Distinct pathophysiological pathways support stratification of Sjögren's disease based on symptoms, clinical, and routine biological data.

Objective: Recently, three distinct phenotypes of Sjögren's disease (SjD) patients have been described, based on cluster analysis: B-cell active with low symptoms (BALS), high systemic activity (HSA), and low systemic activity with high symptoms (LSAHS). We aimed to assess whether these clusters were associated with distinct biomarkers and the prognostic value of IFN signature.

Methods: The ASSESS cohort is a 20-year prospective cohort of SjD patients.

mA RNA methylation controls salivary gland epithelial cell function and has a protective role in Sjögren's disease.

Objectives: The RNA epitranscriptomic modification known as -methyladenosine (mA) represents a novel mechanism of gene regulation that is poorly understood in human autoimmune diseases. Our research explores the role of this RNA mA modification in salivary gland epithelial cells (SGEC) and its impact on the pathogenesis of Sjögren's disease (SjD).

Methods: SGECs from SjD patients and controls were analysed for mA writers METTL3 and METTL14 expression using RNA-seq, quantitative PCR and immunohistochemistry.

Clinical, biological, prognostic characteristics of patients with immune-mediated thrombotic thrombocytopenic purpura and Sjögren's disease.

Objectives: The association between immune-mediated thrombotic thrombocytopenic purpura (iTTP) and Sjögren disease (SjD) has been poorly investigated. This study presents the first retrospective cohort of iTTP-SjD aiming to identify risk factors for iTTP occurrence in SjD patients and examine their clinical course.

Methods: Patients with iTTP-SjD were identified within the French TTP Registry based on American College of Rheumatology/European League Against Rheumatism 2016 criteria.

Variants in the autoimmune disease risk locus influence the regulatory network in immune cells and salivary gland.

Fine mapping and bioinformatic analysis of the genetic risk association in Sjögren's Disease (SjD) and Systemic Lupus Erythematosus (SLE) identified five common SNPs with functional evidence in immune cell types: rs4938573, rs57494551, rs4938572, rs4936443, rs7117261. Functional interrogation of nuclear protein binding affinity, enhancer/promoter regulatory activity, and chromatin-chromatin interactions in immune, salivary gland epithelial, and kidney epithelial cells revealed cell type-specific allelic effects for all five SNPs that expanded regulation beyond effects on and expression. Mapping the local chromatin regulatory network revealed several additional genes of interest, including .

Immune Checkpoint Inhibitor-induced Sicca Syndrome.

The development of sicca in patients treated with immune checkpoint inhibitors (ICIs) is undoubtedly an underestimated complication, but one whose functional consequences and impact on quality of life are significant for patients. This update aims to review the frequency of this complication and different clinical pictures. The authors also propose a diagnostic and therapeutic approach to guide clinicians in daily practice.

Identification of distinct subgroups of Sjögren's disease by cluster analysis based on clinical and biological manifestations: data from the cross-sectional Paris-Saclay and the prospective ASSESS cohorts.

Background: Sjögren's disease is a heterogenous autoimmune disease with a wide range of symptoms-including dryness, fatigue, and pain-in addition to systemic manifestations and an increased risk of lymphoma. We aimed to identify distinct subgroups of the disease, using cluster analysis based on subjective symptoms and clinical and biological manifestations, and to compare the prognoses of patients in these subgroups.

Methods: This study included patients with Sjögren's disease from two independent cohorts in France: the cross-sectional Paris-Saclay cohort and the prospective Assessment of Systemic Signs and Evolution of Sjögren's Syndrome (ASSESS) cohort.

Inclusion-body myositis associated with Sjögren's disease: clinical characteristics and comparison with other Sjögren-associated myositis.

Objectives: To describe the characteristics of patients with Sjögren's disease (SjD) and inclusion-body myositis (IBM), and how they compare to SjD patients with other inflammatory myopathies (IM).

Methods: Patients were retrospectively recruited from 13 French centers and included if they met the ACR/EULAR criteria for SjD and for IM. They were categorized as SjD-IBM if sub-criteria for IBM were met, or as SjD-other IM if not.

Characterisation of airway disease associated with Sjögren disease.

Objective: Although airway disease associated with Sjögren's disease (Sjo-AD) is common, it is poorly studied compared with interstitial lung disease (ILD). In this study, we aimed to assess factors associated with Sjo-AD, the characteristics and prognosis of this manifestation.

Methods: We performed a retrospective multicentric study involving nine centres.

Pregnancy outcomes in women with primary Sjögren's syndrome: an analysis of data from the multicentre, prospective, GR2 study.

Background: Adverse pregnancy outcomes in women with primary Sjögren's syndrome have only been evaluated retrospectively using heterogeneous methods and with contradictory results. We aimed to describe adverse pregnancy, delivery, and birth outcome risks in pregnant women with primary Sjögren's syndrome compared with those of a matched general population in France, and to identify factors predictive of disease flares or adverse pregnancy outcomes.

Methods: We conducted a multicentre, prospective, cohort study in France using the GR2 (Groupe de Recherche sur la Grossesse et les Maladies Rares) registry.

[F]FDG-PET/CT in Polymyalgia Rheumatica: An Update and Future Aspects.

Polymyalgia rheumatica (PMR) is an inflammatory disorder usually diagnosed in patients older than 50 years of age. It is characterized by sudden onset pain and prolonged morning stiffness in the scapular and/or pelvic girdle, sometimes debilitating and accompanied by constitutional symptoms such as weight loss. In approximately 20% of the cases, it is linked to giant cell arteritis (GCAV) representing a disease continuum.

PET/CT in Inflammatory and Auto-immune Disorders: Focus on Several Key Molecular Concepts, FDG, and Radiolabeled Probe Perspectives.

Chronic immune diseases mainly include autoimmune and inflammatory diseases. Managing chronic inflammatory and autoimmune diseases has become a significant public health concern, and therapeutic advancements over the past 50 years have been substantial. As therapeutic tools continue to multiply, the challenge now lies in providing each patient with personalized care tailored to the specifics of their condition, ushering in the era of personalized medicine.

Comparison between primary Sjögren's disease patients with high or low level of dryness.

Objectives: To describe primary Sjögren's disease (SjD) patients presenting no or low level of dryness and to compare them with SjD patients with oral or ocular dryness features.

Methods: All patients diagnosed with SjD according to AECG or ACR/EULAR criteria in our tertiary reference centre were included. Patients with high or low subjective symptoms or objective signs of dryness were compared.

[Inflammatory diseases of the salivary].

INFLAMMATORY DISEASES OF THE SALIVARY GLANDS. Salivary glands can be affected by inflammatory pathologies. They are most often manifested by the occurrence of swelling of the main salivary glands that can be associated with a dryness.

French national diagnostic and care protocol for Sjögren's disease.

Sjögren's disease (SD), also known as Sjögren's syndrome (SS) or Gougerot-Sjögren's syndrome in France, is a rare systemic autoimmune disease in its primary form and is characterised by tropism for the exocrine glandular epithelia, particularly the salivary and lacrimal glands. The lymphocytic infiltration of these epithelia will clinically translate into a dry syndrome which, associated with fatigue and pain, constitutes the symptom triad of the disease. In about one third of patients, SD is associated with systemic complications that can affect the joints, skin, lungs, kidneys, central or peripheral nervous system, and lymphoid organs with an increased risk of B-cell lymphoma.

Expert Perspective: Challenges in Sjögren's Disease.

The management of Sjögren's disease is challenging because of several factors. Indeed, the clinical presentations are heterogeneous, and one must be able to identify prognostic markers to adapt the follow-up. In addition, there is no validated treatment.

Association Between Bruton's Tyrosine Kinase Gene Overexpression and Risk of Lymphoma in Primary Sjögren's Syndrome.

Objective: We undertook this study to analyze whole blood gene expression and to investigate the role of B cell genes in primary Sjögren's syndrome-related non-Hodgkin lymphoma (primary SS-NHL).

Methods: Peripheral whole blood samples were collected from 345 well-phenotyped patients with primary SS enrolled in the prospective Assessment of Systemic Signs and Evolution in Sjögren's Syndrome (ASSESS) cohort. Transcriptomic analysis was performed using human Clariom S Arrays (Affymetrix).

Impact of patient ancestry on heterogeneity of Sjögren's disease.

Objectives: We aimed to compare disease characteristics between primary Sjögren's syndrome (pSS) patients of African ancestry (AA) and Caucasian ancestry.

Methods: We conducted a retrospective, case-control study in a French national and European referral centre for pSS. All patients with pSS of AA were matched with two Caucasians patients having similar follow-up duration.