TY - JOUR T1 - Outcome of Patients with Systemic Sclerosis in the Intensive Care Unit JF - The Journal of Rheumatology JO - J Rheumatol SP - 1406 LP - 1412 DO - 10.3899/jrheum.141617 VL - 42 IS - 8 AU - Frédéric Pène AU - Tarik Hissem AU - Alice Bérezné AU - Yannick Allanore AU - Guillaume Geri AU - Julien Charpentier AU - Jérôme Avouac AU - Loïc Guillevin AU - Alain Cariou AU - Jean-Daniel Chiche AU - Jean-Paul Mira AU - Luc Mouthon Y1 - 2015/08/01 UR - http://www.jrheum.org/content/42/8/1406.abstract N2 - 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. ER -