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Anti-synthetase syndrome: a rare and challenging diagnosis for bilateral ground-glass opacities—a case report with literature review

BACKGROUND: Anti-synthetase syndrome (ASS) is an uncommon immune-mediated entity characterized by myositis, interstitial lung disease (ILD), non-erosive arthritis, and less common features such as fever, Raynaud’s phenomenon, and skin changes in association with anti-aminoacyl-transfer-RNA antibodie...

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Detalles Bibliográficos
Autores principales: Alfraji, Nasam, Mazahir, Usman, Chaudhri, Moiuz, Miskoff, Jeffrey
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7787399/
https://www.ncbi.nlm.nih.gov/pubmed/33407281
http://dx.doi.org/10.1186/s12890-020-01388-0
Descripción
Sumario:BACKGROUND: Anti-synthetase syndrome (ASS) is an uncommon immune-mediated entity characterized by myositis, interstitial lung disease (ILD), non-erosive arthritis, and less common features such as fever, Raynaud’s phenomenon, and skin changes in association with anti-aminoacyl-transfer-RNA antibodies, most commonly anti-Jo-1 antibodies. CASE PRESENTATION: We present a challenging and rare case of ASS-associated ILD presenting with unexplained respiratory symptoms and bilateral infiltrates on chest imaging during the COVID-19 pandemic. High clinical suspicion for ASS with early appropriate therapy with corticosteroids and immunosuppressive agents led to marked clinical improvement. CONCLUSION: High index of suspicion for ASS is mandated in patients with unexplained ILD. A comprehensive autoimmune work-up is important as an early treatment with corticosteroids with or without immunomodulators improves patient outcomes and survival in an otherwise poor prognostic disease.