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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
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Outcome of Patients with Systemic Sclerosis in the Intensive Care Unit
Frédéric Pène1, Tarik Hissem2, Alice Bérezné2
1From 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.
Objective:
Patients 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.
Methods:
We 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.
Results:
Forty-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%.
Conclusion:
This 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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