Introduction
Acute chest syndrome (ACS) can develop rapidly after a severe vaso-occlusive crisis (VOC), and its progression can be sudden. Early identification of high-risk patients is therefore a key priority in order to:
- improve medical management,
- prevent severe respiratory complications,
- reduce pressure on emergency departments through better patient triage.
Against this background, the PRESEV 1 and PRESEV 2 studies were developed to build and then validate a predictive score for acute chest syndrome in people with sickle cell disease presenting with a severe VOC.
Both studies are being conducted by SAMG (Soutien et Actions contre les Maladies du Globule Rouge). The predictive score was developed in PRESEV 1 and validated through PRESEV 2.
Ultimately, this work will enable:
- earlier detection of patients at risk,
- better referral of patients,
- optimisation of hospital resources,
- reduced morbidity and mortality associated with acute chest syndrome.
PRESEV 1
PRESEV 1 is a completed, prospective, single-centre study conducted at Henri Mondor University Hospital in Créteil. It included adults with sickle cell disease who were admitted through the emergency department with a severe vaso-occlusive crisis requiring hospitalisation.
Its primary aim was to identify, at an early stage, patients at risk of developing acute chest syndrome (ACS), a serious and potentially life-threatening complication of sickle cell disease.
ACS was defined by the onset of:
- abnormal findings on chest examination and/or chest pain;
- together with a new lung infiltrate identified on chest imaging.
A total of 226 patients were included in the study. Of these, 16.4% developed secondary ACS, on average on the third day of their hospital stay.
The PRESEV 1 findings identified several predictive factors for ACS and were used to build a predictive score that can be applied as soon as the patient arrives at the emergency department.
The score is designed to support patient triage:
- low-risk patients may be suitable for enhanced monitoring at home;
- high-risk patients require early and close monitoring in hospital.
PRESEV 2
PRESEV 2 is an ongoing international multicentre study conducted in Europe and sub-Saharan Africa. Unlike PRESEV 1, this new phase includes both adults and children with sickle cell disease.
Its primary aim is to validate the predictive score developed in PRESEV 1 at scale, using varied and independent populations.
This multicentre validation will:
- confirm the score’s robustness and reproducibility;
- assess its performance across geographical settings and age groups;
- determine whether adaptations are needed for local clinical practice.
Expanding the study across several continents is particularly important because the largest burden of sickle cell disease is in African populations.
In the longer term, PRESEV 2 could:
- identify patients at risk of ACS earlier;
- improve the referral and triage of people with sickle cell disease;
- make better use of hospital resources;
- improve the overall care of severe vaso-occlusive crises.
Conclusion
PRESEV 1 used early clinical information from adults admitted with a severe VOC to create a predictive score for acute chest syndrome.
PRESEV 2 is testing this score in several countries and centres, with adults and children from different populations.
In the longer term, this work could:
- identify patients at risk earlier;
- improve patient referral and triage;
- optimise hospital resources;
- reduce morbidity and mortality associated with acute chest syndrome.
