Achieving Medication-Free Remission in Patients With Juvenile Dermatomyositis.

Objective: Prognostic factors associated with medication discontinuation in children with juvenile dermatomyositis (JDM) remain largely elusive. We aim to identify the predictors of medication-free remission (MFR) in children with JDM.

Methods: In this retrospective study, patients diagnosed with JDM according to Peter & Bohan criteria and followed for ≥18 months at a tertiary care center from 2006 through 2022 were included.

The value of quantitative muscle ultrasound in monitoring muscle and fascia inflammatory activity in juvenile dermatomyositis patients.

Background: Currently, the activity of juvenile dermatomyositis (JDM) is mainly assessed based on clinical manifestations, creatine kinase (CK) level, and magnetic resonance imaging (MRI), but certain limitations arise in these approaches for children. Thus, this cross-sectional study aimed to explore the value of ultrasound in evaluating muscle inflammation via the dynamic analysis of muscle ultrasound characteristics in children with active or stable JDM.

Methods: The data of a group of children who were diagnosed with JDM and admitted to the Rheumatology and Immunology Department of the Capital Pediatric Research Institute Children's Hospital between June 2022 and November 2023, and a normal control group were collected.

Radiographs in Pediatric Rheumatology: Where Do We Stand?

Rheumatic disorders in children include inflammatory arthritis, inflammatory bone disorders such as chronic nonbacterial osteomyelitis (CNO), connective tissue disorders, and vasculitides (juvenile dermatomyositis, scleroderma). The diagnosis in these children is based on a combination of history, clinical examination, and laboratory investigations. Radiographs play an important role in children with arthritis, who have atypical presentation or for assessment of disease-related damage and differentiation from mimics.

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.

Lupus erythematosus and dermatomyositis.

Dr. Irwin Braverman, a luminary in our field of dermatology, united his love of internal medicine with dermatology to pioneer our understanding of the cutaneous manifestations of systemic disease. His meticulous documentation of physical examination findings in his book Skin Signs of Systemic Disease became fundamental to the training of dermatologists worldwide for decades.

The role of nailfold video-capillaroscopy in the assessment of dermatomyositis.

Objectives: The clinical manifestations of dermatomyositis (DM) are diverse, nailfold video-capillaroscopy (NVC) can reflect microangiopathy, a process believed to contribute significantly to the clinical manifestations of DM. We aimed to explore the distinctive alterations and implications of nailfold capillary for evaluating disease progression in individuals with DM.

Methods: We gathered clinical data from 76 DM patients who underwent NVC in the Affiliated Hospital of Nantong University between September 2017 and September 2022.

Mixed and undifferentiated autoimmune connective tissue diseases.

Dr. Irwin M. Braverman has advanced our understanding of cutaneous manifestations of autoimmune connective tissue diseases (AI-CTD).

Low serum S100A6 levels are associated RP-ILD risk in anti-MDA5-positive dermatomyositis.

Introduction: Anti-MDA5-positive dermatomyositis (anti-MDA5-DM) is a rare autoimmune disease that often leads to rapid-progressive interstitial lung disease (RP-ILD). The lack of effective prediction and treatment methods makes RP-ILD a major risk factor for death in patients with this condition. S100A6 is a member of the S100 Ca2 + - binding protein family, which plays important roles in inflammation, tumor, injury, and fibroblast reparation.

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.

A Bayesian latent class approach to causal inference with longitudinal data.

Bayesian methods are becoming increasingly in demand in clinical and public health comparative effectiveness research. Limited literature has explored parametric Bayesian causal approaches to handle time-dependent treatment and time-dependent covariates. In this article, building on to the work on Bayesian g-computation, we propose a fully Bayesian causal approach, implemented using latent confounder classes which represent the patient's disease and health status.

Successful tocilizumab treatment for rapidly progressive interstitial lung disease with anti-MDA5-positive juvenile dermatomyositis: a case report and literature review.

Background: Currently, no established integrated treatment regimen exists for anti-melanoma differentiation-associated gene 5 (anti-MDA5)-positive juvenile dermatomyositis (JDM) complicated by rapidly progressive interstitial lung disease (RP-ILD). We present a case of refractory anti-MDA5-positive JDM with RP-ILD that was successfully treated using a combination of tocilizumab and plasma exchange, along with a review of the relevant literature.

Methods: A literature review was conducted to gain insights into the clinical features and treatment strategies for managing refractory anti-MDA5-positive JDM complicated by RP-ILD.

Refractory skin ulcers and afebrile bacteremia with Staphylococcus aureus in anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis: a case report.

The skin ulcers sometimes appear in patients with anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive dermatomyositis (DM), and usually associates with disease activity. Here, we report a case of 41-year-old woman with anti-MDA5 antibody-positive DM, who developed refractory skin ulcers during the remission induction therapy, which were proven to be associated with clinically silent Staphylococcus aureus bacteremia with septic thrombi in her lung. The patient was referred to our hospital for the treatment of amyopathic DM with interstitial lung disease.

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.

Anti-Ro-Positive Dermatomyositis Presenting as a Clinical Mimic of Guillain-Barré Syndrome: A Case Report.

Dermatomyositis is a multi-system, connective tissue immune-mediated inflammatory condition characterised by myositis and distinct skin manifestations, with a higher prevalence in women. Symptoms typically appear in adulthood, though a juvenile form exists. Early signs may include Gottron's papules and heliotrope rash, with proximal muscle weakness as the second most common initial symptom.

Analysis of the Potential Link Between Dermatomyositis and Cancer.

Background: Dermatomyositis (DM) is an inflammatory muscle disease that increases the risk of cancer, although the precise connection is not fully understood. The aim of this study was to investigate the mechanisms linking DM to cancer and identify potential therapeutic targets.

Methods: We conducted differential gene expression analysis on the GSE128470 dataset and employed WGCNA to pinpoint key genes related to DM.