PT - JOURNAL ARTICLE AU - Etienne-Marie Jutant AU - Guillaume Voiriot AU - Vincent Labbé AU - Laurent Savale AU - Hayat Mokrani AU - Patrick Van Dreden AU - Grigorios Gerotziafas AU - Muriel Fartoukh TI - Endothelial dysfunction and hypercoagulability in severe sickle-cell acute chest syndrome AID - 10.1183/23120541.00496-2021 DP - 2021 Oct 01 TA - ERJ Open Research PG - 00496-2021 VI - 7 IP - 4 4099 - http://openres.ersjournals.com/content/7/4/00496-2021.short 4100 - http://openres.ersjournals.com/content/7/4/00496-2021.full SO - erjor2021 Oct 01; 7 AB - Rationale Acute pulmonary hypertension (PH) may develop during sickle-cell acute chest syndrome (ACS), and is associated with an increased mortality. Its mechanisms remain poorly known. We questioned whether there is endothelial dysfunction and hypercoagulability in severe ACS, with and without acute PH.Methods In a prospective monocentre cohort follow-up study, all sickle-cell adult patients with ACS admitted to the intensive care unit underwent transthoracic echocardiography and measurement of biomarkers of coagulation, endothelial activation and platelet and erythrocyte activation. Acute PH was defined as a high echocardiographic probability of PH. The biological profiles of sickle-cell patients were analysed at the time of ACS, contrasting with the existence of acute PH, and compared with steady-state and with non-sickle-cell controls (healthy subjects and community-acquired pneumonia).Results Most patients (36 patients with 39 ACS episodes; 23 males; median age 27 years) had thoracic pain, dyspnoea and computed tomography scan lung consolidation. Acute PH was diagnosed in seven (19%) patients. Erythrocyte- and platelet-derived microparticles and the pro-coagulant activity of microparticles were higher in ACS patients with acute PH, compared with their counterparts. Compared with healthy controls, ACS patients had higher levels of tissue factor, fibrin monomers, D-dimer, release of pro-coagulant microparticles and erythrocyte- and platelet-derived microparticles. Compared with community-acquired pneumonia patients, ACS patients had increased levels of fibrin monomers and erythrocyte- and platelet-derived microparticles.Conclusions Severe ACS is characterised by endothelial dysfunction and hypercoagulability, with a marked pro-coagulant profile in cases of associated PH.Severe sickle-cell acute chest syndrome is associated with an activation of the pulmonary vascular endothelium and of coagulation, with higher levels of pro-coagulant microparticles in cases of associated acute pulmonary hypertension https://bit.ly/3sjkaYy