8. An autoinflammatory syndrome resembling Castleman’s disease with excellent response to IL-6 blockade

Abstract

Introduction Autoinflammatory diseases are an emerging group, characterised by recurrent inflammatory episodes due to dysregulated innate immunity. Common features include fevers, rash, arthralgia, lymphadenopathy and systemic symptoms. Castleman’s disease is a rare lymphoproliferative disease, associated with the overproduction of interleukin-6 (IL-6). Its two main variants: unicentric and multicentric differ in aetiology and clinical outcomes. The cytokine storm driven by IL-6 can mimic autoinflammatory disease. We present the case of an acquired autoinflammatory syndrome in a 33-year-old male with the clinical phenotype of Castleman’s but no culprit lymph node detected radiologically. Symptoms dramatically improved with yocilizumab, an IL-6 blocker.