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 - Université de Bourgogne Accéder directement au contenu
Article Dans Une Revue Annals of Intensive Care Année : 2018

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

1 CHU Tenon [AP-HP]
2 ICAN - Unité de Recherche sur les Maladies Cardiovasculaires, du Métabolisme et de la Nutrition = Research Unit on Cardiovascular and Metabolic Diseases
3 Service de réanimation médicale
4 Service de Réanimation Médicale, Hôpital de la Croix-Rousse, Hospices Civils de Lyon, Université de Lyon, Lyon, France
5 CHU Pitié-Salpêtrière [AP-HP]
6 Service de Réanimation Médicale (CHU de Dijon)
7 UNICANCER/Lille - Centre Régional de Lutte contre le Cancer Oscar Lambret [Lille]
8 LIRIC - Lille Inflammation Research International Center - U 995
9 RID-AGE - Facteurs de Risque et Déterminants Moléculaires des Maladies liées au Vieillissement - U 1167
10 Reanimation Médicale et Infectieuse [Bichat]
11 CHU de Poitiers - Centre hospitalier universitaire de Poitiers = Poitiers University Hospital
12 CHU Saint-Antoine [AP-HP]
13 Service de Réanimation Médicale [CHU Grenoble]
14 Service de Réanimation Médico-Chirurgicale [Avicenne]
15 Service de Réanimation Médicale [Strasbourg]
16 Service de Réanimation Médicale [CHU Rouen]
17 Service de réanimation médicale [CHU HEGP]
18 CHRU Montpellier - Centre Hospitalier Régional Universitaire [Montpellier]
19 PhyMedExp - Physiologie & médecine expérimentale du Cœur et des Muscles [U 1046]
20 CHU - BREST - Réa Med - CHRU Brest - Service de Réanimation Médicale
21 Centre Hospitalier Le Mans (CH Le Mans)
22 Service de Réanimation Médicale, Hôpital Saint Louis, Paris, France
23 Immunologie clinique [CHU St-Louis]
24 Centre Hospitalier Victor Dupouy
Constance Vuillard
  • Fonction : Auteur
Nicolas de Prost
Matthieu Schmidt
Guillaume Dumas
Eric Maury
  • Fonction : Auteur
  • PersonId : 981199
Sylvie Ricome
  • Fonction : Auteur
  • PersonId : 914569
Gwenhael Colin
  • Fonction : Auteur
Christophe Guitton
  • Fonction : Auteur
  • PersonId : 898392

Résumé

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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Dates et versions

hal-02005941 , version 2 (25-02-2020)
hal-02005941 , version 1 (10-02-2023)

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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, 2018, 8 (1), pp.87. ⟨10.1186/s13613-018-0433-3⟩. ⟨hal-02005941v2⟩
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