Article
Rheumatology
Xiaomin Chen, Shan Jiang, Zihao Zhou, Xin Xu, Senhong Ying, Lifeng Du, Kairui Qiu, Yesha Xu, Jinyu Wu, Xiaobing Wang
Summary: The study found a significant upregulation of IL-21-inducible genes in the minor salivary glands of pSS patients, which correlated with disease activity and cell subset enrichment. IL-21 scores were positively associated with antibody positivity and high IgG levels, while hypoxia and IFN-related genes showed strong correlation with IL-21 signature gene expression.
Article
Immunology
Akinsola Oyelakin, Erich Horeth, Eun-Ah Christine Song, Sangwon Min, Monika Che, Brandon Marzullo, Christopher J. Lessard, Astrid Rasmussen, Lida Radfar, R. Hal Scofield, David M. Lewis, Donald U. Stone, Kiely Grundahl, Scott S. De Rossi, Zoya Kurago, A. Darise Farris, Kathy L. Sivils, Satrajit Sinha, Jill M. Kramer, Rose-Anne Romano
Summary: Primary Sjogren's syndrome is a systemic autoimmune disease primarily affecting women, leading to loss of saliva and tears. Currently, there is no effective cure due to limited understanding of disease etiology and pathology.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Endocrinology & Metabolism
S. Rastelli, M. D'Amario, E. Iacomino, M. Lacarbonara, D. Guerra, M. Capogreco
Summary: Sjogren's Syndrome is a systemic autoimmune disease that mainly affects women in the age group of 50-80 years, causing glandular secretory disorders and significantly impacting the patient's quality of life. Early diagnosis plays a crucial role.
JOURNAL OF BIOLOGICAL REGULATORS AND HOMEOSTATIC AGENTS
(2022)
Article
Medicine, General & Internal
Ana Isabel Lopes, Raquel Machado-Neves, Mrinalini Honavar, P. Ricardo Pereira
Summary: The minor salivary glands' biopsy is an effective and simple diagnostic procedure for Sjogren's syndrome and amyloidosis. Its use can increase the number of patients meeting the classification criteria for Sjogren's syndrome and appears to be useful in diagnosing amyloidosis.
EUROPEAN JOURNAL OF INTERNAL MEDICINE
(2021)
Review
Medicine, Research & Experimental
Simone Negrini, Giacomo Emmi, Monica Greco, Matteo Borro, Federica Sardanelli, Giuseppe Murdaca, Francesco Indiveri, Francesco Puppo
Summary: Sjogren's syndrome is a chronic autoimmune disease characterized by dryness in the eyes and mouth, with systemic manifestations and unclear pathogenesis. Diagnosis is based on clinical symptoms and specific tests, while treatment ranges from local to systemic medications. New classification criteria and disease activity scores have been developed for research and clinical practice.
CLINICAL AND EXPERIMENTAL MEDICINE
(2022)
Article
Immunology
Tamandeep K. K. Bharaj, Lara A. A. Aqrawi, Siren Fromreide, Roland Jonsson, Johan G. G. Brun, Silke Appel, Kathrine Skarstein
Summary: A study stratified 85 patients to investigate the heterogeneity of minor salivary glands in primary Sjogren's syndrome, identifying variations in tissue characteristics, immune cell proportions, and clinical correlations at different disease stages.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Biochemistry & Molecular Biology
Ioanna E. Stergiou, Loukas Chatzis, Asimina Papanikolaou, Stavroula Giannouli, Athanasios G. Tzioufas, Michael Voulgarelis, Efstathia K. Kapsogeorgou
Summary: The Akt pathway is specifically activated in MSGs of pSS patients, providing support for novel therapeutic targets.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Biochemistry & Molecular Biology
Fredrik Fineide, Xiangjun Chen, Thomas Bjellaas, Valeria Vitelli, Tor Paaske Utheim, Janicke Liaaen Jensen, Hilde Kanli Galtung
Summary: The study revealed significant differences in the lipidomic profiles of saliva and tears in pSS patients compared to healthy controls, with individual lipid species in stimulated saliva showing similarities to glandular biopsies. This opens up intriguing avenues for further research and may lead to innovative diagnostic methodologies and treatment modalities for alleviating pSS-related sicca symptoms.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Oncology
Maria Janka, Attila Zalatnai
Summary: This study compared the histomorphological changes and clinical findings in patients with pathologically confirmed Sjogren's syndrome and those with negative histology. It found that only 31.8% of Sjogren's histologies showed acinar loss, suggesting that decreased saliva production may be due to immune-mediated hypofunction rather than direct acinar damage. This is the first systemic study in Hungary to investigate the correlation between pathological alterations and clinical findings.
PATHOLOGY & ONCOLOGY RESEARCH
(2023)
Article
Immunology
Donghyun Kim, Ye Jin Jeong, Yerin Lee, Jihoon Choi, Young Min Park, Oh Chan Kwon, Yong Woo Ji, Sung Jun Ahn, Hyung Keun Lee, Min-Chan Park, Jae-Yol Lim
Summary: Recent studies have shown that the oral microbiome in patients with Sjogren's syndrome (SS) differs significantly from that in healthy individuals. The composition and diversity of the oral microbiome are correlated with clinical features of SS, especially ductal structures and salivary flow, suggesting that the oral microbiome may serve as a potential diagnostic biomarker for SS.
FRONTIERS IN IMMUNOLOGY
(2022)
Review
Rheumatology
Valerie Devauchelle-Pensec, Alen Zabotti, Guillermo Carvajal-Alegria, Nenad Filipovic, Sandrine Jousse-Joulin, Salvatore De Vita
Summary: Salivary gland ultrasonography plays an important role in detecting structural abnormalities in primary Sjogren's Syndrome, and could potentially be used for classification. The ongoing HarmonicSS project, supported by the EU, aims to apply artificial intelligence to SGUS in pSS for future study. Many questions remain unresolved, but data collected so far suggest that SGUS will be a significant tool in the study of pSS in the near future.
Article
Biology
Arso M. Vukicevic, Milos Radovic, Alen Zabotti, Vera Milic, Alojzija Hocevar, Sara Zandonella Callegher, Orazio De Lucia, Salvatore De Vita, Nenad Filipovic
Summary: This study proposed a deep learning-based solution for automated segmentation of salivary gland ultrasound images to address the low inter/intra observer reliability in diagnosing primary Sjögren's syndrome. After training with different architectures, the FCN-DenseNet network showed superior performance in accuracy and speed, indicating its potential for wider clinical applications. Further work will focus on integrating methods for pSS scoring to establish SGUS as an effective noninvasive diagnostic tool.
COMPUTERS IN BIOLOGY AND MEDICINE
(2021)
Article
Dentistry, Oral Surgery & Medicine
M. C. Hesterman, S. V. Furrer, B. S. Fallon, M. L. Weller
Summary: This study found that hepatitis delta virus (HDV) was detected in the minor salivary gland (MSG) tissue of Sjogren's disease (SjD) patients without hepatitis B virus (HBV) coinfection. The expression of HDV antigen (HDAg) can trigger an SjD-like phenotype in vivo. HDAg was detected in different cell types and subcellular locations in the MSG, and HDV genomic RNA was localized in the MSG nuclei. The intensity of HDAg showed negative correlations with lymphocytic inflammation and certain autoimmune disease comorbidities in SjD patients.
JOURNAL OF DENTAL RESEARCH
(2023)
Article
Biochemistry & Molecular Biology
Alessandra Falleni, Stefania Moscato, Giovanni Fulvio, Enza Polizzi, Margherita Bernardeschi, Francesco Bianchi, Valentina Donati, Manuela Cabiati, Chiara Ippolito, Silvia Del Ry, Chiara Baldini, Letizia Mattii
Summary: This study identified the presence of Cx26, Cx32, and Cx43 proteins in human salivary glands and their localization in duct cells, acinar cells, and myoepithelial cells. This suggests that Cxs may play a role in salivary gland dysfunctions.
Article
Rheumatology
L. G. Chatzis, V Pezoulas, P. V. Voulgari, C. Baldini, T. P. Exarchos, D. Fotiadis, C. P. Mavragani, F. N. Skopouli, H. M. Moutsopoulos, A. G. Tzioufas, A. Goules
Summary: This study described the clinical spectrum of Sjogren's syndrome patients with combined seronegativity, revealing that such patients have a milder disease phenotype. Triple seronegative patients showed lower frequency of peripheral nervous involvement compared to controls, while quadruple seronegative patients presented less frequently with persistent lymphadenopathy and lymphoma.
CLINICAL AND EXPERIMENTAL RHEUMATOLOGY
(2021)
Review
Rheumatology
Juliana Imgenberg-Kreuz, Astrid Rasmussen, Kathy Sivils, Gunnel Nordmark
Summary: Primary Sjogren's syndrome (pSS) is a multifactorial disease with underlying genetic predisposition, epigenetic mechanisms and environmental factors playing roles in disease development. The strongest association signal lies within the HLA genes, with most genetic risk variants found at intergenic regions whose functional impact remains unclear. Epigenetic mechanisms like DNA methylation and histone modifications modulate gene expression, forming a dynamic link between the genome and phenotypic manifestations in pSS.
Article
Rheumatology
L. Vasaitis, G. Nordmark, E. Theander, C. Backlin, K. E. Smedby, J. Askling, L. Ronnblom, C. Sundstrom, E. Baecklund
SCANDINAVIAN JOURNAL OF RHEUMATOLOGY
(2020)
Article
Genetics & Heredity
Christian Lundtoft, Pascal Pucholt, Juliana Imgenberg-Kreuz, Jonas Carlsson-Almlof, Maija-Leena Eloranta, Ann-Christine Syvanen, Gunnel Nordmark, Johanna K. Sandling, Ingrid Kockum, Tomas Olsson, Lars Ronnblom, Niklas Hagberg
Article
Genetics & Heredity
Karin Bolin, Juliana Imgenberg-Kreuz, Dag Leonard, Johanna K. Sandling, Andrei Alexsson, Pascal Pucholt, Malena Loberg Haarhaus, Jonas Carlsson Almlof, Joanne Nititham, Andreas Jonsen, Christopher Sjowall, Anders A. Bengtsson, Solbritt Rantapaa-Dahlqvist, Elisabet Svenungsson, Iva Gunnarsson, Ann-Christine Syvanen, Karoline Lerang, Anne Troldborg, Anne Voss, Oyvind Molberg, Soren Jacobsen, Lindsey Criswell, Lars Ronnblom, Gunnel Nordmark
Summary: The genetic variations in BANK1 are associated with lupus nephritis, and evidence suggests genetic regulation of DNA methylation within the BANK1 locus, indicating a role for BANK1 in the pathogenesis of lupus nephritis.
GENES AND IMMUNITY
(2021)
Review
Medicine, General & Internal
P. W. Franks, E. Melen, M. Friedman, J. Sundstrom, I Kockum, L. Klareskog, C. Almqvist, S. E. Bergen, K. Czene, S. Hagg, P. Hall, K. Johnell, A. Malarstig, A. Catrina, H. Hagstrom, M. Benson, J. Gustav Smith, M. F. Gomez, M. Orho-Melander, B. Jacobsson, J. Halfvarson, D. Repsilber, M. Oresic, C. Jern, B. Melin, C. Ohlsson, T. Fall, L. Ronnblom, M. Wadelius, G. Nordmark, A. Johansson, R. Rosenquist, P. F. Sullivan
Summary: Genomic approaches to diagnosis, prognostication, prevention and treatment may optimize medical practice. Research is underway to determine the polygenic basis of many complex diseases, which will likely necessitate models for prediction and diagnosis that incorporate genetic and non-genetic data.
JOURNAL OF INTERNAL MEDICINE
(2021)
Article
Immunology
Juliana Imgenberg-Kreuz, Johanna K. Sandling, Katrine Braekke Norheim, Svein Joar Auglaend Johnsen, Roald Omdal, Ann-Christine Syvanen, Elisabet Svenungsson, Lars Ronnblom, Maija-Leena Eloranta, Gunnel Nordmark
Summary: The study introduced a DNA methylation-based IFN score to identify patients with activation of the IFN system in primary Sjogren's syndrome (pSS). High DNAm IFN scores were associated with younger age at symptom onset and diagnosis, positive SSA and SSB antibodies, hypergammaglobulinemia, low C4, and lymphoma in pSS patients. The DNAm IFN score could be a promising tool for patient stratification, treatment decisions, monitoring, and inclusion in clinical trials.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Rheumatology
Gabriela Hernandez-Molina, Belchin Kostov, Pilar Brito-Zeron, Arjan Vissink, Thomas Mandl, Anneline C. Hinrichs, Luca Quartuccio, Chiara Baldini, Raphaele Seror, Antonia Szanto, David Isenberg, Roberto Gerli, Gunnel Nordmark, Astrid Rasmussen, Roser Solans-Laque, Benedikt Hofauer, Damien Sene, Sandra G. Pasoto, Maureen Rischmueller, Sonja Praprotnik, Tamer A. Gheita, Debashish Danda, Berkan Armagan, Yasunori Suzuki, Valeria Valim, Valerie Devauchelle-Pensec, Soledad Retamozo, Marika Kvarnstrom, Agata Sebastian, Fabiola Atzeni, Roberto Giacomelli, Steven E. Carsons, Seung-Ki Kwok, Hideki Nakamura, Virginia Fernandes Moca Trevisani, Alejandra Flores-Chavez, Xavier Mariette, Manuel Ramos-Casals
Summary: This study characterized 414 patients with primary SS who developed haematological malignancies and analyzed the impact of SS- and lymphoma-related features on presentation patterns and outcomes. The results showed that B-cell lymphomas, especially MALT lymphoma, predominated in the salivary glands and had a favorable prognosis.
Article
Rheumatology
Christian Lundtoft, Pascal Pucholt, Myriam Martin, Matteo Bianchi, Emeli Lundstrom, Maija-Leena Eloranta, Johanna K. Sandling, Christopher Sjowall, Andreas Jonsen, Iva Gunnarsson, Solbritt Rantapaa-Dahlqvist, Anders A. Bengtsson, Dag Leonard, Eva Baecklund, Roland Jonsson, Daniel Hammenfors, Helena Forsblad-d'Elia, Per Eriksson, Thomas Mandl, Sara Magnusson Bucher, Katrine B. Norheim, Svein Joar Auglaend Johnsen, Roald Omdal, Marika Kvarnstrom, Marie Wahren-Herlenius, Antonella Notarnicola, Helena Andersson, Oyvind Molberg, Louise Pyndt Diederichsen, Jonas Almlof, Ann-Christine Syvanen, Sergey Kozyrev, Kerstin Lindblad-Toh, Bo Nilsson, Anna M. Blom, Ingrid E. Lundberg, Gunnel Nordmark, Lina Marcela Diaz-Gallo, Elisabet Svenungsson, Lars Ronnblom
Summary: Copy number variation of C4A is associated with the autoimmune repertoire in systemic autoimmune diseases, with a low C4A copy number strongly linked to the presence of autoantibodies. These findings have implications for understanding the mechanisms of autoimmune diseases and patient stratification based on genetic profiles.
ARTHRITIS & RHEUMATOLOGY
(2022)
Article
Multidisciplinary Sciences
Bhuwan Khatri, Kandice L. Tessneer, Astrid Rasmussen, Farhang Aghakhanian, Tove Ragna Reksten, Adam Adler, Ilias Alevizos, Juan-Manuel Anaya, Lara A. Aqrawi, Eva Baecklund, Johan G. Brun, Sara Magnusson Bucher, Maija-Leena Eloranta, Fiona Engelke, Helena Forsblad-d'Elia, Stuart B. Glenn, Daniel Hammenfors, Juliana Imgenberg-Kreuz, Janicke Liaaen Jensen, Svein Joar Auglaend Johnsen, Malin Jonsson, Marika Kvarnstrom, Jennifer A. Kelly, He Li, Thomas Mandl, Javier Martin, Gaetane Nocturne, Katrine Braekke Norheim, Oyvind Palm, Kathrine Skarstein, Anna M. Stolarczyk, Kimberly E. Taylor, Maria Teruel, Elke Theander, Swamy Venuturupalli, Daniel J. Wallace, Kiely M. Grundahl, Kimberly S. Hefner, Lida Radfar, David M. Lewis, Donald U. Stone, C. Erick Kaufman, Michael T. Brennan, Joel M. Guthridge, Judith A. James, R. Hal Scofield, Patrick M. Gaffney, Lindsey A. Criswell, Roland Jonsson, Per Eriksson, Simon J. Bowman, Roald Omdal, Lars Ronnblom, Blake Warner, Maureen Rischmueller, Torsten Witte, A. Darise Farris, Xavier Mariette, Marta E. Alarcon-Riquelme, Caroline H. Shiboski, Marie Wahren-Herlenius, Wan-Fai Ng, Kathy L. Sivils, Indra Adrianto, Gunnel Nordmark, Christopher J. Lessard
Summary: This study identifies ten novel genetic risk regions associated with Sjogren's disease, implicating genes involved in immune and salivary gland function.
NATURE COMMUNICATIONS
(2022)
Article
Rheumatology
Christian Lundtoft, Christopher Sjowall, Solbritt Rantapaa-Dahlqvist, Anders A. Bengtsson, Andreas Jonsen, Pascal Pucholt, Yee Ling Wu, Emeli Lundstrom, Maija-Leena Eloranta, Iva Gunnarsson, Eva Baecklund, Roland Jonsson, Daniel Hammenfors, Helena Forsblad-d'Elia, Per Eriksson, Thomas Mandl, Sara Bucher, Katrine B. Norheim, Svein Joar Auglaend Johnsen, Roald Omdal, Marika Kvarnstrom, Marie Wahren-Herlenius, Lennart Truedsson, Bo Nilsson, Sergey Kozyrev, Matteo Bianchi, Kerstin Lindblad-Toh, Chack-Yung Yu, Gunnel Nordmark, Johanna K. Sandling, Elisabet Svenungsson, Dag Leonard, Lars Ronnblom
Summary: This study reveals that a genetic pattern involving partial deficiencies of C2 and C4A in the classical complement pathway is a strong risk factor for SLE and primary SS, highlighting the central role of the complement system in the pathogenesis of both diseases.
ARTHRITIS & RHEUMATOLOGY
(2022)
Letter
Biochemistry & Molecular Biology
Thoas Fioretos, Valtteri Wirta, Lucia Cavelier, Eva Berglund, Mikaela Friedman, Michael Akhras, Johan Botling, Hans Ehrencrona, Lars Engstrand, Gisela Helenius, Therese Fagerqvist, David Gisselsson, Sofia Gruvberger-Saal, Ulf Gyllensten, Markus Heidenblad, Kina Hoglund, Bo Jacobsson, Maria Johansson, Asa Johansson, Maria Johansson Soller, Marene Landstrom, Par Larsson, Lars-Ake Levin, Anna Lindstrand, Lovisa Lovmar, Anna Lyander, Malin Melin, Ann Nordgren, Gunnel Nordmark, Paula Molling, Lars Palmqvist, Richard Palmqvist, Dirk Repsilber, Per Sikora, Bianca Stenmark, Peter Soderkvist, Henrik Stranneheim, Tobias Strid, Craig E. Wheelock, Mia Wadelius, Anna Wedell, Anders Edsjo, Richard Rosenquist
Article
Rheumatology
Joe Scott Berry, Jessica Tarn, John Casement, Pierre-Marie Duret, Lauren Scott, Karl Wood, Svein-Joar Johnsen, Gunnel Nordmark, Valerie Devauchelle-Pensec, Raphaele Seror, Benjamin Fisher, Fransesca Barone, Simon J. Bowman, Michele Bombardieri, Dennis Lendrem, Renaud Felten, Jacques-Eric Gottenberg, Wan-Fai Ng
Summary: Proteomic and network analysis identified distinct pathobiology and potential therapeutic targets for different subtypes of Sjogren's syndrome (SS). Stratification of patients using the Newcastle Sjogren's Stratification Tool (NSST) revealed an association between high levels of serum IL-6 and improvement in fatigue score. These findings provide insights into the underlying mechanisms and treatment strategies of SS subtypes.
ANNALS OF THE RHEUMATIC DISEASES
(2023)
Article
Rheumatology
Lina-Marcela Diaz-Gallo, Vilija Oke, Emeli Lundstrom, Kerstin Elvin, Yee Ling Wu, Susanna Eketjall, Agneta Zickert, Johanna T. Gustafsson, Andreas Jonsen, Dag Leonard, Daniel J. Birmingham, Gunnel Nordmark, Anders A. Bengtsson, Lars Ronnblom, Iva Gunnarsson, Chack-Yung Yu, Leonid Padyukov, Elisabet Svenungsson
Summary: Our study identified four distinct subgroups among patients with SLE based on autoantibody profiles, which differ in associations with HLA-DRB1 alleles and immunological and clinical features, indicating diverse disease pathways.
ACR OPEN RHEUMATOLOGY
(2022)
Article
Rheumatology
S. Retamozo, N. Acar-Denizli, I. F. Horvath, W-F Ng, A. Rasmussen, X. Dong, X. Li, C. Baldini, P. Olsson, R. Priori, R. Seror, J-E Gottenberg, A. A. Kruize, G. Hernandez-Molina, A. Vissink, P. Sandhya, B. Armagan, L. Quartuccio, A. Sebastian, S. Praprotnik, E. Bartoloni, S-K Kwok, M. Kvarnstrom, M. Rischmueller, R. Solans-Laque, D. Sene, S. G. Pasoto, Y. Suzuki, D. A. Isenberg, V. Valim, G. Nordmark, H. Nakamura, V Fernandes Moca Trevisani, B. Hofauer, A. Siso-Almirall, R. Giacomelli, V Devauchelle-Pensec, M. Bombardieri, F. Atzeni, D. Hammenfors, B. Maure, S. E. Carsons, T. Gheita, I Sanchez-Berna, M. Lopez-Dupla, J. Morel, N. Inanc, E. Fonseca-Aizpuru, C. Morcillo, C. Vollenweider, S. Melchor, M. Vazquez, E. Diaz-Cuiza, S. Consani-Fernandez, B. de-Miguel-Campo, A. Szanto, S. Bombardieri, A. Gattamelata, A. Hinrichs, J. Sanchez-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-Zeron
Summary: This study analyzed the impact of age at diagnosis on the phenotypic features of primary Sjogren's syndrome. The results showed that older patients had a higher frequency of oral dryness symptoms, while autoantibodies and immunological markers decreased with age. The study concluded that age at diagnosis plays a critical role in disease expression and should be considered in diagnostic and treatment approaches.
CLINICAL AND EXPERIMENTAL RHEUMATOLOGY
(2021)
Article
Rheumatology
Johannes Mofors, Albin Bjork, Elina Richardsdotter Andersson, Marika Kvarnstrom, Helena Forsblad D'Elia, Sara Magnusson-Bucher, Leonid Padyukov, Ingrid Kockum, Jan Hillert, Per Eriksson, Thomas Mandl, Gunnel Nordmark, Lars Alfredsson, Marie Wahren-Herlenius