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Amyopathic Dermatomyositis with a Rapidly Progressing Interstitial Pneumonia.
Eur J Case Rep Intern Med
November 2024
Department of Lung Diseases and Thoracic Surgery, Pauls Stradins Clinical University Hospital, Riga, Latvia.
Background: Clinically amyopathic dermatomyositis (CADM) is a rare subtype of idiopathic inflammatory myositis often linked with the presence of autoantibodies targeting melanoma differentiation-associated protein 5 (MDA5). Patients with CADM are at increased risk of developing rapidly progressing interstitial lung disease, which significantly increases both morbidity and mortality compared to other forms of inflammatory myopathies. While there is no standardized treatment regimen, current therapeutic strategies are generally focused on combination immunosuppressive therapies.
View Article and Find Full Text PDFAntimelanoma differentiation-associated gene 5 (anti-MDA5) antibody-positive dermatomyositis: clinical features and outcomes in a racially diverse patient cohort.
BMC Rheumatol
January 2025
Montefiore Medical Center, Albert Einstein College of Medicine, Rheumatology, Bronx, NY, USA.
Background: The anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody-positive dermatomyositis is known for its association with rapidly progressive interstitial lung disease (RP-ILD) and ulcerative skin lesions, often presenting with or without muscle involvement. The aim of this study was to identify distinct clinical and laboratory features that could be used to evaluate disease progression in an ethnically diverse cohort of anti-MDA5 dermatomyositis patients at a U.S.
View Article and Find Full Text PDFAnti-MDA5 Positive Juvenile Dermatomyositis With Macrophage Activation Syndrome: A Case Report.
Cureus
December 2024
Pediatric Medicine, Tawam Hospital, Abu Dhabi, ARE.
Article Synopsis
- There are various inflammatory myopathies, including juvenile dermatomyositis (JDM), which can cause muscle inflammation and weakness, with macrophage activation syndrome (MAS) being a severe complication, though it is not frequently reported.
- A case study of a six-year-old girl with proximal muscle weakness and skin manifestations, leading to a JDM diagnosis, showed elevated lab markers and confirmed muscle involvement through MRI.
- The girl initially improved with steroid therapy, but later tested positive for anti-MDA5 antibodies, indicating a worse prognosis and highlighting the importance of recognizing MAS as a possible complication in JDM patients with these antibodies.
[Clinical characteristics and prognostic analysis of concurrent pneumonia in patients with idiopathic inflammatory myopathy].
Zhonghua Jie He He Hu Xi Za Zhi
January 2025
Department of Pulmonary and Critical Care Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing100730, China.
To describe the clinical characteristics and to explore the prognostic factors of concurrent pneumonia (PJP) in patients with idiopathic inflammatory myopathy (IIM). We retrospectively enrolled consecutive IIM patients diagnosed with PJP at our center between January 2014 and December 2022. Fifty-eight IIM-PJP patients were enrolled in our study, with the age of 26-79 (56.
View Article and Find Full Text PDFClinical features and prognosis of anti-MDA5-positive dermatomyositis with coexistent anti-aminoacyl-tRNA synthetase antibodies.
Clin Rheumatol
January 2025
Department of Rheumatology and Immunology, The Second Affiliated Hospital of Soochow University, Suzhou, Jiangsu, China.
Objectives: This study aimed to evaluate the incidence and clinical significance of coexistence of anti-aminoacyl-tRNA synthetase (anti-ARS) antibody in patients with anti-melanoma differentiation-associated gene 5-positive dermatomyositis (anti-MDA5 + DM).
Methods: We assessed a cohort of 246 consecutive patients with anti-MDA5 + DM. Clinical characteristics and survival rates were compared between patients with and without anti-ARS antibodies.
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