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<p>Castleman disease (CD) encompasses a heterogeneous group of rare lymphatic disorders characterised by distinctive histopathological changes in lymph nodes. Clinically, patients present with lymphadenopathy, fever, and systemic inflammation, making CD a relevant differential diagnosis in rheumatology. Dysregulated interleukin (IL)-6 production is central to its pathogenesis, driving excessive B‑cell and plasma cell activation. The unicentric form (UCD) is distinguished from a multicentric form (MCD), the latter comprising three subtypes: HHV-8-associated MCD, POEMS-associated MCD, and idiopathic MCD (iMCD). The iMCD subtype represents a diagnosis of exclusion requiring histological confirmation by lymph node excision. Diagnosis is challenging due to substantial clinical and serological overlap with autoimmune, autoinflammatory, and lymphoproliferative disorders; false-positive autoantibodies arising from polyclonal B‑cell activation further complicate classification. Therapeutically, UCD is generally curable by surgical resection. For iMCD, IL‑6 blockade with siltuximab or tocilizumab is standard of care, with glucocorticoids reserved for short-term bridging. HHV-8-associated MCD is treated with rituximab, while POEMS-associated MCD requires treatment directed at the underlying plasma cell disorder. This case report illustrates the diagnostic challenges of this rare disease and underscores the value of a structured, multimodal workup.</p>

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Idiopathischer multizentrischer Morbus Castleman als differenzialdiagnostische Herausforderung

  • 期刊论文

  • 发表日期：

    2026年7月6日

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  • Peter Pavol,
  • Ester Szemanova,
  • Andrea Rubbert-Roth,
  • Stefan Wolfrum

摘要

Castleman disease (CD) encompasses a heterogeneous group of rare lymphatic disorders characterised by distinctive histopathological changes in lymph nodes. Clinically, patients present with lymphadenopathy, fever, and systemic inflammation, making CD a relevant differential diagnosis in rheumatology. Dysregulated interleukin (IL)-6 production is central to its pathogenesis, driving excessive B‑cell and plasma cell activation. The unicentric form (UCD) is distinguished from a multicentric form (MCD), the latter comprising three subtypes: HHV-8-associated MCD, POEMS-associated MCD, and idiopathic MCD (iMCD). The iMCD subtype represents a diagnosis of exclusion requiring histological confirmation by lymph node excision. Diagnosis is challenging due to substantial clinical and serological overlap with autoimmune, autoinflammatory, and lymphoproliferative disorders; false-positive autoantibodies arising from polyclonal B‑cell activation further complicate classification. Therapeutically, UCD is generally curable by surgical resection. For iMCD, IL‑6 blockade with siltuximab or tocilizumab is standard of care, with glucocorticoids reserved for short-term bridging. HHV-8-associated MCD is treated with rituximab, while POEMS-associated MCD requires treatment directed at the underlying plasma cell disorder. This case report illustrates the diagnostic challenges of this rare disease and underscores the value of a structured, multimodal workup.