ArticleFrontiers in medicine2020
Interstitial Lung Disease-Complicated Anti-MDA5 Antibody in Clinically Amyopathic Dermatomyositis Patients: Report of Two Cases With Distinct Clinical Features.
Article in Frontiers in medicine, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 10 papers.
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Who cites it
10 citing papers in PubMed, 16 citations in OpenAlex.
- Article
- Extracorporeal membrane oxygenation for idiopathic inflammatory myopathy-associated interstitial lung disease: a narrative review.Journal of thoracic disease · 2026Review
- Acute fibrinous and organising pneumonia (AFOP): a case series highlighting diverse aetiologies and clinical outcomes in three patients.European clinical respiratory journal · 2026Article
- Elevated serum interleukin-38 levels in polymyositis and dermatomyositis: diagnostic implications and correlations with inflammatory markers.Frontiers in immunology · 2026Article
- MDA5-positive dermatomyositis without muscle weakness and rash: a case report and literature review.Frontiers in medicine · 2024Article
- Narrative review of autoantibodies in idiopathic inflammatory myopathies.Annals of translational medicine · 2023Review
- Lung transplantation for anti-MDA5-positive dermatomyositis-associated rapid progressive interstitial lung disease: report of two cases and review of the literature.Clinical rheumatology · 2023Review
- Slowly progressive interstitial lung disease preceding typical dermatomyositis symptoms in anti-melanoma differentiation-associated gene 5 antibody-positive clinically amyopathic dermatomyositis.Respiratory medicine case reports · 2021Article
- Dermatomyositis With Anti-MDA5 Antibodies: Bioclinical Features, Pathogenesis and Emerging Therapies.Frontiers in immunology · 2021Review
- Spontaneous Pneumomediastinum due to Anti-Melanoma Differentiation-Associated Protein 5 Requiring a Bilateral Lung Transplant.Case reports in rheumatology · 2021Article
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Authors and funding
8 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Two patients presented simultaneously to our hospital with distinct clinical features associated with the presence of anti-MDA5 antibodies: the first one was admitted for a skin rash resembling to a toxic epidermal necrosis (Lyell syndrome) and the second one presented with pulmonary manifestations attributed to a diffuse fibrosing interstitial pneumonitis on chest CT-scan. In addition to the skin lesions involving 40% of the body surface area, the first patient developed a rapid diffuse interstitial pneumonitis with respiratory distress justifying the initiation of a systemic immunosuppressive treatment. However, she died 3 weeks after her admission from mesenteric thrombosis associated with septic shock. The second patient respiratory condition worsened despite an intensive immunosuppressive treatment with high doses of intravenous methylprednisolone and cyclophosphamide and plasmapheresis, and required lung transplantation. Anti-MDA5 antibody titer declined and disappeared on anti-rejection treatment. These two cases underline the diagnostic conundrum and the therapeutic difficulties in patients with anti-MDA5 antibodies and clinically amyopathic dermatomyositis (CADM) or interstitial lung disease (ILD), who may undergo rapidly-progressive and fatal outcome. Presence of anti-MDA5 antibodies should always be suspected when confronted to CADM patients with cutaneous ulcerations or ILD to allow a rapid and adapted treatment initiation.
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