Literature DB >> 33643437

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

Caroline Sampson1, Jennifer Taylor1, Luke Dyson2, Mostafa Hassanein1.   

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. © The Intensive Care Society 2019.

Entities:  

Keywords:  Antisynthetase syndrome; extra-corporeal membrane oxygenation; severe acute respiratory failure

Year:  2019        PMID: 33643437      PMCID: PMC7890757          DOI: 10.1177/1751143719840260

Source DB:  PubMed          Journal:  J Intensive Care Soc        ISSN: 1751-1437


  10 in total

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Review 5.  Antisynthetase syndrome.

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6.  Severe respiratory failure as a presenting feature of an interstitial lung disease associated with anti-synthetase syndrome (ASS).

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7.  Outcome of Patients with Interstitial Lung Disease Treated with Extracorporeal Membrane Oxygenation for Acute Respiratory Failure.

Authors:  Franziska C Trudzinski; Franziska Kaestner; Hans-Joachim Schäfers; Sebastian Fähndrich; Frederik Seiler; Philip Böhmer; Oliver Linn; Ralf Kaiser; Hendrik Haake; Frank Langer; Robert Bals; Heinrike Wilkens; Philipp M Lepper
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8.  Acute Respiratory Failure as the First Manifestation of Antisynthetase Syndrome.

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10.  Rituximab in severe, treatment-refractory interstitial lung disease.

Authors:  Gregory J Keir; Toby M Maher; Damien Ming; Reza Abdullah; Angelo de Lauretis; M Wickremasinghe; Andrew G Nicholson; David M Hansell; Athol U Wells; Elisabetta A Renzoni
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  10 in total

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