Polyarteritis nodosa presenting with severe upper gastrointestinal bleeding: a case report.

Background: Polyarteritis nodosa is a relatively uncommon type of systemic necrotizing vasculitis that primarily affects medium-sized arteries. While gastrointestinal involvement is known in polyarteritis nodosa, heavy gastrointestinal bleeding due to gastric ulceration is relatively uncommon. We present the case of an 81-year-old male of Chinese ethnicity who experienced severe gastrointestinal bleeding as a result of polyarteritis nodosa and an innovative treatment approach for a better patient outcomes.

Granulomatosis with polyangiitis involving both ischemic and hemorrhagic cerebrovascular disease: A case report and literature review.

Background: Granulomatosis with polyangiitis (GPA) is a necrotizing small-vessel vasculitis associated with antineutrophilic cytoplasmic antibodies (ANCAs). GPA can have multisystem involvement; however, central nervous system (CNS) manifestations are uncommon. Here, for this first time, we report a rare case of GPA with both ischemic and hemorrhagic CNS involvement.

An update on myositis autoantibodies and insights into pathogenesis.

Myositis-specific autoantibodies (MSAs) are hallmarks of idiopathic inflammatory myopathies (IIMs) and have become increasing valuable in disease diagnosis, phenotyping, and classification. In addition to their clinical utility, emerging data, including findings from several animal studies, suggest that MSAs and autoreactive T cells substantially contribute to the etiopathogenesis of IIMs. This review aims to provide an updated perspective on myositis autoantibodies by focusing on relevant clinical and translational studies.

Validation of the 2018 (New) ENMC Classification Criteria for Dermatomyositis in Chinese Patients with Idiopathic Inflammatory Myopathies.

Objectives: To validate the 2018 European Neuromuscular Centre classification (ENMC) criteria, compare its performance to the 1975 Bohan & Peter (B&P) and 2017 European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) criteria for dermatomyositis (DM), and describe characteristics of different myositis-specific autoantibody (MSA)-positive patients defined by the ENMC-DM criteria.

Methods: Medical records and data on MSAs and muscle biopsies were retrospectively obtained from 1370 Chinese patients with idiopathic inflammatory myopathy (IIM) between 2008 and 2020. Patients were diagnosed with DM by at least two rheumatologists and classified according to the ENMC-DM, EULAR/ACR, and B&P criteria.

Revisiting PPAR agonists: novel perspectives in the treatment of primary biliary cholangitis.

Introduction: In primary biliary cholangitis (PBC), approximately 40% of the patients respond incompletely to first-line treatment with ursodeoxycholic acid (UDCA), resulting in a poorer prognosis. Although obeticholic acid (OCA) is approved as a second-line therapy, it is not well-tolerated by patients with significant itching or advanced cirrhosis. Peroxisome proliferator-activated receptor (PPAR) agonists, including fibrates traditionally known as antihyperlipidemic agents, have emerged as potent alternatives for treating PBC patients with an incomplete response to UDCA.

Association of anti-HMGCR antibodies of the IgM isotype with refractory immune-mediated necrotizing myopathy.

Objective: Anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) autoantibodies are one of the myositis-specific antibodies which is associated with immune-mediated necrotizing myopathy (IMNM). However, the relationship between anti-HMGCR isotypes and prognosis has not yet been fully investigated. This study was conducted to gain insight into the association between anti-HMGCR isotypes and clinical, and prognosis in IMNM patients who were positive for anti-HMGCR antibodies.

Clinical characteristics of idiopathic inflammatory myopathies patients with anti-PM/Scl antibodies.

Objectives: To analyze the clinical features of idiopathic inflammatory myopathies (IIMs) patients with anti-PM/Scl antibodies.

Methods: In this retrospective cohort study, we compared the clinical manifestations between patients who were solely positive for anti-PM/Scl antibodies (isolated anti-PM/Scl group) and those with a coexistence of anti-PM/Scl antibodies and myositis-specific antibodies (MSAs) (double-positive group).

Results: Sixty-five IIMs patients positive for anti-PM/Scl antibodies were included, among whom 51 (78.

Distinct phenotype and prognosis of immune-mediated necrotizing myopathy based on clinical-serological-pathological classification.

Objective: The aim of the study was to investigate the characteristics and prognosis of patients with immune-mediated necrotizing myopathy (IMNM) based on clinical, serological and pathological classification.

Methods: A total of 138 patients with IMNM who met the 2018 European Neuromuscular Center criteria for IMNM including 62 anti-SRP, 32 anti-HMGCR-positive and 44 myositis specific antibody-negative were involved in the study. All patients were followed up and evaluated remission and relapse.

Antimelanoma differentiation antigen 5-positive dermatomyositis: an update.

Purpose Of Review: Antimelanoma differentiation antigen 5-dermatomyositis (MDA5-DM) is a complex and serious systemic autoimmune disease that primarily affects the skin and lungs. In this review, we aimed to provide new insights into the clinical features, pathogenesis, and practical management approach for this disease.

Recent Findings: Although lung lesions are prominent in most patients with MDA5-DM, they are now recognized as heterogeneous diseases.

Clinical and prognostic associations of anti-Jo-1 antibody levels in patients with antisynthetase syndrome.

Objective: To investigate the association of serum anti-Jo-1 antibody levels with the disease activity and prognosis in anti-Jo-1-positive patients with antisynthetase syndrome (ASS).

Methods: This study included 115 anti-Jo-1-positive patients with ASS who were admitted to China-Japan Friendship Hospital between 2009 and 2019. Anti-Jo-1 antibody serum levels at initial admission and follow-up were determined by enzyme-linked immunosorbent assay (ELISA).

Prognostic value of elevated lipoprotein (a) in patients with acute coronary syndromes: a systematic review and meta-analysis.

Background: Elevated lipoprotein (a) level was recognized as an independent risk factor for significant adverse cardiovascular events in acute coronary syndrome (ACS) patients. Despite this recognition, the consensus in the literature regarding the prognostic significance of elevated lipoprotein (a) in ACS was also limited. Consequently, we conducted a thorough systematic review and meta-analysis to evaluate the prognostic relevance of elevated lipoprotein (a) level in individuals diagnosed with ACS.

Listening to patients, for the patients: The COVAD Study-Vision, organizational structure, and challenges.

Background: The pandemic presented unique challenges for individuals with autoimmune and rheumatic diseases (AIRDs) due to their underlying condition, the effects of immunosuppressive treatments, and increased vaccine hesitancy.

Objectives: The COVID-19 vaccination in autoimmune diseases (COVAD) study, a series of ongoing, patient self-reported surveys were conceived with the vision of being a unique tool to gather patient perspectives on AIRDs. It involved a multinational, multicenter collaborative effort amidst a global lockdown.

Longitudinal study of patients with anti-SAE antibody positive dermatomyositis: a multicenter cohort study in China.

Objectives: To describe the longitudinal study and long-term prognosis of multicentre large inception cohort of patients with anti-SAE positive DM.

Methods: We retrospectively recruited patients with anti-SAE+DM in four tertiary referral centers from China between March 2005 and December 2022. Long-term survival analysis was performed in the enrolled patients.

Dynamic change in red cell distribution width as a predictor for short-time mortality in dermatomyositis-associated rapid progressive interstitial lung disease.

Aim: We aimed to explore a new and readily available practical marker for rapidly progressive interstitial lung disease (RP-ILD) and poor short-term outcomes in patients with idiopathic inflammatory myopathies (IIM).

Methods: A total of 1822 consecutive patients with IIM between 2009 and 2021 were evaluated retrospectively. All proven cases of naïve ILD with complete medical records were included.

Polymyositis is a rare and favourable outcome subtype of idiopathic inflammatory myopathy in Chinese patients.

Objectives: To investigate the prevalence and characteristics of typical polymyositis (PM) in Chinese patients with idiopathic inflammatory myopathy (IIM).

Methods: Patients diagnosed with IIM according to the 2017 EULAR/ACR criteria were included. Serological aspects including myositis-specific antibodies (MSA) and pathological data were re-evaluated.

Performance of the 2017 EULAR/ACR Classification Criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups: a scoping review.

The 2017 EULAR/ACR classification criteria for adult/juvenile idiopathic inflammatory myopathies (IIM) were established using a data-driven approach by an international group of myositis experts to allow classification of IIM and its major subtypes. Since their publication, the performance of the criteria has been tested in multiple cohorts worldwide and significant limitations have been identified. Moreover, the understanding and classification of IIM have evolved since 2017.

Novel endotypes of antisynthetase syndrome identified independent of anti-aminoacyl transfer RNA synthetase antibody specificity that improve prognostic stratification.

Objectives: To systemically analyse the heterogeneity in the clinical manifestations and prognoses of patients with antisynthetase syndrome (ASS) and evaluate the transcriptional signatures related to different clinical phenotypes.

Methods: A total of 701 patients with ASS were retrospectively enrolled. The clinical presentation and prognosis were assessed in association with four anti-aminoacyl transfer RNA synthetase (ARS) antibodies: anti-Jo1, anti-PL7, anti-PL12 and anti-EJ.

Anti-Toxoplasma gondii antibodies as a risk factor for the prevalence and severity of systemic lupus erythematosus.

Background: Systemic lupus erythematosus (SLE) is a complex systemic autoimmune disease characterized by the presence of numerous autoantibodies. The interaction of infectious agents (viruses, bacteria and parasites) and a genetically susceptible host may be a key mechanism for SLE. Toxoplasma gondii is a widespread intracellular parasite that has been implicated in the pathogenesis of autoimmune diseases.

Fatty infiltration in the posterior muscles of the lower extremities as an MRI feature in antimitochondrial antibody-associated myopathy.

Objective: Idiopathic inflammatory myopathy (IIM) with antimitochondrial M2 antibody (AMA-M2) has been associated with distinct clinical characteristics. In this study, we explore the magnetic resonance imaging (MRI) findings of the muscles of the lower extremities in AMA-M2-positive IIM to gain more insight.

Methods: MRI of 22 lower extremity muscles was retrospectively evaluated in 14 patients with AMA-M2-positive IIM and 37 age- and sex-matched patients with AMA-M2-negative IIM.

Spontaneous pneumomediastinum in anti-MDA5-positive dermatomyositis: Prevalence, risk factors, and prognosis.

Objective: To depict the clinical panorama of spontaneous pneumomediastinum (SPM) in anti-MDA5 antibody-positive dermatomyositis (anti-MDA5+ DM).

Methods: A total of 1352 patients with idiopathic inflammatory myopathy (IIM), including 384 anti-MDA5+ DM patients were retrospectively enrolled. The clinical profiles of anti-MDA5+ DM-associated SPM were analyzed.

Expansion of exhausted CD8+ T cells associates with increased pulmonary fungal infection risk in anti-melanoma differentiation associated gene 5 dermatomyositis.

Objectives: Anti-MDA5+ dermatomyositis was associated with poor prognosis due to the high incidence of rapid progressive interstitial lung disease, pulmonary infection. The aim of this study is to investigate the abundance and clinical relevance of exhaustion markers on peripheral CD8 T cells from patients with idiopathic inflammatory myopathy (IIM).

Methods: Twenty-nine healthy controls (HCs) and 71 patients with IIM were enrolled, including 42 with anti-MDA5+ and 18 with anti-MDA5- dermatomyositis (DM) and 11 with anti-synthetase syndrome (ASS).