Causes

All cases of UCD and idiopathic MCD have an unknown cause. There are no known risk factors; there is no evidence of any food, lifestyle, or environmental exposure associated with these diseases. There is no known account of a patient contracting Castleman disease directly from another individual.

Signs and Symptoms

Castleman disease is a group of rare disorders characterized by lymph node enlargement, specific microscopic changes to the lymph nodes, and a range of symptoms and laboratory findings. Castleman disease has a broad spectrum of disease severity, ranging from mild compressive symptoms and fatigue to life-threatening multi-organ failure. Symptoms of Castleman disease often overlap with symptoms of other more common illnesses which makes diagnosis difficult. A Castleman disease diagnosis can only be made with a lymph node biopsy that shows characteristic Castleman disease features and when all other illnesses have been evaluated and excluded. More…

Diagnosis

Castleman disease (CD) can be difficult to diagnose as it is both a rare disease and an imitator of many other diseases. Patients can have the same symptoms as some cancers and some autoimmune diseases. Due to the complexity of the disease, physicians often need to rule out many other diseases before CD is suspected. More…

Prognosis

UCD: The average length of survival after UCD diagnosis is greater than 10 years, and life expectancy is usually not changed by UCD.

MCD: The 5-year overall survival rate of HIV negative (presumed HHV8-negative) MCD was 65% in a 2012 case series published prior to the approval of anti-IL-6 therapy (1). A study in 2021 found a 75% 5-year overall survival rate among iMCD patients (2). More…