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• W4387247836 abstract "SESSION TITLE: Pulmonary Manifestations of Systemic Disease Case Report Posters 9 SESSION TYPE: Case Report Posters PRESENTED ON: 10/10/2023 09:40 am - 10:25 am INTRODUCTION: The usual interstitial pneumonia (UIP) pattern of CT findings in interstitial lung disease is commonly associated with idiopathic pulmonary fibrosis (IPF). However, this pattern can be seen with other causes of interstitial lung disease (ILD) including connective tissue related ILD (CTD-ILD). Often patients with CTD-ILD present with clinical features of underlying connective tissue disease (CTD), however as our understanding of the association between ILD and CTD has grown, more patients presenting without evidence of CTD are being diagnosed with CTD-ILD. The distinction between IPF and CTD-ILD in patients with a UIP pattern is important as treatment of the underlying connective tissue disease is key to management and improves prognosis unlike IPF. One rare form of CTD-ILD, anti-synthetase syndrome, is an idiopathic immune myositis typically associated with a triad of myositis, arthritis, and ILD. Here we present a case of anti-synthetase syndrome with anti-PL-7 antibody and ILD with UIP pattern as the sole presenting clinical manifestation initially misdiagnosed as IPF. CASE PRESENTATION: A 70-year-old male presented to the pulmonary clinic for management of chronic hypoxic respiratory failure. He was diagnosed 5 years prior with IPF based on CT findings, without further workup. He was started on nintedanib with minimal progression in his disease, radiographically, symptomatically and on pulmonary function tests, over the five years since his initial diagnosis. He required 1 liter of continuous oxygen. Pulmonary function tests showed FVC 68%, TLC 60% and DLCO 44% predicted. High resolution CT scan of the chest showed UIP pattern with heterogenous subpleural honeycombing, traction bronchiectasis, and anterior upper lobe sign. History and physical exam were significant for prior tobacco use, rhinitis, and jet fuel exposure without symptoms of autoimmune disease. Patient was referred for lung transplant evaluation and work up was completed which revealed positive PL-7 antibody. The patient did not have any additional symptoms of anti-synthetase syndrome including myopathy, mechanic's hands, arthritis, or Raynaud phenomenon. Rheumatology was consulted and agreed with the diagnosis of anti-synthetase syndrome, with ILD as the sole current manifestation and initiated mycophenolate for immunosuppression. Clinical follow up demonstrated symptomatic improvement with decreased dyspnea, increased exercise tolerance, and increased FVC and TLC to 79% and 72% predicted respectively. DISCUSSION: Here we presented a case of anti-synthetase syndrome presenting solely with ILD that was misdiagnosed as IPF due to imaging pattern. While the UIP pattern is commonly associated with IPF it is also seen in other causes of pulmonary fibrosis such as CTD-ILD. CONCLUSIONS: This case highlights the importance of a complete workup for treatable systemic diseases when patients present with a new diagnosis of ILD with UIP pattern. Failure to do so will result in increased morbidity and mortality, particularly when findings consistent with CTD-ILD are present. REFERENCE #1: Vij R, Strek ME. Diagnosis and treatment of connective tissue disease-associated interstitial lung disease. Chest. 2013 Mar;143(3):814-824 REFERENCE #2: Mecoli CA, Christopher-Stine L. Management of Interstitial Lung Disease in Patients With Myositis Specific Autoantibodies. Curr Rheumatol Rep. 2018 Apr 10;20(5):27 REFERENCE #3: Jiang M, Dong X, Zheng Y. Clinical characteristics of interstitial lung diseases positive to different anti-synthetase antibodies. Medicine (Baltimore). 2021 May 14;100(19):e25816 DISCLOSURES: No disclosure on file for Roxanne Garcia Orr No relevant relationships by Dylan McCreary No relevant relationships by Lauren Ridenour No relevant relationships by Clement Singarajah" @default.
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• W4387247836 date "2023-10-01" @default.
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• W4387247836 title "ANTI-SYNTHETASE SYNDROME: A CASE OF MISTAKEN IDENTITY" @default.
• W4387247836 doi "https://doi.org/10.1016/j.chest.2023.07.3668" @default.
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