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001-es BibID:	BIBFORM083561
035-os BibID:	(WOS)000488952900016 (Scopus)85071621125
Első szerző:	Retamozo, Soledad
Cím:	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 / S. Retamozo, N. Acar-Denizli, A. Rasmussen, I. F. Horváth, C. Baldini, R. Priori, P. Sandhya, G. Hernandez-Molina, B. Armagan, S. Praprotnik, M. Kvarnstrom, R. Gerli, A. Sebastian, R. Solans, M. Rischmueller, S. G. Pasoto, V. Valim, G. Nordmark, A. A. Kruize, H. Nakamura, B. Hofauer, R. Giacomelli, V. Fernandes Moça Trevisani, V. Devauchelle-Pensec, F. Atzeni, T. A. Gheita, S. Consani-Fernández, A. Szántó, K. Sivils, A. Gattamelata, D. Danda, L. Kilic, E. Bartoloni, S. Bombardieri, J. Sánchez-Guerrero, M. Wahren-Herlenius, X. Mariette, M. Ramos-Casals, P. Brito-Zerón, Sjögren Big Data Consortium
Dátum:	2019
ISSN:	0392-856X
Megjegyzések:	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.
Tárgyszavak:	Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban folyóiratcikk primary Sjögren's syndrome ESSDAI Raynaud's phenomenon pleuritis pericarditis uveitis congenital heart block pulmonary arterial hypertension
Megjelenés:	Clinical and Experimental Rheumatology. - 37 Suppl. 118 : 3 (2019), p. 97-106. -
További szerzők:	Acar-Denizli, Nihan Rasmussen, Astrid Horváth Ildikó Fanny (1980-) (belgyógyász, allergológus, klinikai immunológus) Baldini, Chiara Priori, Roberta Sandhya, Pulukool Hernandez-Molina, Gabriela Armagan, Berkan Praprotnik, Sonja Kvarnstrom, Marika Gerli, Roberto Sebastian, Agata Solans, Roser Rischmueller, Maureen Pasoto, Sandra Valim, Valeria Nordmark, Gunnel Kruize, Aike A. Nakamura, Hideki Hofauer, Benedikt Giacomelli, Roberto Fernandes Moça Trevisani, Virginia Devauchelle-Pensec, Valerie Atzeni, F. Gheita, Tamer A. Consani-Fernández, S. Szántó Antónia (1977-) (belgyógyász, allergológus és klinikai immunológus) Sivils, Kathy Gattamelata, Angelica Danda, Debashish Kilic, Levent Bartoloni, Elena Bombardieri, Stefano Sanchez-Guerrero, Jorge Wahren-Herlenius, Marie Mariette, Xavier Ramos-Casals, Manuel Brito-Zerón, Pilar Sjögren Big Data Consortium
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