .032), and total ESSDAI score ≥8 (P = .029). Conclusion FT-CMR can reveal subclinical LV regional dysfunction in patients with pSS without cardiovascular disease. Furthermore, patients with pSS and Raynaud's phenomenon, a focus score ≥2, or an ESSDAI score ≥8 were considered to be at high risk for myocardial dysfunction.

Although dry mouth, dry eye, and swollen salivary glands are the hallmark manifestations of Sjögren’s syndrome (pSS), a wide spectrum of other conditions should be considered for differential diagnosis. The diagnostic challenge is mainly encountered in patients presenting with dry eyes and/or dry mouth, who do not meet the full established classification criteria for pSS. Presented case-based review highlights the relationship between dry eye, parotid swelling, and psychiatric disorders. The obsessive-compulsive disorder may be separately be a cause of dryness symptoms even were not using any psychiatric drugs. The presented review widely discussed this problem and the aim is to shed new light on the interpretation of the dryness symptom and the necessity of individual patient assessment, excluding causes other than pSS before making a final diagnosis and making a decision on the treatment method.

Primary Sjögren's syndrome (pSS) is a complex autoimmune disease characterized by aberrant immune cell action against secretory glands throughout the body. A number of studies have previously identified unique characteristics in the circulating expression profile of white blood cells of pSS patients. However, the molecular progression pattern of pSS is unclear. Through a systematic analysis of pSS transcriptome information, we found that pSS transcriptomes display broad heterogeneity, but cannot be distinguished from the broad range of possible profiles of healthy controls. Instead, only sample learning using a subset of pre-identified signature genes could achieve partial separation through a trajectory governed by interferon activity. Interestingly, this trajectory is correlated with a decrease in dendritic cell counts. Our study thus highlights a major limitation to the utility of broad blood transcriptome analysis in the context of pSS, while also identifying several factors that influence the divergence between patient samples.

ObjectiveThis work aims to evaluate the long-term safety of rituximab (RTX) in primary Sjögren syndrome (pSS) and to determine the safety and the efficacy of long-term treatment with B cell depleting therapy in pSS patients with active systemic disease.MethodsA historical cohort study, enrolling 35 patients with pSS treated with RTX between 2008 and 2019 in a single rheumatologic unit, was performed. When patients experienced adverse events, the treatment was suspended and patients’ data were recorded.ResultsThe included patients were mainly female (91%), with a mean age of 54 years. During the time of observation, 13 patients (37.1%) suspended RTX treatment (10 cases per 100 patient-years, 95% CI 0.06–0.17). Baseline demographics, disease characteristics, European Alliance of Associations for Rheumatology (EULAR) Sjögren’s Syndrome Disease Activity Index (ESSDAI) values, and treatment were comparable across RTX-suspended and nonsuspended groups. Patients exposed to RTX had been followed for 35.82 ± 32.56 months, and the time of observation varied from 6 to 96 months. All the patients except one experienced a significant and persisting meaningful improvement of their ESSDAI (≥ 3 points) during the long-term follow-up. For the duration of the follow-up, 13 (37%) patients discontinued RTX treatment. Four out of 13 (30.8%) discontinued the treatment after the first administration due to infusion-related reactions. During subsequent RTX courses, the main cause of withdrawal was hypogammaglobulinemia onset (7 patients). In 2 patients, hypogammaglobulinemia was associated with severe infections.ConclusionLong-term RTX administration was shown to be a safe, well tolerated, and effective treatment in patients with active systemic disease, significantly reducing ESSDAI and controlling disease activity.

This study aimed to describe a patient with Sjögren syndrome who developed Plummer-Vinson syndrome, and to review the literature and describe shared aspects of this rare association. A systematic screening of articles was conducted in PubMed/MEDLINE, LILACS, SciELO, Scopus, Web of Science, and Cochrane, dating 1940 to 2020. All the articles included the association between Sjögren syndrome and Plummer-Vinson syndrome. No language restriction was applied. The following terms were used: "Sjögren syndrome" or "sicca syndrome" and "Plummer-Vinson syndrome" or "Paterson-Kelly syndrome." We performed our analysis by adding our present case, with a total of 4 cases. Three out of four were female (75%), age varied from 56 to 58 years old. In 2 cases, Sjögren syndrome preceded Plummer-Vinson syndrome diagnosis, and in 1 report, Plummer-Vinson syndrome appeared before Sjögren syndrome. Disease duration varied from 7 to 20 years. In two cases, autoantibodies were available, and antinuclear antibodies and anti-Ro/SS-A were positive in both, and anti-La/SS-B in one of them was associated with anti-dsDNA; however, no data regarding lupus was available in the article. Treatment involved iron supplementation in 3/3. Two out of three received parenteral iron supplementation, and in these two cases, mechanical esophageal dilatation was needless. In the other case, an additional endoscopic esophageal dilatation was necessary to receive the oral iron supplement. All 3 cases had a good outcome. This case illustrates a patient with Sjögren syndrome who developed the rare Plummer-Vinson syndrome. In Sjögren syndrome, the presence of iron-deficiency anemia, dysphagia, and weight loss should alert the physician to search for associated Plummer-Vinson syndrome.

Objective Patients with idiopathic pulmonary fibrosis (IPF) commonly present with sicca symptoms. This study aimed to assess labial minor salivary glands (LMSGs) in those patients to rule out Sjogren's syndrome (SS), in which sicca symptoms are the clinical hallmark. Study Design Cases of patients with IPF with sicca symptoms referred to the oral medicine clinic at the University of Florida within the last 13 years were selected with institutional review board approval. Demographic characteristics, clinical findings, laboratory results, and histomorphologic parameters were retrospectively analyzed. Results A total of 12 patients (9 men and 3 women, ages 55-76 years) were identified. History of exposure to asbestos or chemicals, smoking, and medication information was obtained. All patients reported sicca symptoms with 57% of those exhibiting objective or borderline dryness. Anti-SSA/Ro and anti-SSB/La were positive in 25% and 8% of the cases, respectively. Microscopically, 1 out of 12 patients was biopsy positive in the absence of anti-SSA/Ro, fulfilling the 2016 SS criteria with positive sialometry. Conclusions A LMSG biopsy is critical to identify SS in patients with diagnosed IPF and present sicca symptoms, especially those with negative serology, as revealed in our study.

Liver and renal involvement is a rare event in Sjögren’s syndrome. Sjögren’s syndrome is characterized by the progressive immune-mediated destruction of epithelial tissues of the salivary and lacrimal glands. Sensory ganglionitis, accompanied by T-cell invasion, occurs in patients with Sjögren’s syndrome, resulting in sensory neuropathy of the face or limbs. Patients are assessed by quantitative sensory testing. A 76-year-old woman presented with numbness of her left face and was subsequently diagnosed with Sjögren’s syndrome and primary biliary cirrhosis, and was found to have renal failure. Detection of her serum anti-Ro/SSA antibody was strongly positive. Shirmer’s test or a salivary volume in the gum test also showed positive results. Her somatosensory disturbance severity was higher in the trigeminal area than in the forearm, suggesting that the trigeminal nerve is more susceptible than other parts of the nervous system in patients with Sjögren’s syndrome and primary biliary cirrhosis. A simple sensory test could be performed during regular check-ups, as sensory deficits might develop after patients are diagnosed with Sjögren’s syndrome and primary biliary cirrhosis.

Objectives This study investigated the associations of mast cells with immune-mediated inflammation and fibrosis in patients with primary Sjögren’s syndrome (pSS); it also explored the underlying pathophysiology of pSS-related sialadenitis. Methods Twenty-two patients with pSS and 10 patients with sicca (control individuals) underwent labial salivary gland biopsies. Sections were subjected to staining and immunofluorescence analyses. HMC-1 human mast cells were cocultured with fibroblasts in vitro; fibroblasts were also grown in HMC-1 conditioned medium. mRNA levels of collagen Type I (Col1a) and transforming growth factor (TGF)β1 were analysed in cultured cells. Results Mast cell numbers in labial salivary glands were significantly greater in patients with pSS than in control individuals. In salivary glands from patients with pSS, mast cell number was significantly correlated with fibrosis extent; moreover, mast cells were located near fibrous tissue and expressed TGFβ1. Col1a and TGFβ1 mRNAs were upregulated in cocultured fibroblasts and HMC-1 cells, respectively. Fibroblasts cultured in HMC-1 conditioned medium exhibited upregulation of Col1a mRNA; this was abrogated by TGFβ1 neutralizing antibodies. Conclusions Mast cell numbers were elevated in patients with pSS-related sialadenitis; these cells were located near fibroblasts and expressed TGFβ1. TGFβ1 could induce collagen synthesis in fibroblasts, which might contribute to fibrosis.

Introduction We aimed to study the prevalence of a history of COVID-19 infection among patients suffering from systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), Sjögren’s syndrome (SjS) or psoriatic arthritis (PsA), and the potential influence of long-term hydroxychloroquine (HCQ) intake. Methods We performed an observational monocentric cohort study at the Adolphe de Rothschild Foundation Hospital ophthalmology division (Paris, France). Electronic medical records (EMR) data were searched for keywords associated with SLE, RA, SjS, or PsA. Patients were contacted by phone and were interviewed using a standardized questionnaire. The primary outcome was the occurrence of a positive COVID-19 test result during the study period. We determined the adjusted association between various antirheumatic drugs intake, COVID-19 risk factors, and occurrence of COVID-19 using a logistic regression model. This study is registered on ClinicalTrials.gov (Identifier: NCT04345159). Results Patients were recruited between Apr 17, 2020, and Apr 30, 2020 and were recontacted between Oct 6, 2020, and Nov 2, 2020. A total of 569 patients were included, of whom 459 patients were eligible for data analysis. One hundred and eighty-one patients were treated with long-term HCQ and 18 patients had tested positive for COVID-19. No antirheumatic drug intake, including HCQ intake, was significantly associated with an increased or decreased risk of developing COVID-19 infection. Conclusions No antirheumatic drug intake was associated with an increased or decreased risk of developing COVID-19 infection in our cohort of patients suffering from immune-mediated inflammatory diseases.

Background Subclavian steal syndrome (SSS) caused by Sjogren's syndrome is rare, especially for elderly patients with risk factors for atherosclerosis. The current report presents the uncommon etiology and treatment of SSS, aiming to improve doctor's clinical experience. Case summary A 69-year-old man was diagnosed with hypertension and acute cerebral infarction presenting with left upper limb weakness and pain even gradually aggravating to left limb hemiplegia 30 years ago. He was managed with antihypertensive and antithrombotic therapy; however, his condition was recurrent, and he never had any further examination. It was found that the difference of the bilateral upper arm systolic pressure was over 20 mmHg, and Doppler examination showed that the blood flow of the left vertebral artery was reversed, suggesting SSS. Further tests revealed a benign lymphoepithelial lesion in salivary gland tissue, confirming the Sjogren's syndrome. Conclusion The patient was found to have hypertension when he was 33 years old, and the blood pressure of both sides was asymmetric, which was ignored. The patient's symptoms of dizziness and upper limb weakness were misdiagnosed as general cerebral infarction. It is necessary to test the aorta computed tomography angiography to prove secondary hypertension factors such as Sjogren's syndrome.

Background/aim Sjögren’s syndrome (SS) is an autoimmune disease and its pathogenesis is still not completely clear. The wingless (Wnt)/β-catenin pathway has recently been shown to play an important role in inflammation. This study aims to determine the serum and saliva levels of Dickkopf (DKK)1 and sclerostin and to evaluate Wnt-1 and Wnt-3a expression in the salivary gland in patients with primary SS. Materials and methods This study included 30 patients diagnosed with SS, 30 patients diagnosed with systemic lupus erythematosus (SLE), and 29 healthy controls. Serum and saliva levels of DKK1 and sclerostin were measured and the expressions of Wnt1 and Wnt3a in the salivary gland were measured immunohistochemically. Results Serum DKK1 and sclerostin levels were lower in the SS and SLE groups compared to the control group (both p < 0.001). Saliva DKK1 levels were higher in the SS group compared to the control and SLE groups (p = 0.004 and p = 0.009, respectively). Wnt1 and Wnt3a expression were found in salivary gland tissue samples in 71.4% of primary SS patients and relatively frequent than control group. Conclusions Serum DKK1 and sclerostin levels in primary SS and SLE were decreased. Moreover, levels of Wnt1 and Wnt3a expression in the salivary gland were also elevated in primary SS. Therefore, it can be concluded that the Wnt/β-catenin pathway activities may be altered in case of glandular inflammation.

Background/aim: This study aimed to investigate the prevalence of sicca symptoms and secondary Sjogren's syndrome (SjS) in patients with systemic sclerosis (SSc). Also this study aimed to evaluate the expression of alpha-smooth muscle actin (alpha-SMA) in minor salivary gland (MSG) specimens, a possible marker of fibrosis responsible for myofibroblastic transformation. Materials and methods: Patients with SSc who were followed in Rheumatology outpatient clinic at a university hospital evaluated. The questionnaire of sicca symptoms and classification of SjS were evaluated according to the American-European Consensus Group (AECG) criteria. Histopathologic evaluations were done in MSG specimens investigating the presence of focal lymphocytic sialadenitis and glandular fibrosis, also assessing the expression of alpha-SMA. Results: This cross-sectional study included 102 patients with SSc [91 females (89%), mean age 52.5 +/- 12 years]. In this cohort 76 (75%) patients had sicca symptoms and 36 (35.3%) patients fulfilled the AECG criteria for SjS; all with limited form. Having SjS found to be associated with older age and the presence of positive anti-SS-A antibodies. On histopathologic examinations, glandular fibrosis was observed in 67 (80%) and lymphocytic sialadenitis was detected in 38 (45%) patients; but only 7 samples were positive for alpha-SMA. Conclusion: This study suggested sicca symptoms were found to be very common among patients with SSc. Also secondary SjS was detected in nearly one-third of patients with SSc; especially in limited subtype. Anti SS-A positivity and older age were detected as predictors for SjS. Histopathologic evaluations showed significant glandular fibrosis but rare alpha-SMA staining in patients with SSc.

Cancer immunotherapy, which seeks to stimulate a patient's own immune system to combat cancer, is quickly becoming a central pillar of cancer therapeutics and has resulted in the development of many novel anticancer therapies. One subtype of cancer immunotherapy, immune checkpoint inhibitors (ICIs), has revolutionized cancer treatment and changed the standard of care for multiple indications. However, the advent of ICIs has produced a wide variety of inflammatory side effects termed immune-related adverse events (IRAEs), including ICI-induced Sicca syndrome. This article outlines the clinical features of ICI-induced Sicca syndrome and assesses its reported incidence in clinical trials, case series, and case reports across numerous cancers and treatment modalities. Presentations of ICI-induced Sicca syndrome in patients with pre-existing Sjӧgren's disease and with extra-glandular manifestations will also be explored. The pathophysiological mechanisms underlying IRAEs, including ICI-induced Sicca syndrome, will be evaluated through an examination of existing literature. Finally, the various treatment and management strategies as well as aims for future work will be discussed and reviewed.

This clinical quandary details a Mexican man, aged 77 years, who presented to the oncology clinic with a sternal mass. Based on the results, the patient fulfilled the 2016 American College of Rheumatology/European League Against Rheumatism classification criteria for Sjögren syndrome, thus the diagnosis triggered by immune checkpoint inhibitors was definitively established.

Introduction Onconeuronal antibodies directed against intracellular antigens are strongly associated with paraneoplastic syndromes and their detection in the absence of cancer is unusual. We herein report a case of anti-Ma2 encephalitis associated with Sjogren's syndrome (SS). Case report An 81-year-old woman followed for a cutaneous lupus with vasculitis associated with SS presented a flare of her disease with neurological worsening including walking difficulty, hypersialorrhea and dysphagia. A paraneoplastic origin of the symptoms was suspected and anti-Ma2 antibodies were positive in serum. The search for an underlying neoplasia was negative. The diagnosis of anti-Ma2 encephalitis secondary to a SS was made. In the literature, the association of anti-Ma2 encephalitis and SS has been previously reported twice. Cases of patients with other onconeuronal antibodies associated with SS have been also reported. Anti-Ma2 encephalitis is a rare condition with a wide spectrum of symptoms associated with a cancer in more than 90% of the cases. Anti-Ma2 encephalitis has also been described after the use of immune check points inhibitors underscoring the role of autoimmunity in its pathogenesis. Conclusion Anti-Ma2 encephalitis is essentially associated with neoplasia but can occur in Sjogren's syndrome.

The aims of this pilot investigation were to calculate the levels of sensitivity and specificity of salivary glands ultrasonography (SGUS) in diagnosing Sjögren syndrome (SS) and to assess the ultrasonographic findings of parotid and submandibular glands.Patients diagnosed with SS or dry mouth and healthy controls were enrolled. Bilateral parotid and submandibular glands were assessed for (1) parenchymal inhomogeneity (PIH), (2) median size of the glands, (3) visibility of glandular posterior borders, and (4) size of sialolith, if present.This study included 34 female patients, of whom 12 had SS (35.3%), 12 had dry mouth (35.3%), and 10 were healthy controls (29.4%). Patients with SS showed higher PIH scores in all glands with the median differences being statistically higher in the right and left parotids and left submandibular glands (P.001, P = .012, and P.001, respectively). SGUS, with a PIH cutoff ≥2, showed a sensitivity of 100% and a specificity of 81.6% for detecting SS. The majority of SS had invisible glandular posterior borders (P.001). Median size of the glands and size of the sialolith did not show any statistically significant differences between groups.SGUS is a noninvasive imaging modality with good sensitivity and specificity that might be valuable as a diagnostic aid for SS.

Sjögren’s syndrome (SS) is a systemic autoimmune disease, characterized by lymphocytic infiltration of the secretory glands. This leads to dryness of the main mucosal surfaces such as the mouth, eyes, nose, larynx, pharynx, and vagina. Although there is little morbidity data at the initial diagnosis, SS may be a serious disease, with extra mortality caused by hematological cancer. The cause of SS is unknown, but factors postulated to play a role include genetic and environmental factors, hormonal abnormality, and viral infection. Under the influence of these factors, the immune system becomes abnormal and the tissue is damaged. In this study, we summarize recent developments in our understanding of the relationship between SS and viral infections, including Epstein–Barr virus (EBV), hepatitis C virus (HCV), human T cell lymphotropic virus type 1 (HTLV-1), cytomegalovirus (CMV), and human immunodeficiency virus (HIV).

PURPOSE: To examine whether “activated” dendritic cells (aDCs) could serve as a biomarker of systemic immune disorders in individuals with dry eye (DE) symptoms. Secondarily, to examine the impact of a topical anti-inflammatory agent on aDC number. METHODS: Retrospective analysis was conducted to identify individuals with DE symptoms who had in-vivo confocal microscopy (IVCM) imaging between October 2018 and July 2020 at the Miami Veterans Hospital. aDCs were manually quantified based on morphology. Receiver operating curve (ROC) analysis examined relationships between aDC number and systemic immune disease status. Individuals were then grouped by aDC number (≥2 versus

Background Although the presentations of Sjogren's syndrome (SS) are variable, ranging from mild dryness to wider systemic involvement, ranulas as early clinical signs were scarcely reported. Here, we present an adult patient with SS, who developed a unilateral simple ranula and was diagnosed primary SS 3 years later. We also provide a review of cases of SS and ranulas from 1980 to 2020. Case summary A 22-year-old girl was found to have a left painless floor-of-mouth lesion 3 years ago, without obvious trauma or inducement. The diagnosis of a unilateral (left) simple ranula was made, and the ranula was surgically treated. Within 3 years after the ranula surgery, she developed acute lymphadenectasis in unilateral parotid twice without inducement, and ultrasonic examination revealed diffuse lesions in bilateral parotids and submandibular glands, which strongly suggested SS. Serologic tests and the unstimulated whole saliva flow rate confirmed the SS diagnosis. Conclusion Our study underlines that ranulas are early clinical signs of SS. As early diagnosis and early intervention of SS are important to obtain better outcomes, our findings underline the need for histopathological test after sublingual adenectomy and imaging detection of exocrine glands for the patients with ranulas.

Objective: Interstitial lung disease (ILD) is a common feature of connective tissue disease (CTD). The diagnosis of CTD-ILD can be challenging and is important for therapeutic decisions. In this st...

PURPOSE OF REVIEW: We review the clinical manifestations of three less common connective tissue disease-associated interstitial lung diseases (ILDs): Sjogren’s syndrome, mixed connective tissue disease, and systemic lupus erythematosus. RECENT FINDINGS: Sjogren’s syndrome is classically associated with lymphocytic interstitial pneumonia and cystic lung disease, but the most common type of ILD in Sjogren’s patients is nonspecific interstitial pneumonia. ILD is prevalent in mixed connective tissue disease and associated with worse survival. Systemic lupus erythematosus-associated ILD, while rare, is more common in those with connective tissue disease overlap syndromes. Regardless of underlying cause, a subset of patients with fibrotic connective tissue disease-associated ILD develop a progressive course for which antifibrotic agents and lung transplantation should be considered. SUMMARY: An understanding of the characteristics of ILD in Sjogren’s syndrome, mixed connective tissue disease, and systemic lupus erythematosus is important for the pulmonary specialist. Future research should identify risk factors for progression and develop additional treatment modalities for both connective tissue disease-related autoimmune features and progressive ILD.

To assess the relationship between resilience and several diseases and individual features in primary Sjӧgren's Syndrome (SS) patients. Resilience was assessed using the Resilience Scale (RS-14). Disease activity, damage, and reported symptoms were assessed by means of ESSDAI (EULAR Sjogren's syndrome disease activity index), SSDDI (Sjӧgren's Syndrome Disease Damage Index) and ESSPRI (EULAR Sjӧgren's Syndrome Patient Reported Index). EuroQol, HADS (Hospital Anxiety and Depression Scale), SF-12 (Short-form 12 health survey), FAS (Fatigue Assessment Scale), FACIT-F (Functional Assessment of Chronic Illness Therapy – Fatigue), and IPAQ (International Physical Activity Questionnaire) questionnaires were submitted to evaluate physical and mental well-being of the recruited patients. Data about the autoimmune profile, systemic manifestations, and current therapy were collected. Educational qualifications and work activities were also considered. Descriptive, correlational, and linear regression analysis were performed. 74 consecutive women with primary SS and 74 sex and age-matched healthy subjects as a control group were recruited. SS patients displayed a moderate value of resilience (median 78.5) with no significant difference compared to controls (p = 0.38). An inverse relationship was found between resilience and mood disorders such as anxiety (p = 0.038) and depression (p

Case Presentation A 63-year-old, non-smoking Asian woman presented to our hospital due to abnormal findings on chest radiography. She had no history of dust exposure. Chest radiography and CT imaging showed patchy ground-glass attenuation (GGA) in the bilateral lower lung lobes, a ground-glass nodule in the right lower lung lobe (diameter, 9.8 mm), and some thin-walled cysts in both lungs ( Fig 1 ). Thickening of the interlobular septa, mediastinal lymphadenopathy, and pleural effusion were not evident. Video-assisted thoracic surgery was performed for the examination of the nodule and the background lung disease, and the nodule was histologically diagnosed as lung adenocarcinoma. Simultaneously, the lung background showed diffuse lymphocytic infiltration in the alveolar septum and peribronchovascular interstitium ( Fig 2 ). There were no symptoms suggestive of autoimmune diseases such as dryness, arthralgia, skin rash, or fever. The patient was followed up without treatment for the interstitial lung disease.

BACKGROUND During the course of disease, some patients with primary Sjogren's syndrome (pSS) develop interstitial lung disease (ILD), which leads to a poor prognosis. There is currently a lack of methods to identify high-risk patients with ILD and predict the prognosis. The purpose of this study was to analyze the predictive value of the neutrophil to lymphocyte ratio (NLR) for the occurrence of ILD and its prognosis in patients with pSS. METHODS According to the inclusion and exclusion criteria, patients with pSS admitted to our hospital from May 2009 to November 2020 were included. The patients were divided into either an ILD group or a non-interstitial pneumonia [non-ILD (NILD)] group. We compared the baseline data of the two groups of participants, and the participants were followed up (≥1 year) at clinic visits. Logistic multivariate regression analysis was used to analyze the risk factors related to ILD and prognosis in patients with pSS. RESULTS A total of 217 patients with pSS were included, of which 71 (32.7%) participants were diagnosed with ILD (ILD group) at the time of pSS diagnosis, and 146 (67.3%) participants had no obvious ILD (NILD group). Based on follow-up results, logistic multivariate analysis revealed that NLR [relative risk (RR) =1.81, 95% confidence interval (CI): 1.15 to 4.73], age (RR =1.43, 95% CI: 1.06 to 3.66), non-regular treatment (RR =1.39, 95% CI: 1.03 to 3.38), ALB

Objective To evaluate the safety and utility of core needle biopsy (CNB) for diagnosis of salivary gland lymphoma in Sjogren's syndrome (SS). Methods We analyzed data from consecutive SS patients who underwent ultrasound-guided major salivary gland CNB for lymphoma diagnosis and determined whether CNB yielded an actionable diagnosis without need for further intervention. Results CNB were performed in 24 patients to evaluate discrete parotid (n=6) or submandibular (n=2) gland masses or diffuse enlargement (n=16; 15 parotid). One patient had 3 CNB of the same mass. Of the 26 CNB, 24 included flow cytometry using CNB and/or fine needle aspirate material and 14 targeted sonographically-identified focal lesions. No patient reported complications. In the 23 patients with one CNB, final diagnoses were marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT; n=6), atypical lymphoid infiltration (n=3), benign lymphoepithelial sialadenitis (BLEL, n=9), normal gland tissue (n=4), and lymphoepithelial cyst (n=1). In the patient with serial CNB, the initial one without flow cytometry was benign but the next two showed atypical lymphoid infiltration. Monoclonal lymphoid infiltration was detected in 12 patients: six with MALT lymphoma, three benign and three atypical lymphoid infiltration. Of the latter three, one was treated with rituximab and two with expectant observation. The diagnosis changed from atypical lymphoid infiltration to MALT lymphoma in one patient following biopsy of inguinal adenopathy 6 months post-CNB. CNB provided actionable results and avoided open excisional biopsies in all cases. Conclusion CNB is safe and useful in the evaluation of suspected salivary gland lymphoma in SS.

BACKGROUND Neuromyelitis optica spectrum disorder (NMOSD) is a demyelinating autoimmune disease that affects the central nervous system. It typically manifests as optic neuritis or extensive longitudinal myelitis, with or without the presence of anti-aquaporin protein 4 autoantibodies (immunoglobulin G). CASE SUMMARY We report the case of a 45-year-old woman with a history of Sjogren's syndrome who was diagnosed with NMOSD accompanied by spinal cord injury and left calf intermuscular vein thrombosis. The patient received hormone shock and gamma globulin therapy in the acute phase and standard rehabilitation treatment during convalescence. Upon discharge, the patient was able to control urination and defecation, stand independently, and walk short distances with the aid of a walker. CONCLUSION This case suggests that pharmacotherapy and standard rehabilitation treatment can improve the prognosis of NMSOD patients.

Sjögren’s syndrome (SS) is an autoimmune disease characterized by inflammation with lymphoid infiltration and destruction of the salivary glands. Although many genome-wide association studies have revealed disease-associated risk alleles, the functions of the majority of these alleles are unclear. Here, we show previously unrecognized roles of GTF2I molecules by using two SS-associated single nucleotide polymorphisms (SNPs), rs73366469 and rs117026326 (GTF2I SNPs). We found that the risk alleles of GTF2I SNPs increased GTF2I expression and enhanced nuclear factor-kappa B (NF-κB) activation in human salivary gland cells via the NF-κB p65 subunit. Indeed, the knockdown of GTF2I suppressed inflammatory responses in mouse endothelial cells and in vivo. Conversely, the over-expression of GTF2I enhanced NF-κB reporter activity depending on its p65-binding N-terminal leucine zipper domain. GTF2I is highly expressed in the human salivary gland cells of SS patients expressing the risk alleles. Consistently, the risk alleles of GTF2I SNPs were strongly associated with activation of the IL-6 amplifier, which is hyperactivation machinery of the NF-κB pathway, and lymphoid infiltration in the salivary glands of SS patients. These results demonstrated that GTF2I expression in salivary glands is increased in the presence of the risk alleles of GTF2I SNPs, resulting in activation of the NF-κB pathway in salivary gland cells. They also suggest that GTF2I could be a new therapeutic target for SS.

To evaluate the lower labial minor salivary glands (MSGs) flow rate using fluorescein dye in healthy individuals and patients with Sjögren's syndrome (SS) and Stevens-Johnson syndrome (SJS).Thirty consecutive patients with SS (The mean salivary flow rate from the lower labial MSGs of normal individuals showed decade-wise decline (2.7 ± 0.29The fluorescein-assisted evaluation of the lower lip can be used as a quantitative method for measuring the labial salivary flow rate in SS and SJS patients.

Objective . A case study was used to discuss the effects of Traditional Chinese Medicine (TCM) treatments on Sjogren's syndrome. Clinical features and outcomes . A 45-year-old woman suffered from dry eyes, dry mouth, and fatigue for six months and was diagnosed with Sjogren's syndrome. She had received regular treatment with hydroxychloroquine (HCQ) and artificial tears as well as artificial saliva for nearly one year, but the results were unsatisfactory. Therefore, she sought CHM for further intervention. After 7 months of Yi-Guan-Jian with Huai-Xiao-Mai (Triticum aestivum Linn.) and Tian-Hua-Fen (Trichosanthis Radix), on the seventh treatment with TCM, she reported no fatigue or sleep dysfunction and relief of dry eyes and mouth. Neither complications nor side effects were noted during the CHM treatment. Conclusions . From this case, we concluded that CHM may be an effective and safe alternative therapy for the treatment of Sjogren's syndrome.

SS is usually described as having severe fatigue, dryness, diffuse pain, glandular swelling, and various extraglandular (systemic) manifestations. Clinical trials have generally failed because the vast majority of enrolled patients had no extraglandular manifestations at the time of enrolment but suffered from fatigue, dryness and pain that did not significantly respond to the study medication. A number of hypotheses on the pathogenesis of pSS have been put forward, including disturbances of innate and adaptive immunity as well as abnormalities of the interface between immune disorders and the neuro-endocrine system related to lacrimal and secretory gland dysfunction. Thus, future therapies must be designed for improvement of the symptoms of dry eyes and dry mouth, extraglandular disease, and fatigue and cognitive deficits. Given the inadequacies and limitations of current treatment options, we suggest that innovative directions involving interactions with neuroscientists and neuropsychiatrists together or combined with new immune targeting may be hold promise for better treating pSS.

The information about comorbidities (excluding lymphoma) in primary Sjögren’s syndrome (pSS) is relatively scarce. Cardiovascular disease, infections, musculoskeletal conditions or malignancy are likely the most relevant comorbid conditions in pSS. Different infections (particularly oral candidal infections) and fibromyalgia are extremely frequent in the daily clinical practice. On the other hand, the incidence of cardiovascular events and cancer in pSS is low, so information about them comes from large epidemiological studies or meta-analysis. For this reason, preclinical vascular disease is investigated by different techniques, demonstrating the presence of early atherosclerosis in pSS patients. Coronary events could be slightly more frequent in pSS than in the general population. The overall risk of malignancy in pSS patients seems to be slightly increased, likely due to excess occurrence of lymphoma. An association between pSS and thyroid cancer might exist, although it should be confirmed by further investigations.

To assess the risk factors for severe visual impairment (SVI) and corneal complications in primary and secondary Sjögren syndrome (SS).Retrospective case series.Ocular data of all consecutive SS patients presenting to an eye-care network and receiving a diagnosis according to 2012 American College of Rheumatology criteria over the past 8 years were reviewed.risk factors associated with SVI (best-corrected visual acuity20/200) and vision-threatening corneal complications (ulceration or perforation) at presentation were evaluated using multivariate analysis and odds ratios (OR).Of the 919 patients, 285 (31%) had primary and 634 (69%) had secondary SS. The most common cause of secondary SS was rheumatoid arthritis (98.1%), followed by systemic lupus erythematosus (0.79%), psoriasis (0.79%), and scleroderma (0.6%). Among the 1,838 eyes, SVI was noted in 10%, and 2.5% had corneal complications at presentation. The presence of corneal scarring (P.00001; OR: 3.00), corneal ulceration (P.00001; OR: 12.96), low Schirmer values (P = .0084; OR: 0.93), cataract (P = .0036; OR: 2.4), glaucoma (P = .04; OR: 4.09), and age at diagnosis (P = .005; OR: 1.02) were independent risk factors for developing SVI. The risk factors for corneal complications were presence of scleritis (P.0001; OR: 8.9) and a diagnosis of secondary SS (P = .009; OR: 2.94).In patients with SS, severity of dryness, corneal ulceration and scarring, cataract, and glaucoma are factors associated with poor visual acuity. Eyes with scleritis have a greater risk of developing vision-threatening corneal complications and therefore should be monitored closely.

Background The aim of this study was to evaluate the in vivo confocal microscopic morphology of corneal subbasal nerves and its relationship with clinical parameters in patients with primary Sjögren’s syndrome in China. Methods This was a case control study of 22 dry eye disease (DED) patients with primary Sjögren’s syndrome (pSS) and 20 control subjects with non-Sjögren dry eye disease (NSDE). Each patient underwent an evaluation of ocular surface disease using the tear film break-up time (TBUT), noninvasive tear film break-up time (NIKBUT), noninvasive tear meniscus height (NIKTMH), corneal staining (National Eye Institute scale, NEI), Schirmer I test, meibography, and corneal subbasal nerve analysis with in vivo confocal microscopy (IVCM). The right eye of each subject was included in this study. Results SS patients showed a shorter TBUT (P = 0.009) and Schirmer I test results (P = 0.028) than the NSDE group. However, there was no significant difference in NIKBUT between the two groups (P = 0.393). The nerve density of subbasal nerves, number of nerves and tortuosity of the SS group were significantly lower than those of the NSDE group (P = 0.001, P P = 0.039, respectively). In the SS group, the mean nerve length was correlated with age and the Schirmer I test (r = − 0.519, P = 0.013 and r = 0.463, P = 0.035, respectively). Corneal staining was correlated with nerve density and the number of nerves (r = − 0.534, P = 0.013 and r = − 0.487, P = 0.025, respectively). Conclusions Sjögren syndrome dry eye (SSDE) patients have more severe clinical dry eye parameters than non-Sjögren dry eye disease (NSDE) patients. Compared with NSDE patients, we found that SSDE patients showed decreased corneal subbasal nerve density and numbers.

Objective The aim at the current study was to investigate the clinical characteristics and risk factors of Raynaud’s phenomenon (RP) in patients with primary Sjögren’s syndrome (pSS). Methods Retrospective analysis of the medical records of 333 new-onset pSS patients was performed. Demographic, clinical, and serological data were compared between individuals with and without RP. Logistic regression analysis was used to identify risk factors. Results RP was present in 11.41% of the pSS patients. pSS-RP patients were younger (49.74±14.56 years vs. 54.46±13.20 years, p=0.04) and exhibited higher disease activity (11 [5.75–15] vs. 7 [4–12], p=0.03) than those without. The prevalence of lung involvement was significantly higher in pSS patients with RP (60.53% vs. 17.29%; pp=0.003, p=0.04), anti-RNP positive status (OR=79.41, 95% CI 12.57–501.78; pp=0.002) as prognostic factors for pSS-RP. Conclusion The presence of RP defined a subset of pSS with a unique phenotype, manifesting as increased lung involvement and a higher frequency of anti-RNP antibodies and ACA, as well as greater disease activity. These results suggest that RP has clinical and prognostic value of pSS patients. Further prospective studies with a larger number of subjects are warranted to confirm our findings and assess the prognostic and treatment implications of RP in pSS patients. Key Points• Raynaud’s phenomenon (RP) was present in 38 (11.41%) of 333 patients with primary Sjögren’s syndrome (pSS), with patients with RP exhibiting a younger age and higher disease activity.• The presence of RP indicates a subset of pSS with a unique phenotype, with manifestations including increased lung involvement and a higher frequency of anti-RNP antibodies and anti-centromere antibodies.• Patients with pSS and RP need close follow-up and long-term observation (including assessment of microangiopathy), with specific attention paid to the possible development of clinical features of systemic sclerosis.