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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:	BIBFORM092150
035-os BibID:	(cikkazonosító)639975 (WoS)000635978100001 (Scopus)85103554422
Első szerző:	Szabó Krisztina (Molekuláris biológus)
Cím:	The imbalance of circulating follicular T helper cell subsets in primary Sjögren's syndrome associates with serological alterations and abnormal B-cell distribution / Krisztina Szabó, Ilona Jámbor, Antónia Szántó, Ildikó Fanny Horváth, Tünde Tarr, Britt Nakken, Péter Szodoray, Gábor Papp
Dátum:	2021
ISSN:	1664-3224
Megjegyzések:	Since B-cell hyperactivity and pathologic antibody response are key features in the immunopathogenesis of primary Sjögren's syndrome (pSS), the role of follicular T helper (TFH) cells as efficient helpers in the survival and differentiation of B cells has been emerged. Our aim was to investigate whether a change in the balance of circulating (c)TFH subsets and follicular regulatory T (TFR) cells could affect the distribution of B cells in pSS. Peripheral blood of 38 pSS patients and 27 healthy controls was assessed for the frequencies of cTFH cell subsets, TFR cells and certain B cell subpopulations by multicolor flow cytometry. Serological parameters, including anti-SSA, anti-SSB autoantibodies, immunoglobulin and immune complex titers were determined as part of the routine diagnostic evaluation. Patients with pSS showed a significant increase in activated cTFH cell proportions, which was associated with serological results. Frequencies of cTFH subsets were unchanged in pSS patients compared to healthy controls. The percentages and number of cTFR cells exhibited a significant increase in autoantibody positive patients compared to patients with seronegative pSS. The proportions of transitional and naïve B cells were significantly increased, whereas subsets of memory B cells were significantly decreased and correlated with autoantibody production. Functional analysis revealed that the simultaneous blockade of cTFH and B cell interaction with anti-IL-21 and anti-CD40 antibodies decreased the production of IgM and IgG. Imbalance in TFH subsets and TFR cells indicates an ongoing over-activated humoral immune response, which contributes to the characteristic serological manifestations and the pathogenesis of pSS.
Tárgyszavak:	Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban folyóiratcikk follicular T helper cell follicular regulatory T cell Chemokine receptors B cells primary Sjögren's syndrome
Megjelenés:	Frontiers in Immunology. - 12 (2021), p. 1-13. -
További szerzők:	Jámbor Ilona (1985-) (orvosi biotechológus) Szántó Antónia (1977-) (belgyógyász, allergológus és klinikai immunológus) Horváth Ildikó Fanny (1980-) (belgyógyász, allergológus, klinikai immunológus) Tarr Tünde (1976-) (belgyógyász, allergológus és klinikai immunológus) Nakken, Britt Szodoray Péter (1973-) (belgyógyász, orvos) Papp Gábor (1984-) (belgyógyász)
Pályázati támogatás:	121327 PD_16 124177 OTKA
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