• Frédéric Pène, Tarik Hissem, Alice Bérezné, Yannick Allanore, Guillaume Geri, Julien Charpentier, Jérôme Avouac, Loïc Guillevin, Alain Cariou, Jean-Daniel Chiche, Jean-Paul Mira, and Luc Mouthon.
• From the Réanimation médicale, and Médecine Interne, and Rhumatologie A, Centre National de Référence des maladies systémiques et autoimmunes rares, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP); Faculté de Médecine, Université Paris Descartes, Sorbonne Paris Cité, Paris, France.F. Pène, MD, PhD, Réanimation médicale, Hôpital Cochin, AP-HP, and Faculté de Médecine, Université Paris Descartes, Sorbonne Paris Cité; T. Hissem, MD, Réanimation médicale, Hôpital Cochin, AP-HP; A. Bérezné, MD, Médecine Interne, and Centre National de Référence des maladies systémiques et autoimmunes rares, Hôpital Cochin, AP-HP; Y. Allanore, MD, PhD, Rhumatologie A, Hôpital Cochin, AP-HP, and Faculté de Médecine, Université Paris Descartes, Sorbonne Paris Cité; G. Geri, MD, Réanimation médicale, Hôpital Cochin, AP-HP, and Faculté de Médecine, Université Paris Descartes, Sorbonne Paris Cité; J. Charpentier, MD, Réanimation médicale, Hôpital Cochin, AP-HP; J. Avouac, MD, PhD, Rhumatologie A, Hôpital Cochin, AP-HP, and Faculté de Médecine, Université Paris Descartes, Sorbonne Paris Cité; L. Guillevin, MD, Médecine Interne, and Centre National de Référence des maladies systémiques et autoimmunes rares, Hôpital Cochin, AP-HP, and Faculté de Médecine, Université Paris Descartes, Sorbonne Paris Cité; A. Cariou, MD, PhD; J.D. Chiche, MD, PhD; J.P. Mira, MD, PhD, Réanimation médicale, Hôpital Cochin, AP-HP, and Faculté de Médecine, Université Paris Descartes, Sorbonne Paris Cité; L. Mouthon, MD, PhD, Médecine Interne, and Centre National de Référence des maladies systémiques et autoimmunes rares, Hôpital Cochin, AP-HP, and Faculté de Médecine, Université Paris Descartes, Sorbonne Paris Cité. frederic.pene@cch.aphp.fr.
• J Rheumatol. 2015 Aug 1; 42 (8): 1406-12.

ObjectivePatients with systemic sclerosis (SSc) are prone to disease-specific or treatment-related life-threatening complications that may warrant intensive care unit (ICU) admission. We assessed the characteristics and current outcome of patients with SSc admitted to the ICU.MethodsWe performed a single-center retrospective study over 6 years (November 2006-December 2012). All patients with SSc admitted to the ICU were enrolled. Short-term (in-ICU and in-hospital) and longterm (6-mo and 1-yr) mortality rates were studied, and the prognostic factors were analyzed.ResultsForty-one patients with a median age of 50 years [interquartile range (IQR) 40-65] were included. Twenty-nine patients (72.5%) displayed diffuse cutaneous SSc. The time from diagnosis to ICU admission was 78 months (IQR 34-128). Twenty-eight patients (71.7%) previously had pulmonary fibrosis, and 12 (31.5%) had pulmonary hypertension. The main reason for ICU admission was acute respiratory failure in 27 patients (65.8%). Noninvasive ventilation was first attempted in 13 patients (31.7%) and was successful in 8 of them, whereas others required endotracheal intubation within 24 h. Altogether, 13 patients (31.7%) required endotracheal intubation and mechanical ventilation. The overall in-ICU, in-hospital, 6-month, and 1-year mortality rates were 31.8%, 39.0%, 46.4%, and 61.0%, respectively. Invasive mechanical ventilation was the worst prognostic factor, associated with an in-hospital mortality rate of 84.6%.ConclusionThis study provides reliable prognostic data in patients with SSc who required ICU admission. The devastating outcome of invasive mechanical ventilation in patients with SSc requires a reappraisal of indications for ICU admission and early identification of patients likely to benefit from noninvasive ventilation.

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