Effectiveness of Rituximab in IgG4 Related Disease

Fernando Lopez-Gutierrez¹, Javier Loricera², Cristina Hormigos³, Dalifer Freites Nuñez⁴, Maria Rodriguez-Laguna⁵, Patricia Moya Albarado⁶, Marta López I Gómez⁷, Hèctor Corominas⁸, Maite Silva-Díaz⁹, GUILLERMO GONZALEZ ARRIBAS⁹, Angel Garcia-Aparicio¹⁰, Judit Font Urgelles¹¹, Ivette Casafont-Sole¹¹, Pablo Martínez Calabuig¹², Elisabet Castaneda¹³, Carolina Merino¹⁴, Raquel Zas¹⁵, Juan Molina-Collada¹⁶, Rafael Benito Melero-Gonzalez¹⁷, Eva Galindez-Agirregoikoa¹⁸, Andrea Hernández-Martín¹⁹, Lucia Pantoja²⁰, Ignacio Brana Abascal²¹, Vega Jovani²², Elia Valls-Pascual²³, Natalia Mena Vázquez²⁴, Adela Gallego- Flores²⁵, Noelia Cabaleiro Raña²⁶, Raul Veroz²⁷, Mariano Andres²⁸, Santos Castañeda²⁹ and Ricardo Blanco-Alonso³⁰, and Rituximab in IgG4 Related Disease Spanish Cooperative Group, ¹Rheumatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Cantabria, Spain, ²Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain, ³Hospital Clínico San Carlos, Madrid, Madrid, Spain, ⁴Hospital Clínico San Carlos. Madrid. Spain., Madrid, Spain, ⁵Resident in Rheumatology, Madrid, Spain, ⁶Hospital de San Pau, Barcelona, Spain, ⁷Hospital Universitario Alava, Vitoria, Pais Vasco, Spain, ⁸Hospital de la Santa Creu i Sant Pau, Barcelona, Spain, ⁹Complejo Hospitalario Universitario de A Coruña, A Coruña, Spain, ¹⁰Hospital Universitario de Toledo, Toledo, Spain, ¹¹Hospital Universitari Germans Trias i Pujol, Badalona, Spain, ¹²HOSPITAL GENERAL UNIVERSITARIO VALENCIA SPAIN, Ontinyent, Comunidad Valenciana, Spain, ¹³Hospital Universitario Infanta Sofía, Madrid, Madrid, Spain, ¹⁴Hospital Universitario Puerta de Hierro Majadahonda., Majadahonda (Madrid), Spain, ¹⁵Hospital Universitario 12 de Octubre, Madrid, Madrid, Spain, ¹⁶Hospital General Universitario Gregorio Marañón, Madrid, Spain, ¹⁷CHU Ourense, O Carballino, Spain, ¹⁸BASURTO UNIVERSITY HOSPITAL, BILBAO, Spain, ¹⁹Hospital Universitario de Gran Canaria Doctor Negrín, Las Palmas, Spain, ²⁰Complejo Hospitalario Segovia, Segovia, Castilla y Leon, Spain, ²¹Hospital Universitario Central de Asturias, Oviedo, Asturias, Spain, ²²National Health system, Alicante, Spain, ²³Hospital General de Valencia, Valencia, Comunidad Valenciana, Spain, ²⁴IBIMA, Málaga, Andalucia, Spain, ²⁵Hospital Perpetuo Socorro, Badajoz, Spain, ²⁶Hospital Universitario Montecelo, Pontevedra, Galicia, Spain, ²⁷Hospital de Merida, Merida, Extremadura, Spain, ²⁸Hospital General Universitario de Alicante, Alicante, Spain, ²⁹Hospital Universitario de la Princesa, Madrid, Spain, ³⁰Division of Rheumatology, Hospital Universitario Marqués de Valdecilla. IDIVAL, Immunopathology group, Santander, Spain

Meeting: ACR Convergence 2024

Keywords: B-Lymphocyte, IgG4 Related Disease

Session Information

Date: Saturday, November 16, 2024

Title: 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 disease often associated with elevated serum IgG4 levels. High dose corticosteroids are the cornerstone of treatment, but relapses and side-effects are frequent, requiring synthetic and/or biologic immunosuppressants. Rituximab (RTX) seems to be effective in IgG4-RD.

Methods: Multicentre retrospective observational study of patients with IgG4-RD treated with RTX. Outcomes were clinical and serologic response, as well as safety.

Results: We included 54 patients (38 men/16 women; mean age±SD 53.5±14.6 years) with IgG4-RD, treated with RTX (Table 1). The most affected organs were lymph nodes (n=28; 51.8%), retroperitoneum (n=18; 33.3%), kidney (n=16; 29.6%), orbit (n=15; 27.7%), aorta (n=13; 24.07%), lung/pleura (n=12; 22.2%), pancreas (n=12; 22.2%), salivary glands (n=10; 18.5%), ear nose and throat (n=9; 16.6%), lacrimal glands (n=8; 14.8%), , liver/biliary duct (n=8; 14.8%), pachymeninges (n=3; 5.5%) and mesenterium (n=2; 3.7%). All but 4 patients (7.4%) had received oral corticosteroids, and 15 (27.7%) patients also received corticosteroid boluses. 30 (55.5%) patients received conventional cDMARDs: methotrexate (MTX) (n=17; 31.5%), azathioprine (n=11; 20.4%), and mycophenolate mophetil (n=2(3.7%). Median time from diagnosis to RTX initiation was 6 (range 0-72) months, with a median time of follow-up of 27 (range 1-132) months. Main induction treatment schedule with RTX was 1g x2, two weeks apart (n=41; 75.9%), 500mg x2, two weeks apart (n=9; 16.6%) and 375 mg/m2 weekly x4 (n=3; 5.5%). 38 (70.3%) patients received maintenance treatment with RTX (Table 2). After 12 and 24 moths of follow up, complete and partial clinical improvement was observed in 21 (52.5%) and 17 (42.5%), and in 20 (64.5%) and 9 (29%) patients, respectively. Only 6 relapses were observed. Prednisone could be discontinued at 24 months in 17 (50%) patients. 3 patients died during follow up (1 of acute coronary syndrome, 1 of respiratory tract infection and one of cancer related complications). One patient needed ICU admission because of Influenza pneumonia, and 2 developed a larynx and a breast cancer, respectively.

Conclusion: RTX seems to be an effective and relatively safe therapy in IgG4-RD. Maintenance treatment with RTX seems to be associated with a low rate of relapse.

Disclosures: F. Lopez-Gutierrez: AstraZeneca, 12, Formation/Congress attendance, 12, Formation/Congress attendance, Novartis, 12, Formation/congress attendance; J. Loricera: AstraZeneca, 2, 6, Celgene, 2, 6, Eli Lilly, 5, Janssen, 5, Merck/MSD, 2, 5, 6, Novartis, 12, Formation/Congress attendance, Pfizer, 5, Roche, 2, 5, 6, UCB, 2, 5, 6; C. Hormigos: None; D. Freites Nuñez: None; M. Rodriguez-Laguna: None; P. Moya Albarado: None; M. López I Gómez: None; H. Corominas: None; M. Silva-Díaz: None; G. GONZALEZ ARRIBAS: None; A. Garcia-Aparicio: None; J. Font Urgelles: None; I. Casafont-Sole: None; P. Martínez Calabuig: None; E. Castaneda: None; C. Merino: None; R. Zas: None; J. Molina-Collada: None; R. Melero-Gonzalez: None; E. Galindez-Agirregoikoa: AbbVie/Abbott, 6, Amgen, 6, Eli Lilly, 6, Janssen, 6, Novartis, 6, Pfizer, 6, UCB, 6; A. Hernández-Martín: None; L. Pantoja: None; I. Brana Abascal: None; V. Jovani: None; E. Valls-Pascual: None; N. Mena Vázquez: None; A. Gallego- Flores: None; N. Cabaleiro Raña: None; R. Veroz: None; M. Andres: Grunenthal, 5, Menarini, 6; S. Castañeda: Bristol-Myers Squibb(BMS), 2, 6, Eli Lilly, 2, 6, Merck/MSD, 2, 5, 6, Pfizer, 5, Roche, 2, 6, UCB, 2, 5; R. Blanco-Alonso: AbbVie, 2, 5, 6, Bristol-Myers Squibb, 2, 6, Galapagos, 6, Janssen, 2, 6, Lilly, 2, 6, MSD, 2, 5, 6, Pfizer, 2, 6, Roche, 2, 5, 6.

To cite this abstract in AMA style:

Lopez-Gutierrez F, Loricera J, Hormigos C, Freites Nuñez D, Rodriguez-Laguna M, Moya Albarado P, López I Gómez M, Corominas H, Silva-Díaz M, GONZALEZ ARRIBAS G, Garcia-Aparicio A, Font Urgelles J, Casafont-Sole I, Martínez Calabuig P, Castaneda E, Merino C, Zas R, Molina-Collada J, Melero-Gonzalez R, Galindez-Agirregoikoa E, Hernández-Martín A, Pantoja L, Brana Abascal I, Jovani V, Valls-Pascual E, Mena Vázquez N, Gallego- Flores A, Cabaleiro Raña N, Veroz R, Andres M, Castañeda S, Blanco-Alonso R. Effectiveness of Rituximab in IgG4 Related Disease [abstract]. Arthritis Rheumatol. 2024; 76 (suppl 9). https://acrabstracts.org/abstract/effectiveness-of-rituximab-in-igg4-related-disease/. Accessed .

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