Table 1.

Progress made for iMCD research, 2012-2018

State of iMCD research in 2012State of iMCD research in 2018
Research coordination  
 No physician, researcher, or patient communities 400+ physicians and researchers connected through the CDCN and 2000+ patients connected virtually and through annual meetings 
 No registry or biorepository ACCELERATE Natural History Registry and Biorepository are enrolling patients around the world via e-consent 
Defining iMCD  
 Different subclassification systems being used Uniform classification system published in Blood 
 No diagnostic criteria for iMCD International, evidence-based diagnostic criteria published in 201713  
Characterizing pathogenesis of iMCD and advancing treatment options  
 Prevailing model of pathogenesis: an IL-6 secreting, lymph node “tumor” disorder New model guiding research: complex cytokine storm disorder with an unknown etiology; several hypothesized etiologies under intense investigation 
 No genomic alterations identified in iMCD First somatic mutation in iMCD published in 201820 ; other candidates are currently undergoing validation 
 No FDA-approved treatments for iMCD Siltuximab became the first and only FDA-approved treatment of iMCD in 2014 
 No treatment guidelines International, evidence-based treatment guidelines in press39  
 No drugs in development directed at any targets other than IL-6 First clinical trial of relapsed/refractory iMCD with a drug directed at a target other than IL-6/IL-6R expected to begin enrollment in 2019 
State of iMCD research in 2012State of iMCD research in 2018
Research coordination  
 No physician, researcher, or patient communities 400+ physicians and researchers connected through the CDCN and 2000+ patients connected virtually and through annual meetings 
 No registry or biorepository ACCELERATE Natural History Registry and Biorepository are enrolling patients around the world via e-consent 
Defining iMCD  
 Different subclassification systems being used Uniform classification system published in Blood 
 No diagnostic criteria for iMCD International, evidence-based diagnostic criteria published in 201713  
Characterizing pathogenesis of iMCD and advancing treatment options  
 Prevailing model of pathogenesis: an IL-6 secreting, lymph node “tumor” disorder New model guiding research: complex cytokine storm disorder with an unknown etiology; several hypothesized etiologies under intense investigation 
 No genomic alterations identified in iMCD First somatic mutation in iMCD published in 201820 ; other candidates are currently undergoing validation 
 No FDA-approved treatments for iMCD Siltuximab became the first and only FDA-approved treatment of iMCD in 2014 
 No treatment guidelines International, evidence-based treatment guidelines in press39  
 No drugs in development directed at any targets other than IL-6 First clinical trial of relapsed/refractory iMCD with a drug directed at a target other than IL-6/IL-6R expected to begin enrollment in 2019 
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