Severe interstitial lung disease risk prediction in anti-melanoma differentiation-associated protein 5 positive dermatomyositis: the STRAD-Ro52 model.

Objective: Anti-melanoma differentiation-associated gene 5-positive dermatomyositis-associated interstitial lung disease (MDA5DM-ILD) often leads to acute respiratory failure and endangers lives. This study quantitatively analysed chest high-resolution computed tomography (HRCT) images to assess MDA5DM-ILD and establish a risk prediction model for severe ILD within six months.

Methods: We developed a 'Standardized Threshold Ratio Analysis & Distribution' (STRAD) to analyse lung HRCT images.

Meta-analyses uncover the genetic architecture of Idiopathic Inflammatory Myopathies.

Objective: Idiopathic inflammatory myopathies (myositis, IIMs) are rare, systemic autoimmune disorders that lead to muscle inflammation, weakness, and extra-muscular manifestations, with a strong genetic component influencing disease development and progression. Previous genome-wide association studies identified loci associated with IIMs. In this study, we imputed data from two prior genome-wide myositis studies and analyzed the largest myositis dataset to date to identify novel risk loci and susceptibility genes associated with IIMs and its clinical subtypes.

Regional ischaemic immune myopathy: unraveling a rare subtype of dermatomyopathy.

Regional ischaemic immune myopathy (RIIM) is a subtype of dermatomyopathy which has distinct histopathology features of regional muscle fibre necrosis and regeneration. This case report aims to provide an in-depth exploration of RIIM, emphasising its clinical features and the crucial role of muscle biopsy as a diagnostic investigation, as well as the potential role of immunomodulatory therapies.

Pneumocystis jirovecii pneumonia increases the 3-months mortality of anti-MDA5-antibody-positive dermatomyositis patients.

Background: Anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis (anti-MDA5+DM) patients are associated with considerable mortality, and opportunistic infections including Pneumocystis jirovecii pneumonia (PJP)is the main cause. This study was to identify clinical characteristics, risk factors, and prognostic factors of PJP diagnosed by bronchoalveolar lavage fluid (BALF) metagenomic next-generation sequencing (mNGS) in anti-MDA5+ DM patients.

Methods: In this retrospective observational study, all patients admitted with suspected pneumonia were detected for mNGS in BALF.

Occult ovarian adenocarcinoma manifesting as dermatomyositis.

A woman in her 60s presented with erythematous lesions predominantly over the joints. After evaluation by dermatology and rheumatology, she was diagnosed with dermatomyositis and initiated on oral steroids and immunosuppressants. She was subsequently referred to gynaecology services for further evaluation of possible malignancies.

Altered metabolic profiles of dermatomyositis with different myositis-specific autoantibodies associated with clinical phenotype.

Introduction: Dermatomyositis (DM) is an idiopathic inflammatory myopathy. Because of clinical heterogeneity, the metabolite profile of DM patients with different myositis-specific autoantibodies (MSAs) remains elusive. This study aimed to explore the metabolomics characteristics of the serum in DM with different MSAs, low or high disease activity, and interstitial lung disease.

Rationale, objectives and design of a national prospective database for idiopathic inflammatory myopathies: the Australian Myositis Registry.

Background: Idiopathic inflammatory myopathies (IIMs) are a group of autoimmune diseases characterised by inflammation of skeletal muscle and other organ systems. They have high morbidity and mortality but, in part because of their rarity and heterogeneity, improving understanding and outcomes remains challenging. To address these problems, numerous IIM registries exist globally, but no national registry yet exists in Australia.

Relationship between high-resolution computed tomography quantitative imaging analysis and physiological and clinical features in antisynthetase syndrome-related interstitial lung disease.

Objectives: To explore the association between the extent of CT abnormalities by quantitative imaging analysis (QIA) and clinical/physiological disease parameters in patients with antisynthetase syndrome associated interstitial lung disease (ARS-ILD).

Methods: We analysed 20 patients with antisynthetase antibodies and active ILD enrolled in the Abatacept in Myositis-Associated Interstitial Lung Disease study. High-resolution chest CT was obtained at weeks 0, 24 and 48 and QIA scored the extent of ground glass (quantitative score for ground glass), fibrosis (quantitative score for lung fibrosis, QLF) and total ILD (quantitative ILD, QILD).

Myocardial Performance Index to assess cardiac function in autoimmune connective tissue disease: a systematic review and meta-analysis.

Objectives: This study aimed to evaluate cardiac function using Myocardial Performance Index (MPI) in autoimmune connective tissue disease (ACTD) patients without cardiovascular abnormalities.

Methods: A systematic search of databases including Medline, Google Scholar, ProQuest, Scopus and Cochrane Library was conducted to identify relevant studies on ACTD and MPI from 1995 to 2023. ACTD included in the search were rheumatoid arthritis (RA), systemic sclerosis (SSc), systemic lupus erythematosus (SLE), Sjögren syndrome (SjD), polymyositis and dermatomyositis.

Nailfold microscopy in adult-onset dermatomyositis in association with myositis antibodies.

Dermatomyositis (DM) is an immune mediated inflammatory disease classically associated with muscular and cutaneous involvement. Existing studies have suggested characteristic nailfold findings may be observed in DM, indicating a potential role for nailfold microscopic examination in the diagnosis of DM. To that end, we performed a systematic review of literature pertaining to nailfold microscopic, capillaroscopic, and dermoscopic findings observed in patients with DM, with a secondary review of the association of nailfold microscopic findings with myositis-specific antibody (MSA) and myositis-associated antibody (MAA) status.

Autoantibodies against a subunit of mitochondrial respiratory chain complex I in inclusion body myositis.

Background: Autoantibodies are found in up to 80 % of patients with idiopathic inflammatory myopathies (IIM) and are associated with distinct clinical phenotypes. Autoantibodies targeting cytosolic 5'-nucleotidase 1A (anti-NT5C1A) are currently the only known serum biomarker for the subgroup inclusion body myositis (IBM), although detected even in other autoimmune diseases. The aim of the study was to identify new autoimmune targets in IIM.

A retrospective study for clinical characteristics of 293 patients with dermatomyositis.

This retrospective study aimed to investigate differences in clinical characteristics between different antibody phenotypes in patients with dermatomyositis (DM). Two hundred and ninety-three patients with DM were included in this study from September 2018 to September 2023. We collected basic clinical data from the patients, using statistical methods to analyze the clinical characteristics, and used survival analysis and COX regression to assess the prognosis of the patients.

Identification and validation of interferon-stimulated gene 15 as a biomarker for dermatomyositis by integrated bioinformatics analysis and machine learning.

Background: Dermatomyositis (DM) is an autoimmune disease that primarily affects the skin and muscles. It can lead to increased mortality, particularly when patients develop associated malignancies or experience fatal complications such as pulmonary fibrosis. Identifying reliable biomarkers is essential for the early diagnosis and treatment of DM.

Innovation in Dermatomyositis.

Dermatomyositis (DM) is a rare autoimmune disease defined by the presence of characteristic cutaneous findings, an increased cancer risk, and variable extracutaneous pathology involving the muscles, lungs, gastrointestinal tract, heart, and/or joints. Although the pathogenesis of DM remains incompletely understood, the discovery of myositis-specific autoantibodies has been an important step forward in understanding disease heterogeneity in DM and stratifying risk for extracutaneous disease and malignancy. Moreover, the recent elucidation of key immunologic drivers of DM has laid the groundwork for the development of novel, targeted treatments in the DM therapeutic pipeline.

Atypical presentation of anti-small ubiquitin-like modifier 1 and melanoma differentiation-associated gene 5 antibody positive dermatomyositis presenting with significant inflammatory myopathy on biopsy and normal creatine kinase levels: A case report.

Idiopathic inflammatory myopathies are characterized by chronic inflammation of skeletal muscle. The main subtypes of idiopathic inflammatory myopathies include dermatomyositis, polymyositis, and necrotizing autoimmune myopathies. Dermatomyositis is characterized by symmetrical proximal muscle weakness, distinctive skin lesions, and systemic manifestations.

Neutrophil and mononuclear leukocyte pathways and upstream regulators revealed by serum proteomics of adult and juvenile dermatomyositis.

Objectives: Serum protein abundance was assessed in adult and juvenile dermatomyositis (DM and JDM) patients to determine differentially regulated proteins, altered pathways, and candidate disease activity biomarkers.

Methods: Serum protein expression from 17 active adult DM and JDM patients each was compared to matched, healthy control subjects by a multiplex immunoassay. Pathway analysis and protein clustering of the differentially regulated proteins were examined to assess underlying mechanisms.

Anti-MDA5 positivity: describing the frequency and spectrum of clinically evident MDA5 disease.

To evaluate experience in a tertiary rheumatology service with melanoma differentiation-association-protein-5 (MDA5) disease and testing, patients with positive autoantibody results were reviewed for the presence of clinical disease. Anti-MDA5 positivity was detected in 2% of myositis-specific antibody tests. Of these, 29% did not have features consistent with anti-MDA5 disease.