Sanchit Chawla, MD
Internal Medicine resident
n/a, United States
Disclosure information not submitted.
Dina Alayan, MD
Internal Medicine resident
Cleveland Clinic, United States
Disclosure information not submitted.
Bassel Akbik, MD
Intensivist
Cleveland Clinic
Cleveland, Ohio, United States
Disclosure information not submitted.
Title: Rare case of Anti-synthetase syndrome (AS) manifesting as acute respiratory distress syndrome (ARDS)
Introduction: AS is an autoimmune condition, characterized by antibodies directed against an aminoacyl transfer RNA synthetase. AS has a highly heterogenous disease pattern due to different organ involvement. The initial clinical presentation can be variable. We present an atypical case of AS with ARDS.
Description: A 72-year-old female, retired teacher, with no significant past medical history presented to the emergency department with complaints of progressively worsening shortness of breath for 3 to 4 weeks, associated with fever, nausea, vomiting, and diarrhea. Otherwise review of symptoms was negative. Patient was hypotensive, tachycardic and hypoxic requiring hi-flow nasal canula (FiO2 90%). A chest CT was negative for thromboembolic disease but did show bilateral patchy ground glass opacities with traction bronchiectasis and confluent consolidation in both upper lobes. Patient was admitted to the medical intensive care unit and initiated on antibiotics, but her hypoxemia progressively worsened requiring mechanical ventilation for ARDS. She required multiple proning sessions. Her bronchoscopy with bronchoalveolar lavage was negative for infectious etiology including COVID test. Given her persistent hypoxia, patient was started on methylprednisolone due to concerns for interstitial lung disease (ILD). Patient’s oxygenation significantly improved, and she was extubated 10 days later. Further investigations revealed positive anti-PL-7 directed against threonyl t-RNA synthetase. Case was discussed in the ILD multidisciplinary conference with pulmonary and thoracic surgery. In the light of her ongoing oxygen requirements, there was high suspicion of rapidly progressive form of anti-synthetase syndrome. Therefore, she was started on 60 mg prednisone, tacrolimus, and cyclophosphamide. Patient was discharged to a skilled-nursing facility.
Discussion: AS can be a challenging diagnosis, especially in patients without any muscular or rheumatological symptoms that present with ARDS. It is critical to have a broad differential for patients with non-resolving ARDS. ILD as the initial presentation of AS is a major cause of mortality. Currently there is limited evidence-based recommendations to guide therapy. Additional randomized clinical trials would be needed to support guidelines directing the management of AS ILD flare.