Systemic manifestations of primary Sjögren's syndrome out of the ESSDAI classification: prevalence and clinical relevance in a large international, multi-ethnic cohort of patients

Sjögren Big Data Consortium

Research output: Contribution to journalArticle

Abstract

OBJECTIVES: To analyse the frequency and characterise the systemic presentation of primary Sjögren's syndrome (SS) out of the ESSDAI classification in a large international, multi-ethnic cohort of patients. METHODS: The Big Data Sjögren Project Consortium is an international, multicentre registry based on world-wide data-sharing and cooperative merging of pre-existing clinical SS databases from leading centres in clinical research in SS from the five continents. A list of 26 organ-by-organ systemic features not currently included in the ESSDAI classification was defined according to previous studies; these features were retrospectively recorded. RESULTS: Information about non-ESSDAI features was available in 6331 patients [5,917 female, mean age at diagnosis 52 years, mainly White (86.3%)]. A total of 1641 (26%) patients had at least one of the ESSDAI systemic features. Cardiovascular manifestations were the most frequent organ-specific group of non-ESSDAI features reported in our patients (17% of the total cohort), with Raynaud's phenomenon being reported in 15%. Patients with systemic disease due to non-ESSDAI features had a lower frequency of dry mouth (90.7% vs. 94.1%, p<0.001) and positive minor salivary gland biopsy (86.7% vs. 89%, p=0.033), a higher frequency of anti-Ro/SSA (74.7% vs. 68.7%, p<0.001), anti-La/SSB antibodies (44.5% vs. 40.4%, p=0.004), ANA (82.7% vs. 79.5%, p=0.006), low C3 levels (17.4% vs. 9.7%, p<0.001), low C4 levels (14.4% vs. 9.6%, p<0.001), and positive serum cryoglobulins (8.6% vs. 5.5%, p=0.001). Systemic activity measured by the ESSDAI, clinESSDAI and DAS was higher in patients with systemic disease out of the ESSDAI in comparison with those without these features (p<0.001 for all comparisons). CONCLUSIONS: More than a quarter of patients with primary SS may have systemic manifestations not currently included in the ESSDAI classification, with a wide variety of cardiovascular, digestive, pulmonary, neurological, ocular, ENT (ear, nose, and throat), cutaneous and urological features that increase the scope of the systemic phenotype of the disease. However, the individual frequency of each of these non-ESSDAI features was very low, except for Raynaud's phenomenon.

Original languageEnglish
Pages (from-to)97-106
Number of pages10
JournalClinical and Experimental Rheumatology
Volume37
Issue number3
Publication statusPublished - May 1 2019

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Raynaud Disease
amsonic acid
Cryoglobulins
Minor Salivary Glands
Information Dissemination
Pharynx
Nose
Ear
Registries
Mouth
Databases
Phenotype
Biopsy
Lung
Skin
Antibodies
Serum
Research

ASJC Scopus subject areas

  • Rheumatology
  • Immunology and Allergy
  • Immunology

Cite this

@article{d15457124ebd402d91fd5673004f9127,
title = "Systemic manifestations of primary Sj{\"o}gren's syndrome out of the ESSDAI classification: prevalence and clinical relevance in a large international, multi-ethnic cohort of patients",
abstract = "OBJECTIVES: To analyse the frequency and characterise the systemic presentation of primary Sj{\"o}gren's syndrome (SS) out of the ESSDAI classification in a large international, multi-ethnic cohort of patients. METHODS: The Big Data Sj{\"o}gren Project Consortium is an international, multicentre registry based on world-wide data-sharing and cooperative merging of pre-existing clinical SS databases from leading centres in clinical research in SS from the five continents. A list of 26 organ-by-organ systemic features not currently included in the ESSDAI classification was defined according to previous studies; these features were retrospectively recorded. RESULTS: Information about non-ESSDAI features was available in 6331 patients [5,917 female, mean age at diagnosis 52 years, mainly White (86.3{\%})]. A total of 1641 (26{\%}) patients had at least one of the ESSDAI systemic features. Cardiovascular manifestations were the most frequent organ-specific group of non-ESSDAI features reported in our patients (17{\%} of the total cohort), with Raynaud's phenomenon being reported in 15{\%}. Patients with systemic disease due to non-ESSDAI features had a lower frequency of dry mouth (90.7{\%} vs. 94.1{\%}, p<0.001) and positive minor salivary gland biopsy (86.7{\%} vs. 89{\%}, p=0.033), a higher frequency of anti-Ro/SSA (74.7{\%} vs. 68.7{\%}, p<0.001), anti-La/SSB antibodies (44.5{\%} vs. 40.4{\%}, p=0.004), ANA (82.7{\%} vs. 79.5{\%}, p=0.006), low C3 levels (17.4{\%} vs. 9.7{\%}, p<0.001), low C4 levels (14.4{\%} vs. 9.6{\%}, p<0.001), and positive serum cryoglobulins (8.6{\%} vs. 5.5{\%}, p=0.001). Systemic activity measured by the ESSDAI, clinESSDAI and DAS was higher in patients with systemic disease out of the ESSDAI in comparison with those without these features (p<0.001 for all comparisons). CONCLUSIONS: More than a quarter of patients with primary SS may have systemic manifestations not currently included in the ESSDAI classification, with a wide variety of cardiovascular, digestive, pulmonary, neurological, ocular, ENT (ear, nose, and throat), cutaneous and urological features that increase the scope of the systemic phenotype of the disease. However, the individual frequency of each of these non-ESSDAI features was very low, except for Raynaud's phenomenon.",
author = "{Sj{\"o}gren Big Data Consortium} and Soledad Retamozo and Nihan Acar-Denizli and Astrid Rasmussen and Horv{\'a}th, {Ildik{\'o} Fanny} and Chiara Baldini and Roberta Priori and Pulukool Sandhya and Gabriela Hernandez-Molina and Berkan Armagan and Sonja Praprotnik and Marika Kvarnstrom and Roberto Gerli and Agata Sebastian and Roser Solans and Maureen Rischmueller and Pasoto, {Sandra G.} and Valeria Valim and Gunnel Nordmark and Aike Kruize and Hideki Nakamura and Benedikt Hofauer and Roberto Giacomelli and {Fernandes Mo{\cc}a Trevisani}, Virginia and Valerie Devauchelle-Pensec and Fabiola Atzeni and Gheita, {Tamer A.} and Sandra Consani-Fern{\'a}ndez and Antonia Sz{\'a}nt{\'o} and Kathy Sivils and Angelina Gattamelata and Debashish Danda and Levent Kilic and Elena Bartoloni and Stefano Bombardieri and Jorge S{\'a}nchez-Guerrero and Marie Wahren-Herlenius and Xavier Mariette and Manuel Ramos-Casals and Pilar Brito-Zer{\'o}n",
year = "2019",
month = "5",
day = "1",
language = "English",
volume = "37",
pages = "97--106",
journal = "Clinical and Experimental Rheumatology",
issn = "0392-856X",
publisher = "Clinical and Experimental Rheumatology S.A.S.",
number = "3",

}

TY - JOUR

T1 - Systemic manifestations of primary Sjögren's syndrome out of the ESSDAI classification

T2 - prevalence and clinical relevance in a large international, multi-ethnic cohort of patients

AU - Sjögren Big Data Consortium

AU - Retamozo, Soledad

AU - Acar-Denizli, Nihan

AU - Rasmussen, Astrid

AU - Horváth, Ildikó Fanny

AU - Baldini, Chiara

AU - Priori, Roberta

AU - Sandhya, Pulukool

AU - Hernandez-Molina, Gabriela

AU - Armagan, Berkan

AU - Praprotnik, Sonja

AU - Kvarnstrom, Marika

AU - Gerli, Roberto

AU - Sebastian, Agata

AU - Solans, Roser

AU - Rischmueller, Maureen

AU - Pasoto, Sandra G.

AU - Valim, Valeria

AU - Nordmark, Gunnel

AU - Kruize, Aike

AU - Nakamura, Hideki

AU - Hofauer, Benedikt

AU - Giacomelli, Roberto

AU - Fernandes Moça Trevisani, Virginia

AU - Devauchelle-Pensec, Valerie

AU - Atzeni, Fabiola

AU - Gheita, Tamer A.

AU - Consani-Fernández, Sandra

AU - Szántó, Antonia

AU - Sivils, Kathy

AU - Gattamelata, Angelina

AU - Danda, Debashish

AU - Kilic, Levent

AU - Bartoloni, Elena

AU - Bombardieri, Stefano

AU - Sánchez-Guerrero, Jorge

AU - Wahren-Herlenius, Marie

AU - Mariette, Xavier

AU - Ramos-Casals, Manuel

AU - Brito-Zerón, Pilar

PY - 2019/5/1

Y1 - 2019/5/1

N2 - OBJECTIVES: To analyse the frequency and characterise the systemic presentation of primary Sjögren's syndrome (SS) out of the ESSDAI classification in a large international, multi-ethnic cohort of patients. METHODS: The Big Data Sjögren Project Consortium is an international, multicentre registry based on world-wide data-sharing and cooperative merging of pre-existing clinical SS databases from leading centres in clinical research in SS from the five continents. A list of 26 organ-by-organ systemic features not currently included in the ESSDAI classification was defined according to previous studies; these features were retrospectively recorded. RESULTS: Information about non-ESSDAI features was available in 6331 patients [5,917 female, mean age at diagnosis 52 years, mainly White (86.3%)]. A total of 1641 (26%) patients had at least one of the ESSDAI systemic features. Cardiovascular manifestations were the most frequent organ-specific group of non-ESSDAI features reported in our patients (17% of the total cohort), with Raynaud's phenomenon being reported in 15%. Patients with systemic disease due to non-ESSDAI features had a lower frequency of dry mouth (90.7% vs. 94.1%, p<0.001) and positive minor salivary gland biopsy (86.7% vs. 89%, p=0.033), a higher frequency of anti-Ro/SSA (74.7% vs. 68.7%, p<0.001), anti-La/SSB antibodies (44.5% vs. 40.4%, p=0.004), ANA (82.7% vs. 79.5%, p=0.006), low C3 levels (17.4% vs. 9.7%, p<0.001), low C4 levels (14.4% vs. 9.6%, p<0.001), and positive serum cryoglobulins (8.6% vs. 5.5%, p=0.001). Systemic activity measured by the ESSDAI, clinESSDAI and DAS was higher in patients with systemic disease out of the ESSDAI in comparison with those without these features (p<0.001 for all comparisons). CONCLUSIONS: More than a quarter of patients with primary SS may have systemic manifestations not currently included in the ESSDAI classification, with a wide variety of cardiovascular, digestive, pulmonary, neurological, ocular, ENT (ear, nose, and throat), cutaneous and urological features that increase the scope of the systemic phenotype of the disease. However, the individual frequency of each of these non-ESSDAI features was very low, except for Raynaud's phenomenon.

AB - OBJECTIVES: To analyse the frequency and characterise the systemic presentation of primary Sjögren's syndrome (SS) out of the ESSDAI classification in a large international, multi-ethnic cohort of patients. METHODS: The Big Data Sjögren Project Consortium is an international, multicentre registry based on world-wide data-sharing and cooperative merging of pre-existing clinical SS databases from leading centres in clinical research in SS from the five continents. A list of 26 organ-by-organ systemic features not currently included in the ESSDAI classification was defined according to previous studies; these features were retrospectively recorded. RESULTS: Information about non-ESSDAI features was available in 6331 patients [5,917 female, mean age at diagnosis 52 years, mainly White (86.3%)]. A total of 1641 (26%) patients had at least one of the ESSDAI systemic features. Cardiovascular manifestations were the most frequent organ-specific group of non-ESSDAI features reported in our patients (17% of the total cohort), with Raynaud's phenomenon being reported in 15%. Patients with systemic disease due to non-ESSDAI features had a lower frequency of dry mouth (90.7% vs. 94.1%, p<0.001) and positive minor salivary gland biopsy (86.7% vs. 89%, p=0.033), a higher frequency of anti-Ro/SSA (74.7% vs. 68.7%, p<0.001), anti-La/SSB antibodies (44.5% vs. 40.4%, p=0.004), ANA (82.7% vs. 79.5%, p=0.006), low C3 levels (17.4% vs. 9.7%, p<0.001), low C4 levels (14.4% vs. 9.6%, p<0.001), and positive serum cryoglobulins (8.6% vs. 5.5%, p=0.001). Systemic activity measured by the ESSDAI, clinESSDAI and DAS was higher in patients with systemic disease out of the ESSDAI in comparison with those without these features (p<0.001 for all comparisons). CONCLUSIONS: More than a quarter of patients with primary SS may have systemic manifestations not currently included in the ESSDAI classification, with a wide variety of cardiovascular, digestive, pulmonary, neurological, ocular, ENT (ear, nose, and throat), cutaneous and urological features that increase the scope of the systemic phenotype of the disease. However, the individual frequency of each of these non-ESSDAI features was very low, except for Raynaud's phenomenon.

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VL - 37

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JO - Clinical and Experimental Rheumatology

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SN - 0392-856X

IS - 3

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