Development and implementation of the International AIDA Network Castleman’s disease registry

Castleman's disease (CD) consists of a wide spectrum of rare disorders classified into unicentric CD and multicentric CD (MCD), based on the diffusion of disease distribution and the severity of clinical manifestations. While unicentric CD is characterized by a single lymph node involvement, MCD is defined by multiple lymph node station involvement with more prominent systemic symptoms. MCD is further subdivided into HHV-8 associated MCD, polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, skin changes (POEMS)-associated MCD, and idiopathic MCD (iMCD), which is also subdivided into iMCD-TAFRO (thrombocytopenia, anasarca, fever, reticulin fibrosis, organomegaly) and iMCD-NOS (not otherwise specified). The rarity of the disease makes it still poorly understood, as current insight is largely based on case reports and relatively small patient cohorts. Therefore, knowledge about the clinical details of the disease, histological correlations, complications, prognostic factors, and optimal treatment management remains incomplete. The potential offered by the creation of online data sharing makes the development of a registry specifically dedicated to CD a necessary step to conduct solid research on this condition. Building on the experience and widespread international reach of the AutoInflammatory Disease Alliance (AIDA) Network, the development of this registry can allow the recruitment of a sufficient number of patients to conduct robust research in all the fields of the disease. Moreover, the AIDA Network itself will enable multidisciplinary and integrated collaboration among the various figures necessary for the optimal diagnostic, clinical, and therapeutic management of patients affected by CD in its different forms.

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Journal
Frontiers in Medicine
Published
2025-09-03
DOI
https://doi.org/10.3389/fmed.2025.1579182
Primary Topic
Viral-associated cancers and disorders
Type
article
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article

Development and implementation of the International AIDA Network Castleman’s disease registry

Monica Bocchia, Gaafar Ragab, Moustafa Ali Saad, Αναστάσιος Καραμανάκος et al.
Frontiers in Medicine
Viral-associated cancers and disorders
article

Development and implementation of the International AIDA Network Castleman’s disease registry

Monica Bocchia, Gaafar Ragab, Moustafa Ali Saad, Αναστάσιος Καραμανάκος, Florenzo Iannone, Bruno Frediani, Fatma Alıbaz-Öner, Giuseppe Lopalco, Valeria Caggiano, Antonio Vitale, Lampros Fotis, Hamit Küçük, Lorenzo Dagna, Abdurrahman Tufan, Haner Di̇reskeneli̇, Piero Ruscitti, Samar Tharwat, Alberto Balistreri, Mahmoud Ghanema, Emanuele Cencini, José Hernández‐Rodríguez, Ewa Więsik–Szewczyk, Luca Cantarini, Mariusz Sikora, Giacomo Emmi, Andrés González García, Mark Beecher, Pravin Hissaria, Ilenia Di Cola, Ezgi Deniz Batu, Claudia Fabiani, Pier Luigi Zinzani, Rahime Duran, Seza Özen, Stefano Lazzi, Jessica Sbalchiero
article en

Abstract

Castleman's disease (CD) consists of a wide spectrum of rare disorders classified into unicentric CD and multicentric CD (MCD), based on the diffusion of disease distribution and the severity of clinical manifestations. While unicentric CD is characterized by a single lymph node involvement, MCD is defined by multiple lymph node station involvement with more prominent systemic symptoms. MCD is further subdivided into HHV-8 associated MCD, polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, skin changes (POEMS)-associated MCD, and idiopathic MCD (iMCD), which is also subdivided into iMCD-TAFRO (thrombocytopenia, anasarca, fever, reticulin fibrosis, organomegaly) and iMCD-NOS (not otherwise specified). The rarity of the disease makes it still poorly understood, as current insight is largely based on case reports and relatively small patient cohorts. Therefore, knowledge about the clinical details of the disease, histological correlations, complications, prognostic factors, and optimal treatment management remains incomplete. The potential offered by the creation of online data sharing makes the development of a registry specifically dedicated to CD a necessary step to conduct solid research on this condition. Building on the experience and widespread international reach of the AutoInflammatory Disease Alliance (AIDA) Network, the development of this registry can allow the recruitment of a sufficient number of patients to conduct robust research in all the fields of the disease. Moreover, the AIDA Network itself will enable multidisciplinary and integrated collaboration among the various figures necessary for the optimal diagnostic, clinical, and therapeutic management of patients affected by CD in its different forms.

Frontiers in MedicineVol. 12
University of Siena (IT), Damietta University (EG), Royal Adelaide Hospital (AU), Cairo University (EG), Vita-Salute San Raffaele University (IT), Mansoura University (EG), Universidad de Alcalá (ES), National and Kapodistrian University of Athens (GR), University of L'Aquila (IT), Monash Medical Centre (AU), Ospedale di Cattinara (IT), Azienda USL di Bologna (IT), IRCCS Ospedale San Raffaele (IT), Azienda Ospedaliera Universitaria Senese (IT), Central Clinical Hospital (PL), Consorci Institut D'Investigacions Biomediques August Pi I Sunyer (ES), Hospital Universitario Ramón y Cajal (ES), Evangelismos Hospital (GR), University of Bari Aldo Moro (IT), Hacettepe University (TR), Marmara University (TR), University of Bologna (IT), Gazi University (TR)
Openalex Percentile: Top 67%
Viral-associated cancers and disorders
DOIOpen Access PDFOpenAlex
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