Salvage Autologous Transplant in Relapsed Multiple Myeloma: Long-Term Follow-Up of the Phase 3 GMMG ReLApsE Trial.

The multicenter, phase III GMMG ReLApsE trial (EudraCT-No:2009-013856-61) randomized relapsed and/or refractory multiple myeloma (RRMM) patients equally to lenalidomide/dexamethasone (LEN/DEX, 25mg days 1-21/40mg weekly, 4-week cycles) re-induction, salvage high dose chemotherapy (sHDCT, melphalan 200mg/m2), autologous stem cell transplantation (ASCT) and LEN maintenance (10mg/day; transplant arm, n=139) versus continuous LEN/DEX (control arm, n=138). Ninety-four percent of patients had received frontline HDCT/ASCT. We report an updated analysis of survival endpoints with a median follow-up of 99 months.

Conditional requirement for dimerization of the membrane-binding module for BTK signaling in lymphocyte cell lines.

Bruton's tyrosine kinase (BTK) is a major drug target in immune cells. The membrane-binding pleckstrin homology and tec homology (PH-TH) domains of BTK are required for signaling. Dimerization of the PH-TH module strongly stimulates the kinase activity of BTK in vitro.

Utilising bioinformatics and systems biology methods to uncover the impact of dermatomyositis on interstitial lung disease.

Objectives: Dermatomyositis (DM) is frequently associated with interstitial lung disease (ILD); however, the molecular mechanisms underlying this association remain unclear. This study aimed to employ bioinformatics approaches to identify potential molecular mechanisms linking DM and ILD.

Methods: GSE46239 and GSE47162 were analysed to identify common differentially expressed genes (DEGs).

The burden of illness of the organ manifestations of systemic sclerosis: a pragmatic, targeted review.

Objectives: This structured, targeted literature review aimed to assess the mortality, humanistic and economic burden of eight organ manifestations which are commonly experienced by systemic sclerosis patients.

Methods: Identification of relevant literature was carried out by searching in Ovid MEDLINE and EMBASE, PubMed, and NHS Economic Evaluation Database in August 2023. Studies reporting original data on patients with systemic sclerosis with at least one of eight organ manifestations (interstitial lung disease and/or pulmonary hypertension, skin, peripheral vascular, musculoskeletal, gastrointestinal, cardiac or renal involvement) published within the last 15 years were included.

Cluster analysis identifies the differential impact of disease activity and severity on functional status and patient satisfaction in rheumatoid arthritis: the FRANK registry.

Objectives: The purpose of the present study was to investigate the differential impact of disease activity and severity on functional status and patient satisfaction in rheumatoid arthritis (RA) using cluster analysis on data from the FRANK registry.

Methods: Data from 3,619 RA patients in the FRANK registry were analysed. Patients were grouped using hierarchical and k-means cluster analyses based on age, physician's global assessment (PhGA), patient's pain assessment (PtPA), and Steinbrocker stage.

The association between radiographic progression, functional impairment, markers of dyslipidaemia and inflammation in patients with hand osteoarthritis: a five-year longitudinal study.

Objectives: This study aimed to investigate the associations between radiographic damage, serum biomarkers, and clinical assessments in Czech patients with hand osteoarthritis (HOA) over a five-year follow-up period.

Methods: The study cohort comprised 129 patients diagnosed with HOA, including 72 patients with an erosive subtype and 57 patients with a non-erosive subtype. Radiographs were evaluated using the Kallman scoring system by two independent readers.

Prevalence and Significance of the Presence of Anti-transglutaminase and Anti-endomysium Antibodies in Patients with Early Inflammatory Joint Disease.

Introduction: Celiac disease (CD) affects the small intestine, leading to a progressive disappearance of intestinal villi, and can be found in association with several other autoimmune and inflammatory conditions. The main objective of this study was to determine the prevalence and the clinical significance of anti-transglutaminase and anti-endomysium antibodies in patients diagnosed with early rheumatoid arthritis (RA) and spondyloarthritis (SpA).

Methods: We measured anti-transglutaminase and anti-endomysium antibodies in biobanked serum samples at inclusion in two French prospective multicenter cohorts of patients with suspected early rheumatoid arthritis (ESPOIR, n = 713) and spondyloarthritis (DESIR, n = 709).

Hepatitis B vaccine and juvenile idiopathic arthritis: comparison of the seropositivity rates with healthy children at the time of diagnosis and booster dose response under treatment.

Introduction/objectives: The study aimed to determine whether in children with newly diagnosed juvenile idiopathic arthritis (JIA) hepatitis B surface antibody (anti-HBs) differs from healthy children and to see whether the revaccination is safe and effective under JIA treatment.

Methods: Patients who were followed up with a diagnosis of JIA between January 2020 and February 2024 were included. The control group consisted of healthy children matched for age and gender.

Refractory psoriatic arthritis: emerging concepts in whole process management.

Psoriatic arthritis (PsA) is a chronic and progressive inflammatory arthritis associated with psoriasis, mainly affecting the axial and peripheral joints, characterized by a wide range of complex phenotypes, significant heterogeneity, and a multifactorial etiology. To effectively address the distinct challenges in managing PsA, a pivotal emphasis is placed on clarifying the concept of refractory PsA. Here, we propose a distinction between refractory PsA, differentiating between difficult-to-treat PsA (D2T PsA) and Pseudo-D2T PsA.

Associated diseases and their effects on disease course in patients with chronic non-bacterial osteomyelitis: retrospective experience from a single center.

Objective: Chronic non-bacterial osteomyelitis (CNO) is a rare autoinflammatory bone disease associated with other chronic inflammatory diseases such as familial Mediterranean fever (FMF), juvenile idiopathic arthritis (JIA), spondylarthropathies, inflammatory bowel disease (IBD), and pyoderma gangrenosum. We aimed to describe the clinical and follow-up characteristics of patients with CNO and to compare findings between patients with and without comorbidities.

Methods: The clinical records of patients with CNO who were followed up in our pediatric rheumatology clinic between 2018 and 2023 were reviewed.

Fracture liaison service (FLS) is associated with lower subsequent fragility fracture risk and mortality: NoFRACT (the Norwegian capture the fracture initiative).

Unlabelled: Subsequent fracture rates and associated mortality were compared before and after the introduction of fracture liaison service (FLS). In 100,198 women and men, FLS was associated with 13% and 10% lower risk of subsequent fragility fractures and 18% and 15% lower mortality. The study suggests that FLS may prevent fractures.

Low Bone Mineral Density and Associated Factors Among People Living With HIV in Kerman, Iran: A Cross-Sectional Study in 2021-2022.

Chronic diseases such as osteoporosis and low bone mineral density (BMD) are significant public health concerns for people living with HIV (PLWH), especially with the increased life expectancy because of antiretroviral therapy (ART). This study evaluated the prevalence and associated factors of low BMD among 94 PLWH in Kerman, Iran, from September 2021 to February 2022. Using dual-energy X-ray absorptiometry, BMD was measured, with low BMD defined by specific T-scores and Z-scores.

Polyarteritis nodosa with life-threatening intracranial aneurysms in a child, and treatment with infliximab.

Background: Polyarteritis nodosa (PAN) is a rare and serious form of systemic necrotizing vasculitis that predominantly affects medium and small-sized arteries, with central nervous system involvement being particularly uncommon. Treatment strategies are tailored according to the extent and severity of the disease. While conventional therapy includes glucocorticoids and conventional disease-modifying-rheumatic drugs (cDMARDs), biologic agents may be critical for severe and refractory cases.

Anti-SRP myositis: a diagnostic and therapeutic challenge.

Background: Anti-signal recognition protein (anti-SRP) myopathy is a rare idiopathic inflammatory myopathy in children. Herein, a 3-year-old patient with severe anti-SRP myopathy showing a rapidly progressive disease course is presented in order to increase the awareness of pediatricians about idiopathic inflammatory myopathies.

Case Presentation: A previously healthy 3-year-old girl presented with progressive symmetrical proximal muscle weakness that caused difficulty in climbing stairs for two months prior to evaluation, and a marked elevation of the serum creatine kinase levels.

Assessment of Health Literacy in Parents of Children With Familial Mediterranean Fever Using the Turkish Health Literacy Scale.

Background Health literacy (HL) refers to the ability of individuals to find, understand, and use information and resources to make informed health-related decisions and actions for themselves and others. Managing chronic diseases in children and adolescents requires active family involvement. The primary objective of the study is to evaluate the HL levels of parents of children diagnosed with familial Mediterranean fever (FMF).

Protocol for the MERINO study: A randomized placebo-controlled trial assessing the efficacy, safety, and cost-effectiveness of methotrexate in people with erosive hand osteoarthritis.

Objective: Previous studies on the efficacy of methotrexate in people with hand osteoarthritis (OA) have shown conflicting results. The MERINO trial aims to investigate the efficacy and safety of methotrexate in people with painful inflammatory erosive hand OA.

Design: In total 163 participants with erosive hand OA, synovitis by ultrasound, and finger joint pain of 40-80 ​mm on a visual analogue scale (VAS) will be recruited from a rheumatology outpatient clinic.

Paradoxical Inflammatory Bowel Disease Induced by Golimumab in a Patient With Ankylosing Spondylitis: A Case Report and Systematic Review.

Paradoxical reactions (PRs) to biologic medications, such as psoriasis, arthritis, and inflammatory bowel disease (IBD), have been increasingly recognized. The aim of reporting this case is to establish an association between golimumab and exacerbation or new (de novo) IBD in patients with axial spondyloarthritis (SpA). Our case involves a young patient with juvenile-onset ankylosing spondylitis (AS) who developed de novo IBD following golimumab therapy for active spinal disease.

Macrophage Activation Syndrome/Secondary Hemophagocytic Lymphohistiocytosis in Adult-Onset Still's Disease: An Uncommon Initial Presentation in a Young Nepalese Female: A Case Report.

Hemophagocytic lymphohistiocytosis (HLH), is a fatal systemic hyperinflammatory syndrome. HLH may be due to immunosuppression, infections, cancer, or autoimmune diseases with fever and cytopenia. HLH which occurs in adult-onset Stills disease (AOSD) is called secondary HLH, also known as macrophage activation syndrome (MAS).

Effects of Genetic Risk and Lifestyle Habits on Gout: A Korean Cohort Study.

Background: Gout is a type of inflammatory arthritis caused by monosodium urate crystal deposits, and the prevalence of this condition has been increasing. This study aimed to determine the combined effects of genetic risk factors and lifestyle habits on gout, using data from a Korean cohort study. Identifying high-risk individuals in advance can help prevent gout and its associated disorders.

Hypomethylating agents for patients with VEXAS without myelodysplastic syndrome: Clinical outcome and longitudinal follow-up of vacuolization and UBA1 clonal dynamics.

VEXAS syndrome is a haemato-inflammatory disease caused by somatic UBA1 mutations and characterized by cytoplasmic vacuoles in myeloid and erythroid precursor cells. Although there is currently no standard treatment algorithm for VEXAS, patients are generally treated with anti-inflammatory therapies focused on symptom management, with only partial effectiveness. Hypomethylating agents (HMA) have shown promise in VEXAS patients with concomitant myelodysplastic syndrome (MDS), while the efficacy of HMA in VEXAS patients without MDS is largely unknown.

Investigating the impact of microbiome-changing interventions on food decision-making: MIFOOD study protocol.

Background: Obesity is a multifactorial disease reaching pandemic proportions with increasing healthcare costs, advocating the development of better prevention and treatment strategies. Previous research indicates that the gut microbiome plays an important role in metabolic, hormonal, and neuronal cross-talk underlying eating behavior. We therefore aim to examine the effects of prebiotic and neurocognitive behavioral interventions on food decision-making and to assay the underlying mechanisms in a Randomized Controlled Trial (RCT).

Specific plasma metabolite profile in intestinal Behçet's syndrome.

Background: Intestinal Behçet's syndrome (IBS) has high morbidity and mortality rates with serious complications. However, there are few specific biomarkers for IBS. The purposes of this study were to investigate the distinctive metabolic changes in plasma samples between IBS patients and healthy people, active IBS and inactive IBS patients, and to identify candidate metabolic biomarkers which would be useful for diagnosing and predicting IBS.

Genetic insight into dissecting the immunophenotypes and inflammatory profiles in the pathogenesis of Sjogren syndrome.

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).