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Systemic phenotype related to primary Sjögren's syndrome in 279 patients carrying isolated anti-La/SSB antibodies

Acar-Denizli, Nihan ; Horváth, Ildiko-Fanny ; Mandl, Thomas LU ; Priori, Roberta ; Vissink, Arjan ; Hernandez-Molina, Gabriela ; Armagan, Berkan ; Praprotnik, Sonja ; Sebastian, Agata and Bartoloni, Elena , et al. (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.

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published
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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
2021-12-23 14:50:07
@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},
}