Introducing the international AIDA Network Castleman disease registry

Idiopathic multicentric Castleman disease (iMCD) is a rare and life-threatening lymphoproliferative disorder characterized by systemic inflammation, lymph node enlargement, and multi-organ dysfunction. The most common clinical manifestations include fever, night sweats, fatigue, weight loss, and anemia, alongside potential complications like organ failure. Additionally, other clinical manifestations, such as ocular inflammatory manifestations, may variably occur in some patients and are usually secondary to systemic inflammation or the lymphoproliferative involvement characteristic of the disease.  

Prognosis varies, with some patients responding well to treatment while others experience severe, refractory disease. Management typically involves immunomodulatory therapies, such as anti-IL-6 agents, corticosteroids, and supportive care tailored to individual needs. The Registry will collect data finalized to better describe the clinical history of patients with iMCD in terms of presentation, complications, long-term outcome and therapeutic management.