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• W4387248081 abstract "SESSION TITLE: Pulmonary Manifestations of Systemic Disease Case Report Posters 11 SESSION TYPE: Case Report Posters PRESENTED ON: 10/10/2023 09:40 am - 10:25 am INTRODUCTION: Idiopathic inflammatory myopathies are a group of autoimmune disorders generally characterized by progressive muscle inflammation leading to marked weakness. These disorders include dermatomyositis, polymyositis, inclusion body myosotis and antisynthetase syndrome (AS). The subtypes can be differentiated by the variation of extra-muscular symptoms and disease-specific serologic marker. Antisynthetase syndrome is a specific constellation of the following symptoms: fever, myositis, inflammatory arthritis, Raynaud's phenomenon, and Interstitial Lung Disease (ILD) though not all need be present. CASE PRESENTATION: A 44-year-old Caucasian male with a history of recent subsegmental pulmonary embolism who presented to the hospital with his third bout of pneumonia in 4 months. He endorsed dysphagia, odynophagia and voice change during this timeframe. He is an active smoker and recently stopped daily drinking of alcohol. He works as a cashier and was very healthy prior to this. Family history is non-contributory.On admission, patient was septic treated with intravenous fluids and empiric antibiotics. Chest CT scan showed extensive ground-glass opacities and bilateral consolidations (Figure 1). Respiratory workup was grossly negative including COVID-19. Physical exam of mild left foot drop coupled with muscle pain and dysphagia were concerning for and given an assumptive diagnosis of Amyotrophic Lateral Sclerosis (ALS) as per neurology consultation. Modified barium swallow, MRI imaging, lumbar puncture and electromyogram were negative. A myositis panel was sent but needed 2 weeks to result.After mild improvement initially, the patient developed new aspiration pneumonia and acute hypoxic respiratory failure. He deteriorated rapidly necessitating intubation and developed acute respiratory distress syndrome requiring maximal ventilatory settings. CT Chest showed further worsening of bilateral ground glass and consolidative airspace disease. He did not improve despite empiric antibiotics and pulse dose steroids.After prolonged ICU course, myositis panel eventually resulted positive for Anti-PL-12 antibody. Patient was started on another 3-day pulse dose regimen of Methylprednisolone 1,000mg followed by Prednisone 60mg daily with Trimethoprim-Sulfamethoxazole prophylaxis and Mycophenolate ramp up to 1500mg BID. He recovered slowly and eventually extubated with discharge to rehab. Patient was seen in the clinic for follow up after discharge. Repeat CT scan showed significant improvement and near resolution of ground-glass opacities (Figure 2). DISCUSSION: Antisynthetase syndrome is a rare condition with incidence of 9 per 100,000 people. Patients can present with an incongruent array of symptoms. Our patient had dysphagia and lung disease but did not display other classic features of AS which were mistaken for ALS. It is very possible that this patient could have been interned to his poor prognosis. Thankfully, the expanded myositis panel revealed AS prompting timely initiation of steroids and Mycophenolate. The patient slowly improved clinically but eventually recovered fully and now is back to functional baseline. CONCLUSIONS: Clinicians, including pulmonologist, should consider antisynthetase syndrome as differential diagnosis in patients who presents with recurrent pneumonia. Its symptomology is often murky and can be easily missed or misdiagnosed leading to significant morbidity and delayed treatment. Expanded myositis panel, which take prolonged laboratory processing, are essential for proper workup and diagnosis. Timely treatment with steroids and Mycophenolate is necessary to treating this condition. REFERENCE #1: Pinal-Fernandez I, Casal-Dominguez M, Huapaya JA, et al. A longitudinal cohort study of the anti-synthetase syndrome: increased severity of interstitial lung disease in black patients and patients with anti-PL7 and anti-PL12 autoantibodies. Rheumatology (Oxford). 2017;56(6):999-1007. doi:10.1093/rheumatology/kex021 REFERENCE #2: Ponce-Gallegos MA, Ramos-Martínez E, García-Carmona A, et al. Genetic Susceptibility to Antisynthetase Syndrome Associated With Single-Nucleotide Variants in the IL1B Gene That Lead Variation in IL-1β Serum Levels. Front Med (Lausanne). 2020;7:547186. Published 2020 Nov 24. doi:10.3389/fmed.2020.547186 REFERENCE #3: Theilacker LR, Brandão FS, Goulart FV, Vaz JL, D'Almeida LO, da Fonseca Salgado MC. Antisynthetase Syndrome: two case report and literature review. Rev Bras Reumatol. 2015;55(2):177-180. doi:10.1016/j.rbr.2013.05.007 DISCLOSURES: No relevant relationships by Guido Barmaimon No relevant relationships by Keith Brenner No disclosure on file for Weekon Choi No disclosure on file for Robert Lee No relevant relationships by Karan Omidvari No relevant relationships by Ahmad Qatanani No relevant relationships by Mehek Rahim No relevant relationships by Keith Rose No relevant relationships by Sean Sadikot" @default.
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• W4387248081 date "2023-10-01" @default.
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• W4387248081 title "ANTISYNTHETASE SYNDROME: A CASE OF MISTAKEN IDENTITY" @default.
• W4387248081 doi "https://doi.org/10.1016/j.chest.2023.07.3539" @default.
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