Abstract
Introduction
This is a unique case of fulminant respiratory failure secondary to a rare cause of rapidly progressing ILD; antisynthetase syndrome (ASS). Failure to deliver timely multi-modal treatment in these cases can lead to increased morbidity and mortality.
Case presentation
A previously healthy 27-year-old male presented to his local hospital with a 1-week history of malaise, shortness of breath and cough. Initial work up including bloods and imaging were suggestive of community acquired multi lobar pneumonia, for which he received treatment as per local guidelines. Unfortunately, despite broad empirical antimicrobial cover, he continued to deteriorate with worsening type-1 respiratory failure requiring intubation and subsequent institution of prone position ventilation. Extensive microbiological investigations yielded no positive results. On day 7 of admission immunological testing revealed an ENA screen positive for Jo-1 antibody and a diagnosis of ASS was made. Despite treatment with immunosuppression the patient’s condition rapidly deteriorated and the decision to support with V-V ECMO was made following MDT consideration as there remained uncertainty as to the extent of reversibility of the underlying condition.
Conclusions
This patient recovered with combination of conventional immunosuppression, therapeutic plasma exchange and ECMO support. This case highlights Antisynthetase syndrome as a cause of reversible interstitial lung disease in the ICU and the importance of multi-disciplinary decision making and aggressive treatment approach in the management of such conditions.