Disease-associated immune cell endotypes in anti-MDA5-positive dermatomyositis using unbiased hierarchical clustering.

Objective: Clinical and prognostic features of Anti-MDA5-Positive Dermatomyositis (MDA5+ DM) are diverse. This study aimed to examine the peripheral immune cell profiles of patients with MDA5+ DM, identify disease endotypes related to the heterogeneous manifestations and prognosis, and guide individualized therapy regimen.

Methods: This inpatient cohort included 123 patients with MDA5+ DM.

Severe gastrointestinal involvements in patients with adult dermatomyositis with anti-NXP2 antibody.

Objective: Gastrointestinal (GI) involvements were scarcely reported in adult anti-nuclear matrix protein 2 (NXP2) dermatomyositis (NXP2DM). In this study, we investigated the clinical, pathological and molecular features as well as treatment options of this rare yet life-threatening disease.

Methods: We retrospectively collected the data of the cohort of NXP2 DM from 2012 to 2022 in our hospital.

Immune profile alterations of systemic lupus erythematosus patients with infections.

This study aimed to elucidate the immune status of systemic lupus erythematosus (SLE) patients with infections. We enrolled 253 SLE patients including 77 patients with infections. Clinical features and immunological parameters were analyzed, with particular reference to neutrophil CD64 (nCD64) expression, myeloid-derived suppressor cells (MDSCs), activated T cells and multiple cytokines.

Tmem65 is critical for the structure and function of the intercalated discs in mouse hearts.

The intercalated disc (ICD) is a unique membrane structure that is indispensable to normal heart function, yet its structural organization is not completely understood. Previously, we showed that the ICD-bound transmembrane protein 65 (Tmem65) was required for connexin43 (Cx43) localization and function in cultured mouse neonatal cardiomyocytes. Here, we investigate the functional and cellular effects of Tmem65 reductions on the myocardium in a mouse model by injecting CD1 mouse pups (3-7 days after birth) with recombinant adeno-associated virus 9 (rAAV9) harboring Tmem65 shRNA, which reduces Tmem65 expression by 90% in mouse ventricles compared to scrambled shRNA injection.

Inflammation, new bone formation and aorta.

Objectives: This study describes the characteristics of vertebral osteophytes in different inflammatory and non-inflammatory diseases aiming to reflect the aortic-vertebrae interaction.

Methods: We conducted a cross-sectional study including 4 group of patients, ankylosing spondylitis (AS, n = 52), Takayasu's arteritis (TKA, n = 31), diffuse idiopathic skeletal hyperostosis (DISH, n = 30), coronary artery disease (CAD, n = 10), 100 and also age-matched healthy controls (HC, n = 143). All subjects underwent a chest computed tomography scan and images of the upper and lower border of 7 adjacent thoracic vertebrae (T5 to T12) were captured.

Trisomy 8 Associated Clonal Cytopenia Featured With Acquired Auto-Inflammation and Its Response to JAK Inhibitors.

Objects: It has been recognized the nexus between trisomy 8 and auto-inflammatory features in myelodysplasia syndrome (MDS). Recent research about VEXAS syndrome proved clonal hematopoiesis could interfere with innate immune system far before occurrence of hematological malignancies. We reported a case series of clonal cytopenia with auto-inflammatory features in trisomy 8 patients.

Prevalence and risk factors of active tuberculosis in patients with rheumatic diseases: a multi-center, cross-sectional study in China.

Evidence of active tuberculosis (ATB) in patients with rheumatic diseases are research priorities but limited data from China have been reported. Research targeting patients not taking anti-TNF biologics are especially insufficient. We aimed to investigate the prevalence and risk factors of ATB in this at-risk population.

Description and Analysis of a Novel Subtype of the Anti-Synthetase Syndrome Characterized by Frequent Attacks of Fever and Systemic Inflammation in a Single-Center Cohort Study.

Objectives: The aim of this study was to investigate anti-synthetase syndrome (ASyS) patients who presented with recurrent episodes of fever and systemic inflammation.

Methods: A retrospective cohort of Chinese ASyS patients (n=126) in our center (between January 2013 and January 2020) was included. Patients presenting with concomitant autoimmune rheumatic diseases or malignancies were subsequently excluded.

Methotrexate (MTX) Plus Hydroxychloroquine versus MTX Plus Leflunomide in Patients with MTX-Resistant Active Rheumatoid Arthritis: A 2-Year Cohort Study in Real World.

Purpose: To compare the efficacy, safety, and cost-effectiveness of methotrexate (MTX) plus hydroxychloroquine (HCQ) vs MTX plus leflunomide (LEF) in established rheumatoid arthritis (RA) with inadequate response to MTX monotherapy in a real-world Chinese cohort.

Patients And Methods: A prospective RA cohort (n=549) was screened with eligible patients who had inadequate response (disease activity score in 28 joints using erythrocyte sedimentation rate, DAS28-ESR>3.2) to initial MTX monotherapy and subsequently received either MTX+HCQ or MTX+LEF.

Rituximab or cyclosporin in refractory immune thrombocytopenia secondary to connective tissue diseases: a real-world observational retrospective study.

Immune thrombocytopenia (ITP) is a common complication of connective tissue diseases (CTD). However, refractory and recurrent cases are frequent, who often need intensive immunotherapy. In the real world to compare the efficacy and safety of two common options, rituximab (RTX) and cyclosporine (CsA), in patients with refractory CTD-ITP, we conducted this retrospective study.

Systemic lupus erythematosus gastrointestinal involvement: a computed tomography-based assessment.

Systemic lupus erythematosus (SLE) gastrointestinal (GI) complication is characterized by multi-segment and multi-compartment involvement. The aim of this study is to develop a computed tomography (CT) image-based system for disease evaluation. SLE patients with GI involvement from two independent cohorts were retrospectively included.

Varicella zoster virus infections increase the risk of disease flares in patients with SLE: a matched cohort study.

Objective: To explore whether varicella zoster virus (VZV) infection could increase the risk of disease flares in patients with SLE.

Methods: Patients who had VZV reactivations between January 2013 and April 2018 were included from the SLE database (n=1901) of Shanghai Ren Ji Hospital, South Campus. Matched patients with SLE were selected as background controls with a 3:1 ratio.

Publisher Correction: A Pilot Study on Tocilizumab for Treating Refractory Adult-Onset Still's Disease.

A correction to this article has been published and is linked from the HTML and PDF versions of this paper. The error has been fixed in the paper.

CD4+CXCR4+ T cells as a novel prognostic biomarker in patients with idiopathic inflammatory myopathy-associated interstitial lung disease.

Backgroud: There is an unmet need for the development of new biomarkers for idiopathic inflammatory myopathy-associated interstitial lung disease (IIM-ILD).

Methods: Peripheral CD4+CXCR4+ T cells, stromal cell-derived factor-1 and Krebs von den Lungen-6 were measured in patients with IIM-ILD (n = 85) and controls. The relation to pulmonary functions, high-resolution CT scores, specific clinical phenotypes and survival was analysed.

A Pilot Study on Tocilizumab for Treating Refractory Adult-Onset Still's Disease.

To investigate the efficacy and safety of Tocilizumab (TCZ) in patients with refractory adult-onset Still's disease (AOSD). We enrolled 8 female patients from October 2013 to July 2014. All patients fulfilled Japan's Yamaguch AOSD classification and recognized as refractory AOSD.

The performance of different anti-dsDNA autoantibodies assays in Chinese systemic lupus erythematosus patients.

To compare the performance of different commercial anti-dsDNA autoantibody assays, including multiplex-based immunoassay (Bio-Plex), Farr radioimmunoassay (Farr), ELISA, chemiluminescent immunoassay (CLIA), and Crithidia Luciliae indirect immunofluorescence test (CLIFT) in Chinese patients with systemic lupus erythematosus (SLE). SLE patients (n = 119) as well as healthy controls (n = 200) and disease controls (n = 100) were recruited, and serum anti-dsDNA autoantibodies were detected by Bio-Plex, Farr, two ELISA assays (Medical & Biological Laboratories-ELISA, EUROIMMU-ELISA), CLIA, and a standard CLIFT. The correlation of anti-dsDNA autoantibody levels to SLE disease activity was calculated, and the specificity and sensitivity of these methods were measured by receiver-operator characteristic (ROC) curve analysis.

Pirfenidone in patients with rapidly progressive interstitial lung disease associated with clinically amyopathic dermatomyositis.

To evaluate the efficacy of pirfenidone in patients with rapidly progressive interstitial lung disease (RPILD) related to clinically amyopathic dermatomyositis (CADM), we conducted an open-label, prospective study with matched retrospective controls. Thirty patients diagnosed with CADM-RPILD with a disease duration <6 months at Renji Hospital South Campus from June 2014 to November 2015 were prospectively enrolled and treated with pirfenidone at a target dose of 1800 mg/d in addition to conventional treatment, such as a glucocorticoid and/or other immunosuppressants. Matched patients without pirfenidone treatment (n = 27) were retrospectively selected as controls between October 2012 and September 2015.

Endoscopic diagnosis of invasion depth for early colorectal carcinomas: a prospective comparative study of narrow-band imaging, acetic acid, and crystal violet.

Several studies have validated the effectiveness of narrow-band imaging (NBI) in estimating invasion depth of early colorectal cancers. However, comparative diagnostic accuracy between NBI and chromoendoscopy remains unclear. Other than crystal violet, use of acetic acid as a new staining method to diagnose deep submucosal invasive (SM-d) carcinomas has not been extensively evaluated.

Quadruple therapy with medications containing either rufloxacin or furazolidone as a rescue regimen in the treatment of Helicobacter pylori-infected dyspepsia patients: a randomized pilot study.

Background: The eradication rates of first-line treatment for Helicobacter pylori infection are not satisfactory. Various regimens including quadruple therapies have been recommended as rescue therapies after the first H. pylori eradication attempt failed.