Evidence map›Paper›PMID 42775474›Full record

ArticlePediatric blood & cancer2026

Pediatric Idiopathic Multicentric Castleman Disease Is Often Severe But Responsive to Siltuximab.

Bridget Austin, Sheila K Pierson, Kelley C Dacus, Mateo Sarmiento Bustamante, Saishravan Shyamsundar, Larissa Borys, Kayla McHugh, Abiola Irvine, Yoojin Jo, Adam Bagg and 8 more

Abstract read
PubMed Full text at PMC Publisher
In one paragraph

Article in Pediatric blood & cancer, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

0numbers the graph read from it
0cells of the map it votes in
0citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

0 citing papers in PubMed.

No citing paper in PubMed yet.

4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

18 authors.

Bridget AustinCenter for Cytokine Storm Treatment & Laboratory, Department of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA.ORCID https://orcid.org/0009-0003-5549-882X
Sheila K PiersonCenter for Cytokine Storm Treatment & Laboratory, Department of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA.ORCID https://orcid.org/0000-0002-3736-1411
Kelley C DacusRecordati Rare Diseases Inc., Bridgewater, New Jersey, USA.ORCID https://orcid.org/0009-0005-1323-6818
Mateo Sarmiento BustamanteCenter for Cytokine Storm Treatment & Laboratory, Department of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
Saishravan ShyamsundarCenter for Cytokine Storm Treatment & Laboratory, Department of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA.ORCID https://orcid.org/0000-0002-0160-8910
Larissa BorysCenter for Cytokine Storm Treatment & Laboratory, Department of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
Kayla McHughCenter for Cytokine Storm Treatment & Laboratory, Department of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
Abiola IrvineCenter for Cytokine Storm Treatment & Laboratory, Department of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
Yoojin JoCenter for Cytokine Storm Treatment & Laboratory, Department of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
Adam BaggDepartment of Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA.ORCID https://orcid.org/0000-0002-1669-468X
Mary Jo LechowiczDepartment of Hematology and Medical Oncology, Emory University School of Medicine and Winship Cancer Institute, Atlanta, Georgia, USA.
Daisy AlapatDepartment of Pathology, College of Medicine, University of Arkansas for Medical Sciences, Little Rock, Arkansas, USA.ORCID https://orcid.org/0000-0002-3414-8375
Amy ChadburnDivision of Hematopathology, Department of Pathology and Laboratory Medicine, Weill Cornell Medicine, New York, New York, USA.ORCID https://orcid.org/0000-0002-9499-8638
Megan S LimDepartment of Pathology and Laboratory Medicine, Memorial Sloan Kettering Cancer Center, New York City, New York, USA.ORCID https://orcid.org/0000-0002-0415-2867
Gordan SrkalovicUniversity of Michigan Health-Sparrow Herbert-Herman Cancer Center, Lansing, Michigan, USA.ORCID https://orcid.org/0000-0003-3519-0409
Frits van RheeMyeloma Center, Winthrop P. Rockefeller Cancer Institute, University of Arkansas for Medical Sciences, Little Rock, Arkansas, USA.ORCID https://orcid.org/0000-0001-9959-1282
David C FajgenbaumCenter for Cytokine Storm Treatment & Laboratory, Department of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA.ORCID https://orcid.org/0000-0002-7367-8184
Joshua D BrandstadterCenter for Cytokine Storm Treatment & Laboratory, Department of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA.ORCID https://orcid.org/0000-0003-0652-5791

Funding

mTOR as a Central Regulator of iMCD Pathogenesis and Novel Therapeutic TargetR01HL141408 · NHLBI · UNIVERSITY OF PENNSYLVANIA · PI Taku Kambayashi · 2018 to 2026
$6.1M
Lymph Node Stromal Cells in the Pathogenesis, Diagnosis, and Treatment of Unicentric Castleman DiseaseK08HL171943 · NHLBI · UNIVERSITY OF PENNSYLVANIA · PI Joshua D. Brandstadter · 2025 to 2026
$334k
FDA HHS R01 FD007632NHLBI NIH HHS K08 HL171943NHLBI NIH HHS R01 HL141408Recordati Rare Diseases Incthe NIH National Heart, Lung, & Blood Institute K08HL171943the NIH National Heart, Lung, & Blood Institute R01HL141408the US Food and Drug Administration R01FD007632
6 · The paper itself

Abstract

backgroundIdiopathic multicentric Castleman disease (iMCD) is a potentially fatal immunologic disorder marked by widespread lymphadenopathy and inflammation. Siltuximab, an interleukin-6 (IL-6) inhibitor, is the only FDA-approved treatment for adult patients with iMCD. Limited data exist on pediatric iMCD or its responsiveness to siltuximab. We hypothesize that pediatric iMCD patients will present and respond similarly to adult patients. PROCEDURE: We queried ACCELERATE, the largest global iMCD registry with expert-confirmed diagnoses. We analyzed two iMCD cohorts: pediatric patients (18 years of age or younger at symptom onset) and adults. We assessed both disease severity, using a modified CHA (C-reactive protein [CRP], hemoglobin, albumin) score, and response to treatment. A response was defined as normalization in greater than 50% of abnormal clinical parameters and was considered durable if no new regimens were started within 1 year of initiating the index regimen.

resultsPediatric iMCD patients (N = 30) had higher CHA scores at diagnosis (1.67 vs. 1.31, p = 0.024) than adult iMCD cases (N = 96), indicating greater disease severity and higher CRP. Pediatric and adult iMCD patients had similar responses to siltuximab ± CS (12/14, 86% vs. 35/48, 73%, p = 0.48) as well as other treatments.

conclusionsIn the largest pediatric iMCD analysis to date, we found more severe disease and similar responses to siltuximab in children compared to adults.

Indexed as

iMCDpediatricsiltuximab

Identifiers

PMID42775474
PMCPMC13688268

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.