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Pneumomediastinum in MDA5+ Dermatomyositis: A Case Series.
Mediterr J Rheumatol
December 2024
Department of Clinical Immunology and Rheumatology, KGMU, Lucknow, India.
MDA5+ DM, or anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis (DM), is a rare autoimmune illness that primarily affects women of Asian origin. The typical presentation of MDA5+ DM includes a variety of cutaneous lesions accompanied by either no muscular weakness (amyopathic) or hypomyopathic features. In patients with MDA5+ DM, rapid progression of interstitial lung disease is a frequent manifestation associated with poor prognosis.
View Article and Find Full Text PDFEffectiveness and safety of tofacitinib calcineurin inhibitor in interstitial lung disease secondary to anti-MDA5-positive dermatomyositis: a multi-centre cohort study.
Eur Respir J
January 2025
Department of Rheumatology, Renji Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China
Objective: To compare the effectiveness and safety of tofacitinib (TOF) calcineurin inhibitor (CNI) as initial immunosuppressive regimen for anti-melanoma differentiation-associated gene 5-positive dermatomyositis with interstitial lung disease (MDA5+DM-ILD).
Methods: Adult Chinese patients with newly-diagnosed MDA5+DM-ILD (ILD course<3 months) from five tertiary referral centres between April 2014 and January 2023 were included for this retrospective cohort study. The primary effectiveness endpoint was lung transplantation-free survival within 1 year.
Anti-melanoma differentiation-associated gene 5 antibody-positive interstitial lung disease masquerading as rheumatoid arthritis-associated interstitial lung disease or overlapping with the same: a phenomenon driven by a probable genetic predisposition.
Clin Rheumatol
January 2025
Department of Pulmonology & Interventional Pulmonology, Caritas Hospital and Institute of Health Sciences, Thellakom, Kottayam, Kerala, India.
Rheumatoid arthritis (RA) is a systemic, progressive illness marked by persistent synovitis that causes substantial functional disability. Treatment delays frequently affect health-related quality of life. Extra-articular features are prevalent findings in RA, which leads to significant morbidity and mortality.
View Article and Find Full Text PDFDevelopment and Evaluation of the Dermatomyositis Outcomes for Muscle and Skin as an Outcome Measure in Dermatomyositis Clinical Trials.
JID Innov
March 2025
Corporal Micheal J. Crescenz Veterans Affairs Medical Center, Philadelphia, Pennsylvania, USA.
The Total Improvement Score (TIS), which is used as the primary efficacy measure in dermatomyositis (DM) clinical trials, lacks a skin-specific measure. However, skin is a defining feature of DM. In this study, data were analyzed from the phase 3 trial of lenabasum in DM.
View Article and Find Full Text PDFClinicopathological Features of Mixed Connective Tissue Disease-Related Myositis: A Case Series.
Muscle Nerve
January 2025
Department of Neurology, Tohoku University Graduate School of Medicine, Sendai, Japan.
Introduction: Mixed connective tissue disease (MCTD) patients often have myositis, however, myopathological and clinical data for MCTD are limited. Recent reports have shown that the pathology of MCTD myositis resembles that of immune-mediated necrotizing myopathy (IMNM), whereas earlier reports described perifascicular atrophy or inflammatory infiltrates predominantly in the perivascular region in MCTD myositis. We aim to describe the clinical and myopathological features of MCTD myositis.
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