Clinical Presentation of Igg4-Related Disease. Multicenter Study and Literature Review

Rafael Gálvez Sánchez¹, Fernando Lopez Gutierrez², Javier Loricera³, Pablo Martínez Calabuig⁴, Jorge Juan Fragío Gil⁵, Roxana González Mazario⁶, Cristina Hormigos Martin⁷, DALIFER FREITES⁸, Maria Rodriguez Laguna⁹, Patricia Moya Albarado¹⁰, Marta López i Gómez¹¹, Hector Corominas¹², Maite Silva-Diaz¹³, GUILLERMO GONZALEZ ARRIBAS¹⁴, Angel Garcia-Aparicio¹⁵, Judit Font-Urgelles¹⁶, Ivette Casafont-Solé¹⁶, elisabeth Castañeda¹⁷, Carolina Merino¹⁸, Raquel Zas¹⁹, Juan Molina-Collada²⁰, Sergio Rodríguez Montero²¹, Rafael B. Melero-González²², Eva Galíndez Agirregoikoa²³, Andrea Hernández²⁴, Lucia Pantoja²⁵, Ignacio Braña²⁶, Vega Jovaní²⁷, Elia Valls-Pascual²⁸, Natalia Mena Vázquez²⁹, Adela Gallego³⁰, Noelia Cabaleiro Raña³¹, Raul Veroz³², Mariano Andrés Collado³³, Santos Castañeda³⁴ and Ricardo Blanco³⁵, ¹Division of Rheumatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, Immunopathology Group, Santander , Spain, Santander, Spain, ²Hospital Universitario Marqués de Valdecilla, Immunopathology Group -IDIVAL, Reumatología, Santander, Santander, Spain, ³Hospital Universitario Marqués de Valdecilla, IDIVAL, Immunopathology Group, Santander , Spain, Santander, Spain, ⁴Hospital General Universitario de Valencia, Spain/ Uversidad Catolica de Valencia San Vicente Martir, Valencia, Spain, Ontinyent, Spain, ⁵Hospital General Universitario, Valencia, Spain, ⁶Hospital General de Valencia, Valencia, Spain, ⁷Hospital Clínico San Carlos, Madrid, Spain, ⁸Rheumatology Service, San Carlos Clinical Hospital, Madrid, Madrid, Spain, ⁹Hospital Clinico San Carlos, Madrid, Spain, ¹⁰Hospital de la Santa Creu i Sant Pau, Barcelona, Spain, ¹¹Hospital Universitario de Araba, Vitoria, Pais Vasco, Spain, ¹²Hospital de Sant Pau, Barcelona, Spain, ¹³Complexo Hospitalario Universitario A Coruña, A Coruña, Galicia, Spain, ¹⁴Facultativo especialista, PALMA DE MALLORCA, Galicia, Spain, ¹⁵Hospital Universitario de Toledo, Toledo, Spain, ¹⁶Hospital Universitari Germans Trias i Pujol, Badalona, Spain, ¹⁷Hospital Universitario Infanta Sofía, Madrid, Madrid, Spain, ¹⁸Rheumatology department. Hospital Universitario Puerta de Hierro Majadahonda, Majadahonda (Madrid), Madrid, Spain, ¹⁹Hospital Universitario Doce de Octubre, Madrid, Spain, ²⁰Department of Rheumatology, Hospital General Universitario Gregorio Marañón. Instituto de Investigación Sanitaria Gregorio Marañón (IiSGM), Madrid, Spain, Madrid, Spain, ²¹Hospital Universitario de Valme, Sevilla, Andalucia, Spain, ²²COMPLEXO HOSPITALARIO UNIVERSITARIO DE OURENSE, O Carballino, Spain, ²³BASURTO UNIVERSITY HOSPITAL, BILBAO, Spain, ²⁴Hospital Universitario de Gran Canaria Dr Negrin, Palmas de Gran Canaria, Canarias, Spain, ²⁵Complejo Hospitalario Segovia, Segovia, Spain, ²⁶Hospital Universitario Central de Asturias, Oviedo, Spain, ²⁷Hospital General Universitario de Alicante, Reumatología, Alicante, Alicante, Comunidad Valenciana, Spain, ²⁸Hospital General de Valencia, Valencia, Comunidad Valenciana, Spain, ²⁹Hospital Regional Universitario de Malaga, Malaga, Spain, Málaga, Spain, ³⁰Complejo Hospitalario don Benito Villanueva, Badajoz, Spain, ³¹Hospital Universitario Montecelo, Rheumatology, Pontevedra, Galicia, Spain, ³²Hospital de Merida, Merida, Extremadura, Spain, ³³Hospital General Universitario de Alicante, Madrid, Comunidad Valenciana, Spain, ³⁴Hospital Universitario de La Princesa, IIS-Princesa, Madrid, Madrid, Spain, ³⁵Rheumatology Division, Hospital Universitario Marqués de Valdecilla, IDIVAL, Immunopathology Group, Santander, Spain, Santander, Cantabria, Spain

Meeting: ACR Convergence 2025

Keywords: autoimmune diseases, IgG4 Related Disease

Session Information

Date: Sunday, October 26, 2025

Title: (0233–0279) Miscellaneous Rheumatic & Inflammatory Diseases Poster I

Session Type: Poster Session A

Session Time: 10:30AM-12:30PM

Background/Purpose: 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. Our objetives were 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 135mg/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. The figure 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

TABLE. Main features of the patients with IgG4-RD in series of more than 100 patients and in current series

FIGURE. Involved organs in series of more than 100 patients and in current series. Data are in %

Disclosures: R. Gálvez Sánchez: None; F. Lopez Gutierrez: Roche, Galápagos, Novartis, UCB Pharma, MSD, Celgene, Astra Zeneca, Grünenthal, Janssen, Abbvie, Lilly, Pfizer, 12, support for attending meetings and/or travel; J. Loricera: AbbVie/Abbott, 5, AstraZeneca, 2, 5, 6, Celgene, 2, 5, 6, Eli Lilly, 5, Janssen, 5, Merck/MSD, 2, 5, 6, Novartis, 2, 5, 6, Pfizer, 5, Roche, 2, 5, 6, UCB, 2, 5, 6; P. Martínez Calabuig: None; J. Fragío Gil: Quibim, 1; R. González Mazario: None; C. Hormigos Martin: None; D. FREITES: None; M. Rodriguez Laguna: None; P. Moya Albarado: None; M. López i Gómez: None; H. Corominas: None; M. Silva-Diaz: None; G. GONZALEZ ARRIBAS: None; A. Garcia-Aparicio: AbbVie/Abbott, 6, AstraZeneca, 6, GlaxoSmithKlein(GSK), 1, 2, 6, UCB, 6; J. Font-Urgelles: None; I. Casafont-Solé: None; e. Castañeda: None; C. Merino: None; R. Zas: None; J. Molina-Collada: None; S. Rodríguez Montero: Abbvie, Pfizer, Roche, UCB, Novartis, 12, Financial supporto for attending meetings or travel; R. Melero-González: None; E. Galíndez Agirregoikoa: None; A. Hernández: None; L. Pantoja: None; I. Braña: None; V. Jovaní: None; E. Valls-Pascual: None; N. Mena Vázquez: None; A. Gallego: None; N. Cabaleiro Raña: None; R. Veroz: None; M. Andrés Collado: Grünenthal, Menarini, 6, 12, Attending meetings or travel; S. Castañeda: None; R. Blanco: AbbVie/Abbott, 2, 5, 6, Bristol-Myers Squibb(BMS), 2, 6, Eli Lilly, 2, 6, Janssen, 2, 6, Merck/MSD, 2, 5, 6, Pfizer, 2, 6, Roche, 2, 5, 6.

To cite this abstract in AMA style:

Gálvez Sánchez R, Lopez Gutierrez F, Loricera J, Martínez Calabuig P, Fragío Gil J, González Mazario R, Hormigos Martin C, FREITES D, Rodriguez Laguna M, Moya Albarado P, López i Gómez M, Corominas H, Silva-Diaz M, GONZALEZ ARRIBAS G, Garcia-Aparicio A, Font-Urgelles J, Casafont-Solé I, Castañeda e, Merino C, Zas R, Molina-Collada J, Rodríguez Montero S, Melero-González R, Galíndez Agirregoikoa E, Hernández A, Pantoja L, Braña I, Jovaní V, Valls-Pascual E, Mena Vázquez N, Gallego A, Cabaleiro Raña N, Veroz R, Andrés Collado M, Castañeda S, Blanco R. Clinical Presentation of Igg4-Related Disease. Multicenter Study and Literature Review [abstract]. Arthritis Rheumatol. 2025; 77 (suppl 9). https://acrabstracts.org/abstract/clinical-presentation-of-igg4-related-disease-multicenter-study-and-literature-review/. Accessed .

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