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.

Immune-Related Genes Associated with Interstitial Lung Disease in Dermatomyositis.

Background: Interstitial lung disease (ILD) is one of the significant complications of dermatomyositis (DM), but the mechanisms by which it occurs remain incompletely elucidated. This study aimed to explore further the possible genetic mechanisms by which this complication occurs.

Methods: Gene expression profiles for DM (GSE39454, GSE46239, GSE143323) and ILD (GSE32537, GSE110147, GSE150910) were downloaded from the Gene Expression Omnibus (GEO) database.

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.

Janus kinase inhibition (JAKi) therapy in refractory anti-synthetase syndrome: A retrospective cohort study.

Objectives: To evaluate the efficacy and safety of Janus kinase inhibitors (JAKi) in the treatment of refractory anti-synthetase syndrome (ASS) in real-world clinical settings.

Methods: The medical records of all refractory ASS patients who were treated with JAKi from October 2020 to June 2023 were retrospectively reviewed.

Results: Twenty patients were included, and all (100 %) patients had interstitial lung disease (ILD).

Interstitial Lung Disease in Patients with Mixed Connective Tissue Disease: A Retrospective Study.

Objective: To investigate the clinical features, severity and prognosis of interstitial lung disease (ILD) in patients with mixed connective tissue disease (MCTD).

Methods: We performed a retrospective study on clinical data of MCTD patients admitted to China-Japan Friendship Hospital between October 2012 and October 2022. Data including long-term follow-up were retrieved from medical records.

Effectiveness and safety of eltrombopag in connective tissue disease patients with refractory immune thrombocytopenia: a retrospective study.

Objectives: We aimed to investigate the safety and effectiveness of eltrombopag for adult patients with refractory immune thrombocytopenia (ITP) secondary to connective tissue disease (CTD).

Methods: This is a single-centre, retrospective cohort and propensity score-matched study. Data from CTD-ITP patients treated with eltrombopag between January 2019 and January 2023 were retrospectively analysed.

Nintedanib could potentially lead to improvements in anti-melanoma differentiation-associated 5 dermatomyositis-associated interstitial lung disease.

Objectives: To determine the efficacy and safety of nintedanib in patients with anti-melanoma differentiation-associated gene 5 antibody positive dermatomyositis-associated interstitial lung disease (anti-MDA5+ DM-ILD).

Methods: The study was a retrospective cohort design that evaluated patients with anti-MDA5+ DM who either received or did not receive nintedanib. Clinical symptoms, laboratory tests, and survival were compared in the two groups using a propensity score-matched analysis.

Macrophage activation syndrome in juvenile dermatomyositis: a case report and a comprehensive review of the literature.

Background: Macrophage activation syndrome (MAS) is a severe and life-threatening syndrome associated with autoimmune diseases. The coexistence of MAS and juvenile dermatomyositis (JDM) is not well reported. This report describes a case of JDM with MAS and summarizes the clinical characteristics and prognosis of MAS in patients with JDM.

Clinical characteristics of myositis patients with isolated anti-U1 ribonucleoprotein antibody resemble immune-mediated necrotizing myopathy.

Background: Anti-U1 ribonucleoprotein (U1RNP) antibodies were associated with connective tissue diseases (CTDs), but the clinical characteristics of this antibody in Chinese myositis patients have not been studied.

Objective: We aim to analyze the clinical features of myositis patients who test positive for anti-U1RNP antibodies and delineate a subgroup of myositis.

Design: This is a retrospective cohort study.

High prevalence and mortality of Pneumocystis jirovecii pneumonia in anti-MDA5 antibody-positive dermatomyositis.

Objectives: To identify potential risk factors and prognostic factors of Pneumocystis jirovecii pneumonia (PJP) infection in anti-melanoma differentiation-associated gene 5 antibody-positive DM (anti-MDA5+ DM) patients, and to evaluate the diagnostic performance of metagenomic next-generation sequencing (mNGS).

Methods: Anti-MDA5+ DM patients who underwent mNGS or real-time PCR for PJP detection were recruited. The potential risk factors for PJP occurrence and death were analysed via Logistic regression and Cox proportional hazards regression, respectively.

Inflammatory myopathy following coronavirus disease 2019 vaccination: A systematic review.

Introduction: Reports of unexpected side effects have accompanied the vaccination of larger proportions of the population against coronavirus disease 2019 (COVID-19), including a few cases of inflammatory myopathy (IM). In a bid to improve understanding of the clinical course of vaccine complications, a systematic review of reported cases of IM following COVID-19 vaccination has been conducted.

Methods: The PRISMA guideline 2020 was followed.

The clinical features and prognoses of anti-MDA5 and anti-aminoacyl-tRNA synthetase antibody double-positive dermatomyositis patients.

Objective: To explore the clinical features and prognoses of dermatomyositis (DM) associated with a double-positive anti-MDA5 and anti-aminoacyl-tRNA synthetase (anti-ARS) antibody presentation.

Methods: We retrospectively analyzed 1280 consecutive patients with idiopathic inflammatory myopathy (IIM). Individuals with anti-MDA5 and anti-ARS antibodies (anti-MDA5+/ARS+) were compared to anti-MDA5-/ARS+ and anti-MDA5+/ARS- control individuals based on clinical, pulmonary radiological characteristics, treatment, and follow-up information.

Anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis complicated with macrophage activation syndrome.

Background: Anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody-positive dermatomyositis (DM) has low survival rate, whereas macrophage activation syndrome (MAS) is a severe and life-threatening syndrome associated with autoimmune diseases. Their coexistence is very rare. This study aimed to describe the prevalence, clinical characteristics, and outcomes of anti-MDA5 antibodies-positive DM patients complicated with MAS.

Thromboembolic events in idiopathic inflammatory myopathy: a retrospective study in China.

Objective: To investigate the clinical characteristics and risk factors for thromboembolic events in patients with idiopathic inflammatory myopathy (IIM).

Methods: We retrospectively analyzed 1144 consecutive patients with IIM for arterial and venous thromboses and compared them with age- and sex-matched IIM patients without thrombosis. Logistic regression analysis was used to analyze risk factors for thrombosis.

The Clinical Phenotype of Chinese Patients With Autoimmune Pancreatitis Differs Significantly From Western Patients.

Aim: To characterize the clinical features of autoimmune pancreatitis (AIP) in China and compare differences between our Chinese cohort and Western cohorts.

Methods: This was a retrospective study of patients with AIP that was carried out in the China-Japan Friendship Hospital between January 2010 and April 2021. We included a total of 50 patients (46 males and 4 females) aged between 27 and 86 years who fulfilled the international Consensus Diagnostic (ICD) Criteria.

Interstitial Lung Disease Is a Major Characteristic of Patients Who Test Positive for Anti-PM/Scl Antibody.

Objective: This study aimed to analyze the clinical features of anti-PM/Scl antibodies in Chinese patients.

Method: We reviewed the clinical data of anti-PM/Scl antibody-positive patients, including their long-term follow-up.

Results: A total of 30 patients carried anti-PM/Scl antibodies, 21 (70%) were females, and the mean age was 55.

Interstitial lung disease is not rare in immune-mediated necrotizing myopathy with anti-signal recognition particle antibodies.

Objectives: The purpose was to clarify the characteristics of interstitial lung disease (ILD) in immune-mediated necrotizing myopathy (IMNM) patients with anti-signal recognition particle (SRP) antibodies.

Methods: Medical records of IMNM patients with anti-SRP antibodies were reviewed retrospectively.

Results: A total of 60 patients were identified.

Clinical heterogeneities and prognoses of patients with myositis specific antibody negative dermatomyositis: a retrospective study in China.

Objectives: The clinical features of myositis specific antibody negative dermatomyositis (MSA negative DM) varied greatly, and there were few reports in the literatures. This study aimed to describe and expand the clinical phenotypes and prognoses of MSA negative DM patients.

Methods: MSA negative DM patients were identified from January 2010 to June 2020.

Clinical role of bronchoalveolar lavage in dermatomyositis-associated interstitial lung disease.

Objective: To investigate the role of bronchoalveolar lavage (BAL) in DM-associated interstitial lung disease (ILD).

Methods: We retrospectively reviewed the medical records of patients with DM-ILD who underwent bronchoscopy between October 2015 and September 2019. We then collated clinical features, laboratory data and bronchoscopy findings.

The effects of infliximab in treating idiopathic inflammatory myopathies: A review article.

Anti-TNF treatment may be useful for patients with idiopathic inflammatory myopathies (IIMs). The purpose of this study is to assess the efficacy of infliximab (IFX) in the management of IIMs. Two databases (ie, PubMed and China National Knowledge Infrastructure) were searched up to Nov 2020 for studies investigating skin lesions and muscular weakness in patients with IIMs treated with IFX.

Anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive dermatomyositis responds to rituximab therapy.

Objectives: The purpose of this study was to assess the efficacy of rituximab (RTX) in the management of anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody-positive dermatomyositis (DM), with or without rapidly progressive interstitial lung disease (RP-ILD).

Methods: Medical records of DM patients with anti-MDA5 antibodies treated with RTX therapy were reviewed retrospectively. Skin rash data, lung function tests, chest high-resolution computed tomography (HRCT), and serum markers were compared before and after RTX.

Interstitial lung disease is a major characteristic of anti-KS associated ant-synthetase syndrome.

Background: Anti-KS autoantibodies are rare myositis-specific autoantibodies that have been described to target asparaginyl-transfer RNA synthetase.

Methods: Here, we review the published literature on critical issues concerning the detection of anti-KS antibodies and the clinical features associated with their presence.

Results: Seven articles are reviewed, in all of which immunoprecipitation was employed for the detection of anti-KS antibodies.

Tracheobronchial amyloidosis in primary Sjögren syndrome: A case report.

Rationale: Tracheobronchial amyloidosis (TBA) associated with Sjögren syndrome is very rare. Here, we describe a case with this phenomenon, in order to better understand the condition.

Patient Concerns: A 52-year-old woman presented after 6 months of coughing, sputum, and dyspnea.