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You’ve Been Diagnosed with Castleman Disease — But What If It’s IgG4-RD?

Castleman disease is a group of rare disorders that keep your immune system active when it shouldn’t. Typically, your immune system turns on to fight off infections like colds or the flu, then calms down when the threat is gone. But with CD, the immune system stays stuck in high gear, causing too much inflammation, which can harm your organs and make you very sick.

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  Accelerate Registry

ACCELERATE is the first-ever registry for Castleman disease patients. It’s a research study designed to help physicians and researchers better understand Castleman disease, uncover patterns in the disease, and improve treatment.

  Castleman Disease Collaborative Network

The Castleman Disease Collaborative Network (CDCN) is dedicated to accelerating research and treatment for Castleman disease, supporting patients on their journeys, and revolutionizing biomedical research for countless other diseases.

Articles of Interest

Behind the Mystery: Castleman Disease

Castleman disease is sometimes referred to as a mix between an autoimmune disease, an infectious disease, and a cancer with symptoms that mimic many other disorders. Because Castleman disease is rare, healthcare professionals often correctly and rightfully suspect other diseases first.

Castleman Disease Support Group

This is a group for patients and loved ones who are battling Castleman disease. This is a safe, private, loving community dedicated to supporting anyone with questions, advice, or just wanting someone to talk to. We urge people to also visit the Castleman Disease Collaborative

Castleman Disease: Pearls and Myths

We have all experienced misinformation about our disease. The CDCN wants to bring accurate information to the community and one of the ways we are doing this is through a book and new publication by Dr. David Fajgenbaum and Dr. Luke Chen: A Clinician’s Pearls and Myths.

His Doctors Were Stumped. Then He Took Over.

Castleman disease had been known since the 1950s but has remained largely a mystery. A hallmark of the condition is enlarged lymph nodes, and most people with the disease — about two-thirds — have a form that affects just one part of the body and can usually be cured through surgery. The form ravaging Dr. Fajgenbaum’s body — multicentric Castleman disease — is even more rare and deadly. Only about 1,200 to 1,500 people are discovered to have it every year in the United States. It defied classification, occupying a no man’s land between cancer and immune disease. Doctors weren’t sure of the cause in patients like him: Some believed it was a type of cancer, while others thought it was an inherited genetic disorder, or was triggered by a virus.

Idiopathic Multicentric Castleman Disease Occurring Shortly after mRNA SARS-CoV-2 Vaccine

Though rare systemic inflammatory and immune-mediated adverse events have been reported following SARS-CoV-2 immunizations, we report here the first case of biopsy-confirmed iMCD that appears to be associated with SARS-CoV-2 immunizations.

Living with Castleman Disease

Castleman disease results in a variety of symptoms that can be hard on patients. These symptoms can have an impact on their social and professional life. Intense fatigue, nausea, night sweats, and chronic pain can make daily life difficult for patients. Some treatments can relieve their nausea and chronic pain, but they must learn to live with latent fatigue. It is important for those around you to understand that there is a difference between fatigue in a healthy person and a person with CD.

No evidence for active viral infection in unicentric and idiopathic multicentric Castleman disease by Viral-Track analysis

Castleman disease (CD) is a rare hematologic disorder characterized by pathologic lymph node changes and a range of symptoms due to excessive cytokine production. While uncontrolled infection with human herpesvirus-8 (HHV-8) is responsible for the cytokine storm in a portion of multicentric CD (HHV-8-associated MCD) cases, the etiology of unicentric CD (UCD) and HHV-8-negative/idiopathic MCD (iMCD) is unknown.

Overview of Castleman disease

Castleman disease (CD) describes a group of at least 4 disorders that share a spectrum of characteristic histopathological features but have a wide range of etiologies, presentations, treatments, and outcomes. CD was first described in the 1950s by Benjamin Castleman as localized mediastinal lymph node enlargement characterized by increased numbers of lymphoid follicles with germinal center involution and marked capillary proliferation, including follicular and interfollicular endothelial hyperplasia.

The clinical picture of Castleman disease: a systematic review and meta-analysis

Castleman disease (CD) describes a spectrum of rare disorders with characteristic histopathology and enlarged lymph nodes. Unicentric CD (UCD) mainly occurs as a benign, localized lymphoid hyperplasia, often presenting as a solitary, indolent lymph node swelling. In contrast, multicentric CD (MCD) is potentially life threatening, with multifocal lymphadenopathy, systemic inflammation, and constitutional symptoms. Etiology of idiopathic MCD (iMCD) is unknown; various potential causes for pathological overproduction and dysregulation of proinflammatory cytokines (eg, interleukin-6) have been discussed.

This Doctor Found His Own Miracle Drug. Now He Wants to Do It for Others

New database aims to match rare-disease patients with drugs on pharmacy shelves.

Unraveling Castleman Disease

Castleman disease is a rare immune system disorder. There are about 5,000 to 6,000 patients diagnosed each year in the United States, which makes it about as rare as ALS or Lou Gehrig's disease certainly has more awareness of it. But Castleman disease has similarly prevalence, about 5,000 diagnosed each year in the US or similar incidents. Castleman disease really describes a group of disorders that all look the same under the microscope. So if you look at a lymph node from anyone with any form of Castleman disease, they look really, really similar under the microscope. But the disease is very heterogeneous, meaning that depending on what subtype of Castleman disease, you have a very different clinical course, different prognosis, different treatments. And so on one end of the spectrum is unicentric Castleman disease where patients present with a single lymph node or a single region of enlarged lymph nodes. And those patients typically have a more mild symptomatic course.

“I have a very rare illness": The unique way patients and scientists are fighting Castleman disease

Meet two men who have the rare immune system disorder: a doctor who created a groundbreaking registry for it—and a patient who signed up for it.

Resources

StatPearls

Castleman disease is a lymphoproliferative disorder with a nondefinite etiology and can be classified into subtypes based on clinical and histological manifestations. Therefore, this condition's high morbidity and complications can be avoided with early diagnosis and prompt treatment.