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Abstract Number: 1564

Severe, Life-Threatening Phenotype of Primary Sjögren Syndrome: Clinical Characterization and Outcomes in 1580 Patients (GEAS-SS Registry)

Soledad Retamozo1,2,3, Alejandra Flores-Chavez4, Belchin Kostov5, Roser Solans6, Guadalupe Fraile7, Brenda Maure8, Carlos Feijoo9, Francisco-Javier Rascón10, Roberto Pérez-Alvarez11, Mónica Zamora12, Alicia García-Pérez13, Miguel Lopez-Dupla14, Jesús Canora15, Mar Ripoll16, Eva Fonseca17, Pablo Guisado18, Blanca Pinilla19, Gloria de-la-Red20, Antonio-J. Chamorro21, César Morcillo22, Patricia Fanlo23, Mª José Soto-Cárdenas24, Manuel Ramos-Casals25 and Pilar Brito-Zerón4,22, 1Instituto de Investigaciones en Ciencias de la Salud, Universidad Nacional de Córdoba, Consejo Nacional de Investigaciones Científicas y Técnicas (INICSA-UNC-CONICET), Cordoba, Argentina, 2Laboratory of Systemic Autoimmune Diseases “Josep Font”, CELLEX, Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Department of Systemic Autoimmune Diseases, ICMID, Hospital Clinic, Barcelona, Barcelona, Spain, 3Rheumatology Unit, Hospital Privado Universitario de Córdoba, Institute University of Biomedical Sciences University of Córdoba (IUCBC), Cordoba, Argentina, 4Laboratory of Autoimmune Diseases Josep Font, IDIBAPS, ICMiD, Hospital Clínic, Barcelona, Spain, Barcelona, Spain, 5Primary Care Research Group, IDIBAPS, Centre d’Assistència Primària ABS Les Corts, GESCLINIC, Barcelona, Spain., Barcelona, Spain, 6Systemic Autoimmune Diseases Unit, Hospital Vall d’Hebron, Barcelona, Barcelona, Spain, 7Systemic Autoimmune Diseases Unit, Hospital Ramón y Cajal, Madrid, Madrid, Spain, 8Department of Internal Medicine, Complejo Hospitalario Universitario, Vigo, Vigo, Spain, 9Systemic Autoimmune Diseases Unit, Hospital Parc Taulí, Sabadell, Sabadell, Spain, 10Systemic Autoimmune Diseases Unit, Hospital Son Espases, Palma de Mallorca, Palma de Mallorca, Spain, 11Department of Internal Medicine, Hospital do Meixoeiro, Vigo, Vigo, Spain, 12Systemic Autoimmune Diseases Unit, Hospital Virgen de las Nieves, Granada, Granada, Spain, 13Systemic Autoimmune Diseases Unit, Hospital Universitario Central de Asturias, Oviedo, Oviedo, Spain, 14Systemic Autoimmune Diseases Unit, Hospital Joan XXIII, Tarragona, Tarragona, Spain, 15Systemic Autoimmune Diseases Unit, Hospital de Fuenlabrada, Madrid, Madrid, Spain, 16Department of Internal Medicine, Hospital Infanta Sofía, Madrid, Madrid, Spain, 17Systemic Autoimmune Diseases Unit, Hospital de Cabueñes, Gijón, Gijón, Spain, 18Department of Internal Medicine, Complejo Hospitalario Ruber Juan Bravo, Madrid, Madrid, Spain, 19Systemic Autoimmune Diseases Unit Medicine, Hospital Gregorio Marañón, Madrid, Madrid, Spain, 20Systemic Autoimmune Diseases Unit, Hospital Esperit Sant, Santa Coloma de Gramenet, Santa Coloma de Gramenet, Spain, 21Systemic Autoimmune Diseases Unit, Hospital Universitario de Salamanca, Salamanca, Salamanca, Spain, 22Systemic Autoimmune Diseases Unit, Hospital CIMA-Sanitas, Barcelona, Barcelona, Spain, 23Systemic Autoimmune Diseases Unit, Hospital Virgen del Camino, Pamplona, Pamplona, Spain, 24Department of Medicine, University of Cadiz, Cadiz, Cadiz, Spain, 25University of Barcelona, Hospital Clínic, Barcelona, Barcelona, Spain

Meeting: 2018 ACR/ARHP Annual Meeting

Keywords: outcomes and phenotypes, Sjogren's syndrome

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Session Information

Date: Monday, October 22, 2018

Title: Sjögren's Syndrome – Basic and Clinical Science Poster

Session Type: ACR Poster Session B

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

Background/Purpose: To analyse the clinical features and outcomes of patients presenting with life-threatening systemic disease in a large cohort of Spanish patients with primary Sjogren syndrome (SjS).

Methods: The GEAS-SS multicenter registry was formed in 2005 with the aim of collecting a large series of Spanish patients with primary SS, and included more than 20 Spanish reference centers with substantial experience in the management of SS patients. By January 2018, the database included 1580 consecutive patients fulfilling the 2002 classification criteria for primary SS. Severe, life-threatening systemic disease was defined as an activity level scored as “High” in at least one ESSDAI domain.

Results: Among 1580 patients, 208 (13%) were classified as presenting a severe, potentially life-threatening systemic disease: 193 presented one ESSDAI domain classified as high, 14 presented two high scored domains and only one presented three high activity domains. The ESSDAI domains involved consisted of lymphadenopathy in 78 (37%) cases, CNS in 28 (13%), PNS in 25 (12%), pulmonary in 25 (12%), renal in 21 (10%), cutaneous in 19 (9%), articular in 18 (9%), hematological in 7 (3%) and muscular in 4 (2%). Patients with severe systemic disease were more frequently men (p=0.001) and had a higher frequency of anemia (p<0.001), lymphopenia (p<0.001), rheumatoid factor (p=0.021), low C3 levels (p=0.015), low C4 levels (p<0.001) and cryoglobulins (p<0.001). From a therapeutic point of view, systemic patients received more frequently glucocorticoids (p<0.001), immunosuppressants (p<0.001), intravenous immunoglobulins (p=0.008) and rituximab (p<0.001). We found an overall mortality rate of 20% in severe systemic patients, a rate that reach to 33% in patients presenting two or more high systemic involvements; these patients had a higher frequency of low C4 levels (p=0.012) and cryoglobulins (p=0.001) in comparison with those with a single severe organ involved.

Conclusion: A 13% of patients with primary SjS develop a potentially life-threatening systemic disease (mainly lymphoma, but also severe internal organ involvements including nervous system, the lungs and the kidneys). This subset of patients requires intensive therapeutic management with a mortality rate of nearly 20% of cases.


Disclosure: S. Retamozo, None; A. Flores-Chavez, None; B. Kostov, None; R. Solans, None; G. Fraile, None; B. Maure, None; C. Feijoo, None; F. J. Rascón, None; R. Pérez-Alvarez, None; M. Zamora, None; A. García-Pérez, None; M. Lopez-Dupla, None; J. Canora, None; M. Ripoll, None; E. Fonseca, None; P. Guisado, None; B. Pinilla, None; G. de-la-Red, None; A. J. Chamorro, None; C. Morcillo, None; P. Fanlo, None; M. J. Soto-Cárdenas, None; M. Ramos-Casals, None; P. Brito-Zerón, None.

To cite this abstract in AMA style:

Retamozo S, Flores-Chavez A, Kostov B, Solans R, Fraile G, Maure B, Feijoo C, Rascón FJ, Pérez-Alvarez R, Zamora M, García-Pérez A, Lopez-Dupla M, Canora J, Ripoll M, Fonseca E, Guisado P, Pinilla B, de-la-Red G, Chamorro AJ, Morcillo C, Fanlo P, Soto-Cárdenas MJ, Ramos-Casals M, Brito-Zerón P. Severe, Life-Threatening Phenotype of Primary Sjögren Syndrome: Clinical Characterization and Outcomes in 1580 Patients (GEAS-SS Registry) [abstract]. Arthritis Rheumatol. 2018; 70 (suppl 9). https://acrabstracts.org/abstract/severe-life-threatening-phenotype-of-primary-sjogren-syndrome-clinical-characterization-and-outcomes-in-1580-patients-geas-ss-registry/. Accessed .
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