Australian clinical practice guideline: diagnosis and treatment of idiopathic multicentric Castleman disease

Talaulikar, D; Ng, JY; Bryant, A; Bosco, A; Caterson, HC; Giri, P; Hissaria, P; Harrison, SJ; Lade, S; Leslie, C; Perram, J; Prince, HM; Radeski, D; Rischmueller, M; Wallington-Gates, C; Weber, N; Hoi, A

Author(s): Talaulikar, D; Ng, JY; Bryant, A; Bosco, A; Caterson, HC; Giri, P; Hissaria, P; Harrison, SJ; Lade, S; Leslie, C; Perram, J; Prince, HM; Radeski, D; Rischmueller, M; Wallington-Gates, C; Weber, N; Hoi, A;
Details: Publication Year 2026-07,Volume 56,Issue #7,Page 1218-1239
Journal Title: Internal Medicine Journal
Publication Type: Guideline
Abstract: 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.
Publisher: Wiley
Keywords: Humans; *Castleman Disease/diagnosis/drug therapy/therapy/epidemiology; Rituximab/therapeutic use; Australia/epidemiology; Antibodies, Monoclonal/therapeutic use; Idiopathic multicentric Castleman disease; clinical practice guideline
Department(s): Haematology; Pathology
Publisher's Version: https://doi.org/10.1111/imj.70460
Open Access at Publisher's Site: https://doi.org/10.1111/imj.70460
Terms of Use/Rights Notice: Refer to copyright notice on published article.

Creation Date: 2026-06-04 04:32:20

Last Modified: 2026-08-13 12:12:34