Autoinflammatory & rare systemic diseases
IgG4-related Castleman disease: clinical-pathological features and therapeutic implications
Authors' conclusion
IgG4-CD is characterised by constitutional symptoms, elevated ESR and IgG4 levels, and high-grade lymph node plasmacytosis. Good response to anti-CD20 therapy and a favourable prognosis mark a difference with iMCD.
Study context
- Population
- Patients with igG4-related disease (IgG4-RD) and idiopathic multicentric Castleman disease (iMCD) referred to San Raffaele Hospital (Milan, Italy) and Ramón y Cajal Hospital (Madrid, Spain); IgG4-CD defined by fulfillment of international diagnostic criteria for CD and IgG4-RDpatients with IgG4-CD included, median age 63 years (range 49–71) at IgG4-CD diagnosis
- Participants
- 8 patients
- Exposure
- IgG4-CD compared with IgG4-RD and iMCD patients
- IgG4-CDn = Eight
- Outcome
- Constitutional symptoms
Main finding
As reported in the abstract
- All IgG4-CD patients presented with constitutional symptoms
Observational data: associations, not effects.
Further findings
| Outcome | P value |
|---|---|
| C-reactive proteinIgG4-CD vs iMCD · lower in IgG4-CD | P < 0.0001 |
| Erythrocyte sedimentation rate (ESR)IgG4-CD vs IgG4-RD · higher in IgG4-CD | P = 0.0334 |
Lymph node biopsy findings
- All lymph node biopsies showed high-grade plasmocytosis and features of CD
IgG4-CD extranodal involvement
- Salivary and lacrimal glands, pancreas, and bile ducts
Response to treatment · longest available follow-up
- All patients responded to glucocorticoids or second-line therapy with rituximab with a favourable outcome
Original publication
IgG4-related Castleman disease: clinical-pathological features and therapeutic implications. Clin Exp Rheumatol. Published 23 February 2026. doi:10.
Composed by AI from the published abstract and checked against it. How Briefs are made →