Case Presentation:  We present a case of a 55-year-old Caucasian male with manifestations of dermatomyositis complicated with rapidly progressive interstitial lung disease (RP-ILD). Diagnosis of anti-MDA5 positive dermatomyositis was made.

Discussion:  Myositis specific antibodies (MSA) can be used for diagnosis and predicting prognosis in patients with polymyositis and dermatomyositis. Anti-MDA5 positive dermatomyositis should be considered in patients presenting with dermatomyositis and a disease course resembling antisynthetase syndrome in the absence of antisynthetase autoantibodies, especially if a remarkably high ferritin is noted. Anti-MDA5 autoantibodies have been associated with RP-ILD and adverse outcome. In patients with anti-MDA5 autoantibodies, early diagnosis and aggressive immunosuppressive treatment may improve prognosis.

Conclusion:  This case highlights the importance of determining MSA in patients with dermatomyositis and associated interstitial lung disease, as this has implications for diagnosis, prognosis and therapy.

Download full-text PDF

Source
http://dx.doi.org/10.1080/17843286.2017.1420521DOI Listing

Publication Analysis

Top Keywords

anti-mda5 positive
12
positive dermatomyositis
12
interstitial lung
12
lung disease
12
dermatomyositis complicated
8
complicated rapidly
8
rapidly progressive
8
progressive interstitial
8
anti-mda5 autoantibodies
8
dermatomyositis
7

Similar Publications

Unlabelled: The aim of this study was to assess the 2017 EULAR/ACR classification criteria performance for determining idiopathic inflammatory myopathies (IIMs) in a cohort of patients with anti-MDA5 antibody-positive IIM-related interstitial lung disease (anti-MDA5+IIM-ILD). The outcomes of patients, who did not meet the EULAR/ACR criteria, and who had interstitial pneumonia and exhibited an autoimmune phenotype associated with anti-MDA5 positivity were also investigated.

Methods: This retrospective study recruited adult patients from four hospitals in China who were diagnosed with anti-MDA5 antibody-positive IIM-related interstitial lung disease.

View Article and Find Full Text PDF

[Sequential autoimmunity? The rare case of MDA5 dermatomyositis and sarcoidosis].

Z Rheumatol

February 2025

Sektion für Rheumatologie und Entzündliche Systemerkrankungen in der III. Medizin, Universitätsklinikum Hamburg-Eppendorf (UKE), Martinistr. 52, 20246, Hamburg, Deutschland.

Background: Anti-MDA5 positive dermatomyositis is a rare subtype of idiopathic inflammatory myopathies often accompanied by rapidly progressive interstitial lung disease and severe cutaneous ulcerations. It is associated with a high mortality. The simultaneous occurrence of sarcoidosis with multisystemic involvement of myositis has only been described very rarely in the literature to date.

View Article and Find Full Text PDF

Clinical Features of Dermatomyositis Associated with Myositis-Specific Antibodies in Moroccan Patients.

Clin Pract

February 2025

Laboratory of Inflammation Clinical Immunology and Allergy(LICIA), Faculty of Medicine and Pharmacy of Casablanca, Hassan II University, Casablanca 20250, Morocco.

Dermatomyositis (DM) is a rare idiopathic inflammatory myopathy characterized by muscle weakness and typical cutaneous rash. Dermatomyositis-specific antibodies, such as anti-TIF1γ, anti-SAE, anti-Mi2, anti-MDA5, and anti-NXP2, have been associated with specific clinical phenotypes. Our study aimed to describe the clinical profile of Moroccan patients with DM and clinical associations with myositis-specific antibodies.

View Article and Find Full Text PDF

A case report highlights the challenges faced in managing a 66-year-old Chinese woman diagnosed with anti-MDA5 antibody-positive dermatomyositis (MDA5-DM) complicated by rapidly progressive interstitial lung disease (RP-ILD). Despite aggressive therapeutic interventions, her condition rapidly deteriorated, emphasizing the severity and devastating nature of this subtype of DM. A salient feature of her clinical presentation was the marked elevation of interferon (IFN)-γ and IFN-α levels, underscoring the pivotal role that IFNs play in driving the pathogenesis and progression of MDA5-DM-related RP-ILD.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!