Clinical manifestations, imaging and treatment of Sjögren's disease: one year in review 2024.

Sjögren's disease is a systemic autoimmune disorder characterised by hyperactivation of B-cells and cytokine production. The condition may evolve from an asymptomatic, indolent course, with glandular involvement, to several extra-glandular systemic manifestations up to lymphoma development. Recent efforts have been undertaken to identify patient phenotypes at risk of developing specific extraglandular manifestations in order to improve patient management.

The clinical phenotype of isolated ocular or oral dryness in Sjögren's disease.

Objectives: To assess if isolated mouth or eye dryness constitutes distinct clinical phenotypes in Sjögren's disease (SjD).

Methods: We analysed 1765 patients meeting the 2016 ACR-EULAR SjD criteria, followed up at four centres in Greece and Italy (Universities of Pisa, Italy, and Athens, Harokopion, and Ioannina, Greece). Patients with isolated mouth or eye dryness were identified and matched 1:2 with those experiencing both symptoms, according to age at SjD diagnosis, gender, and disease duration.

Infections in Sjögren's disease: a clinical concern or not?

Patients with autoimmune diseases are particularly prone to infections due to both the underlying immune dysfunction and the use of immunosuppressive therapies. Sjögren's disease (SjD) serves as a valuable model for studying the complex interplay between autoimmunity and infections. This review focuses on the infection risks associated with SjD, emphasising key areas such as oral, respiratory, and urogenital infections, along with complications arising from systemic infections.

18F-Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography in Large-Vessel Vasculitis During Active and Inactive Disease Stages Is Associated with the Metabolic Profile, but Not the Macrophage-Related Cytokines: A Proof-of-Concept Study.

Giant cell arteritis (GCA) is an autoimmune/autoinflammatory disease affecting large vessels in patients over 50 years old. The disease presents as an acute inflammatory response with two phenotypes, cranial GCA and large-vessel vasculitis (LV)-GCA, involving the thoracic aorta and its branches. 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET-CT) is among the imaging techniques contributing to diagnosing patients with systemic disease.

Eradication Reverses DNA Damage Response Pathway but Not Senescence in Human Gastric Epithelium.

infection induces DNA Double-Strand Breaks (DSBs) and consequently activates the DNA Damage Response pathway (DDR) and senescence in gastric epithelium. We studied DDR activation and senescence before and after the eradication of the pathogen. Gastric antral and corpus biopsies of 61 patients with infection, prior to and after eradication treatment, were analyzed by means of immunohistochemistry/immunofluorescence for DDR marker (γH2AΧ, phosporylated ataxia telangiectasia-mutated (pATM), p53-binding protein (53BP1) and p53) expression.

The Role of Nailfold Capillaroscopy in Evaluating Patients with Interstitial Lung Disease Related to Connective Tissue Disease.

Capillaroscopy is a non-invasive and safe imaging method that allows the evaluation of the microcirculation of the small vessels of the skin. The method's main advantage is the early detection of microvascular changes that may occur in certain connective tissue diseases (CTDs). Today, the presence of specific autoantibodies and capillaroscopic findings are generally accepted and emerge as a powerful diagnostic tool for detecting underlying CTDs in patients with Raynaud's phenomenon.

FHBF: Federated hybrid boosted forests with dropout rates for supervised learning tasks across highly imbalanced clinical datasets.

Although several studies have deployed gradient boosting trees (GBT) as a robust classifier for federated learning tasks (federated GBT [FGBT]), even with dropout rates (federated gradient boosting trees with dropout rate [FDART]), none of them have investigated the overfitting effects of FGBT across heterogeneous and highly imbalanced datasets within federated environments nor the effect of dropouts in the loss function. In this work, we present the federated hybrid boosted forests (FHBF) algorithm, which incorporates a hybrid weight update approach to overcome ill-posed problems that arise from overfitting effects during the training across highly imbalanced datasets in the cloud. Eight case studies were conducted to stress the performance of FHBF against existing algorithms toward the development of robust AI models for lymphoma development across 18 European federated databases.

Boosting the performance of MALT lymphoma classification in patients with primary Sjögren's Syndrome through data augmentation: a case study.

Sjögren's Syndrome (SS) patients with mucosa associated lymphoid tissue lymphomas (MALTLs) and diffuse large B-cell lymphomas (DLBCLs) have 10-year survival rates of 80% and 40%, respectively. This highlights the unique biologic burden of the two histologic forms, as well as, the need for early detection and thorough monitoring of these patients. The lack of MALTL patients and the fact that most studies are single cohort and combine patients with different lymphoma subtypes narrow the understanding of MALTL progression.

Senescent cells in giant cell arteritis display an inflammatory phenotype participating in tissue injury via IL-6-dependent pathways.

Objectives: Age is the strongest risk factor of giant cell arteritis (GCA), implying a possible pathogenetic role of cellular senescence. To address this question, we applied an established senescence specific multimarker algorithm in temporal artery biopsies (TABs) of GCA patients.

Methods: 75(+) TABs from GCA patients, 22(-) TABs from polymyalgia rheumatica (PMR) patients and 10(-) TABs from non-GCA/non-PMR patients were retrospectively retrieved and analysed.

NMR-based metabolomics in giant cell arteritis and polymyalgia rheumatica sequential sera differentiates active and inactive disease.

Objectives: Giant Cell Arteritis-(GCA) is an inflammatory disease following a chronic, relapsing course. The metabolic alterations related to the intense inflammatory process during the active phase and to the rapid impact of steroid treatment, remain unknown. The study aims to investigate the serum metabolome in active and inactive disease state.

Utilisation of primary healthcare services by Sjögren's syndrome patients in the Community of Madrid and associated factors: a population-based cross-sectional study.

Objectives: To describe the utilisation of primary health care (PHC) services and factors associated with its use by patients diagnosed with Sjögren's syndrome (SS).

Methods: Population-based cross-sectional cohort of SS patients in Madrid, Spain (SIERMA). Sociodemographic, diagnostic, clinical and PHC service utilisation variables were studied by bivariate analyses and regression models.

Tocilizumab improves clinical outcome in patients with active corticosteroid-resistant moderate-to-severe Graves' orbitopathy: an observational study.

Background: Graves' orbitopathy (GO) is an autoimmune disorder affecting the orbital fat and muscles. A significant role of IL-6 in the pathogenesis of GO has been described and tocilizumab (TCZ), an IL-6 inhibitor targeting IL-6R has been given in some patients. The aim of our case study was to evaluate the therapeutic outcome of TCZ in non-responders to first line treatments with corticosteroids.

Prevalence and comorbidities of Sjogren's syndrome patients in the Community of Madrid: A population-based cross-sectional study.

Objectives: To estimate the prevalence, sociodemographic characteristics and comorbidities of Sjogren's syndrome (SS) patients in the Community of Madrid.

Methods: A population-based cross-sectional cohort of SS patients was derived from the information system for rare diseases in the Community of Madrid (SIERMA) and confirmed by a physician. The prevalence per 10,000 inhabitants among people aged ≥18years in June 2015 was calculated.

The clinical phenotype of primary Sjögren's syndrome patients with lymphadenopathy.

Objectives: Previous cohort studies have shown that around 10% of patients with primary Sjögren's syndrome (pSS) develop lymphadenopathy during their disease course. However, no studies have described their clinical phenotype. The present study aims to describe the clinical manifestations and laboratory findings of pSS patients presenting long-standing lymphadenopathy.

Small airways dysfunction in patients with systemic sclerosis and interstitial lung disease.

Background: A number of studies report small airways involvement in patients with systemic sclerosis (SSc). Furthermore, small airways dysfunction is increasingly recognized in patients with interstitial lung disease (ILD) of idiopathic or autoimmune etiology. The objectives of this study were to evaluate small airways function in SSc patients with ILD and explore the effect of treatment on small airways function by using conventional and contemporary pulmonary function tests (PFTs).

Experimental models of Sjögren's syndrome: differences and similarities with human disease.

Mouse models have been employed extensively to provide pathogenetic insights into many complex human disorders including systemic autoimmune diseases. The explosion of biotechnology and molecular biology have simplified the procedures to design and generate mouse models with the phenotype of interest. In this line, more than 30 mouse models have been proposed or developed to resemble Sjögren's syndrome (SS) in humans, in an attempt to better understand the pathophysiology of the disease and design more effective treatments.

Towards the identification of novel autoantibodies in Sjögren's syndrome.

Primary Sjögren's syndrome may be difficult to diagnose when antibodies against Ro/SSA are lacking, and can be grouped in at least four clusters indicating different pathophysiological pathways. Novel biomarkers, in particular autoantibodies, would be helpful in diagnosing Sjögren's syndrome and in further identification and characterisation of the clusters.In this review, we describe new technologies that may be utilised in the rapid identification of novel autoantibodies, and an example of how well characterised patients, here from the HarmonicSS cohort, are a prerequisite in the discovery of clinically meaningful biomarkers.

Clinical and laboratory findings of primary Sjögren's syndrome patients without sicca symptoms.

Objectives: Primary Sjögren's syndrome (pSS) is a systemic autoimmune disease characterised by oral and eye dryness. A minority of patients can present without dryness but studies on their clinico-laboratory manifestations are scarce. Our purpose was to describe the clinical phenotype of pSS patients lacking sicca symptoms.