Chronic osteo-articular changes in patients with sickle cell disease

Advances in Rheumatology

article

Ferreira, Taciana Fernandes Araújo¹  dos Santos, Ana Paula Teixeira¹  Leal, Alexandra Silva²  de Araújo Pereira, Gilberto²  Silva, Sheila Soares²  Moraes-Souza, Helio² 

[1] Clinical Hospital of Federal University Triângulo Mineiro, Functional Units Rom;Federal University of Triângulo Mineiro

关键词: Osteonecrosis;    Osteoarthritis;    Osteopenia;    Sickle cell disease;    Hydroxyurea;   

DOI  :  10.1186/s42358-021-00169-5

学科分类：地球科学(综合)

来源: Springer

【 摘 要 】

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. Fifty-five patients were referred to a medical consultation with a specialized assessment of the locomotor system, followed by laboratory tests and radiographic examinations. 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. There was a high prevalence of chronic osteoarticular alterations, which was statistically associated only with the non-regular use of hydroxyurea.

【 授权许可】

CC BY