Life-threatening respiratory failure requiring extra-corporeal membrane oxygenation secondary to the anti-synthetase syndrome.

Abstract

Veno-venous extra-corporeal membrane oxygenation (VV ECMO) provides support in severe acute respiratory failure (SARF) refractory to maximal conventional ventilatory support. ECMO does not treat the lungs per se, but allows time for the underlying condition to reverse or resolve. Common indications include acute respiratory distress syndrome (ARDS) and life-threatening asthma. On occasion, rarer conditions causing respiratory failure are diagnosed during ECMO support. The anti-synthetase syndrome (ASS) comprises of a group of conditions characterised by the presence of anti-aminoacyl-tRNA-synthetase antibodies with one or more of interstitial lung disease, inflammatory myositis and/or arthritis. Mainstay of management is immunosuppression. Here, we present two patients requiring ECMO support for SARF, whose respiratory function failed to respond to usual treatment for their assumed pneumonia. Both showed a rapid improvement in respiratory function and oxygenation once immunosuppressive therapy was instigated. Further testing revealed anti-synthetase antibodies, therefore both went on to receive further immunosuppression and an ultimately good outcome. Despite life-threatening respiratory failure, VV ECMO support allowed time for stabilisation, diagnosis and treatment. Outcomes in acute inflammatory interstitial pneumonitis are improved if immunosuppressive treatment is initiated as soon as possible. Our experience with these two cases have led to an institutional change in practice to send an urgent auto-antibody screen (including extractable nuclear antigen panel) on admission for all our SARF patients.