Year 2020 / Volume 27 / Number 4

Special Article

Clinical pathophysiology in patients with sickle cell disease: the transition from acute to chronic pain

Rev. Soc. Esp. Dolor. 2020; 27(4): 257-268 / DOI: 10.20986/resed.2020.3814/2020

Borja Mugabure, Silvia González, Amaia Uría, Antía Osorio


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.


Los pacientes con enfermedad de células falciformes (ECF), también denominada drepanocítica, sufren un dolor intenso que suele comenzar durante la infancia y aumenta su gravedad a lo largo de la vida, lo que lleva a la hospitalización y a una mala calidad de vida a lo largo de los años. Una característica única de la ECF son las crisis vaso-oclusivas (CVO), caracterizadas por episodios recurrentes e impredecibles de dolor agudo. La obstrucción microvascular durante una CVO provoca una disminución del suministro de oxígeno a la periferia y una lesión por isquemia y posterior reperfusión, inflamación, estrés oxidativo y disfunción endotelial, todo lo cual puede perpetuar un microambiente nocivo que provoca dolor. Por otro lado, además de los dolores agudos episódicos, los pacientes con ECF también padecen dolor crónico, definido como dolor casi diario durante un periodo de 6 meses, asociado a trastornos psicosociales. Pueden deberse a lesiones crónicas como úlceras cutáneas, necrosis avascular ósea o infartos en diversos órganos. Asimismo, la sensibilización central parece estar directamente involucrada en la cronicidad del dolor y existe un componente de dolor neuropático claramente infradiagnosticado e infratratado. El tratamiento actual del dolor moderado a intenso en la ECF se basa principalmente en la administración de los opioides, vía oral, de liberación rápida ambulatoria o en forma de analgesia controlada por el paciente vía intravenosa intrahospitalaria. Sin embargo, el uso de opioides a largo plazo está asociado con múltiples efectos secundarios. Esta revisión presenta los últimos avances en la comprensión de la fisiopatología del dolor en la ECF y se describen los mecanismos subyacentes que pueden ayudar a desarrollar nuevas estrategias terapéuticas y/o preventivas para mejorar el dolor en la ECF.

Complete Article


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Mugabure B, González S, Uría A, Osorio A. Clinical pathophysiology in patients with sickle cell disease: the transition from acute to chronic pain. Rev Soc Esp Dolor 2020; 27(4): 257-268 / DOI: 1020986/resed20203814/2020

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Recibido: 17/04/2020

Aceptado: 07/07/2020

Prepublicado: 07/07/2020

Publicado: 04/08/2020

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