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

Soledad Retamozo^1,2,3, 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-Zerón^4,22, 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

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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