The Castleman Disease Collaborative Network (CDCN), a global nonprofit organization dedicated to accelerating research and treatments for Castleman disease (CD), a rare immunological disease, announced a collaboration with Janssen Research & Development to conduct a proteomics study of idiopathic multicentric Castleman disease (iMCD), a deadly subtype of CD. This collaborative study has the potential to improve diagnosis and treatment for iMCD patients. The collaboration combines CDCN’s strong expertise in iMCD research and its world-wide network of physician researchers with Janssen’s industry leading iMCD research resources.

The causes of iMCD, one of three subtypes of CD, are unknown. About 1,0001new cases of iMCD are diagnosed each year in the United States and approximately 35% of patients die within five years of diagnosis2. iMCD patients experience a wide spectrum of symptoms, from mild flu-like symptoms to acute sepsis-like multiple organ system failure due to uncontrolled immune system activation. Siltuximab, an anti-interleukin-6 monoclonal antibody developed by Janssen, was approved by the FDA for treatment of iMCD in 2014. However, many questions still exist related to iMCD, particularly for patients that do not respond to siltuximab.

“Ever since I was diagnosed with iMCD ten years ago, I have been dedicated to doing whatever I can to improve CD research and awareness. We have made progress, but have important work ahead of us to understand the causes of CD, and develop diagnostic tests and treatments for it,” stated, Greg Pacheco, board president of CDCN.

Recently, CDCN brought together a team of physicians and researchers to establish the first-ever diagnostic criteria for iMCD, which was published last month in the journal, Blood. These criteria include clinical and pathology features, and diseases to exclude. However, the diagnostic process can be time-consuming as there is no definitive test for iMCD. Therefore, the aim of this study is to identify molecules whose levels are specifically altered in the blood of iMCD patients. The hope is that these molecules can be used to shorten the time required to diagnose iMCD for critically ill patients and as therapeutic targets for treatment of iMCD patients that do not respond to current therapies. To accomplish these goals, the collaborators will measure the level of approximately 1,300 analytes in more than 260 serum samples collected from approximately 100 iMCD patients at various time points during active disease.

This unprecedented collaboration reflects a trend towards greater collaboration between industry, research foundations, and academia, as well as continued interest in drug development for rare diseases. Janssen will fund a significant portion of the project and contribute iMCD serum samples that were collected as part of a previous clinical trial. CDCN’s role in the collaboration includes, through its network of physician scientists, partnering with seven academic institutes from around the globe to contribute iMCD, related disease and ‘normal’ serum samples and analyzing the results of the proteomic screen. CDCN will also fund a portion of the project.

“It is unusual and creative for a pharmaceutical company to open up their banks of samples for research and to do it in such a collaborative fashion. Janssen’s commitment to fully leveraging its iMCD resources to advance high-impact, patient-centric research demonstrates its commitment to helping patients,” stated Pacheco. “The results of this ambitious research project have the potential to shed significant light on a poorly understood disease and improve survival for all CD patients.”