This series of bite-sized episodes will take you on the winding journey that patients with idiopathic multicentric Castleman disease (iMCD) often undergo to achieve an accurate diagnosis. Once the condition is finally identified, even the most astute specialists can find themselves challenged by the treatment, management, and monitoring of these patients. Join Drs. Corey Casper and Sudipto Mukherjee as they explain the ups and downs of this rare disease.
The Feverish Case of the Fatigued and Fibrotic
The Feverish Case of the Fatigued and Fibrotic
Welcome to CME on ReachMD. This episode is part of our MinuteCME curriculum.
Prior to beginning the activity, please be sure to review the faculty and commercial support disclosure statements as well as the learning objectives.
This is CME on ReachMD, and I'm Dr. Corey Casper. Here with me today is Dr. Sudipto Mukherjee.
Let's discuss the relapsing and remitting symptoms that our patients have with Castleman’s disease. So, Dr. Mukherjee, what can you tell us about the systemic symptoms? How can we prepare our patients for what's in store for them over the course of their disease?
Thank you, Dr. Casper, for this very interesting question. And I think it all begins with a diagnosis of iMCD [idiopathic multicentric Castleman disease]. And before launching into any treatment, that is one of the key times when the provider and the patient needs to sit down and have a very broad and a frank discussion about what are we getting into. iMCD is a chronic disease; none of the therapies are curative. And when the treatment is started, the current recommendation is to continue the treatment indefinitely or lifelong until disease progression or unacceptable toxicities. So we may be talking about in some patients for a few years, in some patients up to a decade or more of treatment. That's the most data we have on siltuximab-responding patients as of now.
So in these patients, we have to counsel them that because this is a chronic disease, even on treatment there can be instances where there might be acute flare-ups, acute exacerbations that may require the patient to be hospitalized or may end up in the ICU on life support. Why it happens, we do not know. There are many hypotheses, and one of them that has been shown has been differential levels of interleukin-6 in different body compartments. For example, in patients who have a pulmonary failure, interleukin-6 levels in the pleural fluid would be sometimes as high as 6- or 8-fold than the levels in the circulating plasma. There are other factors in play, which we do not really know about. And we even don't know who are the patients who will relapse and remit, and who are the ones who will not. But typically, in these patients who relapse, in most of the cases, there is an underlying driver, and it still remains that the common biological underpinning is overproduction of interleukin-6.
How we best manage these patients is through best supportive care. Sometimes it may require the use of high-dose steroids to calm down the cytokine storm, and in several instances mostly reported either in personal clinical experience, including my experience, and in some case reports, is more frequent use of interleukin-6 [inhibitor] from once every 3 weeks to even once weekly. These have not been rigorously tested or validated, but there are some reports that these have been used to manage these significant adverse outcomes in the clinical journey of these patients when they relapse.
And clearly, for a patient to be on a lifelong journey with these therapies, especially knowing that these therapies, when they work, they do have improved the survival of these patients, it is critically important to get some buy-in from the patient and the caregivers who take care of these patients. And letting them know about the anticipated difficulties or complications through their clinical journey, and setting up realistic goals is critically important in shared decision-making, because that will allow adherence to the treatment and better compliance, and which hopefully will turn into better clinical outcomes.
Thank you, Dr. Mukherjee, for sharing your extensive clinical experience with us.
The most important thing in this chronic disease that has its ups and downs is that close partnership between the patient and the provider for that therapeutic relationship that allows that close partnership to select and to adhere to the most effective therapies. I think patients need to know that there are multiple treatment options, that there are lots of different ways of dealing with the ups and downs of Castleman’s disease, but that it's a journey, and it’s one that, together, the patient and the provider and maybe even the greater community of Castleman’s providers and patients can support and can be again a long and healthy life.
So with that, I want to end our brief but great discussion. Unfortunately, our time is up. Thank you all for tuning in.
You have been listening to CME on ReachMD. This activity is provided by Prova Education and is part of our MinuteCME curriculum.
To receive your free CME credit, or to download this activity, go to ReachMD.com/Prova. Thank you for listening.
In accordance with the ACCME Standards for Integrity and Independence, Global Learning Collaborative (GLC) requires that individuals in a position to control the content of an educational activity disclose all relevant financial relationships with any ineligible company. GLC mitigates all conflicts of interest to ensure independence, objectivity, balance, and scientific rigor in all its educational programs.
Corey Casper, MD, MPH
Clinical Professor of Medicine and Global Health
University of Washington
Affiliate Professor, Fred Hutch Cancer Center
Research: Amyris, ImmunityBio (IBRX), Janssen
Ownership Interest: Viracta Therapeutics
Receives Royalties: UpToDate
Patent Holder: AAHI
Consulting Fees: EUSA Pharma
Sudipto Mukherjee, MD, PhD, MPH
Director, Rare Cancers & Blood Diseases
Cleveland Clinic Taussig Cancer Institute
Research: BMS/Celgene, Jazz Pharma, Novartis
Receives Royalties: McGraw Hill
Consulting Fees: BioPharm, Blueprint Medicines, BMS/Celgene, Genentech and AbbVie, EUSA, Novartis
- Cindy Davidson has nothing to disclose.
- Stephen Chavez has nothing to disclose.
- Libby Lurwick has nothing to disclose.
- Colleen Resnick has nothing to disclose.
- Mara Siegel has nothing to disclose.
After participating in this educational activity, participants should be better able to:
- Discuss the clinical burden and impact associated with a delayed diagnosis of Castleman disease
- Improve the speed and accuracy of a diagnosis of Castleman disease through increased recognition of its clinical presentation and knowledge of published diagnostic criteria
- Incorporate guideline-concordant care into the treatment of patients with Castleman disease
- Employ shared decision-making strategies to increase patient participation and promote advocacy, clinical trial enrollment, and research
This activity is designed to meet the educational needs of US hematologist oncologists, rheumatologists, infectious disease specialists, immunologists, and other HCPs who are involved in diagnosing, managing, and treating iMCD.
In support of improving patient care, Global Learning Collaborative (GLC) is jointly accredited by the Accreditation Council for Continuing Medical Education (ACCME), the Accreditation Council for Pharmacy Education (ACPE), and the American Nurses Credentialing Center (ANCC) to provide continuing education for the healthcare team.
Global Learning Collaborative (GLC) designates this enduring activity for a maximum of 1.0 AMA PRA Category 1 Credits™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.
Global Learning Collaborative (GLC) designates this activity for 1.0 nursing contact hour. Nurses should claim only the credit commensurate with the extent of their participation in the activity.
Prova Education designs and executes continuing education founded on evidence-based medicine, clinical need, gap analysis, learner feedback, and more. Our mission is to serve as an inventive and relevant resource for clinical content and educational interventions across a broad spectrum of specialties.
Prova Education's methodology demonstrates a commitment to continuing medical education and the innovative assessment of its effects. Our goal is clear—to develop and deliver the very best education in the most impactful manner and to verify its results with progressive outcomes research.
This activity is supported by an independent educational grant from Recordati.
The views and opinions expressed in this educational activity are those of the faculty and do not necessarily represent the views of GLC and Prova Education. This presentation is not intended to define an exclusive course of patient management; the participant should use his/her clinical judgment, knowledge, experience, and diagnostic skills in applying or adopting for professional use any of the information provided herein. Any procedures, medications, or other courses of diagnosis or treatment discussed or suggested in this activity should not be used by clinicians without evaluation of their patients’ conditions and possible contraindications or dangers in use, review of any applicable manufacturer’s product information, and comparison with recommendations of other authorities. Links to other sites may be provided as additional sources of information. Once you elect to link to a site outside of Prova Education you are subject to the terms and conditions of use, including copyright and licensing restriction, of that site.
Reproduction of this material is not permitted without written permission from the copyright owner.
Our site requires a computer, tablet, or mobile device and a connection to the Internet. For best results, a high-speed Internet connection is recommended (DSL/Cable/Fibre). We also recommend using the latest version of your favorite browser to ensure compliance with W3C standards, such as Chrome, Safari, Firefox, or Microsoft Edge.