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A3067 - Why Won't this Pneumonia Go Away
Author Block: L. A. Benninger, R. G. Valentin, I. Faruqi; University of Florida, Gainesville, FL, United States.
Sepsis is one of the leading reasons for admission to the intensive care unit (ICU). While there are numerous causes for sepsis, pneumonia is one of the most frequently encountered. However, due to similarly appearing diseases, pneumonia is often over-diagnosed. We present a unique case of a patient who was admitted to the ICU with a presumed diagnosis of recurrent pneumonia who was found to have another disease, anti-synthetase syndrome (aSS). Our patient is a 37-year-old African American male with past medical history of previously diagnosed rheumatoid arthritis who presented to the medical ICU with dyspnea and found to have hypoxia requiring high-flow nasal cannula and imaging consistent with multifocal infiltrates. This current encounter marked his fourth hospital admission within a six-month period for the same signs and symptoms. He underwent bronchoscopy and was started on broad-spectrum antibiotics. Cultures were negative. Further history from the patient revealed occasional joint pain and muscle weakness but no skin or joint findings on physical exam. Given the appearance on imaging and history of autoimmune disorder, additional testing was completed to reveal a positive anti-PL-12. A diagnosis of aSS was made. He was subsequently started on pulse dose steroids, followed by oral prednisone, as well as increasing doses of mycophenolate mofetil. Once treatment was initiated, our patient’s oxygen requirement rapidly decreased and his symptoms improved. aSS is a separate disease entity within the spectrum of idiopathic inflammatory myopathies. While aSS is a rare diagnosis, understanding and recognition of this syndrome continues to grow. aSS has a constellation of clinical findings including constitutional symptoms, myositis, mechanic’s hands, arthritis and interstitial lung disease (ILD). There are several autoantibodies associated with this syndrome including Jo, PL-7. PL-12, OJ, EJ KS, Zo, and Ha. Patients with autoantibodies PL-7 and PL-12 are more likely to have ILD and gastrointestinal manifestations and less commonly myositis compared to individuals with positive autoantibodies to Jo. Due to aSS’s frequency for progressive disease, the combination of systemic glucocorticoids and a second agent such as azathioprine, mycophenolate mofetil, calcineurin inhibitors or methotrexate are usually initiated. Increasing awareness of this syndrome, as well as prompt diagnosis with initiation of therapy, is vital to improving not only the life span but also the quality of life for these patients.