作者
Nihan Acar-Denizli,Ildiko-Fanny Horvath,Thomas Mandl,Roberta Priori,Arjan Vissink,Gabriela Hernández-Molina,Berkan Armagan,Sonja Praprotnik,Agata Sebastian,Elena Bartoloni,Maureen Rischmueller,Sandra Gofinet Pasoto,Gunnel Nordmark,Hideki Nakamura,Virginia Fernandes Moça Trevisani,Soledad Retamozo,Steven E. Carsons,Brenda Maure-Noia,Isabel Sánchez-Berná,Miguel López-Dupla,Eva Fonseca-Aizpuru,Sheila Melchor Díaz,Marcos Vázquez,P Ericka Díaz Cuiza,Borja de Miguel Campo,Wan-Fai Ng,Astrid Rasmussen,Xu Dong,Xiaomei Li,Chiara Baldini,Raphaèle Seror,Jacques-Eric Gottenberg,Aike A. Kruize,Pulukool Sandhya,Saviana Gandolfo,Seung-Ki Kwok,Marika Kvarnström,Roser Solans,Damien Sène,Yasunori Suzuki,David A. Isenberg,Valeria Valim,Benedikt Hofauer,Roberto Giacomelli,Valérie Devauchelle-Pensec,Fabiola Atzeni,Tamer A. Gheita,Jacques Morel,Raffaella Izzo,Umut Kalyoncu,Antónia Szántó,Peter Olsson,Hendrika Bootsma,Manuel Ramos-Casals,Belchin Kostov,Pilar Brito-Zerón
摘要
OBJECTIVES: To evaluate the systemic phenotype associated with the presence of isolated anti-La/SSB antibodies in a large international registry of patients with primary Sjogren's syndrome (pSS) fulfilling the 2002 classification criteria.METHODS: The Big Data Sjogren Project Consortium is an international, multicentre registry created in 2014. Baseline clinical information from leading centres on clinical research in SS of the 5 continents was collected. Combination patterns of anti-Ro/SSA-La/SSB antibodies at the time of diagnosis defined the following four immunological phenotypes: double positive (combined Ro/SSA and La/SSB,) isolated anti-Ro/SSA, isolated anti-La/SSB, and immunonegative.RESULTS: The cohort included 12,084 patients (11,293 females, mean 52.4 years) with recorded ESSDAI scores available. Among them, 279 (2.3%) had isolated anti-La/SSB antibodies. The mean total ESSDAI score at diagnosis of patients with pSS carrying isolated anti-La/SSB was 6.0, and 80.4% of patients had systemic activity (global ESSDAI score ≥1) at diagnosis. The domains with the highest frequency of active patients were the biological (42.8%), glandular (36.8%) and articular (31.2%) domains. Patients with isolated anti-La/SSB showed a higher frequency of active patients in all ESSDAI domains but two (articular and peripheral nerve) in comparison with immune-negative patients, and even a higher absolute frequency in six clinical ESSDAI domains in comparison with patients with isolated anti-Ro/SSA. In addition, patients with isolated anti-La/SSB showed a higher frequency of active patients in two ESSDAI domains (pulmonary and glandular) with respect to the most active immunological subset (double-positive antibodies). Meanwhile, systemic activity detected in patients with isolated anti-La/SSB was overwhelmingly low. Even in ESSDAI domains where patients with isolated anti-La/SSB had the highest frequencies of systemic activity (lymphadenopathy and muscular), the percentage of patients with moderate or high activity was lower in comparison with the combined Ro/SSA and La/SSB group.CONCLUSIONS: Patients carrying isolated La/SSB antibodies represent a very small subset of patients with a systemic SS phenotype characterised by a significant frequency of active patients in most clinical ESSDAI domains but with a relative low frequency of the highest severe organ-specific involvements. Primary SS still remains the best clinical diagnosis for this subset of patients. (Less)