How Different Systemic Organ Involvements Overlap In Patients with Primary Sjögren Syndrome: Analysis Using A Mathematical Model.

VALIM VALERIA; KOSTOV B; MANDL THOMAS; BALDINI CHIARA; DANDA D; ARMAGAN BERKAN; WAHREN-HERLENIUS MARIE; ELENA BARTOLONI; SUZUKI YASUNORI; GUNNEL NORDMARK; TAKASHI NAKAMURA; HOFAUER B; CARSONS SE; MOREL J; BRITO ZERON PILAR; HERNANDEZ-MOLINA GABRIELA; PASOTO SG; SIVILS KATHY; LI X; GOTTENBERG JE; QUARTUCCIO LUCA; KWOK SK; SENE D; SOLANS ROSER; PIOTR WILAND; HENDRIKA BOOTSMA; RETAMOZO SOLEDAD; DEVAUCHELLE-PENSEC V; FERNANDES MOÇA TREVISANI V; HAMMENFORS DANIEL; ZEHER MARGIT; ATZENI FABIOLA; SEROR R; RAMOS CASALS MANUEL; MARIETTE XAVIER; PRIORI ROBERTA; KRUIZE AA; PRAPROTNIK SONJA; RISCHMUELLER M; ISENBERG DAVID; GUADALUPE FRAILE; GIACOMELLI ROBERTO; BOMBARDIERI MICHELLE; GHEITA TA; VOLLENVEIDER, CRISTINA

Buenos Aires

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

PANLAR

Background and Objectives: To examine the degree of overlap of the differentsystemic organ involved in patients with primary Sjögren syndrome (SjS) ina large international cohort using a mathematical model.Methods: We have quantified at diagnosis systemic involvement (defined accordingto the 10 domains of the ESSDAI score) in patients included in the BigData Sjögren Registry. We quantified how many patients had activity in morethan one domain, how many patients had involvement of an isolated domainand we measured the degree of overlap between the main pairs and triplets ofassociated groups of active domains. Using a mathematical model, we representedthe overlap between groups by means of Venn diagrams.Results: 9118 patients were included in the Registry with ESSDAI valuesavailable at the time at diagnosis: 5758 (63%) had activity in at least oneESSDAI domain. Among them, 3167 (55%) had only one active domain,1532 (27%) two, 628 (11%) three and 431 (7%) four or more active domains.Three domains showed activity at diagnosis in at least 10% of patients: articular(37.9%), glandular (21.4%) and pulmonary (10.4%). The frequency ofpatients having concomitant activity in other organs varied widely in eachdomain: among patients with articular involvement, 54% of cases had anotherdomain active, a figure that was 68% for patients with glandular involvement;88% of patients with muscular involvement had concomitantactivity in other organs, 78% of those with peripheral neuropathy and 74%of those with renal involvement. Numerically, the most frequent associationsamong two different domains were between articular and glandular (n=944),pulmonary (n=487), constitutional (n=464) or cutaneous (n=410) domains,while the most frequent associations among 3 organs were between articular/glandular plus pulmonary (n=224), constitutional (n=216) or lymphadenopathy(n=173) domains. Mathematical model identified the highestdegree of overlap among the different domains for the following pairs: %of patients with muscular activity who had concomitant glandular activity(Δ30.2% with respect to the expected frequency of glandular activity), thosewith lymphadenopathy who had concomitant glandular activity (Δ22.9%),and those with constitutional who had concomitant articular (Δ21.6%) orglandular (Δ21.1%) activities.Conclusions: Primary Sjögren syndrome is presenting at diagnosis as a systemicdisease in two thirds of cases; in more than half the cases, there was onlyone active domain, although 7% of patients presented with a multisystemicdisease affecting 4 or more different organs. An enhanced degree of overlapin comparison with the expected values found for the total cohort was identifiedin the concomitant activity for the clusters glandular-lymphadenopathymuscularand constitutional-articular-glandular