Castleman disease is a rare inflammatory disorder that presents with enlarged lymph nodes with a certain histologic appearance. Like other patients who have rare diseases, patients with Castleman routinely suffer from the ignominies of a delayed diagnosis, questions of whether or not they can get the diagnosis at all, and whether they can get a prompt and correct diagnosis. What’s more, Castleman disease patients only have one FDA-approved medication for their treatment if they have the idiopathic multicentric variety of Castleman disease that can present with a systemic inflammatory syndrome that can potentially cause multi-organ system failure, ICU admissions, and potentially death...
Castleman disease is a rare inflammatory disorder that presents with enlarged lymph nodes with a certain histologic appearance. Like other patients who have rare diseases, patients with Castleman routinely suffer from the ignominies of a delayed diagnosis, questions of whether or not they can get the diagnosis at all, and whether they can get a prompt and correct diagnosis. What’s more, Castleman disease patients only have one FDA-approved medication for their treatment if they have the idiopathic multicentric variety of Castleman disease that can present with a systemic inflammatory syndrome that can potentially cause multi-organ system failure, ICU admissions, and potentially death. And that one FDA-approved treatment is siltuximab, an IL-6 blocker. But about half to maybe more than half of patients will not respond to that one FDA-approved therapy. So the second major challenge that Castleman disease patients face is what to do for patients whose disease does not respond to siltuximab or IL-6 antagonism.
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