Development and Implementation of the International AIDA Network Castleman's Disease Registry

Antonio Vitale¹Jessica Sbalchiero¹Valeria Caggiano¹Stefano Lazzi¹Samar Tharwat²Lampros Fotis³Giuseppe Lopalco⁴Giacomo Emmi⁵Piero Ruscitti⁶Abdurrahman Tufan⁷Gaafar Ragab⁸Pravin Hissaria⁹Lorenzo Dagna¹⁰Ewa Barbara Wiesik-Szewczyk¹¹José Hernández-Rodríguez^1,12Andres González-Garcia¹³Anastasios Karamanakos¹⁴Ezgi Deniz Batu¹⁵Haner Direskeneli¹⁶Florenzo Iannone⁴Ilenia Di Cola⁶Hamit Kucuk⁷Rahime Duran⁷Moustafa Ali Saad¹⁷Mahmoud Ghanema¹⁷Mariusz Sikora¹⁸FATMA ALIBAZ-ONER¹⁶Mark Beecher¹⁹Seza Ozen¹⁵Emanuele Cencini¹Monica Bocchia¹Alberto Balistreri¹Claudia Fabiani¹Bruno Frediani¹Luca Cantarini^1*Pier Luigi Zinzani²⁰

• ¹University of Siena, Siena, Italy
• ²Mansoura University, Mansoura, Dakahlia, Egypt
• ³National and Kapodistrian University of Athens, Athens, Greece
• ⁴University of Bari Aldo Moro, Bari, Italy
• ⁵University of Trieste, Trieste, Friuli-Venezia Giulia, Italy
• ⁶University of L'Aquila, L'Aquila, Abruzzo, Italy
• ⁷Gazi University, Ankara, Ankara, Türkiye
• ⁸Cairo University, Giza, Giza, Egypt
• ⁹Royal Adelaide Hospital, Adelaide, South Australia, Australia
• ¹⁰San Raffaele Hospital (IRCCS), Milan, Lombardy, Italy
• ¹¹University of Warsaw, Warsaw, Masovian, Poland
• ¹²Hospital Clinic of Barcelona, Barcelona, Catalonia, Spain
• ¹³Ramón y Cajal Institute for Health Research, Madrid, Madrid Community, Spain
• ¹⁴Department of Cardiology, Evangelismos General Hospital, Athens, Greece
• ¹⁵Hacettepe University, Ankara, Ankara, Türkiye
• ¹⁶Marmara University, Kadikoy, Istanbul, Türkiye
• ¹⁷New Giza University, Giza, Giza, Egypt
• ¹⁸Military University of Technology in Warsaw, Warsaw, Masovian, Poland
• ¹⁹University of Adelaide, Adelaide, South Australia, Australia
• ²⁰University of Bologna, Bologna, Emilia-Romagna, Italy

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.