Corticosteroids during Acute Painful Crises of Sickle Cell Diseases

Abstract

Background: Sickle cell diseases (SCD) are severe inflammatory processes on vascular endothelium, particularly at the capillary level since the capillary system is the main distributor of hardened red blood cells (RBC) into the tissues. Methods: All patients with the SCD were included. Results: The study included 222 males and 212 females with similar ages (30.8 vs 30.3 years, p>0.05, respectively). Disseminated teeth losses (5.4% vs 1.4%, p<0.001), ileus (7.2% vs 1.4%, p<0.001), cirrhosis (8.1% vs 1.8%, p<0.001), leg ulcers (19.8% vs 7.0%, p<0.001), digital clubbing (14.8% vs 6.6%, p<0.001), coronary heart disease (18.0% vs 13.2%, p<0.05), chronic renal disease (9.9% vs 6.1%, p<0.05), chronic obstructive pulmonary disease (25.2% vs 7.0%, p<0.001), and stroke (12.1% vs 7.5%, p<0.05) were all higher but not acute chest syndrome (2.7% vs 3.7%), pulmonary hypertension (12.6% vs 11.7), deep venous thrombosis and/or varices and/or telangiectasias (9.0% vs 6.6%), and mean age of mortality (30.2 vs 33.3 years) in males (p>0.05 for all). Conclusion: Although the hardened RBC-induced capillary endothelial damage is present in whole body even at birth, severe exacerbations during additional stresses are called as acute painful crises. An increased basal metabolic rate, exaggerated sickling, diffuse capillary endothelial damage, exaggerated capillary endothelial inflammation and edema, generalized tissue hypoxia, and multiorgan insufficiencies may be the main causes of mortality during the crises. Although rapid RBC supports are the main treatment option, corticosteroids should also be added to decrease severity of endothelial inflammation and edema, and to prevent tissue hypoxia and multiorgan insufficiencies during such crises. Key words: Sickle cell diseases, acute painful crises, capillary inflammation, capillary edema, corticosteroids, metabolic syndrome, atherosclerosis