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

Vuillard, Constance; Pineton de Chambrun, Marc; de Prost, Nicolas; Guérin, Claude; Schmidt, Matthieu; Dargent, Auguste; Quenot, Jean-Pierre; Préau, Sébastien; Ledoux, Geoffrey; Neuville, Mathilde; Voiriot, Guillaume; Fartoukh, Muriel; Coudroy, Rémi; Dumas, Guillaume; Maury, Eric; Terzi, Nicolas; Tandjaoui-Lambiotte, Yacine; Schneider, Francis; Grall, Maximilien; Guérot, Emmanuel; Larcher, Romaric; Ricome, Sylvie; Le Mao, Raphaël; Colin, Gwenhaël; Guitton, Christophe; Zafrani, Lara; Morawiec, Elise; Dubert, Marie; Pajot, Olivier; Mentec, Hervé; Plantefève, Gaëtan; Contou, Damien

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.