Effects of corticosteroids in patients with sickle cell disease and acute complications: a systematic review and meta-analysis

Whether corticosteroids improve outcome in patients with acute complications of sickle cell disease (SCD) is still debated. We performed a systematic review of the literature with the aim of estimating effects of corticosteroids on the clinical course of vaso-occlusive crisis (VOC) or acute chest sy...

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Published inHaematologica (Roma) Vol. 107; no. 8; pp. 1914 - 1921
Main Authors Lopinto, Julien, Gendreau, Segolene, Berti, Enora, Bartolucci, Pablo, Habibi, Anoosha, Mekontso Dessap, Armand
Format Journal Article
LanguageEnglish
Published Italy Ferrata Storti Foundation 01.08.2022
Fondazione Ferrata Storti
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Summary:Whether corticosteroids improve outcome in patients with acute complications of sickle cell disease (SCD) is still debated. We performed a systematic review of the literature with the aim of estimating effects of corticosteroids on the clinical course of vaso-occlusive crisis (VOC) or acute chest syndrome (ACS) in patients with SCD. The primary outcome was transfusion requirement during hospitalization. Studies were identified by search of MEDLINE and CENTRAL database. Three randomized clinical trials (RCT) and three retrospective cohort studies (RCS) were included, involving 3,304 participants and 5,562 VOC or ACS episodes. There was no difference between corticosteroids and standard treatment regarding transfusion requirement overall (odds ratio [OR]=0.98, 95% confidence interval [CI]: 0.38-2.53) but there was a significant interaction of the study type (P<0.0001): corticosteroid therapy was associated with a lower risk of transfusion in RCT (OR=0.13, 95% CI: 0.04-0.45) and a higher risk of transfusion in RCS (OR=2.12, 95% CI: 1.33-3.40. In RCT, the length of hospital stay was lower with corticosteroids as compared with standard treatment: mean difference - 24 hours (95% CI: -35 to -14). Corticosteroids were associated with an increased risk of hospital readmission as compared with standard treatment, in RCT, RCS, and the entire cohort: OR=5.91, 95% CI: 1.40-24.83; OR=3.28, 95% CI: 1.46-7.36 and OR=3.21, 95% CI: 1.97-5.24, respectively. Corticosteroids were associated with reduced number of transfusions and length of stay in RCT but not in RCS, with more rehospitalizations overall. Additional RCT should be conducted while minimizing the risk of rehospitalizations.
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The datasets and materials used and analyzed during the current study are available from the corresponding author on reasonable request.
JL, SG, EB and AMD collected the data; JL, SG and AMD analyzed and interpreted the data; JL, SG and AMD drafted the manuscript; JL and AMD contributed to the study conception and design; AMD, AH and PB critically revised the manuscript. All authors read and approved the final manuscript.
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No conflicts of interest to disclose.
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ISSN:0390-6078
1592-8721
1592-8721
DOI:10.3324/haematol.2021.280105