Musculoskeletal complications in sickle cell disease: Pathophysiology, diagnosis and management.

Best Pract Res Clin Rheumatol

ICMR-National Institute of Research in Tribal Health, Jabalpur, India; Academy of Scientific and Innovative Research (AcSIR), Ghaziabad, India; Model Rural Health Research Unit, Jheet, Durg, India. Electronic address:

Published: January 2025

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Article Abstract

Sickle cell disease (SCD) is a mono-genic disorder causing chronic hemolysis, anemia, and vaso-occlusion, leading to musculoskeletal complications such as osteonecrosis, osteoporosis, and bone fractures affecting 50-70% SCD patients. These complications result from a complex interplay of genetic and physiological factors, including abnormal hemoglobin production, chronic inflammation, and oxidative stress. This review discusses the pathophysiology, pre-clinical symptoms, and clinical manifestations of musculoskeletal complications in SCD, as well as current treatment options, including pharmacological interventions, surgical procedures, and bone marrow transplantation. Early detection of pre-clinical symptoms is crucial to prevent progression. Pharmacological interventions (analgesics, anti-inflammatory agents, bone-modifying agents and hydroxyurea), surgical interventions (core decompression, bone grafting, joint replacement and osteotomy) and supportive measures enhance mobility, strength and well-being. A multidisciplinary approach is essential for optimal care, and early diagnosis and management are crucial to prevent long-term damage and improve outcomes. Future research directions include targeted therapies, biomarker investigation and infrastructure development to improve outcomes for SCD individuals with musculoskeletal complications.

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http://dx.doi.org/10.1016/j.berh.2025.102033DOI Listing

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