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Revista de la Sociedad Española del Dolor

Print version ISSN 1134-8046

Abstract

MUGABURE BUJEDO, B; GONZALEZ SANTOS, S; URIA AZPIAZU, A  and  OSORIO LOPEZ, A. Clinical pathophysiology in patients with sickle cell disease: the transition from acute to chronic pain. Rev. Soc. Esp. Dolor [online]. 2020, vol.27, n.4, pp.257-268.  Epub Oct 13, 2020. ISSN 1134-8046.  https://dx.doi.org/10.20986/resed.2020.3814/2020.

Patients with sickle cell disease (SCD) suffer from severe pain that often begins in childhood and increases in severity over the course of a lifetime, leading to hospitalization and poor quality of life over the years. A unique feature of SCD is vase-occlusive crises (VOC) characterized by recurrent and unpredictable episodes of acute pain. Microvascular occlusion during a VOC results in decreased oxygen supply to the periphery and injury from ischemia and subsequent reperfusion, inflammation, oxidative stress and endothelial dysfunction, all of which can perpetuate a harmful pain-causing microenvironment. On the other hand, in addition to episodic acute pain, SCD patients also report chronic pain, defined as almost daily pain over a 6-month period associated to either sicologic or social morbidities. They may be due to chronic lesions such as skin ulcers, avascular bone necrosis or infarctions in various organs. In addition, central sensitization appears to be directly involved in the chronicity of pain and there is a clearly under-diagnosed and under-treated component of neuropathic pain. Current treatment of moderate to severe pain in SCD is based primarily on opioids; either as an oral quick release outpatient or in the form of patient-controlled intravenous analgesia in the hospital. However, long-term opioid use is associated with multiple side effects. This review presents the latest advances in the understanding of the pathology of pain in SCD and describes objectives based on mechanisms that may help to develop new therapeutic and/or preventive strategies to improve pain in SCD.

Keywords : Analgesia; sickle cell disease; acute pain; chronic pain syndrome; opioids.

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