[Research Advances in Pathogenesis of Idiopathic Multicentric Castleman Disease]

Idiopathic multicentric Castleman disease (iMCD) is a rare and life-threatening lymphoproliferative disorder involving systemic inflammatory symptoms,polyclonal lymphoproliferation,cytopenia,and multiple organ dysfunction.Although interleukin-6-mediated cytokine storm is thought to be the key driver of iMCD,more than 50% of the patients fail to respond to the treatments targeting interleukin-6 or its receptors.This underscores the urgent need to identify other cytokines and pathogenic pathways that may underlie iMCD.Fortunately,recent years have witnessed notable research advances in the pathogenesis of iMCD.This article reviews the latest progress in this field,aiming to provide a theoretical foundation for further research and facilitate the optimization of treatment strategies to improve the prognosis of the patients.