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Lookup NU author(s): Professor Fai Ng
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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 Sjögren's syndrome (pSS) fulfilling the 2002 classification criteria. METHODS: The Big Data Sjögren 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.
Author(s): Acar-Denizli N, Horvath I-F, Mandl T, Priori R, Vissink A, Hernandez-Molina G, Armagan B, Praprotnik S, Sebastian A, Bartoloni E, Rischmueller M, Pasoto SG, Nordmark G, Nakamura H, Fernandes Moca Trevisani V, Retamozo S, Carsons SE, Maure-Noia B, Sanchez-Berna I, Lopez-Dupla M, Fonseca-Aizpuru E, Melchor Diaz S, Vazquez M, Diaz Cuiza PE, de Miguel Campo B, Ng W-F, Rasmussen A, Dong X, Li X, Baldini C, Seror R, Gottenberg J-E, Kruize AA, Sandhya P, Gandolfo S, Kwok S-K, Kvarnstrom M, Solans R, Sene D, Suzuki Y, Isenberg DA, Valim V, Hofauer B, Giacomelli R, Devauchelle-Pensec V, Atzeni F, Gheita TA, Morel J, Izzo R, Kalyoncu U, Szanto A, Olsson P, Bootsma H, Ramos-Casals M, Kostov B, Brito-Zeron P
Publication type: Article
Publication status: Published
Journal: Clinical and Experimental Rheumatology
Online publication date: 03/09/2020
Acceptance date: 03/09/2020
ISSN (print): 0396-856X
ISSN (electronic): 1593-098X
Publisher: Pacini Editore SpA
PubMed id: 33095152