Systemic phenotype related to primary Sjögren's syndrome in 279 patients carrying isolated anti-La/SSB antibodies
(2020) In Clinical and Experimental Rheumatology 38 Suppl 126(4). p.85-94- Abstract
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... (More)
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.
(Less)
- author
- author collaboration
- organization
- publishing date
- 2020-10-24
- type
- Contribution to journal
- publication status
- published
- subject
- in
- Clinical and Experimental Rheumatology
- volume
- 38 Suppl 126
- issue
- 4
- pages
- 10 pages
- publisher
- Pacini
- external identifiers
-
- scopus:85094685088
- pmid:33095152
- ISSN
- 0392-856X
- language
- English
- LU publication?
- yes
- id
- 789838a3-8d2c-4995-8f51-3043f763e892
- alternative location
- https://pubmed.ncbi.nlm.nih.gov/33095152/
- date added to LUP
- 2020-10-24 13:29:09
- date last changed
- 2024-03-20 17:08:12
@article{789838a3-8d2c-4995-8f51-3043f763e892,
abstract = {{<p>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.</p><p>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.</p><p>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.</p><p>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.</p>}},
author = {{Acar-Denizli, Nihan and Horváth, Ildiko-Fanny and Mandl, Thomas and Priori, Roberta and Vissink, Arjan and Hernandez-Molina, Gabriela and Armagan, Berkan and Praprotnik, Sonja and Sebastian, Agata and Bartoloni, Elena and Rischmueller, Maureen and Pasoto, Sandra G and Nordmark, Gunnel and Nakamura, Hideki and Fernandes Moça Trevisani, Virginia and Retamozo, Soledad and Carsons, Steven E and Maure-Noia, Brenda and Sánchez-Berná, Isabel and López-Dupla, Miguel and Fonseca-Aizpuru, Eva and Melchor Díaz, Sheila and Vázquez, Marcos and Díaz Cuiza, P Ericka and de Miguel Campo, Borja and Ng, Wan-Fai and Rasmussen, Astrid and Dong, Xu and Li, Xiaomei and Baldini, Chiara and Seror, Raphaele and Gottenberg, Jacques-Eric and Kruize, Aike A and Sandhya, Pulukool and Gandolfo, Saviana and Kwok, Seung-Ki and Kvarnstrom, Marika and Solans, Roser and Sene, Damien and Suzuki, Yasunori and Isenberg, David A and Valim, Valeria and Hofauer, Benedikt and Giacomelli, Roberto and Devauchelle-Pensec, Valerie and Atzeni, Fabiola and Gheita, Tamer A and Morel, Jacques and Izzo, Raffaella and Olsson, Peter and Bootsma, Hendrika and Ramos-Casals, Manuel and Kostov, Belchin and Brito-Zerón, Pilar}},
issn = {{0392-856X}},
language = {{eng}},
month = {{10}},
number = {{4}},
pages = {{85--94}},
publisher = {{Pacini}},
series = {{Clinical and Experimental Rheumatology}},
title = {{Systemic phenotype related to primary Sjögren's syndrome in 279 patients carrying isolated anti-La/SSB antibodies}},
url = {{https://pubmed.ncbi.nlm.nih.gov/33095152/}},
volume = {{38 Suppl 126}},
year = {{2020}},
}