A clinical risk score for pulmonary artery thrombosis during acute chest syndrome in adult patients with sickle cell disease

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Summary Pulmonary artery thrombosis (PAT) is involved in lung vascular dysfunction during acute chest syndrome (ACS) complicating sickle cell disease (SCD). No clinical score is available to identify patients eligible for multi‐detector computed tomography (MDCT) angiography duringACS. This retrospective study aimed to develop a risk score forPATduringACS(PAT‐ACSrisk score). Patients withSCDwere investigated byMDCTduringACS. A logistic regression was performed to determine independent risks factors forPATand to build thePAT‐ACSrisk score. A total of 43 episodes (11·9%) ofPATwere diagnosed in 361 episodes ofACS. Multivariate analysis identified four risk factors, which were included in thePAT‐ACSrisk score: a baseline haemoglobin >82 g/l, the lack of a triggering factor forACS, a platelet count >440 × 109/l and a PaCO2<38 mmHg atACSdiagnosis. The area under the receiver operating characteristic curve for thePAT‐ACSrisk score was 0·74 (95% confidence interval [CI] 0·69–0·79) and differed from that of the revised Geneva score (0·63 (95%CI0·58–0·69);P = 0·04). The negative predictive value of aPAT‐ACSrisk score ≥2 was 94%. In conclusion, we propose a simple clinical risk score to identifySCDpatients at high risk ofPATduringACS.

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