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

RETAMOZO SOLEDAD; ROSER SOLANS; CARLOS FEIJOO; MÓNICA ZAMORA; JESÚS CANORA; PABLO GUISADO-VASCO; ANTONIO-J. CHAMORRO; Mª JOSÉ SOTO-CÁRDENAS; BELCHIN KOSTOV; BRENDA MAURE; ROBERTO PÉREZ-ALVAREZ; MIGUEL LOPEZ-DUPLA; EVA FONSECA; GLORIA DE-LA-RED; PATRICIA FANLO; PILAR BRITO-ZERÓN; ALEJANDRA FLORES-CHAVEZ; GUADALUPE FRAILE; FRANCISCO-JAVIER RASCÓN; ALICIA GARCÍA-PÉREZ; MAR RIPOLL; BLANCA PINILLA; CÉSAR MORCILLO; RAMOS-CASALS MANUEL

Chicago

Congreso; 2018 ACR/ARHP Annual Meeting; 2018

American Collage of Rheumatology

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