A RARE AND ATYPICAL PRESENTATION OF ANTISYNTHETASE SYNDROME

Abstract

SESSION TITLE: Tuesday Fellows Case Report Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/22/2019 01:00 PM - 02:00 PM INTRODUCTION: Antisynthetase syndrome (AS) is a rare disease that can be challenging to diagnose, especially with an atypical presentation. The diagnosis of AS requires the presence of several antisynthetase autoantibodies and one of the following: fever, inflammatory polyarthritis, myositis, interstitial lung disease (ILD), Raynaud’s phenomenon or “mechanic’s hands”. We discuss a rare and atypical presentation of AS with acute respiratory distress syndrome (ARDS). CASE PRESENTATION: A 30-year-old Caucasian female without prior medical history presented to an outside hospital with dyspnea, nonproductive cough and chest discomfort for 2 weeks. A CT angiogram of the chest showed dense interstitial and alveolar opacities throughout the left lung and right lower lobe. She was treated for community acquired pneumonia with IV antibiotics for 5 days, without resolution of symptoms. Her respiratory status worsened and was transferred to our institution where she underwent orotracheal intubation for severe hypoxemia refractory to 100% oxygen via a non-rebreather mask. Further workup with an echocardiogram and bronchoscopy was non-revealing for a cause of ARDS, including a negative sequential lavage. Infectious workup including cultures was also negative and she had no fevers. Her review of systems was revisited and disclosed arthralgias, a rash on her forearms and symmetric weakness in handgrip in the preceding 4 weeks. With a high suspicion for an autoimmune cause of ARDS, an autoimmune panel was ordered and high-dose steroids were initiated. She was successfully weaned off mechanical ventilation on day 5 and eventually transferred out of the ICU. A few days later, her autoimmune workup resulted with a positive antinuclear (speckled pattern, 1:320), anti-ribonucleoprotein and anti-Jo-1 (>8.0) antibodies. Steroids were tapered over 14 days and mycophenolate was initiated and gradually titrated outpatient. Mycophenolate was later changed to azathioprine due to side effect of nausea. At 4 year follow up, her disease and symptoms remain well controlled with azathioprine, rituximab and steroids. DISCUSSION: Though ILD is a common manifestation of AS, severe presentations with ARDS are rare with a few previous reports in the literature 1-3. The patient had a good outcome with initiation of high dose steroids in a timely fashion. This case highlights the importance of considering non-infectious causes of pneumonia and ARDS in cases that do not respond appropriately to standard therapies. It also emphasizes the significance of detailed history taking since reaching a diagnosis is challenging in the absence of confirmatory serologic testing. CONCLUSIONS: It is important to have a broad differential for patients with nonresolving pneumonia, including autoimmune etiologies such as in this case. Early recognition and treatment can result in improved outcomes. Reference #1: Katzap E, Barilla-LaBarca ML, Marder G. Antisynthetase syndrome. Curr Rheumatol Rep. 2011;13(3):175-181. Reference #2: Kanchustambham VK, Saladi S, Mahmoudassaf S, Patolia S. Antisynthetase syndrome (ASS) presenting as acute respiratory distress syndrome (ARDS) in a patient without myositis features. BMJ Case Rep. 2016;2016. Reference #3: Malhotra G, Ramreddy N, Chua S, Iliescu M, Kaur T. A Curious Case of Acute Respiratory Failure: Is It Antisynthetase Syndrome? Case Rep Crit Care. 2016;2016:7379829. DISCLOSURES: No relevant relationships by Rodrigo Cavallazzi, source=Web Response No relevant relationships by Bilal Jalil, source=Web Response No relevant relationships by Hassan Zeb, source=Web Response