Nasam Alfraji1, Usman Mazahir2, Moiuz Chaudhri2, Jeffrey Miskoff2. 1. Department of Internal Medicine, Jersey Shore University Medical Center, Hackensack Meridian Health, Neptune, NJ, 07753, USA. nasam.alfraji@hmhn.org. 2. Department of Pulmonology and Critical Care, Jersey Shore University Medical Center, Neptune, NJ, 07753, USA.
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.
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.
Authors: Ernesto Trallero-Araguás; Josep María Grau-Junyent; Anne Labirua-Iturburu; Francisco José García-Hernández; Manuel Monteagudo-Jiménez; Guadalupe Fraile-Rodriguez; Iñigo Les-Bujanda; Mónica Rodriguez-Carballeira; Luis Sáez-Comet; Albert Selva-O'Callaghan Journal: Semin Arthritis Rheum Date: 2016-03-30 Impact factor: 5.532