SJOGRENSER Registry: Prospective Evaluation of a Cohort of Patients with Primary Sjögren's Syndrome After 8 Years of Follow-up

Mónica Fernández-Castro¹, Zulema Plaza², Jose Rosas³, Victor Martinez-Taboada⁴, Alejandro Olivé⁵, Raúl Menor-Almagro⁶, SARA MANRIQUE⁷, Ruth López-González⁸, Celia Erausquin⁹, Antonio Naranjo⁹, Rocío Caño-Alameda¹⁰, Silvia Gómez-Sabater¹⁰, Jesús Alberto García Vadillo¹¹, Belen Serrano Benavente¹², Sheila Melchor¹³, Paula V. Estrada-Alarcón¹⁴, Angel Garcia-Aparicio¹⁵, Cristina Bohorquez¹⁶, Enrique Júdez¹⁷, Ximena Larco¹⁸, Javier Loricera¹⁹, Nerea Alcorta Lorenzo²⁰, Beatriz Paredes Romero²¹, Jorge González Martín²², María Ángeles Blázquez Cañamero²³, Consuelo Ramos-Giráldez²⁴, Fernando Alonso²⁵, Marta Domínguez²⁶ and Jose Luis Andreu-Sánchez²⁷, ¹Rheumatology Department Hospital Puerta de Hierro Majadahonda, Majadahonda, Spain, ²Universidad Autónoma de Madrid, Madrid, Spain, ³Hospital Marina Baixa, Alicante, Spain, ⁴Rheumatology, Hospital Marqués de Valdecilla, Santander, Spain, ⁵Rheumatology Department Hospital Germans Trias i Pujol, Badalona, Spain, ⁶Rheumatology, Hospital Jerez, Puerto De Santa María, Spain, ⁷Division of Rheumatology, Hospital Regional Universitario Carlos Haya, Malaga, Spain, ⁸Rheumatology Department Hospital Virgen de la Concha, Zamora, Spain, ⁹Rheumatology Department Hospital Universitario de Gran Canaria Doctor Negrín, Las Palmas de Gran Canaria, Spain, ¹⁰Rheumatology Department, Dr. Balmis University General Hospital, Alicante. Institute for Health and Biomedical Research (ISABIAL), Alicante, Spain, ¹¹Rheumatology Department Hospital de la Princesa, Madrid, Spain, ¹²Rheumatology Department Hospital Gregorio Marañón, Madrid, Spain, ¹³Rheumatology Department Hospital Doce de Octubre, Madrid, Spain, ¹⁴Hospital de San Juan Despí Moisès Broggi, Barcelona, Spain, ¹⁵Hospital Universitario de Toledo, Toledo, Spain, ¹⁶Rheumatology Unit, Hospital Universitario Príncipe de Asturias, Alcalá de Henares, Spain, ¹⁷Rheumatology Department Hospital de Albacete, Albacete, Spain, ¹⁸Rheumatology Department Hospital de León, León, Spain, ¹⁹Hospital Universitario Marqués de Valdecilla, Santander, Spain, ²⁰University Hospital Donostia, San Sebastian, Spain, ²¹Rheumatology Department Hospital Infanta Sofía, San Sebastian de los Reyes, Spain, ²²Rheumatology Department Hospital Madrid Norte Sanchinarro, Madrid, Spain, ²³Rheumatology Department Hospital Ramon y Cajal, Madrid, Spain, ²⁴Rheumatology Department Hospital Universitario Virgen de Valme, Sevilla, Spain, ²⁵Spanish Society of Rheumatology, Madrid, Spain, ²⁶Sociedad Española de Reumatología, Madrid, Spain, ²⁷Rheumatology, Hospital Universitario Puerta de Hierro, Majadahonda, Spain

Meeting: ACR Convergence 2023

Keywords: Sjögren's syndrome

Session Information

Date: Monday, November 13, 2023

Title: (1365–1382) Sjögren’s Syndrome – Basic & Clinical Science Poster I

Session Type: Poster Session B

Session Time: 9:00AM-11:00AM

Background/Purpose: Describe the evolution of patients with Sjögren’s syndrome (SS) in relation to the appearance of new systemic manifestations and disease activity, as well as factors associated with an unfavorable outcome.

Methods: SJÖGRENSER PROS (SS-PROS) is an observational, longitudinal, and multicenter study of patients with SS who met 2002 classification criteria under active follow-up in rheumatology clinics of 31 Spanish hospitals that participated in the cross-sectional phase of the study (SJÖGRENSER TRANS; SS-TRANS). For the prospective phase, an 8 years follow-up visit was performed (2021-2022) comparing the results with baseline visit (SS-TRANS 2013-2014, 437 patients). Medical history was reviewed and a medical interview was performed. Epidemiological, clinical and serological variables as well as causes of death were recorded. Continuous and categorical variables were analyzed using means, medians, and frequencies, with their respective deviations and interquartile ranges (Q1-Q3). Student’s T test was used to establish statistical associations, considering p< 0.05 significant.

Results: Up to January 2023, 180 patients have been included, 96% women, mean age 68 years (SD 11.2) and mean evolution time from diagnosis to inclusion of 18 years (SD 7.27). Newly developed systemic involvement was: joint in 47% of patients (arthritis in 17%), hematological (cytopenia) in 44%, lung involvement in 14%, renal in 13%, digestive in 17%. %, hepatic 6%, central and peripheral nervous system (NS) 4% and 3% respectively, and cardiac 2.7%; parotid inflammation was described in 13%. In the last 8 years, 3 new lymphomas have appeared (3/180, 1.6%).

Mean of the ESSDAI in SS-PROS was lower than in SS-TRANS: 2.78 (SD 4.3; 1-4) vs. 3.66 (SD 5.15; 2-4), respectively. By domains, the greatest variations were observed in the articular, hematological and biological domains; while in the rest of the domains, stability was evidenced in ≥94% of the cases (Figure 1). By organs, the most affected organ during follow-up was the lung, with 8 patients who increased the mean value of this domain.

ESSPRI mean improved: 5.24 (SD 2.35; 3.67-7) vs. 4.47 (SD 2.14; 2.67-6), respectively; being greater in the VAS of pain, 5.16 (SD 3.02; 3- 8) vs. 3.98 (SD 2.95; 1- 7), respectively (Figure 2).

Twenty-seven patients out of 180 have died since their inclusion in SS-TRANS (15%), 89% women, with a mean age of 75 years (SD 11.5; 65-84.5).

Comparing baseline data visit (SS-TRANS) from the group of deceased (during SS-PROS) and of non-deceased (remaining under follow-up in SS-PROS), we observed that age (70 years; SD 11; 59-77), disease evolution time (11.5 years; SD 8; 4-17) and ESSDAI (5.22; SD 6.35; 0-8), were higher in the deceased vs. non-deceased (age 59 years, SD 12, p< 0.001; evolution time 9 years, SD7, p=0.078; ESSDAI 3.38, SD 4.88, 0-4, p0.087). There were no differences in ESSPRI.

Conclusion: Patients with SS develop new systemic manifestations over the years, despite maintaining or improving ESSDAI, suggesting the need of a close follow-up. The ESSPRI varies little over time, which represents a great challenge for the scientific community. Mortality in this cohort is 15%. Greater age, time of evolution and baseline ESSDAI were associated with a worse outcome.

Figure 1: ESSDAI analysis by domains comparing SjögrenSER TRANS vs. SjögrenSER PROS

Figure 2: Mean ESSPRI in SjögrenSER TRANS compare with SjögrenSER PROS

Disclosures: M. Fernández-Castro: None; Z. Plaza: None; J. Rosas: None; V. Martinez-Taboada: None; A. Olivé: None; R. Menor-Almagro: None; S. MANRIQUE: None; R. López-González: None; C. Erausquin: AbbVie/Abbott, 12, financial support to assist meetings; A. Naranjo: None; R. Caño-Alameda: None; S. Gómez-Sabater: None; J. García Vadillo: None; B. Serrano Benavente: None; S. Melchor: None; P. Estrada-Alarcón: None; A. Garcia-Aparicio: None; C. Bohorquez: None; E. Júdez: None; X. Larco: None; J. Loricera: None; N. Alcorta Lorenzo: None; B. Paredes Romero: None; J. González Martín: None; M. Blázquez Cañamero: Gedeon Richter, 6, GlaxoSmithKlein(GSK), 6, UCB, 6; C. Ramos-Giráldez: None; F. Alonso: None; M. Domínguez: None; J. Andreu-Sánchez: None.

To cite this abstract in AMA style:

Fernández-Castro M, Plaza Z, Rosas J, Martinez-Taboada V, Olivé A, Menor-Almagro R, MANRIQUE S, López-González R, Erausquin C, Naranjo A, Caño-Alameda R, Gómez-Sabater S, García Vadillo J, Serrano Benavente B, Melchor S, Estrada-Alarcón P, Garcia-Aparicio A, Bohorquez C, Júdez E, Larco X, Loricera J, Alcorta Lorenzo N, Paredes Romero B, González Martín J, Blázquez Cañamero M, Ramos-Giráldez C, Alonso F, Domínguez M, Andreu-Sánchez J. SJOGRENSER Registry: Prospective Evaluation of a Cohort of Patients with Primary Sjögren’s Syndrome After 8 Years of Follow-up [abstract]. Arthritis Rheumatol. 2023; 75 (suppl 9). https://acrabstracts.org/abstract/sjogrenser-registry-prospective-evaluation-of-a-cohort-of-patients-with-primary-sjogrens-syndrome-after-8-years-of-follow-up/. Accessed .

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