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Clinical features and outcome of patients with acute respiratory failure revealing anti-synthetase or anti-MDA-5 dermato-pulmonary syndrome: A French multicenter retrospective study

Annals of Intensive Care Sep 16, 2018

Vuillard C, et al. - Given that anti-synthetase (AS) and anti-melanoma differentiation-associated gene 5 (aMDA-5) syndromes are near one of the other autoimmune inflammatory myopathies potentially responsible for severe acute interstitial lung disease leading to acute respiratory distress syndrome (ARDs) and may be clinically indistinguishable from one another, researchers studied the clinical presentation and the outcome of 47 patients admitted to the intensive care unit (ICU) for acute respiratory failure (ARF) revealing AS or aMDA-5 syndromes in a 13-year retrospective multicenter study in 35 French ICUs. Researchers also examined predictive factors of hospital mortality in this patient population. A total of 42 patients (89%) had ARDS, which was severe in 86%. Corticosteroids, cyclophosphamide, rituximab, intravenous immunoglobulins and plasma exchange were administered to 100%, 72%, 15%, 21% and 17% of patients, respectively. Patients with aMDA-5 dermato-pulmonary syndrome had a higher rate of hospital mortality vs those with AS syndrome. Based on the findings, intensivists were recommended to consider inflammatory myopathies as a cause of ARF of unknown origin.

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