A high polygenic risk score is associated with SSA/SSB antibody positivity and early onset in primary Sjögren's disease.

Objectives: To calculate a polygenic risk score (PRS) based on single nucleotide variants (SNVs) previously associated with primary Sjögren's disease (SjD) with genome-wide significance, and determine the genetic risk for SjD stratified by antibodies, sex and age at diagnosis.

Methods: Patients with SjD (n = 1065) were genotyped using Illumina OmniExpressExome chip. Control genotype data were available (n = 7742).

Long-term follow-up in primary Sjögren's syndrome reveals differences in clinical presentation between female and male patients.

Background: Despite men being less prone to develop autoimmune diseases, male sex has been associated with a more severe disease course in several systemic autoimmune diseases. In the present study, we aimed to investigate differences in the clinical presentation of primary Sjögren's syndrome (pSS) between the sexes and establish whether male sex is associated with a more severe form of long-term pSS.

Methods: Our study population included 967 patients with pSS (899 females and 68 males) from Scandinavian clinical centers.

Evaluation of germinal center-like structures and B cell clonality in patients with primary Sjögren syndrome with and without lymphoma.

Objective: Germinal center (GC)-like structures have previously been observed in minor salivary glands (MSG) of patients with primary Sjögren syndrome (pSS). The aim of our study was to explore the prevalence and features of GC-like structures and B cell clonality in patients with pSS with and without lymphoma.

Methods: Based on a nationwide survey in Norway, we included 21 patients with pSS and with a concomitant lymphoma from whom MSG and/or lymphoma biopsies were available.

Association of genes in the NF-κB pathway with antibody-positive primary Sjögren's syndrome.

Primary Sjögren's syndrome (SS) is a systemic autoimmune inflammatory disease characterized by focal lymphocytic infiltrates in the lachrymal and salivary glands and autoantibodies against the SSA/Ro and SSB/La antigens. Experimental studies have shown an activation of NF-κB in primary SS. NF-κB activation results in inflammation and autoimmunity and is regulated by inhibitory and activating proteins.

Genetic associations to germinal centre formation in primary Sjogren's syndrome.

Background: Primary Sjögren's syndrome (pSS) is an autoimmune rheumatic disease mainly characterised by focal mononuclear cell infiltration in the salivary and lacrimal glands, and by the symptoms xerostomia and keratoconjunctivitis sicca. Germinal centre-like structures (GC) are found in the minor salivary glands of approximately 25% of patients. In this study, we aimed to assess genetic variations in pSS patients with GC-like formations (GC+) compared with patients without such formations (GC-).

Risk of non-Hodgkin's lymphoma in primary Sjögren's syndrome: a population-based study.

Objective: Primary Sjögren's syndrome (SS) is associated with an increased risk of non-Hodgkin's lymphoma (NHL), but the reported prevalence and risk vary considerably. The objective of this study was to determine the risk of NHL in a well-defined population-based primary SS cohort in Norway.

Methods: The authors examined all patients fulfilling the American-European Consensus Group criteria for primary SS from 2 Norwegian counties and compared the data to the Cancer Registry of Norway to identify the primary SS patients who had lymphoma.

Association between genetic variants in the tumour necrosis factor/lymphotoxin α/lymphotoxin β locus and primary Sjogren's syndrome in Scandinavian samples.

Objectives: Lymphotoxin β (LTB) has been found to be upregulated in salivary glands of patients with primary Sjögren's syndrome (pSS). An animal model of pSS also showed ablation of the lymphoid organisation and a marked improvement in salivary gland function on blocking the LTB receptor pathway. This study aimed to investigate whether single-nucleotide polymorphisms (SNP) in the lymphotoxin α (LTA)/LTB/tumour necrosis factor (TNF) gene clusters are associated with pSS.