POS0494 CLINICAL PRESENTATION OF IGG4-RELATED DISEASE. MULTICENTER STUDY AND LITERATURE REVIEW

Le résumé fourni par la source

Background: IgG4-related disease (IgG4-RD) is a systemic fibroinflammatory entity that can involve almost every organ, with characteristic histological features. Its pathogenesis remains poorly understood, clinical features are heterogeneous and unspecific, and recently released classification criteria have poorer performance in early recognition of the disease. Therefore, regrettably IgG4-RD continues to be underdiagnosed. Objectives: a ) To evaluate the clinical characteristics of patients diagnosed with IgG4-RD; and b ) to compare it with other large series. Methods: Observational multicenter study of patients diagnosed with IgG4-RD and literature review. Diagnosis was made accordingly to these criteria : a ) Okazaki; b ) Umehara; c ) ACR/EULAR 2020; and/or d ) clinical, laboratory and imaging suggestive findings [1-3]. For the literature review, we searched PubMed and the Cochrane library from its inception until 30 November 2024, selecting those series with the largest number of patients. Results: We studied 78 patients diagnosed with IgG4-RD (48 males/25 females, mean±SD age; 54.4±15 years). The main affected organs were lymph nodes (n=29; 42,6%), retroperitoneum (n=23; 33.8%), kidney (n=18; 26.5%), orbit (n=16; 23.5%), aorta (n=14; 20.5%), lung/pleura (n=15; 22.%), pancreas (14; 20.6%), salivary glands (n=11; 16.1%), ear nose and throat (n=9; 16.6%), lacrimal glands (n=9; 13.2%), liver/biliary duct (n=11; 16.1%), pachymeninges (n=3; 5.5%), mesenterium (n=2; 3.7%) and other 9 (n=16.6%). IgG4 values were above 135 mg/dL in 23 (33,8%) patients (median 109 mg/dL [IQR 49,5-188]). Median values of CRP and ESR were 2.6 mg/dL [IQR 0.77-13.6] and 29 mm/h [IQR 11-56], respectively. In the literature review, 6 series of more than 100 patients each were selected. The main data from the different series are listed in Table 1. The Figure 1 shows the most frequently affected organs in the different series. The pancreas and salivary glands were less frequently involved in our series. In contrast, aortic, retroperitoneum and lung/pleura were more frequently involved than in most of the other series. Conclusion: IgG4-RD is a very heterogeneous disease with involvement of virtually every organ of the anatomy, usually presenting with involvement of more than one organ. Despite the name of the entity, serum IgG4 is not always elevated. REFERENCES: [1] Okazaki K et al. Int J Rheumatol. 2012. PMID: 22690221. [2] Umehara H, et al. Mod Rheumatol. 2012. PMID: 21881964. [3] Wallace ZS et al. Arthritis Rheumatol. 2020. PMID: 3179325. Figure 1Involved organs in series of more than 100 patients and in current series. Data are in %. Table 1Main features of the patients with IgG4-RD in series of more than 100 patients and in current series.ReferenceCasesSexFemale (F)/ Male (M)Age,median [IQR] or mean±SDDiagnosis criteriaNumber of organs affectedLevel of serum IgG4 (mg/dL), median [IQR]Lanzillotta et al. Rheumatology 2020131F (n=36),M (n=95)62 [53-70]-Umehara: possible (46%), probable (2%), definitive (52%)-1 organ (26%)-More of 1 organ (74%)224 [115-382]An et al.Orphanet J Rare Dis 2022127F (n=35),M (n=92)63 [55-69]-Umehara: possible (82.6%), probable (2.3%), definitive (11.8%)-1 organ (20%)-2-4 organs (68%)-More of 5 organs (9%)980 [390-1520]Zongfei et al.Clin Rheumatol 2021102F (n=25),M (n=77)62 [54.1-65.8]-Okazaki (100%)Median [IQR]: 2 [1-3]399 [199-776]Wang et al.Arthritis Res Ther 2018215F (n=67),M (n=148)54 [46-62]-Umehara: possible (47.9%), probable (4.7%), definitive (47.4%)-1-2 organs (36%)-3-4 organs (47%)-5 or more organs (17%)896 [350-1860]Inoue et al.Medicine (Baltimore) 2015235F (n=189), M(n=46)67-Symptoms+laboratory+image/ Compatible histology (100%)-1 organ (41%)-2 or more organs (59%)470 [ND]Wallace et al.Arthritis Rheumatol 2015125F (n=49),M (n=76)50.3±14.9- Symptoms+laboratory+image/ Compatible histology (100%)-1 organ (38%)-2 organs (24%)-3 or more organs (38%)NDCurrent series68F (n=25),M (n=48)54 ± 15.16-Okazaki: 51,4%-Umehara: possible (5,9%), probable (5%), definitive (45,6%)-ACR/EULAR 2020 (51.4%)-Symptoms+laboratory+image/ Compatible histology (23,5%)-1 organ (28%)-2 organs (26%)-3 or more organs (46%)109 [49.5-188] Acknowledgements: Blanca García Magallón (Hospital Universitario Puerta del Hierro), Maite Odriozola Gil (Hospital Universitario de Valme), Cristina Arciniega (Hospital de Mérida), Maria Lourdes Mateo Soria (Hospital German Trias i Pujol), Santiago Muñoz (Hospital Universitario Infanta Sofía), Iñigo Jesús Rua Figueroa, (Hospital Universitario de Gran Canaria Dr Negrin), Sabela Fernández Aguado, (Hospital de Montecelo). Disclosure of Interests: Fernando López-Gutiérrez Roche, Galápagos, Novartis, UCB Pharma, MSD, Celgene, Astra Zeneca, Grünenthal, Janssen, Abbvie, Lilly, Pfizer, Javier Loricera Roche, Galápagos, Novartis, UCB Pharma, MSD, Celgene, Astra Zeneca and Grünenthal, Janssen, Abbvie, Roche, Novartis, MSD, UCB Pharma, Celgene, Lilly, Pfizer, Galápagos, Pablo Martínez Calabuig: None declared, Jorge Juan Fragío Gil: None declared, Roxana González-Mazarío: None declared, Cristina Hormigos: None declared, Dalifer Freites: None declared, María Rodríguez Laguna: None declared, Patricia Moya: None declared, Marta López I Gómez: None declared, Hèctor Corominas: None declared, Maite Silva-Díaz: None declared, Guillermo González Arribas: None declared, Ángel García-Aparicio: None declared, Judit Font: None declared, Ivette Casafont-Solé: None declared, Elisabet Castaneda: None declared, Carolina Merino Argumánez: None declared, Raquel Zas: None declared, Juan Molina-Collada: None declared, Sergio Rodríguez Montero: None declared, Rafael B Melero-González: None declared, Eva Galíndez-Agirregoikoa: None declared, Andrea Hernández: None declared, Lucía Pantoja: None declared, Ignacio Braña Abascal: None declared, Vega Jovani: None declared, Elia Valls-Pascual: None declared, Natalia Mena-Vázquez: None declared, Adela Gallego: None declared, Noelia Cabaleiro-Raña: None declared, Raúl Veroz González: None declared, Mariano Andrés: None declared, Santos Castañeda: None declared, Ricardo Blanco AbbVie, Pfizer, Roche, Bristol-Myers, Janssen, Lilly, UCB, and MSD, AbbVie, MSD, and Roche. © The Authors 2025. This abstract is an open access article published in Annals of Rheumatic Diseases under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). Neither EULAR nor the publisher make any representation as to the accuracy of the content. The authors are solely responsible for the content in their abstract including accuracy of the facts, statements, results, conclusion, citing resources etc.

Ce résumé expose les affirmations des auteurs. BNTIC ne l’interprète pas comme une validation indépendante des résultats.