Hematology
OTHER
Castleman disease can resemble lupus, requiring careful diagnosis for proper treatment
Frontiers in Medicine
Published March 31, 2026
A medical perspective article discusses how two different diseases, idiopathic multicentric Castleman disease (iMCD) and systemic lupus erythematosus (SLE), can look very similar in patients. They share many symptoms and lab findings, such as fevers, swollen lymph nodes, low blood cell counts, kidney problems, and high levels of inflammation. Some people with iMCD even have autoantibodies in their blood, which are typically associated with lupus. This overlap can make it hard to tell which condition a person has.
The key to getting the right diagnosis is examining a sample of tissue from a swollen lymph node under a microscope. This procedure, called a lymph node biopsy, is central to identifying iMCD. Without this step, a person might be misdiagnosed with lupus. The article notes that cases of iMCD might initially seem to meet the criteria for lupus but then not respond well to standard lupus treatments.
It is important to distinguish between these diseases because they are treated very differently. iMCD typically requires treatments that target specific inflammatory proteins called cytokines, while lupus is often managed with broader immunosuppressive drugs. Using the wrong treatment strategy could delay a patient's recovery. This article is not a new study, but a perspective meant to raise awareness among doctors about this diagnostic challenge to help improve patient care.
View Original Abstract ↓
Castleman disease (CD) is a rare lymphoproliferative disorder with heterogeneous clinical manifestations, particularly in its idiopathic multicentric form (iMCD), which is driven by cytokine dysregulation rather than classical autoimmunity. Owing to overlapping features such as fever, lymphadenopathy, cytopenias, renal involvement, and elevated inflammatory markers, iMCD may closely mimic systemic lupus erythematosus (SLE). This resemblance is further complicated by the presence of autoantibodies in a subset of iMCD patients, increasing the risk of misclassification and delayed diagnosis. In this Perspective, we highlight key clinical scenarios in which CD presents as a lupus-like syndrome, including cases that initially fulfill SLE classification criteria but fail to respond to conventional immunosuppressive therapy. We emphasize the limitations of serologic testing in distinguishing these entities and underscore the central role of lymph node histopathology in establishing the correct diagnosis. Recognizing Castleman disease as an important mimic of SLE has critical therapeutic implications, as iMCD requires cytokine-directed treatment rather than immune suppression alone. These observations also suggest that CD and SLE may share a cytokine-driven inflammatory spectrum in selected patients; however, distinguishing between them remains essential because their therapeutic strategies differ fundamentally. Increased awareness of this diagnostic overlap may help clinicians avoid inappropriate treatment strategies and improve outcomes in patients presenting with atypical or refractory lupus-like disease.
