multicentric Castleman disease

Summary

multicentric Castleman disease (MONDO:0019754) is a disease and 17 clinical trials. Top therapeutic interventions include siltuximab, tocilizumab, and valganciclovir. A subtype of human herpesvirus 8-related tumor — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Prevalence: Unknown (Worldwide)
• Clinical trials: 17

Clinical features

No curated clinical features (Orphanet) for this disease.

Identifiers

Disease identifiers

Field	Value
Canonical name	multicentric Castleman disease
Mondo ID	MONDO:0019754
Orphanet	570438, 93686
DOID	DOID:0111152
ICD-11	1590444463
NCIT	C27855
UMLS	C1334815
MedGen	233197
GARD	0022310
Is cancer (heuristic)	no

Also known as: HHV-8-associated multicentric Castleman disease · Human herpesvirus-8-associated multicentric Castleman disease · idiopathic multicentric Castleman’s disease · MCD · multicentric Angiofollicular lymphoid hyperplasia · multicentric Castleman’s disease · multicentric plasma cell variant of Castleman’s disease

Disease family

This is a subtype of human herpesvirus 8-related tumor. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.

Classification path: disease › human disease › disease by etiologic mechanism › cancer or benign tumor › neoplastic disease or syndrome › neoplasm › virus associated tumor › human herpesvirus 8-related tumor › multicentric Castleman disease

Related subtypes (2): Kaposi’s sarcoma, primary effusion lymphoma

Genetics & variants

GWAS landscape

No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.

Variant details and genetic-evidence tiers

No tiered GWAS variants or ClinVar records for this disease.

Genes & proteins

No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).

Function

No pathway enrichment — requires an associated-gene cohort.

Therapeutics

No druggable-target or therapeutic data for this disease’s cohort.

Clinical trials & evidence

Clinical trials

Clinical trials: 17.

Phase distribution (across all retrieved trials)

Phase	Trials
PHASE2	10
Not specified	5
PHASE1	2

Top trials by phase / activity

NCT	Phase	Status	Title
NCT04585893	PHASE2	ACTIVE_NOT_RECRUITING	Safety and Efficacy of Rituximab for Treatment of Multicentric Castleman Disease in Malawi
NCT06983028	PHASE2	RECRUITING	Atacicept in Multiple Glomerular Diseases
NCT01024036	PHASE2	COMPLETED	A Study to Evaluate the Efficacy and Safety of CNTO328 Plus Best Supportive Care in Multicentric Castleman’s Disease
NCT01400503	PHASE2	COMPLETED	A Study to Evaluate the Safety of Long-term Treatment With Siltuximab in Patients With Multicentric Castleman’s Disease
NCT01441063	PHASE2	COMPLETED	Tocilizumab for KSHV-Associated Multicentric Castleman Disease
NCT03043105	PHASE2	UNKNOWN	TCP Regimen in Newly Diagnosed MCD:a Prospective, Single-center, Single-arm, Phase-II Pilot Trial
NCT03982771	PHASE2	UNKNOWN	BCD Regimen in Newly Diagnosed Idiopathic Multicentric Castleman’s Disease (iMCD)
NCT04009668	PHASE2	COMPLETED	Tumor Necrosis Factor Inhibition in Focal Segmental Glomerulosclerosis and Treatment Resistant Minimal Change Disease
NCT04743687	PHASE2	UNKNOWN	Zanuburutinib in Relapsed and Refractory iMCD: a Prospective, Single-center, Single-arm Trial
NCT04838860	PHASE2	TERMINATED	Siltuximab In Siltuximab-RElapsed/REfractory Multicentric CAstleman Disease
NCT07267026	PHASE1	RECRUITING	A Study to Evaluate the Safety, Tolerability and PK of SK-09
NCT02109224	PHASE1	TERMINATED	Ibrutinib in Treating Relapsed or Refractory B-Cell Non-Hodgkin Lymphoma in Patients With HIV Infection
NCT00006518	Not specified	RECRUITING	Specimen Collections From Participants With HIV Infection, KSHV Infection, Viral-Related Pre-malignant Lesions and Cancer
NCT04571658	Not specified	RECRUITING	NEPTUNE Match Study
NCT05650619	Not specified	RECRUITING	Recurrence Post-transplant Observational Study in Focal Segmental Glomerulosclerosis and Minimal Change Disease
NCT05663502	Not specified	RECRUITING	Collecting Blood and Tissue Sample Donations for Research for HIV/AIDS-Related Cancers
NCT05995834	Not specified	UNKNOWN	Producing a Novel Symptom Burden Scale for People Living With Idiopathic Multicentric Castleman Disease (ISBUS)

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
SILTUXIMAB	4	3
TOCILIZUMAB	4	1
VALGANCICLOVIR	4	1
ZANUBRUTINIB	4	1
ATACICEPT	3	1
CHEMBL5193128	0	1