LIFE-THREATENING PRIMARY SJÖGREN SYNDROME: CLINICAL CHARACTERIZATION AND OUTCOMES IN 1535 PATIENTS (GEAS-SS REGISTRY)

KOSTOV, BELCHIN; BRENDA MAURE; PEREZ ALVAREZ ROBERTO; MIGUEL LOPEZ-DUPLA; EVA FONSECA; GLORIA DE LA RED; PATRICIA FANLO; BRITO ZERON PILAR; FLORES-CHÁVEZ ALEJANDRA; FRAILE, GUADALUPE; FRANCISCO-JAVIER RASCÓN; ALICIA GARCÍA-PÉREZ ; MAR RIPOLL; BLANCA PINILLA; Mª JOSÉ SOTO-CÁRDENAS; RAMOS CASALS MANUEL; RETAMOZO SOLEDAD; SOLANS, ROSER; CARLOS FEIJOO; MÓNICA ZAMORA; MIGUEL ÁNGEL DUARTE MILLÁN ; PABLO GUISADO-VASCO; SANDRA RODRÍGUEZ RODRÍGUEZ; CÉSAR MORCILLO

Amsterdam

Congreso; EUROPEAN LEAGUE AGAINST RHEUMATISM (EULAR); 2018

EULAR

OBJECTIVE. 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).PATIENTS. 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 1535 consecutive patients fulfilling the 2002 classification criteria for primary SS. Life-threatening systemic disease was defined as an activity level scored as ?High? in at least one ESSDAI domain.RESULTS. Among 1535 patients, 209 (14%) were classified as presenting a life-threatening systemic disease: 194 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 22 (10%), cutaneous in 18 (9%), articular in 18 (9%), hematological in 7 (3%) and muscular in 4 (2%); the most frequent clinical presentations in each domain were, respectively, parotid lymphoma (n=41), focal neurological deficit (n=20), ganglionopathy (n=11), usual interstitial pneumonitis (n=9), renal failure (n=11), ulcerated cutaneous vasculitis (n=9), symmetric polyarthritis (m=17), severe thrombocytopenia (n=3) and severe myositis (n=3). With respect to therapeutic approach, 144 (69%) required glucocorticoids, 65 (31%) immunosuppressive agents and 42 (20%) biological therapies. During the follow-up, 36/209 (17%) patients died, mainly due to lymphoma (n=16), pulmonary fibrosis (n=5), end-stage renal failure (n=4), CNS progressive disease (n=3) and systemic vasculitis (n=3).CONCLUSION. A 14% 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.