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.

Comparative Imaging Analysis of Kimura's Disease Using F-FDG PET/CT and [F]AlF-NOTA-FAPI-04 PET/CT.

Kimura's disease is a rare, chronic inflammatory disorder characterized by subcutaneous nodules, eosinophilia, and elevated serum IgE levels. It commonly affects young Asian males and typically presents in the head and neck region. Diagnosis is confirmed via histopathological examination, while treatment options include corticosteroids, surgery, and radiotherapy.

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.

Clinical features of macrophage activation syndrome in adult dermatomyositis: A single-center retrospective case-control study.

Background: Little is known about the features of macrophage activation syndrome (MAS) in dermatomyositis, especially the association between rapidly progressive interstitial lung disease (RP-ILD) and MAS.

Objective: To determine the characteristics of MAS in patients with dermatomyositis and their association with RP-ILD.

Methods: This was a retrospective cohort study of 201 dermatomyositis patients at the First Affiliated Hospital of Zhejiang University over a 10-year period.

Predictors of progression in idiopathic inflammatory myopathies with interstitial lung disease.

The idiopathic inflammatory myopathies (IIMs) are a group of connective tissue diseases that afect multiple organ systems, including the lungs. Interstitial lung disease (ILD) is the most common and heterogeneous complication of IIMs, with its degree ranging from mild to fatal. Thus, it is critical to identify clinical features and validated biomarkers for predicting disease progression and prognosis, which could be beneficial for therapy adjustment.

Development and validation of the AF score for diagnosis of adult-onset Still's disease in fever of unknown origin.

Objective: To develop and validate a diagnostic score to identify adult-onset Still's disease (AOSD) in fever of unknown origin (FUO).

Methods: A single center, retrospective case-control study of inpatients with FUO from January 2018 to December 2021. Using clinical and laboratory data from 178 cases with AOSD and 486 cases with FUO, we developed an AOSD/FUO (AF) score with a Bayesian Model Averaging approach.

Ischaemic heart disease in Behçet's syndrome: a systematic review and meta-analysis.

Objectives: Behçet's syndrome (BS) has been reported with cardiovascular involvement. It's still unclear that BS is associated with the increased risk of ischaemic heart disease (IHD). We aimed to conduct a meta-analysis concerning the incidence of IHD in BS and identify the relationship between IHD and BS.

An efficient multilevel thresholding image segmentation method based on the slime mould algorithm with bee foraging mechanism: A real case with lupus nephritis images.

To improve the diagnosis of Lupus Nephritis (LN), a multilevel LN image segmentation method is developed in this paper based on an improved slime mould algorithm. The search of the optimal threshold set is key to multilevel thresholding image segmentation (MLTIS). It is well known that swarm-based methods are more efficient than the traditional methods because of the high complexity in finding the optimal threshold, especially when performing image partitioning at high threshold levels.

Efficacy and Tolerability of Nintedanib in Idiopathic-Inflammatory-Myopathy-Related Interstitial Lung Disease: A Pilot Study.

To initially clarify the efficacy and tolerability of nintedanib in patients with idiopathic-inflammatory-myopathy-related interstitial lung disease (IIM-ILD). A retrospective, real-world analysis was conducted in IIM-ILD patients who regularly received outpatient visit or hospitalization from January 2018 to March 2020 in three centers. And the patients were divided into two groups depending on presence or absence of nintedanib therapy.

Risk of macrophage activation syndrome in patients with adult-onset Still's disease with skin involvement: A retrospective cohort study.

Background: Small case series and case reports indicated that atypical persistent pruritic eruptions (PPEs), another type of skin lesions seen in adult-onset Still's disease (AOSD), imply a worse prognosis than typical evanescent rashes.

Objective: To investigate clinical characteristics and macrophage activation syndrome (MAS) occurrence in AOSD with PPEs.

Methods: A retrospective cohort study analyzed 150 patients with AOSD with rashes at the First Affiliated Hospital of Zhejiang University from January 2013 to December 2019.

Understanding of cytokines and targeted therapy in macrophage activation syndrome.

Macrophage activation syndrome (MAS) is a potentially life-threatening complication of systemic autoinflammatory/autoimmune diseases, generally systemic juvenile idiopathic arthritis and adult-onset Still's disease. It is characterized by an excessive proliferation of macrophages and T lymphocytes. Recent research revealed that cytokine storm with elevated pro-inflammatory cytokines, including IFN-γ, IL-18, and IL-6, may be central to the pathogenesis of MAS.

Community-Acquired Pneumonia and Hospital-Acquired Pneumonia in Adult Patients with Idiopathic Inflammatory Myopathy: Outcome and Antibiotic Therapy.

Introduction: Community-acquired pneumonia (CAP) and hospital-acquired pneumonia (HAP) are common complications in idiopathic inflammatory myopathy (IIM) patients, and are frequently associated with unfavorable outcome as well as prolonged antibiotic therapy. In this study, we intended to clarify whether clinical pulmonary infection score (CPIS) and multiple serum biomarkers are valuable in predicting unfavorable outcomes and prolonged antibiotic therapy in adult IIM patients complicated with CAP or HAP.

Methods: Data of IIM patients with CAP or HAP who were admitted to three tertiary centers from December 2010 to November 2019 were retrospectively collected.

Acute Exacerbation of Interstitial Lung Disease in Adult Patients With Idiopathic Inflammatory Myopathies: A Retrospective Case-Control Study.

This study aimed at clarifying the prevalence, risk factors, outcome, and outcome-related factors of acute exacerbation of interstitial lung disease (AE-ILD) in patients with idiopathic inflammatory myopathy (IIM). Data of IIM patients who were admitted to the First Affiliated Hospital of Zhejiang University (FAHZJU) from September 2007 to September 2019 were retrospectively collected. And the IIM patients with AE-ILD formed the case group.

Iguratimod Inhibits the Aggressiveness of Rheumatoid Fibroblast-Like Synoviocytes.

Objective: Iguratimod, a novel disease-modifying anti-rheumatic drug for the treatment of rheumatoid arthritis, has been approved in China and Japan. Here, we aimed to find whether iguratimod can inhibit the aggressive behavior and promote apoptosis of rheumatoid fibroblast-like synoviocytes (RA-FLSs).

Methods: The proliferation of RA-FLSs was assessed by 5-ethynyl-2'-deoxyuridine test and Cell Counting Kit-8.

Adult-Onset Still Disease Presenting With Dermatomyositis-Like Persistent Pruritic Lesions.

Adult-onset Still disease (AOSD) is a rare autoinflammatory condition. The presence of an evanescent, salmon-pink, nonpruritic rash is one of the major diagnostic criteria for the disease. The rash occurs with fever and subsides with defervescence.

Hemophagocytic Lymphohistiocytosis: Prevalence, Risk Factors, Outcome, and Outcome-related Factors in Adult Idiopathic Inflammatory Myopathies.

Objective: To clarify the prevalence, risk factors, outcome, and outcome-related factors of hemophagocytic lymphohistiocytosis (HLH) in patients with dermatomyositis (DM), polymyositis (PM), or clinically amyopathic dermatomyositis (CADM).

Methods: Data of patients with DM, PM, or CADM who were admitted to the First Affiliated Hospital of Zhejiang University from February 2011 to February 2019 were retrospectively collected. Patients diagnosed with HLH constituted the case group.

Clinical features and current treatments of adult-onset Still's disease: a multicentre survey of 517 patients in China.

Objectives: As a rare systemic autoinflammatory disease, adult-onset Still's disease (AOSD) has heterogeneous clinical manifestations, response to treatment and outcome. This study tried to assess the clinical characteristics, laboratory tests, and treatments of Chinese AOSD patients, and make a retrospective analysis.

Methods: We collected from 7 hospitals in China a total of 517 Chinese patients with AOSD who satisfied the Yamaguchi criteria.

Histopathological diagnosis of persistent pruritic eruptions associated with adult-onset Still's disease.

Aims: Persistent pruritic eruptions (PPEs), presenting with dyskeratotic keratinocytes histologically, are characteristic skin rash in patients with adult-onset Still's disease (AOSD). The lesions may be histologically similar to other entities that present with dyskeratosis. In the present study, we compared the histopathological features between PPEs and other entities presenting with dyskeratosis.

Comparison of IgG4-Related Lymphadenopathy and Multicentric Castleman's Disease: a Retrospective Study.

Background: It can be difficult to distinguish between IgG4-related lymphadenopathy and multicentric Castleman's disease (MCD) because these conditions cannot be differentially diagnosed using immunohistochemical staining alone. In this study, we analyzed the clinical features of IgG4-related lymphadenopathy and MCD patients.

Methods: We retrospectively analyzed 27 patients with MCD, including 20 with plasma cell-type (PC-type) and 7 with hyaline vascular (HV) features (mixed-type).

Usefulness of tocilizumab for treating rheumatoid arthritis with myelodysplastic syndrome: A case report and literature review.

Rationale: Dysregulated immune function in rheumatoid arthritis (RA) might lead to the development of myelodysplastic syndrome (MDS). Serum interleukin-6 (IL-6) concentrations are increased in both RA and MDS patients.

Patient Concerns: A 58-year-old woman presented with severe RA.

Baseline adiponectin and leptin levels in predicting an increased risk of disease activity in rheumatoid arthritis: A meta-analysis and systematic review.

To determine the pathogenic role of adipokines, such as adiponectin and leptin, in rheumatoid arthritis (RA) by investigating whether serum levels of these adipokines correlated with disease activity in RA patients. Medline, Cochrane, EMBASE and Google Scholar were searched for studies published until 5 November 2015 reporting serum levels of leptin and adiponectin and measures of disease activity including DAS scores and radiographic progression scores (such as total change in SHS scores and number of erosions). Secondary outcomes included pain scores, functional status and health questionnaires.

Erratum to "The Characteristics and Significance of Locally Infiltrating B Cells in Lupus Nephritis and Their Association with Local BAFF Expression".

[This corrects the article DOI: 10.1155/2013/954292.].

Acute lymphocytic leukemia mimicking spondyloarthritis in an adolescent: A case report and review of the literature.

The present study describes the case of an 18-year-old adolescent male exhibiting acute lymphocytic leukemia (ALL), complicated by the onset of the symptom of sacroiliitis mimicking spondyloarthritis. Atypical features including an enlarged spleen, poor effects of non-steroidal anti-inflammatory drug therapy, low levels of hemoglobin, a low platelet count, a low neutrophil count and increased levels of monocytes, indicated the possibility of hematological malignancy. Bone marrow examination confirmed the diagnosis of ALL.