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Review
. 2021 Jan 6;21(1):11.
doi: 10.1186/s12890-020-01388-0.

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

Nasam Alfraji  1 , Usman Mazahir  2 , Moiuz Chaudhri  2 , Jeffrey Miskoff  2
Affiliations
Review

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

Nasam Alfraji et al. BMC Pulm Med. .

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.

Keywords: Anti-synthetase syndrome; Autoimmune disease; Corticosteroids; Interstitial lung disease.

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Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
Chest x ray revealing persistent bibasilar infiltrates
Fig. 2
Fig. 2
Computed tomography angiography of the chest revealing bilateral ground glass opacities, with no filling defects suggesting pulmonary emboli
See this image and copyright information in PMC

References

    1. Devi HG, Pasha MM, Padmaja MS, Halappa S. Anti-synthetase syndrome: a rare cause for ILD. J Clin Diagn Res. 2016;10(3):OD08–OD09. doi: 10.7860/JCDR/2016/16872.7361. - DOI - PMC - PubMed
    1. Marco JL, Collins BF. Clinical manifestations and treatment of antisynthetase syndrome. Best Pract Res Clin Rheumatol. 2020;10:101503. doi: 10.1016/j.berh.2020.101503. - DOI - PubMed
    1. Gallay L, Gayed C, Hervier B. Antisynthetase syndrome pathogenesis: knowledge and uncertainties. Curr Opin Rheumatol. 2018;30(6):664–673. doi: 10.1097/BOR.0000000000000555. - DOI - PubMed
    1. 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):111–127. doi: 10.1097/01.md.0000217525.82287.eb. - DOI - PubMed
    1. Badshah A, Haider I, Pervez S, et al. Anti-synthetase syndrome presenting as interstitial lung disease: a case report. J Med Case Rep. 2019;13:241. doi: 10.1186/s13256-019-2146-0. - DOI - PMC - PubMed

Supplementary concepts

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