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 Romaric Larcher Sylvie Ricome Raphael Le Mao 16 Gwenhael Colin Christophe Guitton 17 Lara Zafrani 18 Elise Morawiec 19 Marie Dubert 20 Olivier Pajot 21 Hervé Mentec 21 Gaetan Plantefeve 21 Damien Contou 21, *
Abstract : Background Anti-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. Results From 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). Conclusions Intensivists 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
Contributeur : Lnc - Université de Bourgogne <>
Soumis le : lundi 4 février 2019 - 12:05:06
Dernière modification le : vendredi 26 juillet 2019 - 01:29:09

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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, BioMed Central, 2018, 8 (1), pp.87. ⟨https://annalsofintensivecare.springeropen.com/articles/10.1186/s13613-018-0433-3⟩. ⟨10.1186/s13613-018-0433-3⟩. ⟨hal-02005941⟩

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