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

Abstract

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 overall [OR=0.98 (95% CI 0.38 to 2.53)], but with a significant interaction of study type (P

Sputum interleukin‐6 level as a marker of severity during acute chest syndrome in children with sickle cell disease

“…Indeed, although ACS pathophysiology remains incompletely understood, increasing evidence has highlighted a key role of inflammation. Steroids have been found in the past to reduce ACS severity, especially duration of oxygen therapy and length of hospitalisation, but they are usually not considered a good option since they increase the risk of severe secondary VOC and readmission by inducing enhanced leucocyte and neutrophil count 13–15 . In recent years, we have shown that IL‐6 levels are dramatically high in sputum and tracheal aspirations of SCD patients during ACS, 5 possibly reflecting pulmonary recruitment of innate immune cells such as monocytes and macrophages whose activation by haemoglobin S leads to increased production of inflammatory cytokines, particularly IL‐6 16 .…”

“…have been found in the past to reduce ACS severity, especially duration of oxygen therapy and length of hospitalisation, but they are usually not considered a good option since they increase the risk of severe secondary VOC and readmission by inducing enhanced leucocyte and neutrophil count. [13][14][15] In recent years, we have shown that IL-6 levels are dramatically high in sputum and tracheal aspirations of SCD patients during ACS, 5 possibly reflecting pulmonary recruitment of innate immune cells such as monocytes and macrophages whose activation by haemoglobin S leads to increased production of inflammatory cytokines, particularly IL-6. 16 Furthermore, sputum IL-6 level could rise at an early stage of ACS development, even before the clinical and radiological diagnostic criteria for ACS are met, and may serve as a predictive marker of ACS occurrence during VOC, independently of CRP level.…”

Impact of prenatal corticosteroid therapy on sickle cell disease in pregnant women

“…The risks and benefits of using corticosteroids in individuals with SCD has recently gained attention. In a meta‐analysis, Lopinto et al 21 were unable to conclude on the benefit of corticosteroids to treat patients hospitalized for VOC or ACS, where the length of hospital stay was reduced when considering three randomized trials, but the risk of readmission increased. The systematic review by Ferreira de Matos et al 22 concluded that corticosteroid administration to SCD patients results in increased risks of sickling complications.…”

Stroke without cerebral arteriopathy in sickle cell disease children: causes and treatment

“…Given the underlying vascular dysfunction, impaired microcirculation may be unable to compensate for a rapid increase in Hb level by increasing vascular resistance and promoting RBC aggregation 25 . In addition, major hyperleukocytosis promoted by inflammation or corticosteroid therapy may increase blood viscosity 26,27 . Involvement of leukocytes, particularly neutrophils in vascular and organ damage in SCD patients is well described beyond 20 x10 9 /L, including in stroke 28 .…”