Abstract Introduction Anti-Jo 1 and Anti-SSA antibodies both independently cause interstitial lung disease(ILD). We present a case with overlap of these antibodies causing rapidly progressive ILD with severe refractory hypoxia requiring ECMO. This scenario is exceedingly rare and underreported. Case-presentation A 61-year-old female with known lupus on chronic Prednisone and Plaquenil, and no known lung disease presented with shortness of breath. Her symptoms began 6 weeks prior when she was hospitalized for pneumonia after mild infiltrates were seen on CT-scan. She was discharged on antibiotics and 2 L home oxygen. Two weeks later she presented for nephrolithiasis but was noted to have persistent infiltrates in the lung bases on CT abdomen. She was referred to see a pulmonologist outpatient for possible ILD. 1 week later she called an ambulance due to acute shortness a breath. She was hypoxic to 77% on 2 L of oxygen and was placed on high-flow nasal cannula. A CT chest showed progressively worsening infiltrates with a reticular interstitial prominence more so in the subpleural distribution. She was given pulse dose steroids and antibiotics with intermittent diuresis. Despite these efforts she continued to have worsening respiratory failure and was intubated. After intubation she had significantly elevated peak pressures and persistent hypoxia with respiratory acidosis. Multiple different modes of ventilation as well as sedation and paralytics were tried. However, she continued to have refractory hypoxia and was cannulated for Veno-venous ECMO. A bronchoalveolar lavage(BAL) was done with no endobronchial evidence of alveolar hemorrhage. Infectious workup was sent, all of which remained negative. Rheumatological workup resulted in positive anti Jo-1 and anti-SSA antibodies. Her oxygen requirement failed to improve despite steroids and antibiotics. Repeat CT showed rapidly worsening disease over 5 days. Hospital course was complicated by shock requiring vasopressors, atrial fibrillation and a large cerebral infarct. Given her poor prognosis, family decided on comfort directed care only. Discussion Anti synthetase syndrome and Sjogren’s disease can both lead to ILD. Anti-Jo-1 may cause acute hypoxic respiratory failure often without preceding muscle symptoms, which makes recognition difficult. Sjogren’s disease with Anti-SSA can also present similarly. Both antibodies lead to severe lung restriction with poor response to standard immunosuppression. This particular overlap of antibodies has significantly high mortality due to rapidly progressive and treatment resistant ILD. While this has been recognized, the need for VV ECMO due to refractory hypoxia is remarkably rare. Published case reviews have not reported ECMO use. This abstract is funded by: None
Khan et al. (Fri,) studied this question.