Osteoarticular involvement in sickle cell disease

Abstract

The osteoarticular involvement in sickle cell disease has been poorly studied and it is mainly characterized by osteonecrosis, osteomyelitis and arthritis. The most frequent complications and those that require hospital care in sickle cell disease patients are painful vaso-occlusive crises and osteomyelitis. The deoxygenation and polymerization of hemoglobin S, which results in sickling and vascular occlusion, occur more often in tissues with low blood flow, such as in the bones. Bone microcirculation is a common place for erythrocyte sickling, which leads to thrombosis, infarct and necrosis. The pathogenesis of microvascular occlusion, the key event in painful crises, is complex and involves activation of leukocytes, platelets and endothelial cells, as well as hemoglobin S-containing red blood cells. Osteonecrosis is a frequent complication in sickle cell disease, with a painful and debilitating pattern. It is generally insidious and progressive, affecting mainly the hips (femur head) and shoulders (humeral head). Dactylitis, also known as hand-foot syndrome, is an acute vaso-occlusive complication characterized by pain and edema in both hands and feet, frequently with increased local temperature and erythema. Osteomyelitis is the most common form of joint infection in sickle cell disease. The occurrence of connective tissue diseases, including rheumatoid arthritis and systemic lupus erythematosus, has rarely been reported in patients with sickle cell disease. The treatment of these complications is mainly symptomatic, and more detailed studies are required to understand the pathophysiological mechanisms involved in the complications and propose more adequate and specific therapies.

Keywords: Anemia, sickle cell/complications; Arthritis; Bone diseases/etiology; Hemoglobin SC Disease; Osteomyelitis; Osteonecrosis.

Figure 1

X-ray of vertebral column showing biconcave vertebral body depressions in a patient with sickle cell disease.Reproduced from Huo et al.⁽¹¹⁾ with the permission of the Yale Journal of Biology and Medicine

Figure 2

X-ray of a 16-year-old patient with sickle cell disease showing necrosis of the femoral head. Reproduced from Mukisi-Mukaza et al.⁽¹²⁾ with permission of Elsevier B.V.

Figure 3

X-ray showing bilateral osteonecrosis of the femur in patient with sickle cell disease. Reproduced from Huo et al.⁽¹¹⁾ with the permission of the Yale Journal of Biology and Medicine

Figure 4

X-ray showing multiple infarcts in the femur and tibia. Reproduced from Huo et al.⁽¹¹⁾ with the permission of the Yale Journal of Biology and Medicine

Figure 5

X-ray showing osteonecrosis of the humeral head and bone infarction in patient with sickle cell disease. Reproduced from Huo et al.⁽¹¹⁾ with the permission of the Journal of Biology and Medicine

Figure 6

X-ray of upper limb showing radial multifocal osteomyelitis in a patient with sickle cell disease. Reproduced from Almeida & Roberts⁽⁴⁾ with permission of John Wiley and Sons

Figure 7

X-ray of the hands of a patient with sickle cell disease and rheumatoid arthritis showing periarticular osteopenia, erosion of pyramidal and pisiform bones, left prestyloid synovitis, decreased joint space in the third left proximal interphalangeal joint and suspected synovitis in the third and fourth proximal right interphalangeal joints. Reproduced from Arana et al.(26) with permission of the Asociación Colombiana de Reumatología e Asociación Centroamericana, Caribe & Andina de Reumatología

Figure 8

Pathophysiology of osteoarticular involvement in sickle cell disease