Inhibition of DJ-1 protects from lupus onset and severity.

The DJ-1 protein, known as an oxidative stress sensor, plays an important role in immunological processes. Using DJ-1 gene knock-out mice, we identified DJ-1 as a positive regulator in systemic lupus erythematosus (SLE). DJ-1 deficiency significantly alleviated skin lesion, splenomegaly, lymphadenopathy, while reducing multiple autoantibodies and immunoglobulin levels.

Risk factors and predictive model for pulmonary arterial hypertension in adult idiopathic-inflammatory-myopathy patients: A cross-sectional study.

Objective: To identify clinical and laboratory risk factors for Pulmonary Arterial Hypertension (PAH) in Idiopathic-Inflammatory-Myopathy (IIM) patients as well as construct a predicting model for PAH.

Methods: An IIM cohort in southeastern China was established, including 504 adult IIM patients who met the inclusion and exclusion criteria, and were hospitalized at four divisions of the First Affiliated Hospital, Zhejiang University School of Medicine (FAHZJU) from January 1st, 2018, to April 30st 2022. Serum cytokine profiles, myositis-specific antibodies as well as other factors of patients who met the inclusion and exclusion criteria were collected and analyzed.

Real-world efficacy and safety of azvudine in hospitalized older patients with COVID-19 during the omicron wave in China: A retrospective cohort study.

Debates persist regarding the efficacy and safety of azvudine, particularly its real-world outcomes. This study involved patients aged ≥60 years who were admitted to 25 hospitals in mainland China with confirmed SARS-CoV-2 infection between December 1, 2022, and February 28, 2023. Efficacy outcomes were all-cause mortality during hospitalization, the proportion of patients discharged with recovery, time to nucleic acid-negative conversion ( ), time to symptom improvement ( ), and time of hospital stay ( ).

Ga-FAPI-04 PET/CT Imaging for Assessing Renal Tubulointerstitial Fibrosis in Lupus Nephritis.

The objective of this study was to evaluate the feasibility of using Ga-labeled fibroblast activation protein inhibitor-04 (Ga-FAPI-04) PET/CT imaging as a molecular tracer and noninvasive tool for assessing active renal tubulointerstitial fibrosis in patients with lupus nephritis (LN). The study included 29 LN patients who underwent Ga-FAPI-04 PET/CT scanning to quantify Ga-FAPI-04 uptake. Renal biopsies were performed within a week of scanning.

Volume-Based Quantitative Measurement of [F]AlF-NOTA-FAPI-04 PET/CT Uptake Reflects the Disease Activity of IgG4-Related Disease.

Background: To investigate the potential utility of quantitative parameters obtained by F-fibroblast activation protein inhibitor positron emission tomography/computed tomography ([F]AlF-NOTA-FAPI-04 PET/CT) in the assessment of organ involvement and disease activity in IgG4-related disease (IgG4-RD).

Methods: This study enrolled patients who underwent [F]AlF-NOTA-FAPI-04 PET/CT scans at the Department of Rheumatology, The First Affiliated Hospital, Zhejiang University School of Medicine from August 2021 to August 2022. The PET/CT images of the included patients were re-evaluated by PET center technicians, and the maximal standardized uptake value (SUV), metabolic lesion volume (MLV), and total lesion FAPI (TL-FAPI) were used to evaluate the involved organs and tissues that abnormally accumulated [F]AlF-NOTA-FAPI-04.

Risk of drug-induced delirium in older patients- a pharmacovigilance study of FDA adverse event reporting system database.

Background: Drug-induced delirium is known risk factors associated with increased morbidity and mortality in older patients. The objective was to evaluate the risk of drug-related delirium in older patients based on the FDA Adverse Event Reporting System (FAERS).

Research Design And Methods: Delirium reports in older patients (age ≥65) extracted from the FAERS database using Open Vigil 2.

An externally validated clinical-laboratory nomogram for myocardial involvement in adult idiopathic-inflammatory-myopathy patients.

Objectives: This study aimed at identifying clinical and laboratory risk factors for myocardial involvement (MI) in idiopathic inflammatory myopathies (IIMs) patients as well as constructing a risk-predicted nomogram for prediction and early identification of MI.

Methods: An IIMs cohort in southeastern China was constructed, including 504 adult IIMs patients who met the inclusion and exclusion criteria, and were hospitalized at four divisions of the First Affiliated Hospital, Zhejiang University School of Medicine from January 1st 2018 to April 30st 2022. After dividing patients into the training cohort and the validation cohort, risk factors for MI were identified through least absolute shrinkage and selection operator regression and multivariate logistic regression.

Mechanisms of rainfall-induced landslides and interception dynamic response: a case study of the Ni changgou landslide in Shimian, China.

Geological hazards, especially landslides and mudslides, are frequent in Caoke County, Sichuan Province, China. In September 2022, the mechanical parameters of the soil were obtained through a basic investigation of the landslide characteristics of Ni changgou. Upon that, the finite element-discrete element method was used to reconstruct the three-dimensional numerical model of the landslide on the right bank of Ni changgou, and the initiation mechanism of rainfall on landslide and the formation of debris flow impact dam process were simulated.

Differential diagnostic performance of PET/CT in adult-onset still's disease and lymphoma: a retrospective pilot study.

Background: Adult-onset still's disease (AOSD) and lymphoma are the common causes of fever of unknown origin (FUO) and show some similar clinical symptoms. This study aimed to establish a reliable and easy-to-used scoring model based on clinical information, laboratory characteristics and F-fluorodeoxyglucose positron emission tomography/computer tomography (F-FDG PET/CT) images for the differential diagnosis of these two diseases.

Methods: A development cohort including 70 AOSD and 37 lymphoma patients was used to establish a scoring model based on the features of PET/CT images.

Ferritin triggers neutrophil extracellular trap-mediated cytokine storm through Msr1 contributing to adult-onset Still's disease pathogenesis.

Hyperferritinemic syndrome, an overwhelming inflammatory condition, is characterized by high ferritin levels, systemic inflammation and multi-organ dysfunction, but the pathogenic role of ferritin remains largely unknown. Here we show in an animal model that ferritin administration leads to systemic and hepatic inflammation characterized by excessive neutrophil leukocyte infiltration and neutrophil extracellular trap (NET) formation in the liver tissue. Ferritin-induced NET formation depends on the expression of peptidylarginine deiminase 4 and neutrophil elastase and on reactive oxygen species production.

Performance of the modified Systemic Manifestation Score for systemic juvenile idiopathic arthritis in Adult-onset Still's disease.

Objectives: To compare the ability of the modified Systemic Manifestation Score (mSMS) and the mPouchot score to distinguish adult-onset Still's disease (AOSD) with high disease severity in a large cohort.

Methods: We scored the disease severity of 174 patients and categorized them into high and low disease severity states. The correlation of mSMS and mPouchot score with ESR, CRP, ferritin, liver function tests, and serum cytokines was investigated.

Enhanced type I interferon signature induces neutrophil extracellular traps enriched in mitochondrial DNA in adult-onset Still's disease.

Adult-onset Still's disease (AOSD) is a rare but clinically well-known auto-inflammatory disorder. Cytokine storm, the hallmark of AOSD, is mediated by neutrophil hyperactivation and enhanced neutrophil extracellular trap (NET) formation. Type I interferons (IFNs), having a primary role in the initiation of proinflammation responses, can induce subsequent inflammatory cytokine production.

The Role of Advanced Imaging in Gout Management.

Gout is a common form of inflammatory arthritis where urate crystals deposit in joints and surrounding tissues. With the high prevalence of gout, the standardized and effective treatment of gout is very important, but the long-term treatment effect of gout is not satisfied because of the poor adherence in patients to the medicines. Recently, advanced imaging modalities, including ultrasonography (US), dual-energy computed tomography (DECT), and magnetic resonance imaging (MRI), attracted more and more attention for their role on gout as intuitive and non-invasive tools for early gout diagnosis and evaluation of therapeutic effect.

The high prevalence of abnormal magnetic resonance imaging findings in non-neuropsychiatric patients with persistently positive anti-phospholipid antibodies.

Objectives: Thrombosis occurring in the central nervous system is common in APS patients, leading to neuropsychiatric symptoms. We investigated the prevalence of silent brain abnormalities on MRI in primary APS (PAPS) patients and aPL carriers and assessed the association between the vascular risk factors, aPL profile, clinical manifestations and MRI abnormalities.

Methods: We consecutively included 44 PAPS patients, 24 aPL carriers and 23 healthy controls with comparable age and gender in a single-centre, observational, cross-sectional study.

Serum VEGF-C as an evaluation marker of disease activity in adult-onset Still's disease.

In view of the possible involvement of vascular endothelial growth factor-C (VEGF-C) in pathogenesis of adult-onset Still's disease (AOSD) based on our previous genome-wide association study (GWAS) results, the primary objective of this study, therefore, was to investigate the correlations between the content of VEGF-C in serum and clinical and biochemical markers of AOSD. Blood samples were collected from 80 patients with AOSD, 26 with rheumatoid arthritis (RA), 30 with systemic lupus erythematosus (SLE) and 31 healthy control subjects. The serum VEGF-C levels were determined using an enzyme-linked immunosorbent assay (ELISA).

Urine Proteomics Differentiate Primary Thrombotic Antiphospholipid Syndrome From Obstetric Antiphospholipid Syndrome.

Antiphospholipid syndrome (APS) is a multisystem disorder characterized by thrombosis and/or recurrent fetal loss. This clinical phenotype heterogeneity may result in differences in response to treatment and prognosis. In this study, we aimed to identify primary thrombotic APS (TAPS) from primary obstetric APS (OAPS) using urine proteomics as a non-invasive method.

Characteristics of purified anti-β2GPI IgG N-glycosylation associate with thrombotic, obstetric and catastrophic antiphospholipid syndrome.

Objective: Anti-β-2 glycoprotein I (anti-β2GPI) antibodies, defined as primary pathogenic antibody in antiphospholipid syndrome (APS). It has been reported that IgG Fc N-glycosylation affects IgG effector, we aim to investigate the association of Fc glycosylation profiles of purified anti-β2GP1 IgG with clinical features of APS.

Methods: We purify anti-β2GPI IgG and total IgG from 82 APS patients including nine catastrophic antiphospholipid syndrome (CAPS) patients, as well as total IgG from 103 healthy controls to quantitatively analyse all detectable Fc N-glycanforms of all IgG subclasses with Multiple Reaction Monitoring (MRM) method based on UPLC-ESI-QqQ mass spectrometry.

Total metabolic lesion volume of lymph nodes measured by F-FDG PET/CT: a new predictor of macrophage activation syndrome in adult-onset Still's disease.

Background: To investigate the potential utility of quantitative parameters obtained by F-fluorodeoxyglucose positron emission tomography/computed tomography (F-FDG PET/CT) in the assessment of disease severity and the occurrence of macrophage activation syndrome (MAS) in adult-onset Still's disease (AOSD).

Methods: Fifty-seven patients with AOSD who underwent pre-treatment F-FDG PET/CT were recruited in this study and compared with 60 age- and sex-matched healthy controls. Clinical features and laboratory data were recorded.

Cytokine Storm in Coronavirus Disease 2019 and Adult-Onset Still's Disease: Similarities and Differences.

The catastrophic outbreak of coronavirus disease 2019 (COVID-19) is currently a public emergency. Adult-onset Still's disease (AOSD) is an autoinflammatory disease characterized by life-threatening complications. Systemic hyperinflammation and cytokine storm play a critical role in the pathogenesis of both COVID-19 and AOSD.

Characteristics and risk factors of relapses in patients with adult-onset Still's disease: a long-term cohort study.

Objectives: To describe the detailed characteristics and explore the potential risk factors of relapses in patients with adult-onset Still's disease (AOSD).

Methods: We enrolled patients with AOSD admitted to the Department of Rheumatology and Immunology, Ruijin Hospital from August 2016 to September 2019. Kaplan-Meier curves and the log rank test were used to estimate the cumulative relapse probability and persistent remission rate before the first occurrence of relapse.

A Cohort Study of Liver Involvement in Patients With Adult-Onset Still's Disease: Prevalence, Characteristics and Impact on Prognosis.

Adult-onset Still's disease (AOSD) is a systemic disorder commonly accompanied by liver involvement. This study aims to illustrate the detailed information of liver abnormalities in patients with AOSD and evaluate the impact on the prognosis. A total number of 128 hospitalized patients, who met the Yamaguchi criteria of AOSD in the Department of Rheumatology and Immunology, Ruijin Hospital from July 2016 to August 2019 were consecutively enrolled and followed up.

Association of the Leukocyte Immunoglobulin-like Receptor A3 Gene With Neutrophil Activation and Disease Susceptibility in Adult-Onset Still's Disease.

Objective: Adult-onset Still's disease (AOSD) is a severe autoinflammatory disease. Neutrophil activation with enhanced neutrophil extracellular trap (NET) formation is involved in the pathogenesis of AOSD. Functional leukocyte immunoglobulin-like receptor A3 (LIR-A3; gene name LILRA3) has been reported to be associated with many autoimmune diseases.

Pregnancy Outcomes in Patients With Adult-Onset Still's Disease: A Cohort Study From China.

Adult-onset Still's disease (AOSD) is an autoinflammatory disease with a higher prevalence rate in young females. The purpose of this study is to investigate whether AOSD has an adverse impact on pregnancy outcomes, or conversely exacerbated by pregnancy. The outcomes of 191 pregnancies were evaluated in 86 female patients with AOSD.

Circulating Neutrophil Extracellular Traps Signature for Identifying Organ Involvement and Response to Glucocorticoid in Adult-Onset Still's Disease: A Machine Learning Study.

Adult-onset Still's disease (AOSD) is an autoinflammatory disease with multisystem involvement. Early identification of patients with severe complications and those refractory to glucocorticoid is crucial to improve therapeutic strategy in AOSD. Exaggerated neutrophil activation and enhanced formation of neutrophil extracellular traps (NETs) in patients with AOSD were found to be closely associated with etiopathogenesis.