Australian clinical practice guideline: diagnosis and treatment of idiopathic multicentric Castleman disease
Idiopathic multicentric Castleman disease (iMCD) is a rare condition. The pathogenesis is incompletely understood; however, interleukin-6 (IL-6) is a major mediator. The clinical presentation is heterogeneous, from mild constitutional symptoms to severe multi-organ failure. The diagnosis is challenging, as it incorporates clinicopathologic criteria and requires careful evaluation to exclude various systemic disorders. Targeting IL-6 activity forms the cornerstone of modern therapy for iMCD, with siltuximab recommended as first-line therapy. Rituximab-based regimens are recommended for second-line therapy. However, many patients do not achieve adequate responses with limited evidence to guide further therapy. In the context of these substantial challenges, herein we provide a multidisciplinary Australasian clinical practice guideline to characterise clinical and pathological features, summarise treatment pathways and discuss clinical outcomes of the condition. The objective is to develop a multidisciplinary clinical practice guideline in the diagnosis and management of iMCD in Australia.
Authors
- Jun Yen Ng (ORCID: https://orcid.org/0000-0002-7995-4696)
- Dipti Talaulikar (ORCID: https://orcid.org/0000-0001-6766-8345)
- Hugh C. Caterson (ORCID: https://orcid.org/0000-0003-2250-6654)
- Jacinta Perram (ORCID: https://orcid.org/0000-0003-0054-7906)
- Dejan Radeski (ORCID: https://orcid.org/0009-0004-5660-7685)
- Maureen Rischmueller (ORCID: https://orcid.org/0000-0001-5057-3286)
- Connull Leslie
- Adam Bryant
- Nicholas Weber
- Pravin Hissaria
- H Miles Prince
- Stephen Lade
- Pratyush Giri
- Annmarie Bosco
- Craig Wallington‐Gates
- Alberta Hoi (ORCID: https://orcid.org/0000-0002-9416-7383)
- Simon J. Harrison (ORCID: https://orcid.org/0000-0003-4555-6582)
Institutions
- Australian National University (AU)
- Royal Adelaide Hospital (AU)
- The University of Melbourne (AU)
- Royal Brisbane and Women's Hospital (AU)
- Epworth Hospital (AU)
- UCL Australia (AU)
- Peter MacCallum Cancer Centre (AU)
- Liverpool Hospital (AU)
- Sir Charles Gairdner Hospital (AU)
- Canberra Hospital (AU)
- UNSW Sydney (AU)
- Queen Elizabeth Hospital (AU)
- St Vincent’s Private Hospital Sydney (AU)
- Sunshine Coast University Hospital (AU)
- Prince of Wales Hospital (AU)
- Monash Health (AU)
- Pathwest Laboratory Medicine (AU)
- ACT Government (AU)
- The University of Adelaide (AU)
- Neuroscience Research Australia (AU)
Publication Details
- Journal
- Internal Medicine Journal
- Published
- 2026-05-30
- DOI
- https://doi.org/10.1111/imj.70460
- Primary Topic
- Viral-associated cancers and disorders
- Type
- article
- Field-Weighted Citation Impact
- 0.00