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Published in: BMC Pulmonary Medicine 1/2021

Open Access 01-12-2021 | Case report

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

Authors: Nasam Alfraji, Usman Mazahir, Moiuz Chaudhri, Jeffrey Miskoff

Published in: BMC Pulmonary Medicine | Issue 1/2021

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Abstract

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.
Literature
4.
go back to reference O’Hanlon TP, Carrick DM, Targoff IN, Arnett FC, Reveille JD, Carrington M, et al. Immunogenetic risk and protective factors for the idiopathic inflammatory myopathies: distinct HLA-A, -B, -Cw, -DRB1, and -DQA1 allelic profiles distinguish European American patients with different myositis autoantibodies. Medicine (Baltim). 2006;85(2):111–27. https://doi.org/10.1097/01.md.0000217525.82287.eb.CrossRef O’Hanlon TP, Carrick DM, Targoff IN, Arnett FC, Reveille JD, Carrington M, et al. Immunogenetic risk and protective factors for the idiopathic inflammatory myopathies: distinct HLA-A, -B, -Cw, -DRB1, and -DQA1 allelic profiles distinguish European American patients with different myositis autoantibodies. Medicine (Baltim). 2006;85(2):111–27. https://​doi.​org/​10.​1097/​01.​md.​0000217525.​82287.​eb.CrossRef
7.
go back to reference Rüegg CA, Maurer B, Laube I, et al. Jo1-antisynthetase syndrome and severe interstitial lung disease with organising pneumonia on histopathology with favourable outcome on early combined treatment with corticosteroids, mycophenolate mofetil and rituximab. BMJ Case Rep. 2019;12:e231006. https://doi.org/10.1136/bcr-2019-231006. CrossRefPubMed Rüegg CA, Maurer B, Laube I, et al. Jo1-antisynthetase syndrome and severe interstitial lung disease with organising pneumonia on histopathology with favourable outcome on early combined treatment with corticosteroids, mycophenolate mofetil and rituximab. BMJ Case Rep. 2019;12:e231006. https://​doi.​org/​10.​1136/​bcr-2019-231006. CrossRefPubMed
Metadata
Title
Anti-synthetase syndrome: a rare and challenging diagnosis for bilateral ground-glass opacities—a case report with literature review
Authors
Nasam Alfraji
Usman Mazahir
Moiuz Chaudhri
Jeffrey Miskoff
Publication date
01-12-2021
Publisher
BioMed Central
Published in
BMC Pulmonary Medicine / Issue 1/2021
Electronic ISSN: 1471-2466
DOI
https://doi.org/10.1186/s12890-020-01388-0

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