Veno-venous ECMO for rapidly progressing interstitial lung disease: A multidisciplinary approach

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.

Keywords: antisynthetase syndrome; myositis related interstitial lung disease; rapidly progressing interstitial lung disease; respiratory failure; veno-venous Extracorporeal Membrane oxygenation.

Fig. 1.

CT Thorax on Day 2 of admission showing extensive bilateral consolidation and surrounding nodular ground glass opacification, in keeping with multi-lobar pneumonia

Fig. 2.

CT Thorax on Day 15 after clinical deterioration showing dense bilateral infiltrates throughout both lung in keeping with ARDS and possible organizing pneumonia. There was no pneumothorax or pleural effusions.

Fig. 3.

CT Thorax performed on Day 39 shortly after discontinuation of ECMO showed nodular and ground glass opacification which have improved significantly in the interim. There was some early traction bronchiectasis.

Fig. 4.

CT Thorax performed Day 92 prior to discharge home showing further improvement of both ground glass opacification and reticular shadowing with minor traction bronchiectasis.

Fig. 5.

CT Thorax performed 2 years after initial presentation showing resolution of bilateral infiltrates. Ongoing upper lobe predominant reticulations and traction bronchiectasis were evident in keeping with interstitial fibrosis.