Molecular Evidence

  • NF-κB is a protein complex that acts as the primary transcription factor for several of the cytokines commonly elevated in iMCD. It has been proposed as one of the important dysregulated pathways in iMCD pathogenesis.
  • Mutations in the gene responsible for turning off NF-κB transcription result in autoinflammatory disorders that demonstrate episodic bouts of intense inflammation.
Clinical Evidence

  • Thalidomide is an NF-κB inhibitor that has been shown to be effective in some iMCD patients.