Chronic osteo-articular changes in patients with sickle cell disease

• Published in: Advances in rheumatology (London, England) Vol. 61; no. 1; p. 11
• Main Authors: Ferreira, Taciana Fernandes Araújo, Dos Santos, Ana Paula Teixeira, Leal, Alexandra Silva, de Araújo Pereira, Gilberto, Silva, Sheila Soares, Moraes-Souza, Helio
• Format: Journal Article
• Language: English
• Published: England BioMed Central 19.02.2021; Sociedade Brasileira de Reumatologia; BMC
• Subjects: Age; Anemia; Arthritis; Autosomal recessive inheritance; Blood levels; Bone marrow; Conformation; Consent; Erythrocytes; Femur; Fractures; Hemoglobin; Hemoglobinopathy; Hydroxyurea; Laboratories; Medical records; Mutation; Osteoarthritis; Osteonecrosis; Osteopenia; Pain; Patients; Phenotypes; RHEUMATOLOGY; Sickle cell disease; Statistical analysis; Brazil; Hydroxyurea; Osteonecrosis; Sickle cell disease; Osteoarthritis; Osteopenia
• ISSN: 2523-3106; 2523-3106
• DOI: 10.1186/s42358-021-00169-5

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 Sβ ), 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.