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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

Constance Vuillard Marc Pineton de Chambrun 1 Nicolas de Prost 2 Claude Guérin 3 Matthieu Schmidt 4 Auguste Dargent 5 Jean-Pierre Quenot 5 Sébastien Préau 6 Geoffrey Ledoux 6 Mathilde Neuville 7 Guillaume Voiriot 8 Muriel Fartoukh 8 Rémi Coudroy 9 Guillaume Dumas 10 Eric Maury 10 Nicolas Terzi 11 Yacine Tandjaoui-Lambiotte 12 Francis Schneider 13 Maximilien Grall 14 Emmanuel Guérot 15 R. Larcher 16, 17 Sylvie Ricome Raphael Le Mao 18 Gwenhael Colin Christophe Guitton 19 Lara Zafrani 20 Elise Morawiec 21 Marie Dubert 22 Olivier Pajot 23 Hervé Mentec 23 Gaetan Plantefeve 23 Damien Contou 23, *
Abstract : BackgroundAnti-synthetase (AS) and dermato-pulmonary associated with anti-MDA-5 antibodies (aMDA-5) syndromes are near one of the other autoimmune inflammatory myopathies potentially responsible for severe acute interstitial lung disease. We undertook a 13-year retrospective multicenter study in 35 French ICUs in order to describe the clinical presentation and the outcome of patients admitted to the ICU for acute respiratory failure (ARF) revealing AS or aMDA-5 syndromes.ResultsFrom 2005 to 2017, 47 patients (23 males; median age 60 [1st–3rd quartiles 52–69] years, no comorbidity 85%) were admitted to the ICU for ARF revealing AS (n = 28, 60%) or aMDA-5 (n = 19, 40%) syndromes. Muscular, articular and cutaneous manifestations occurred in 11 patients (23%), 14 (30%) and 20 (43%) patients, respectively. Seventeen of them (36%) had no extra-pulmonary manifestations. C-reactive protein was increased (139 [40–208] mg/L), whereas procalcitonine was not (0.30 [0.12–0.56] ng/mL). Proportion of patients with creatine kinase ≥ 2N was 20% (n = 9/47). Forty-two patients (89%) had ARDS, which was severe in 86%, with a rate of 17% (n = 8/47) of extra-corporeal membrane oxygenation requirement. Proportion of patients who received corticosteroids, cyclophosphamide, rituximab, intravenous immunoglobulins and plasma exchange were 100%, 72%, 15%, 21% and 17%, respectively. ICU and hospital mortality rates were 45% (n = 21/47) and 51% (n = 24/47), respectively. Patients with aMDA-5 dermato-pulmonary syndrome had a higher hospital mortality than those with AS syndrome (n = 16/19, 84% vs. n = 8/28, 29%; p = 0.001).ConclusionsIntensivists should consider inflammatory myopathies as a cause of ARF of unknown origin. Extra-pulmonary manifestations are commonly lacking. Mortality is high, especially in aMDA-5 dermato-pulmonary syndrome.
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https://hal-univ-bourgogne.archives-ouvertes.fr/hal-02005941
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Soumis le : mardi 25 février 2020 - 14:49:41
Dernière modification le : lundi 19 octobre 2020 - 10:52:26
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Constance Vuillard, Marc Pineton de Chambrun, Nicolas de Prost, Claude Guérin, Matthieu Schmidt, et al.. 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, SpringerOpen, 2018, 8 (1), pp.87. ⟨10.1186/s13613-018-0433-3⟩. ⟨hal-02005941⟩

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