Development Of High Systemic Activity In Primary Sjögren Syndrome: Analysis Of 1487 Spanish Patients (GEAS-SS REGISTRY).

SOLANS LAQUÉ R; FEIJOO MASSÓ C; ZAMORA, MÓNICA; RIPOLL, MAR; DE LA RED, GLORIA; PINILLA, BLANCA; FANLO, PATRICIA; DE MIGUEL B; RAMOS CASALS MANUEL; ALEJANDRA FLORES-CHAVEZ; MAURE, BRENDA; RASCON FRANCISCO JAVIER; DUARTE-MILLÁN, MIGUEL-ANGEL; FONSECA, EVA; SANCHEZ VIZCAÍNO E; AKASBI, MIRIAM; JIMENEZ I; KOSTOV, BELCHIN; RETAMOZO SOLEDAD; FRAILE, GUADALUPE; PEREZ ALVAREZ ROBERTO; LOPEZ-DUPLA, MIGUEL; GUISADO-VASCO, PABLO; CHAMORRO, ANTONIO; SOTO CARDENAS MJ; GATO DIEZ A; ARTEAGA SOFIA; BRITO ZERON PILAR

Buenos Aires

Congreso; XX CONGRESO DE LA LIGA PANAMERICANA DE ASOCIACIONES DE REUMATOLOGÍA (PANLAR); 2018

PANLAR

Background and Objective: To characterize high systemic involvement inprimary Sjögren syndrome (SS) in a large cohort of Spanish patients usingthe EULAR-SS disease activity index (ESSDAI).Methods: The GEAS-SS Study Group was formed in 2005 with the aim ofcollecting a large series of Spanish patients with primary SjS. Systemic involvementwas characterized using ESSDAI definitions for its12 domains,and patients scoring as high in the domains that specifically contain highactivity (lymphadenopathy, articular, cutaneous, pulmonary, renal, peripheralnervous system, central nervous system and muscular) were identifiedand analysed.Results: Of the 1487 patients included in the Registry, 186 (12.5%) presentedwith 197 systemic features classified as high according to the correspondingorgan-by-organ ESSDAI domains. There were 159 women and 27 men, witha mean age of 59,06 years (range 15?89 years) at the time of the diagnosisof high activity. High systemic activity was scored in the lymphadenopathy(n=53), peripheral nervous system (n=31), central nervous system (n=25), pulmonary(n=24), renal (n=21), articular (n=16), skin (n=16), hematological (n=7) andmuscular (n=4) domains. Men were overrepresented in the muscular (33%),lymphadenopathy (19%) and cutaneous (19%) domains. The domains diagnosedat younger ages included muscular (mean age of 45 years), CNS (49,7 years), articular(54,3 yrs) and hematological (55,4 yrs), while cutaneous (63,2 yrs) and pulmonary(67,1 years) were diagnosed at older ages. The main clinical syndromesresponsible for high systemic activity were lymphoma (n=53), ataxic neuronopathy(n=14), pulmonary fibrosis (n=12), diffuse leukocytoclastic vasculitis (n=10), severepolyneuropathy (n=10), myelitis (n=8), meningitis (n=7), multiple mononeuritis(n=7), membranous/membranoproliferative glomerulonephritis (n=6) and severethrombocytopenia (n=5). Notably, 13 (81%) out of the patients presenting withhigh activity in the articular domain (>6 joints involved) were finally diagnosedwith rheumatoid arthritis during their follow-up.Conclusions: Primary Sjögren syndrome at diagnosis is presenting with highsystemic activity in 12.5% of cases; half the cases were related to either the developmentof lymphoma or of severe neurological features. Nearly all the patientspresenting with severe polyarthritis were finally diagnosed withassociated rheumatoid arthritis.