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Multicentric Castleman disease

GtoPdb Disease Summaries

This section gives an overview of the disease, and where available shows the following:

  • Synonyms: Shows known synonyms for the disease.
  • Description: Gives a basic description/definition of the disease.
  • Database Link: External links to the same disease at the Disease Ontology, OMIM or Orphanet sites.
  • Immunopharmacology comments: General comments about the target's role in immunopharmacology, provided by GtoImmuPdb curators.
  • Associated with: Counts are displayed for the total targets the disease is associated with in GtoPdb. The counts of targets and ligands of immunological relevance associated to the disease are also shown.

More information can be found in the help pages.

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Disease ID:1233
Name:Multicentric Castleman disease
Associated with:0 target
2 immuno-relevant ligands
Synonyms
Multicentric giant lymph node hyperplasia
Description
MCD is an aggressive form of Castleman disease (a benign lymphoproliferative disorder) that mainly results from human herpesvirus 8 (HHV8) infection. MCD affects multiple sets of lymph nodes and other tissues throughout the body. Symptoms include fever, diffuse lymphadenopathy, hepatosplenomegaly, involvement of the respiratory system and increased C-reactive protein.
Database Links
OMIM: 148000
Orphanet: ORPHA93686

Targets

GtoPdb Disease Summaries - Targets

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Where available, information is display on the role of the target in the disease; drugs which target the disease and their therapeutic use and side-effects.

If there is mutation data curated in GtoPdb this is indicated, with a link back to the appropriate section on the target detailed view page

Immuno ligand interactions - If available, a table of immuno-relevant ligands is shown. These ligands have been curated as having an association to the disease and possess interaction data with the target in GtoPdb. The approval status of the ligand is shown, along with curator comments and an indication of whether the target is considered the primary target of the ligand.

More information can be found in the help pages.

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Key to terms and symbols

No target related data available for Multicentric Castleman disease

Ligands

GtoPdb Disease Summaries - Ligands

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  • Approved: If the ligand is an approved drug this is indicated, along with approval bodies.
  • Immuno: Immuno icon indicates the ligand is immuno-relevant

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  • Immuno Disease Comments: Curatorial comments specifically added as part of GtoImmuPdb. They give more information on the association between the ligand and disease in the context of immunopharmacology.
  • Clinical Use: General clinical comments relating to the ligand and may not necessarily be specific to the disease in question. With hyperlink to more details on the ligand summary pages.
  • Bioactivty Comments: Curatorial comments specifically about the compounds biological activity - with hyperlink to more details on the ligand summary pages.

More information can be found in the help pages.

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Key to terms and symbols Click ligand name to view ligand summary Click column headers to sort
Ligand References Clinical and Disease comments
siltuximab
Immuno Disease Comments: Approved therapeutic for MCD.
Clinical Use: Approved to treat multicentric Castleman's disease (MCD, a.k.a. giant lymph node hyperplasia), a rare cytokine storm disorder [3-4].

SARS-CoV-2 and COVID-19: Siltuximab was investigated as a mechanism to combat cytokine storm resulting from an exaggerated response of the immune system in patients with severe COVID-19 [1,6-7]. | View clinical data
adalimumab 5
Immuno Disease Comments: Potential of anti-TNF therapy for iMCD has been demonstrated in a single patient case study
Clinical Use: Used in the management of rheumatoid arthritis, juvenile idiopathic arthritis, psoriatic arthritis, ankylosing spondylitis, Crohn's disease and plaque psoriasis.
In 2015 both the EMA and the FDA approved the use of adalimumab as a treatment for hidradenitis suppurativa, a chronic skin disease that causes abscesses and scarring on the skin.
In July 2016, the FDA expanded adalimumab approval as the first non-corticosteroid drug available for use as a treatment for non-infectious intermediate, posterior and panuveitis (forms of autoimmune-driven inflammation of the uvea)- results from Phase 3 clinical trial NCT01138657 are published in [2]. The EMA marketing authorisation for adalimumab Trudexa® was withdrawn at the request of the marketing authorisation holder.

Adalimumab-induced TNF inhibition has been identified as a potential therapy for the rare life-threatening cytokine storm disorder known as idiopathic multicentric Castleman's disease (iMCD) [5]. The only FDA approved drug for iMCD (in early 2025) is the anti-IL-6 monoclonal , but >50% of patients have siltuximab-refractory disease, so additional drugs targeting alternative pathways are needed for those patients. | View clinical data

References

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1. Ghosn L, Assi R, Evrenoglou T, Buckley BS, Henschke N, Probyn K, Riveros C, Davidson M, Graña C, Bonnet H et al.. (2023) Interleukin-6 blocking agents for treating COVID-19: a living systematic review. Cochrane Database Syst Rev, 6 (6): CD013881. [PMID:37260086]

2. Jaffe GJ, Dick AD, Brézin AP, Nguyen QD, Thorne JE, Kestelyn P, Barisani-Asenbauer T, Franco P, Heiligenhaus A, Scales D et al.. (2016) Adalimumab in Patients with Active Noninfectious Uveitis. N Engl J Med, 375 (10): 932-43. [PMID:27602665]

3. Jitaru C, Symeonidis A, Badelita S, Katodritou E, Colita A, Mpanti A, Bancos A, Tigu B, Rotariu P, Urian L et al.. (2024) Siltuximab in Idiopathic Multicentric Castleman Disease: Real-World Experience. J Hematol, 13 (5): 207-215. [PMID:39493599]

4. Lang E, van Rhee F. (2024) Idiopathic multicentric Castleman disease: An update in diagnosis and treatment advances. Blood Rev, 64: 101161. [PMID:38087716]

5. Mumau MD, Gonzalez MV, Ma C, Irvine AH, Sarmiento Bustamante M, Shyamsundar S, Chen LYC, Koslicki D, Fajgenbaum DC. (2025) Identifying and Targeting TNF Signaling in Idiopathic Multicentric Castleman's Disease. N Engl J Med, 392 (6): 616-618. [PMID:39908436]

6. Panda AK, Padhi S, Pati A, Nayak TKS. (2021) Efficacy of IL-6 antagonists in the treatment of COVID-19 patients: further randomized controlled trials are warranted. Hum Cell, 34 (6): 1943-1944. [PMID:34524645]

7. Villaescusa L, Zaragozá F, Gayo-Abeleira I, Zaragozá C. (2022) A New Approach to the Management of COVID-19. Antagonists of IL-6: Siltuximab. Adv Ther, 39 (3): 1126-1148. [PMID:35072887]