Characteristics of idiopathic multicentric Castleman disease in Spain: results of the registry-based ARCANA multicenter study
Year: 2026
Type: article
Study design: Observational Study
Abstract: Objectives: We aimed to estimate the prevalence of iMCD in Spain by applying the current international consensus criteria. Methods: This was an observational, multicenter, cross-sectional study that was conducted in 20 Spanish hospitals. The diagnosis of iMCD and the histological subtype were confirmed by a centralized scientific committee by applying the international consensus criteria. The local clinical, laboratory, and pathology reports were reviewed. Results: Between February 2023 and April 2024, 30 patients with a documented diagnosis of iMCD were included in the study. The estimated prevalence of iMCD was 0·61 cases per 100,000 inhabitants (95% CI, 0·39-0·83); 23 subjects (76·7%) were classified as iMCD-NOS, and 7 (23·3%) were classified as iMCD-TAFRO. Twenty-two patients (73·3%) had received therapy for iMCD prior to their inclusion in the study. There are currently 21 patients (70%) on treatment, the most common being the use of siltuximab (19 patients; 90·4%). Complete response, partial response, and stable disease were reported in 11 (36·7%), 8 (26·7%), and 9 (30%) patients, respectively, with current treatment. Conclusion: The prevalence of clinical cases of iMCD in Spain comparable to that reported in the literature. Siltuximab is currently the most commonly used therapy. Most patients under treatment showed a favorable clinical response.
Source: Frontiers in Oncology
Authors: Jose-Tomas Navarro, Santiago Montes-Moreno, Grisell Starita Fajardo, Manuel Espeso de Haro, Pedro Durán-del-Campo, Eva Gonzalez-Barca, Antonio Gutiérrez, Ana Jiménez-Ubieto, Alejandro Martín García-Sancho, Araceli Rubio-Martínez, Rosario Sánchez-Martínez, Paloma García-Martín, Pablo Lorente-Alegre, Iñigo Olazabal Eizaguirre, Angel Ramirez Payer, Guillermo Rodriguez, Antonio García-Guiñon, Andrés González-García
Institutions: Universitat Autònoma de Barcelona, Josep Carreras Leukaemia Research Institute, Institut Català d'Oncologia, Universidad de Cantabria, Instituto de Investigación Marqués de Valdecilla, Hospital Universitario Ramón y Cajal, Hospital Regional Universitario de Málaga, Hospital Universitario Puerta de Hierro Majadahonda, Institut d'Investigació Biomédica de Bellvitge, Universitat de Barcelona, Hospital Universitario Son Espases, Research Institute Hospital 12 de Octubre, Hospital Universitario 12 De Octubre, Universidad de Salamanca, Instituto de Investigación Biomédica de Salamanca, Centro de Investigación del Cáncer, Centro de Investigación Biomédica en Red de Cáncer, Instituto de Biología Molecular y Celular del Cáncer de Salamanca, Hospital Universitario Miguel Servet, Hospital General Universitario de Alicante Doctor Balmis, Instituto de Investigación Sanitaria y Biomédica de Alicante (ISABIAL), Hospital Arnau de Vilanova, Donostiako Unibertsitate Ospitalea, Hospital Universitario Central de Asturias, Hospital Universitario Virgen del Rocío, Hospital Universitari Arnau de Vilanova, Universidad de Alcalá, Instituto Ramón y Cajal de Investigación Sanitaria
Language: English
Subfield: Rheumatology
Field: Medicine
Domain: Health Sciences
Open Access status: gold
Estimated APC paid: $3,801
Funders: Recordati Rare Diseases, EUSA Pharma
OpenAlex ID: W7222929850