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001-es BibID:BIBFORM091875
035-os BibID:(WOS)000581183900014 (Scopus)85094685088
Első szerző:Acar-Denizli, Nihan
Cím:Systemic phenotype related to primary Sjögren's syndrome in 279 patients carrying isolated anti-La/SSB antibodies / N. Acar-Denizli, I. F. Horváth, T. Mandl, R. Priori, A. Vissink, G. Hernandez-Molina, B. Armagan, S. Praprotnik, A. Sebastian, E. Bartoloni, M. Rischmueller, S. G. Pasoto, G. Nordmark, H. Nakamura, V. Fernandes Moça Trevisani, S. Retamozo, S. E. Carsons, B. Maure-Noia, I. Sánchez-Berná, M. López-Dupla, E. Fonseca-Aizpuru, S. Melchor Díaz, M. Vázquez, P. E. Díaz Cuiza, B. de Miguel Campo, W. F. Ng, A. Rasmussen, X. Dong, X. Li, C. Baldini, R. Seror, Jacques-Eric Gottenberg, A. A. Kruize, P. Sandhya, S. Gandolfo, Seung-Ki Kwok, M. Kvarnstrom, R. Solans, D. Sene, Y. Suzuki, D. A. Isenberg, V. Valim, B. Hofauer, R. Giacomelli, V. Devauchelle-Pensec, F. Atzeni, T. A. Gheita, J. Morel, R. Izzo, U. Kalyoncu, A. Szántó, P. Olsson, H. Bootsma, M. Ramos-Casals, B. Kostov, P. Brito-Zerón, Sjögren Big Data Consortium
Dátum:2020
ISSN:0392-856X
Megjegyzések: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.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
primary Sjögren's syndrome
isolated La/SSB autoantibodies
anti-Ro/SSA antibodies
systemic disease
ESSDAI
big data
Megjelenés:Clinical and Experimental Rheumatology. - 38 : 4 (2020), p. 85-94. -
További szerzők:Horváth Ildikó Fanny (1980-) (belgyógyász, allergológus, klinikai immunológus) Mandl, Thomas Priori, Roberta Vissink, Arjan Hernandez-Molina, Gabriela Armagan, Berkan Praprotnik, Sonja Sebastian, Agata Bartoloni, Elena Rischmueller, Maureen Pasoto, Sandra Nordmark, Gunnel Nakamura, Hideki Fernandes Moça Trevisani, Virginia Retamozo, Soledad Carsons, Steven E. Maure-Noia, B. Sánchez-Berná, I. López-Dupla, Miguel Fonseca-Aizpuru, Eva Melchor Díaz, S. Vázquez, Marta Díaz Cuiza, P. E. Miguel Campo, B. de Ng, Wan Fai Rasmussen, Astrid Dong, X. Li, X. Baldini, Chiara Seror, Raphaele Gottenberg, Jacques-Eric Kruize, Aike A. Sandhya, Pulukool Gandolfo, Saviana Kwok, Seung-Ki Kvarnstrom, Marika Solans, Roser Sene, Damien Suzuki, Yasunori Isenberg, David A. Valim, Valeria Hofauer, Benedikt Giacomelli, Roberto Devauchelle-Pensec, Valerie Atzeni, F. Gheita, Tamer A. Morel, Jacques Izzo, R. Kalyoncu, U. Szántó Antónia (1977-) (belgyógyász, allergológus és klinikai immunológus) Olsson, Peter Bootsma, Hendrika Ramos-Casals, Manuel Kostov, Belchin Brito-Zerón, Pilar Sjögren Big Data Consortium
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2.

001-es BibID:BIBFORM120110
035-os BibID:(WoS)001134013500010 (Scopus)85181176271
Első szerző:Brito-Zerón, Pilar
Cím:Exposure to air pollution as an environmental determinant of how Sjögren's disease is expressed at diagnosis / Brito-Zerón Pilar, Flores-Chávez Alejandra, Ng Wan-Fai, Fanny Horváth Ildiko, Rasmussen Astrid, Priori Roberta, Baldini Chiara, Armagan Berkan, Özkiziltas Burcugül, Praprotnik Sonja, Suzuki Yasunori, Quartuccio Luca, Hernandez-Molina Gabriela, Abacar Kerem, Bartoloni Elena, Rischmueller Maureen, Reis-de Oliveira Fabiola, Fernandes Moca Trevisani Virginia, Jurcut Ciprian, Fugmann Cecilia, Carubbi Francesco, Hofauer Benedikt, Valim Valeria, Pasoto Sandra G., Retamozo Soledad, Atzeni Fabiola, Fonseca-Aizpuru Eva, López-Dupla Miguel, Giacomelli Roberto, Nakamura Hideki, Akasbi Miriam, Thompson Kyle, Szántó Antónia, Farris A. Darise, Villa Martina, Bombardieri Stefano, Kilic Levent, Tufan Abdurrahman, Perdan Pirkmajer Katja, Fujisawa Yuhei, de Vita Salvatore, Inanc Nevsun, Ramos-Casals Manuel, Sjögren Big Data Consortium
Dátum:2023
ISSN:0392-856X 1593-098X
Megjegyzések:Objectives: To analyse how the potential exposure to air pollutants can influence the key components at the time of diagnosis of Sjögren's phenotype (epidemiological profile, sicca symptoms, and systemic disease). Methods: For the present study, the following variables were selected for harmonization and refinement: age, sex, country, fulfilment of 2002/2016 criteria items, dry eyes, dry mouth, and overall ESSDAI score. Air pollution indexes per country were defined according to the OECD (1990-2021), including emission data of nitrogen and sulphur oxides (NO/SO), particulate matter (PM2.5 and 1.0), carbon monoxide (CO) and volatile organic compounds (VOC) calculated per unit of GDP, Kg per 1000 USD. Results: The results of the chi-square tests of independence for each air pollutant with the frequency of dry eyes at diagnosis showed that, except for one, all variables exhibited p-values <0.0001. The most pronounced disparities emerged in the dry eye prevalence among individuals inhabiting countries with the highest NO/SO exposure, a surge of 4.61 percentage points compared to other countries, followed by CO (3.59 points), non-methane (3.32 points), PM2.5 (3.30 points), and PM1.0 (1.60 points) exposures. Concerning dry mouth, individuals residing in countries with worse NO/SO exposures exhibited a heightened frequency of dry mouth by 2.05 percentage points (p<0.0001), followed by non-methane exposure (1.21 percentage points increase, p=0.007). Individuals inhabiting countries with the worst NO/SO, CO, and PM2.5 pollution levels had a higher mean global ESSDAI score than those in lower-risk nations (all p-values <0.0001). When systemic disease was stratified according to DAS into low, moderate, and high systemic activity levels, a heightened proportion of individuals manifesting moderate/severe systemic activity was observed in countries with worse exposures to NO/SO, CO, and PM2.5 pollutant levels. Conclusions: For the first time, we suggest that pollution levels could influence how SjD appears at diagnosis in a large international cohort of patients. The most notable relationships were found between symptoms (dryness and general body symptoms) and NO/SO, CO, and PM2.5 levels.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
Sjögren's syndrome
dryness
systemic
ESSDAI
air pollution
environment
Megjelenés:Clinical And Experimental Rheumatology. - 41 : 12 (2023), p. 2448-2457. -
További szerzők:Flores-Chávez, Alejandra Ng, Wan Fai Horváth Ildikó Fanny (1980-) (belgyógyász, allergológus, klinikai immunológus) Rasmussen, Astrid Priori, Roberta Baldini, Chiara Armagan, Berkan Özkiziltaș, Burcugül Praprotnik, Sonja Suzuki, Yasunori Quartuccio, Luca Hernandez-Molina, Gabriela Abacar, Kerem Bartoloni, Elena Rischmueller, Maureen Reis-de Oliveira, Fabiola Fernandes Moça Trevisani, Virginia Jurcut, Ciprian Fugmann, Cecilia Carubbi, Francesco Hofauer, Benedikt Valim, Valeria Pasoto, Sandra Retamozo, Soledad Atzeni, Fabiola Fonseca-Aizpuru, Eva López-Dupla, Miguel Giacomelli, Roberto Nakamura, Hideki Akasbi, Miriam Thompson, Kyle Szántó Antónia (1977-) (belgyógyász, allergológus és klinikai immunológus) Farris, Darise Villa, Martina Bombardieri, Stefano Kilic, Levent Tufan, Abdurrahman Perdan Pirkmajer, Katja Fujisawa, Yuhei Vita, Salvatore de Inanc, Nevsun Ramos-Casals, Manuel Sjögren Big Data Consortium
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3.

001-es BibID:BIBFORM100740
035-os BibID:(WOS)000671076800063 (Scopus)85108742062
Első szerző:Brito-Zerón, Pilar
Cím:SARS-CoV-2 infection in patients with primary Sjögren syndrome : characterization and outcomes of 51 patients / Brito-Zerón Pilar, Melchor Sheila, Seror Raphaele, Priori Roberta, Solans Roser, Kostov Belchin, Baldini Chiara, Carubbi Francesco, Callejas Jose Luis, Guisado-Vasco Pablo, Hernández-Molina Gabriela, Pasoto Sandra G., Valim Valeria, Sisó-Almirall Antoni, Mariette Xavier, Carreira Patricia, Ramos-Casals Manuel, Sjögren Big Data Consortium, EULAR-SS Task Force Big Data Consortium
Dátum:2021
ISSN:1462-0324 1462-0332
Megjegyzések:Objective: To analyse the prognosis and outcomes of SARS-CoV-2 infection in patients with primary SS. Methods: We searched for patients with primary SS presenting with SARS-CoV-2 infection (defined following and according to the European Centre for Disease Prevention and Control guidelines) among those included in the Big Data Sjögren Registry, an international, multicentre registry of patients diagnosed according to the 2002/2016 classification criteria. Results: A total of 51 patients were included in the study (46 women, mean age at diagnosis of infection of 60 years). According to the number of patients with primary SS evaluated in the Registry (n = 8211), the estimated frequency of SARS-CoV-2 infection was 0.62% (95% CI 0.44, 0.80). All but two presented with symptoms suggestive of COVID-19, including fever (82%), cough (57%), dyspnoea (39%), fatigue/myalgias (27%) and diarrhoea (24%), and the most frequent abnormalities included raised lactate dehydrogenase (LDH) (88%), CRP (81%) and D-dimer (82%) values, and lymphopenia (70%). Infection was managed at home in 26 (51%) cases and 25 (49%) required hospitalization (five required admission to ICU, four died). Compared with patients managed at home, those requiring hospitalization had higher odds of having lymphopenia as laboratory abnormality (adjusted OR 21.22, 95% CI 2.39, 524.09). Patients with comorbidities had an older age (adjusted OR 1.05, 95% CI 1.00, 1.11) and showed a risk for hospital admission six times higher than those without (adjusted OR 6.01, 95% CI 1.72, 23.51) in the multivariate analysis. Conclusion: Baseline comorbidities were a key risk factor for a more complicated COVID-19 in patients with primary SS, with higher rates of hospitalization and poor outcomes in comparison with patients without comorbidities.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
Primary SS
COVID-19
SARS-Cov-2
comorbidities
outcomes
Megjelenés:Rheumatology. - 60 : 6 (2021), p. 2946-2957. -
További szerzők:Melchor, Sheila Seror, Raphaele Priori, Roberta Solans, Roser Kostov, Belchin Baldini, Chiara Carubbi, Francesco Callejas, J. L. Guisado-Vasco, Pablo Hernandez-Molina, Gabriela Pasoto, Sandra Valim, Valeria Sisó-Almirall, Antoni Mariette, Xavier Carreira, Patricia E. Ramos-Casals, Manuel Horváth Ildikó Fanny (1980-) (belgyógyász, allergológus, klinikai immunológus) Szántó Antónia (1977-) (belgyógyász, allergológus és klinikai immunológus) Tarr Tünde (1976-) (belgyógyász, allergológus és klinikai immunológus) Sjögren Big Data Consortium EULAR-SS Task Force Big Data Consortium
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4.

001-es BibID:BIBFORM083560
035-os BibID:(WOS)000574394600030 (Scopus)85078897832
Első szerző:Brito-Zerón, Pilar
Cím:Epidemiological profile and north-south gradient driving baseline systemic involvement of primary Sjögren's syndrome / Pilar Brito-Zerón, Nihan Acar-Denizli, Wan-Fai Ng, Ildiko Fanny Horváth, Astrid Rasmussen, Raphaele Seror, Xiaomei Li, Chiara Baldini, Jacques-Eric Gottenberg, Debashish Danda, Luca Quartuccio, Roberta Priori, Gabriela Hernandez-Molina, Berkan Armagan, Aike A. Kruize, Seung-Ki Kwok, Marika Kvarnstrom, Sonja Praprotnik, Damien Sene, Roberto Gerli, Roser Solans, Maureen Rischmueller, Thomas Mandl, Yasunori Suzuki, David Isenberg, Valeria Valim, Piotr Wiland, Gunnel Nordmark, Guadalupe Fraile, Hendrika Bootsma, Hideki Nakamura, Roberto Giacomelli, Valerie Devauchelle-Pensec, Benedikt Hofauer, Michele Bombardieri, Virginia Fernandes Moça Trevisani, Daniel Hammenfors, Sandra G. Pasoto, Soledad Retamozo, Tamer A. Gheita, Fabiola Atzeni, Jacques Morel, Cristina Vollenweider, Margit Zeher, Kathy Sivils, Bei Xu, Stefano Bombardieri, Pulukool Sandhya, Salvatore De Vita, Antonina Minniti, Jorge Sánchez-Guerrero, Levent Kilic, Eefje van der Heijden, Sung-Hwan Park, Marie Wahren-Herlenius, Xavier Mariette, Manuel Ramos-Casals, Sjögren Big Data Consortium
Dátum:2020
ISSN:1462-0324 1462-0332
Megjegyzések:OBJECTIVE: To characterize the systemic phenotype of primary Sjögren's syndrome at diagnosis by analysing the EULAR-SS disease activity index (ESSDAI) scores. METHODS: The Sjögren Big Data Consortium is an international, multicentre registry based on worldwide data-sharing cooperative merging of pre-existing databases from leading centres in clinical research in Sjögren's syndrome from the five continents. RESULTS: The cohort included 10 007 patients (9352 female, mean 53?years) with recorded ESSDAI scores available. At diagnosis, the mean total ESSDAI score was 6.1; 81.8% of patients had systemic activity (ESSDAI score ?1). Males had a higher mean ESSDAI (8.1 vs 6.0, P?<?0.001) compared with females, as did patients diagnosed at <35?years (6.7 vs 5.6 in patients diagnosed at >65?years, P?<?0.001). The highest global ESSDAI score was reported in Black/African Americans, followed by White, Asian and Hispanic patients (6.7, 6.5, 5.4 and 4.8, respectively; P?<?0.001). The frequency of involvement of each systemic organ also differed between ethnic groups, with Black/African American patients showing the highest frequencies in the lymphadenopathy, articular, peripheral nervous system, CNS and biological domains, White patients in the glandular, cutaneous and muscular domains, Asian patients in the pulmonary, renal and haematological domains and Hispanic patients in the constitutional domain. Systemic activity measured by the ESSDAI, clinical ESSDAI (clinESSDAI) and disease activity states was higher in patients from southern countries (P?<?0.001). CONCLUSION: The systemic phenotype of primary Sjögren's syndrome is strongly influenced by personal determinants such as age, gender, ethnicity and place of residence, which are key geoepidemiological players in driving the expression of systemic disease at diagnosis.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
ethnicity
gender
geoepidemiology
phenotype
primary Sjögren's syndrome
Megjelenés:Rheumatology. - 59 : 9 (2020), p. 2350-2359. -
További szerzők:Acar-Denizli, Nihan Ng, Wan Fai Horváth Ildikó Fanny (1980-) (belgyógyász, allergológus, klinikai immunológus) Rasmussen, Astrid Seror, Raphaele Li, Xiaomei Baldini, Chiara Gottenberg, Jacques-Eric Danda, Debashish Quartuccio, Luca Priori, Roberta Hernandez-Molina, Gabriela Armagan, Berkan Kruize, Aike A. Kwok, Seung-Ki Kvarnstrom, Marika Praprotnik, Sonja Sene, Damien Gerli, Roberto Solans, Roser Rischmueller, Maureen Mandl, Thomas Suzuki, Yasunori Isenberg, David A. Valim, Valeria Wiland, Piotr Nordmark, Gunnel Fraile, Guadalupe Bootsma, Hendrika Nakamura, Hideki Giacomelli, Roberto Devauchelle-Pensec, Valerie Hofauer, Benedikt Bombardieri, Michele Trevisani, Virginia Fernandes Moça Hammenfors, Daniel Pasoto, Sandra Retamozo, Soledad Gheita, Tamer A. Atzeni, Fabiola Morel, Jacques Vollenveider, Cristina Zeher Margit (1957-2018) (belgyógyász, allergológus és klinikai immunológus, reumatológus) Sivils, Kathy Xu, Bei Bombardieri, Stefano Sandhya, Pulukool Vita, Salvatore de Minniti, Antonina Sanchez-Guerrero, Jorge Kilic, Levent Heijden, Eefje van der Park, Sung-Hwan Wahren-Herlenius, Marie Mariette, Xavier Ramos-Casals, Manuel Sjögren Big Data Consortium
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5.

001-es BibID:BIBFORM074743
035-os BibID:(WOS)000446486100015 (Scopus)85055613559
Első szerző:Brito-Zerón, Pilar
Cím:How immunological profile drives clinical phenotype of primary Sjögren's syndrome at diagnosis : analysis of 10,500 patients (Sjögren Big Data Project) / P. Brito-Zerón, N. Acar-Denizli, W. F. Ng, M. Zeher, A. Rasmussen, T. Mandl, R. Seror, X. Li, C. Baldini, Jacques-Eric Gottenberg, D. Danda, L. Quartuccio, R. Priori, G. Hernandez-Molina, B. Armagan, A. A. Kruize, Seung-Ki Kwok, M. Kvarnström, S. Praprotnik, D. Sène, E. Bartoloni, R. Solans, M. Rischmueller, Y. Suzuki, D. A. Isenberg, V. Valim, P. Wiland, G. Nordmark, G. Fraile, H. Bootsma, T. Nakamura, R. Giacomelli, V. Devauchelle-Pensec, A. Knopf, M. Bombardieri, V. Trevisani, D. Hammenfors, S. Pasoto, S. Retamozo, T. A. Gheita, F. Atzeni, J. Morel, C. Vollenveider, I. Horvath, K. Sivils, P. Olsson, S. De Vita, J. Sánchez-Guerrero, L. Kilic, M. Wahren-Herlenius, X. Mariette, M. Ramos-Casals, Sjögren Big Data Consortium
Dátum:2018
ISSN:0392-856X
Megjegyzések:OBJECTIVES:To evaluate the influence of the main immunological markers on the disease phenotype at diagnosis in a large international cohort of patients with primary Sjögren's syndrome (SjS). METHODS:The Big Data Sjögren Project Consortium is an international, multicentre registry created in 2014. As a first step, baseline clinical information from leading centres on clinical research in SjS of the 5 continents was collected. The centres shared a harmonised data architecture and conducted cooperative online efforts in order to refine collected data under the coordination of a big data statistical team. Inclusion criteria were the fulfillment of the 2002 classification criteria. Immunological tests were carried out using standard commercial assays. RESULTS:By January 2018, the participant centres had included 10,500 valid patients from 22 countries. The cohort included 9,806 (93%) women and 694 (7%) men, with a mean age at diagnosis of primary SjS of 53 years, mainly White (78%) and included from European countries (71%). The frequency of positive immunological markers at diagnosis was 79.3% for ANA, 73.2% for anti-Ro, 48.6% for RF, 45.1% for anti- La, 13.4% for low C3 levels, 14.5% for low C4 levels and 7.3% for cryoglobulins. Positive autoantibodies (ANA, Ro, La) correlated with a positive result in salivary gland biopsy, while hypocomplementaemia and especially cryoglo-bulinaemia correlated with systemic activity (mean ESSDAI score of 17.7 for cryoglobulins, 11.3 for low C3 and 9.2 for low C4, in comparison with 3.8 for negative markers). The immunological markers with a great number of statistically-significant associations (p<0.001) in the organ-by-organ ESS- DAI evaluation were cryoglobulins (9 domains), low C3 (8 domains), anti-La (7 domains) and low C4 (6 domains). CONCLUSIONS:We confirm the strong influence of immunological markers on the phenotype of primary SjS at diagnosis in the largest multi-ethnic international cohort ever analysed, with a greater influence for cryoglobulinaemic-related markers in comparison with Ro/La autoantibodies and ANA. Immunological patterns play a central role in the phenotypic expression of the disease already at the time of diagnosis, and may guide physicians to design a specific personalised management during the follow-up of patients with primary SjS.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
primary Sjögren's syndrome
salivary gland biopsy
Ro/La autoantibodies
hypocomplementaemia
cryoglobulinaemia
ESSDAI
Megjelenés:Clinical and Experimental Rheumatology. - 36 : Suppl. 112 (2018), p. S102-S112. -
További szerzők:Acar-Denizli, Nihan Ng, Wan Fai Zeher Margit (1957-2018) (belgyógyász, allergológus és klinikai immunológus, reumatológus) Rasmussen, Astrid Mandl, Thomas Seror, Raphaele Li, X. Baldini, Chiara Gottenberg, Jacques-Eric Danda, Debashish Quartuccio, Luca Priori, Roberta Hernandez-Molina, Gabriela Armagan, Berkan Kruize, Aike A. Kwok, Seung-Ki Kvarnstrom, Marika Praprotnik, Sonja Sene, Damien Bartoloni, Elena Solans, Roser Rischmueller, Maureen Suzuki, Yasunori Isenberg, David A. Valim, Valeria Wiland, Piotr Nordmark, Gunnel Fraile, Guadalupe Bootsma, Hendrika Nakamura, T. Giacomelli, Roberto Devauchelle-Pensec, Valerie Knopf, A. Bombardieri, Michele Trevisani, Virginia Fernandes Moça Hammenfors, Daniel Pasoto, Sandra Retamozo, Soledad Gheita, Tamer A. Atzeni, F. Morel, Jacques Vollenveider, Cristina Horváth Ildikó Fanny (1980-) (belgyógyász, allergológus, klinikai immunológus) Sivils, Kathy Olsson, Peter Vita, Salvatore de Sanchez-Guerrero, Jorge Kilic, Levent Wahren-Herlenius, Marie Mariette, Xavier Ramos-Casals, Manuel Sjögren Big Data Consortium
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6.

001-es BibID:BIBFORM069228
035-os BibID:(WoS)000401138800017 (Scopus)85006013155
Első szerző:Brito-Zerón, Pilar
Cím:Influence of geolocation and ethnicity on the phenotypic expression of primary Sjögren's syndrome at diagnosis in 8310 patients : a cross-sectional study from the Big Data Sjögren Project Consortium / Pilar Brito-Zerón, Nihan Acar-Denizli, Margit Zeher, Astrid Rasmussen, Raphaele Seror, Elke Theander, Xiaomei Li, Chiara Baldini, Jacques-Eric Gottenberg, Debashish Danda, Luca Quartuccio, Roberta Priori, Gabriela Hernandez-Molina, Aike A. Kruize, Valeria Valim, Marika Kvarnstrom, Damien Sene, Roberto Gerli, Sonja Praprotnik, David Isenberg, Roser Solans, Maureen Rischmueller, Seung-Ki Kwok, Gunnel Nordmark, Yasunori Suzuki, Roberto Giacomelli, Valerie Devauchelle-Pensec, Michele Bombardieri, Benedikt Hofauer, Hendrika Bootsma, Johan G. Brun, Guadalupe Fraile, Steven E. Carsons, Tamer A. Gheita, Jacques Morel, Cristina Vollenveider, Fabiola Atzeni, Soledad Retamozo, Ildiko Fanny Horvath, Kathy Sivils, Thomas Mandl, Pulukool Sandhya, Salvatore De Vita, Jorge Sanchez-Guerrero, Eefje van der Heijden, Virginia Fernandes Moça Trevisani, Marie Wahren-Herlenius, Xavier Mariette, Manuel Ramos-Casals, EULAR-SS Task Force Big Data Consortium
Dátum:2017
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
Megjelenés:Annals of the rheumatic diseases. - 76 : 6 (2017), p. 1042-1050. -
További szerzők:Acar-Denizli, Nihan Zeher Margit (1957-2018) (belgyógyász, allergológus és klinikai immunológus, reumatológus) Rasmussen, Astrid Seror, Raphaele Theander, Elke Li, Xiaomei Baldini, Chiara Gottenberg, Jacques-Eric Danda, Debashish Quartuccio, Luca Priori, Roberta Hernandez-Molina, Gabriela Kruize, Aike A. Valim, Valeria Kvarnstrom, Marika Sene, Damien Gerli, Roberto Praprotnik, Sonja Isenberg, David A. Solans, Roser Rischmueller, Maureen Kwok, Seung-Ki Nordmark, Gunnel Suzuki, Yasunori Giacomelli, Roberto Devauchelle-Pensec, Valerie Bombardieri, Michele Hofauer, Benedikt Bootsma, Hendrika Brun, Johan G. Fraile, Guadalupe Carsons, Steven E. Gheita, Tamer A. Morel, Jacques Vollenveider, Cristina Atzeni, Fabiola Retamozo, Soledad Horváth Ildikó Fanny (1980-) (belgyógyász, allergológus, klinikai immunológus) Sivils, Kathy Mandl, Thomas Sandhya, Pulukool Vita, Salvatore de Sanchez-Guerrero, Jorge Heijden, Eefje van der Fernandes Moça Trevisani, Virginia Wahren-Herlenius, Marie Mariette, Xavier Ramos-Casals, Manuel EULAR-SS Task Force Big Data Consortium
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7.

001-es BibID:BIBFORM119113
035-os BibID:(scopus)85181178127 (WoS)001134013500012
Első szerző:Flores-Chávez, Alejandra
Cím:Influence of exposure to climate-related hazards in the phenotypic expression of primary Sjögren's syndrome / Flores-Chávez Alejandra, Brito-Zerón Pilar, Ng Wan-Fai, Szántó Antónia, Rasmussen Astrid, Priori Roberta, Baldini Chiara, Armagan Berkan, Özkiziltas Burcugül, Praprotnik Sonja, Suzuki Yasunori, Quartuccio Luca, Hernández-Molina Gabriela, Inanc Nevsun, Bartoloni Elena, Rischmueller Maureen, Reis-de Oliveira Fabiola, Fernandes Moca Trevisani Virginia, Jurcut Ciprian, Nordmark Gunnel, Carubbi Francesco, Hofauer Benedikt, Valim Valeria, Pasoto Sandra G., Retamozo Soledad, Atzeni Fabiola, Fonseca-Aizpuru Eva, López-Dupla Miguel, Giacomelli Roberto, Nakamura Hideki, Akasbi Miriam, Thompson Kyle, Fanny Horváth Ildiko, Farris A. Darise, Simoncelli Edoardo, Bombardieri Stefano, Kilic Levent, Tufan Abdurrahman, Perdan Pirkmajer Katja, Fujisawa Yuhei, De Vita Salvatore, Abacar Kerem, Ramos-Casals Manuel, Sjögren Big Data Consortium
Dátum:2023
ISSN:0392-856X 1593-098X
Megjegyzések:OBJECTIVES: To analyse how the key components at the time of diagnosis of the Sjögren's phenotype (epidemiological profile, sicca symptoms, and systemic disease) can be influenced by the potential exposure to climate-related natural hazards. METHODS: For the present study, the following variables were selected for harmonisation and refinement: age, sex, country, fulfilment of 2002/2016 criteria items, dry eyes, dry mouth, and overall ESSDAI score. Climate-related hazards per country were defined according to the OECD and included seven climate-related hazard types: extreme temperature, extreme precipitation, drought, wildfire, wind threats, river flooding, and coastal flooding. Climatic variables were defined as dichotomous variables according to whether each country is ranked among the ten countries with the most significant exposure. RESULTS: After applying data-cleaning techniques and excluding people from countries not included in the OECD climate rankings, the database study analysed 16,042 patients from 23 countries. The disease was diagnosed between 1 and 3 years earlier in people living in countries included among the top 10 worst exposed to extreme precipitation, wildfire, wind threats, river flooding, and coastal flooding. A lower frequency of dry eyes was observed in people living in countries exposed to wind threats, river flooding, and coastal flooding, with a level of statistical association being classified as strong (p<0.0001 for the three variables). The frequency of dry mouth was significantly lower in people living in countries exposed to river flooding (p<0.0001) and coastal flooding (p<0.0001). People living in countries included in the worse climate scenarios for extreme temperature (p<0.0001) and river flooding (p<0.0001) showed a higher mean ESSDAI score in comparison with people living in no-risk countries. In contrast, those living in countries exposed to worse climate scenarios for wind threats (p<0.0001) and coastal flooding (p<0.0001) showed a lower mean ESSDAI score in comparison with people living in no-risk countries. CONCLUSIONS: Local exposure to extreme climate-related hazards plays a role in modulating the presentation of Sjögren across countries concerning the age at which the disease is diagnosed, the frequency of dryness, and the degree of systemic activity.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
Sjögren's syndrome
dryness
systemic
ESSDAI
climate
epidemiology
Megjelenés:Clinical And Experimental Rheumatology. - 41 : 12 (2023), p. 2437-2447. -
További szerzők:Brito-Zerón, Pilar Ng, Wan Fai Szántó Antónia (1977-) (belgyógyász, allergológus és klinikai immunológus) Rasmussen, Astrid Priori, Roberta Baldini, Chiara Armagan, Berkan Özkiziltaș, Burcugül Praprotnik, Sonja Suzuki, Yasunori Quartuccio, Luca Hernandez-Molina, Gabriela Inanc, Nevsun Bartoloni, Elena Rischmueller, Maureen Reis-de Oliveira, Fabiola Fernandes Moça Trevisani, Virginia Jurcut, Ciprian Nordmark, Gunnel Carubbi, Francesco Hofauer, Benedikt Valim, Valeria Pasoto, Sandra Retamozo, Soledad Atzeni, Fabiola Fonseca-Aizpuru, Eva López-Dupla, Miguel Giacomelli, Roberto Nakamura, Hideki Akasbi, Miriam Thompson, Kyle Horváth Ildikó Fanny (1980-) (belgyógyász, allergológus, klinikai immunológus) Farris, Darise Simoncelli, Edoardo Bombardieri, Stefano Kilic, Levent Tufan, Abdurrahman Perdan Pirkmajer, Katja Fujisawa, Yuhei Vita, Salvatore de Abacar, Kerem Ramos-Casals, Manuel Sjögren Big Data Consortium
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8.

001-es BibID:BIBFORM104986
035-os BibID:(WOS)000786680100001 (Scopus)85139097101
Első szerző:Hernandez-Molina, Gabriela
Cím:Characterization and outcomes of 414 patients with primary SS who developed haematological malignancies / Hernández-Molina Gabriela, Kostov Belchin, Brito-Zerón Pilar, Vissink Arjan, Mandl Thomas, Hinrichs Anneline C., Quartuccio Luca, Baldini Chiara, Seror Raphaele, Szántó Antonia, Isenberg David, Gerli Roberto, Nordmark Gunnel, Rasmussen Astrid, Solans-Laque Roser, Hofauer Benedikt, Sene Damien, Pasoto Sandra G., Rischmueller Maureen, Praprotnik Sonja, Gheita Tamer A., Danda Debashish, Armagan Berkan, Suzuki Yasunori, Valim Valeria, Devauchelle-Pensec Valerie, Retamozo Soledad, Kvarnstrom Marika, Sebastian Agata, Atzeni Fabiola, Giacomelli Roberto, Carsons Steven E., Kwok Seung-Ki, Nakamura Hideki, Fernandes Moca Trevisani Virginia, Flores-Chávez Alejandra, Mariette Xavier, Ramos-Casals Manuel, Sjögren Big Data Consortium
Dátum:2023
ISSN:1462-0324 1462-0332
Megjegyzések:Abstract Objective To characterize 414 patients with primary SS who developed haematological malignancies and to analyse how the main SS- and lymphoma-related features can modify the presentation patterns and outcomes. Methods By January 2021, the Big Data Sjögren Project Consortium database included 11?966 patients fulfilling the 2002/2016 classification criteria. Haematological malignancies diagnosed according to the World Health Organization (WHO) classification were retrospectively identified. Results There were 414 patients (355 women, mean age 57?years) with haematological malignancies (in 43, malignancy preceded at least one year the SS diagnosis). A total of 376 (91%) patients had mature B-cell malignancy, nearly half had extranodal marginal zone lymphoma (MZL) of mucosa-associated lymphoid tissue (MALT lymphoma) (n?=?197), followed by diffuse large B-cell lymphoma (DLBCL) (n?=?67), nodal MZL lymphoma (n?=?29), chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) (n?=?19) and follicular lymphoma (FL) (n?=?17). Rates of complete response, relapses and death were 80%, 34% and 13%, respectively, with a 5-year survival rate of 86.5% after a mean follow-up of 8?years. There were significant differences in age at diagnosis (younger in MALT, older in CLL/SLL), predominant clinical presentation (glandular enlargement in MALT lymphoma, peripheral lymphadenopathy in nodal MZL and FL, constitutional symptoms in DLBCL, incidental diagnosis in CLL/SLL), therapeutic response (higher in MALT lymphoma, lower in DLBCL) and survival (better in MALT, nodal MZL and FL, worse in DLBCL). Conclusion In the largest reported study of haematological malignancies complicating primary SS, we confirm the overwhelming predominance of B-cell lymphomas, especially MALT, with the salivary glands being the primary site of involvement. This highly-specific histopathological scenario is linked with the overall good prognosis with a 5-year survival rate of nearly 90%.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
Sjögren's Syndrome
haematological malignancy
lymphoproliferative disease
lymphoma
MALT
Megjelenés:Rheumatology. - 62 : 1 (2023), p. 243-255. -
További szerzők:Kostov, Belchin Brito-Zerón, Pilar Vissink, Arjan Mandl, Thomas Hinrichs, Anneline Quartuccio, Luca Baldini, Chiara Seror, Raphaele Szántó Antónia (1977-) (belgyógyász, allergológus és klinikai immunológus) Isenberg, David A. Gerli, Roberto Nordmark, Gunnel Rasmussen, Astrid Soláns-Laqué, Roser Hofauer, Benedikt Sene, Damien Pasoto, Sandra Rischmueller, Maureen Praprotnik, Sonja Gheita, Tamer A. Danda, Debashish Armagan, Berkan Suzuki, Yasunori Valim, Valeria Devauchelle-Pensec, Valerie Retamozo, Soledad Kvarnstrom, Marika Sebastian, Agata Atzeni, Fabiola Giacomelli, Roberto Carsons, Steven E. Kwok, Seung-Ki Nakamura, Hideki Fernandes Moça Trevisani, Virginia Flores-Chávez, Alejandra Mariette, Xavier Ramos-Casals, Manuel Horváth Ildikó Fanny (1980-) (belgyógyász, allergológus, klinikai immunológus) Tarr Tünde (1976-) (belgyógyász, allergológus és klinikai immunológus) Sjögren Big Data Consortium
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9.

001-es BibID:BIBFORM100739
035-os BibID:(WOS)000753131900023 (Scopus)85113866014
Első szerző:Ramos-Casals, Manuel
Cím:Childhood-onset of primary Sjögren's syndrome : phenotypic characterization at diagnosis of 158 children / Ramos-Casals Manuel, Acar-Denizli Nihan, Vissink Arjan, Brito-Zerón Pilar, Li Xiaomei, Carubbi Francesco, Priori Roberta, Toplak Nataŝa, Baldini Chiara, Faugier-Fuentes Enrique, Kruize Aike A., Mandl Thomas, Tomiita Minako, Gandolfo Saviana, Hashimoto Kunio, Hernandez-Molina Gabriela, Hofauer Benedikt, Mendieta-Zerón Samara, Rasmussen Astrid, Sandhya Pulukool, Sene Damien, Trevisani Virginia Fernandes Moca, Isenberg David, Sundberg Erik, Pasoto Sandra G., Sebastian Agata, Suzuki Yasunori, Retamozo Soledad, Xu Bei, Giacomelli Roberto, Gattamelata Angelica, Bizjak Masa, Bombardieri Stefano, Loor-Chavez Richard-Eduardo, Hinrichs Anneline, Olsson Peter, Bootsma Hendrika, Lieberman Scott M., Sjogren Big Data Consortium
Dátum:2021
ISSN:1462-0324 1462-0332
Megjegyzések:Objectives: To characterize the phenotypic presentation at diagnosis of childhood-onset primary SS. Methods: The Big Data Sjögren Project Consortium is an international, multicentre registry using worldwide data-sharing cooperative merging of pre-existing clinical SS databases from the five continents. For this study, we selected those patients in whom the disease was diagnosed below the age of 19 years according to the fulfilment of the 2002/2016 classification criteria. Results: Among the 12 083 patients included in the Sjögren Big Data Registry, 158 (1.3%) patients had a childhood-onset diagnosis (136 girls, mean age of 14.2 years): 126 (80%) reported dry mouth, 111 (70%) dry eyes, 52 (33%) parotid enlargement, 118/122 (97%) positive minor salivary gland biopsy and 60/64 (94%) abnormal salivary US study, 140/155 (90%) positive ANA, 138/156 (89%) anti-Ro/La antibodies and 86/142 (68%) positive RF. The systemic EULAR Sjögren's syndrome disease activity index (ESSDAI) domains containing the highest frequencies of active patients included the glandular (47%), articular (26%) and lymphadenopathy (25%) domains. Patients with childhood-onset primary SS showed the highest mean ESSDAI score and the highest frequencies of systemic disease in 5 (constitutional, lymphadenopathy, glandular, cutaneous and haematological) of the 12 ESSDAI domains, and the lowest frequencies in 4 (articular, pulmonary, peripheral nerve and CNS) in comparison with patients with adult-onset disease. Conclusions: Childhood-onset primary SS involves around 1% of patients with primary SS, with a clinical phenotype dominated by sicca features, parotid enlargement and systemic disease. Age at diagnosis plays a key role in modulating the phenotypic expression of the disease.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
Sjogren's syndrome
autoimmune diseases
childhood
epidemiology
paediatrics
Megjelenés:Rheumatology. - 60 : 10 (2021), p. 4558-4567. -
További szerzők:Acar-Denizli, Nihan Vissink, Arjan Brito-Zerón, Pilar Li, Xiaomei Carubbi, Francesco Priori, Roberta Toplak, Nataŝa Baldini, Chiara Faugier-Fuentes, Enrique Kruize, Aike A. Mandl, Thomas Tomiita, Minako Gandolfo, Saviana Hashimoto, Kunio Hernandez-Molina, Gabriela Hofauer, Benedikt Mendieta-Zerón, Samara Rasmussen, Astrid Sandhya, Pulukool Sene, Damien Trevisani, Virginia Fernandes Moça Isenberg, David A. Sundberg, Erik Pasoto, Sandra Sebastian, Agata Suzuki, Yasunori Retamozo, Soledad Xu, Bei Giacomelli, Roberto Gattamelata, Angelica Bizjak, Masa Bombardieri, Stefano Loor-Chavez, Richard-Eduardo Hinrichs, Anneline Olsson, Peter Bootsma, Hendrika Lieberman, Scott M. Horváth Ildikó Fanny (1980-) (belgyógyász, allergológus, klinikai immunológus) Szántó Antónia (1977-) (belgyógyász, allergológus és klinikai immunológus) Sjögren Big Data Consortium
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10.

001-es BibID:BIBFORM100734
035-os BibID:(WOS)000731864300024 (Scopus)85122843096
Első szerző:Retamozo, Soledad
Cím:Influence of the age at diagnosis in the disease expression of primary Sjögren syndrome. Analysis of 12,753 patients from the Sjögren Big Data Consortium / S. Retamozo, N. Acar-Denizli, I. F. Horváth, W. F. Ng, A. Rasmussen, X. Dong, X. Li, C. Baldini, P. Olsson, R. Priori, R. Seror, Jacques-Eric Gottenberg, A. A. Kruize, G. Hernandez-Molina, A. Vissink, P. Sandhya, B. Armagan, L. Quartuccio, A. Sebastian, S. Praprotnik, E. Bartoloni, Seung-Ki Kwok, M. Kvarnstrom, M. Rischmueller, R. Soláns-Laqué, D. Sene, S. G. Pasoto, Y. Suzuki, D. A. Isenberg, V. Valim, G. Nordmark, H. Nakamura, V. Fernandes Moca Trevisani, B. Hofauer, A. Sisó-Almirall, R. Giacomelli, V. Devauchelle-Pensec, M. Bombardieri, F. Atzeni, D. Hammenfors, B. Maure, S. E. Carsons, T. Gheita, I. Sánchez-Berná, M. López-Dupla, J. Morel, N. Inanc, E. Fonseca-Aizpuru, C. Morcillo, C. Vollenweider, S. Melchor, M. Vázquez, E. Díaz-Cuiza, S. Consani-Fernández, B. De-Miguel-Campo, A. Szántó, S. Bombardieri, A. Gattamelata, A. Hinrichs, J. Sánchez-Guerrero, D. Danda, L. Kilic, S. De Vita, P. Wiland, R. Gerli, S. H. Park, M. Wahren-Herlenius, H. Bootsma, X. Mariette, M. Ramos-Casals, P. Brito-Zerón
Dátum:2021
ISSN:0392-856X
Megjegyzések:Objectives: To analyse how the main components of the disease phenotype (sicca symptoms, diagnostic tests, immunological markers and systemic disease) can be driven by the age at diagnosis of primary Sjögren's syndrome (pSS). Methods: By January 2021, the participant centres had included 12,753 patients from 25 countries that fulfilled the 2002/2016 classification criteria for pSS. The age at diagnosis was defined as the time when the attending physician confirmed fulfilment of the criteria. Patients were clustered according to age at diagnosis. 50 clusters with more than 100 observations (from 27 to 76 years) were used to study the influence of the age at diagnosis in the disease expression. Results: There was a consistent increase in the frequency of oral dryness according to the age at diagnosis, with a frequency of <90% in patients diagnosed at the youngest ages and >95% in those diagnosed at the oldest ages. The smooth curves that best fitted a linear model were the frequency of dry mouth (adjusted R2 0.87) and the frequency of abnormal oral tests (adjusted R2 0.72). Therefore, for each 1-year increase in the age at diagnosis, the frequency of dry mouth increased by 0.13%, and the frequency of abnormal oral diagnostic tests by 0.11%. There was a consistent year-by-year decrease in the frequency of all autoantibodies and immunological markers except for cryoglobulins. According to the linear models, for each 1-year increase in the age at diagnosis, the frequency of a positive result decreased by 0.57% (for anti-Ro antibodies), 0.47% (for RF) and 0.42% (for anti-La antibodies). The ESSDAI domains which showed a more consistent decrease were glandular and lymph node involvement (for each 1-year increase in the age at diagnosis, the frequency of activity decreased by 0.18%), and constitutional, cutaneous, and haematological involvements (the frequency decreased by 0.09% for each 1-year increase). In contrast, other domains showed an ascending pattern, especially pulmonary involvement (for each 1-year increase in the age at diagnosis, the frequency of activity increased by 0.22%), and peripheral nerve involvement (the frequency increased by 0.09% for each 1-year increase). Conclusions: The influence of the age at diagnosis on the key phenotypic features of pSS is strong, and should be considered critical not only for designing a personalised diagnostic approach, but also to be carefully considered when analysing the results of diagnostic tests and immunological parameters, and when internal organ involvement is suspected at diagnosis.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
Sjögren's syndrome
age
disease phenotype
immunological markers
Megjelenés:Clinical and Experimental Rheumatology. - 39 : 6 (2021), p. 166-174. -
További szerzők:Acar-Denizli, Nihan Horváth Ildikó Fanny (1980-) (belgyógyász, allergológus, klinikai immunológus) Ng, Wan Fai Rasmussen, Astrid Dong, X. Li, X. Baldini, Chiara Olsson, Peter Priori, Roberta Seror, Raphaele Gottenberg, Jacques-Eric Kruize, Aike A. Hernandez-Molina, Gabriela Vissink, Arjan Sandhya, Pulukool Armagan, Berkan Quartuccio, Luca Sebastian, Agata Praprotnik, Sonja Bartoloni, Elena Kwok, Seung-Ki Kvarnstrom, Marika Rischmueller, Maureen Soláns-Laqué, Roser Sene, Damien Pasoto, Sandra Suzuki, Yasunori Isenberg, David A. Valim, Valeria Nordmark, Gunnel Nakamura, Hideki Fernandes Moça Trevisani, Virginia Hofauer, Benedikt Sisó-Almirall, Antoni Giacomelli, Roberto Devauchelle-Pensec, Valerie Bombardieri, Michele Atzeni, F. Hammenfors, Daniel Maure, B. Carsons, Steven E. Gheita, Tamer A. Sánchez-Berná, I. López-Dupla, Miguel Morel, Jacques Inanc, Nevsun Fonseca-Aizpuru, Eva Morcillo, C. Vollenveider, Cristina Melchor, Sheila Vázquez, Marta Diaz-Cuiza, E. Consani-Fernández, S. de-Miguel-Campo, B. Szántó Antónia (1977-) (belgyógyász, allergológus és klinikai immunológus) Bombardieri, Stefano Gattamelata, Angelica Hinrichs, Anneline Sanchez-Guerrero, Jorge Danda, Debashish Kilic, Levent Vita, Salvatore de Wiland, Piotr Gerli, Roberto Park, S. H. Wahren-Herlenius, Marie Bootsma, Hendrika Mariette, Xavier Ramos-Casals, Manuel Brito-Zerón, Pilar
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11.

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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