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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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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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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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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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