Chronic osteo-articular changes in patients with sickle cell disease

• Main Authors: Taciana Fernandes Araújo Ferreira, Ana Paula Teixeira dos Santos, Alexandra Silva Leal, Gilberto de Araújo Pereira, Sheila Soares Silva, Helio Moraes-Souza
• Format: Article
• Language: English
• Published: BMC 2021-02-01
• Series: Advances in Rheumatology
• Subjects: Osteonecrosis; Osteoarthritis; Osteopenia; Sickle cell disease; Hydroxyurea
• Online Access: https://doi.org/10.1186/s42358-021-00169-5

Abstract Background Sickle cell disease (SCD) is an autosomal recessive genetic disease in which a mutation occurs in the β-globin chain gene, resulting in abnormal hemoglobin levels. In an environment with reduced oxygen concentration, red blood cells change their conformation, resulting in chronic hemolysis and consequent anemia and vaso-occlusive crises with injuries to several organs, with a significant impairment of the osteoarticular system. This study aimed to verify the chronic osteoarticular alterations and their association with clinical and laboratory characteristics of patients with SCD with a more severe phenotype (SS and Sβ0), on a steady-state fasis. Methods Fifty-five patients were referred to a medical consultation with a specialized assessment of the locomotor system, followed by laboratory tests and radiographic examinations. Results In total, 74.5% patients had hemoglobinopathy SS; 67.3% were female; and 78.2% were non-whites. The mean patient age was 30.5 years. Most patients (61.8%) reported up to three crises per year, with a predominance of high-intensity pain (65.5%). Radiographic alterations were present in 80% patients. A total of 140 lesions were identified, most which were located in the spine, femur, and shoulders. Most lesions were osteonecrosis and osteoarthritis and were statistically associated with the non-use of hydroxyurea. Conclusions There was a high prevalence of chronic osteoarticular alterations, which was statistically associated only with the non-regular use of hydroxyurea.