From the Guidelines
Systemic steroid use is associated with an increased risk of vasocclusive crisis in patients with sickle cell disease, and therefore, should be used with caution. The judicious use of corticosteroids is advised due to the potential relationship between steroid exposure and vaso-occlusive pain for patients with SCD, as noted in the 2019 American Society of Hematology guidelines for sickle cell disease 1. Corticosteroids such as prednisone, methylprednisolone, and dexamethasone should be used at the lowest effective dose for the shortest possible duration, with close monitoring for signs of crisis. Some key considerations for minimizing the risk of vasocclusive crisis in patients with sickle cell disease who require systemic steroids include:
- Using the lowest effective dose and shortest duration of steroid therapy
- Ensuring patients are well-hydrated to reduce blood viscosity
- Implementing prophylactic pain management strategies
- Tapering steroids slowly to avoid rebound inflammation and crisis events, as suggested by the potential relationship between steroid exposure and vaso-occlusive pain 1. The mechanism behind this increased risk involves steroid-induced changes in blood viscosity, vascular tone, and inflammatory responses, which can promote sickling of red blood cells and vessel occlusion.
From the Research
Systemic Steroid Use and Vasocclusive Crisis Risk
- The relationship between systemic steroid use and the risk of vasocclusive crisis in patients with sickle cell disease is complex and has been studied in various contexts, particularly in the management of acute chest syndrome (ACS) [ 2, 3, 4, 5, 6 ].
- Studies have shown that systemic corticosteroids are used in the treatment of ACS due to their anti-inflammatory properties, but their use is also associated with potential risks, including rebound pain crisis, stroke, renal infarction, coma, and even death [ 4 ].
- Some research suggests that the use of systemic steroids in ACS may be associated with more severe disease, as indicated by the need for blood transfusions, intensive care unit admission, respiratory support, and chest X-ray findings with more than one lobe involvement [ 5 ].
- However, the evidence on whether systemic steroid use increases the risk of vasocclusive crisis is not conclusive, with some studies finding no significant difference in readmission rates or length of hospital stay between patients treated with corticosteroids and those who were not [ 3, 5 ].
- Further research is needed to fully understand the effects of systemic steroid use on the risk of vasocclusive crisis in patients with sickle cell disease, including the role of different types of corticosteroids, dosing regimens, and underlying co-morbidities [ 4, 6 ].