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Clinical Characteristics and Outcomes of Patients with Anti-MDA5 Antibody Associated Rapidly Progressive Interstitial Lung Disease (RP-ILD): A Case Series.
Mediterr J Rheumatol
December 2024
Department of Internal Medicine, Government Medical College Kottayam, Kerala, India.
Objective: To describe the clinical profile and treatment outcomes of a longitudinal series of patients with rapidly progressive interstitial lung disease (RP-ILD) associated with anti MDA 5 antibody.
Methods: RP-ILD patients were identified from a prospective cohort of adult patients with idiopathic inflammatory myopathy (IIM). Clinical, demographic, and serological parameters of all patients were recorded using a structured proforma.
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 PDFAcquired leukonychia with lichenoid papules.
JAAD Case Rep
February 2025
Forefront Dermatology, Noblesville, Indiana.
Anti-melanoma Differentiation-Associated Protein 5 (MDA5)-Positive Dermatomyositis With Rapidly Progressive Interstitial Lung Disease (ILD): A Rare and Lethal Entity to Recognize Early.
Cureus
December 2024
Internal Medicine, Unidade Local de Saúde de São José, Lisbon, PRT.
Anti-melanoma differentiation-associated protein 5 (anti-MDA5) clinically linked amyopathic dermatomyositis (CADM) is a rare autoimmune condition strongly linked to rapidly progressive interstitial lung disease (RP-ILD), a life-threatening complication. We present a 63-year-old female patient with anti-MDA5-positive CADM, who developed RP-ILD with an imaging pattern consistent with organizing pneumonia. She presented with Gottron's papules, periungual erythema, progressive dyspnea, and anorexia.
View Article and Find Full Text PDFAmyopathic 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.
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