Review
Biochemistry & Molecular Biology
Nicoletta Del Papa, Antonina Minniti, Maurizio Lorini, Vincenzo Carbonelli, Wanda Maglione, Francesca Pignataro, Nicola Montano, Roberto Caporali, Claudio Vitali
Summary: Evidence suggests that interferons play a key role in the pathogenesis of systemic autoimmune diseases, such as primary Sjogren's syndrome. Overexpression of type I interferon is associated with systemic extra-glandular manifestations and production of autoantibodies, while a milder expression is linked to limited glandular involvement. The potential role of type II interferon and different immunological pathways in subset of pSS patients is an emerging area of study.
Article
Medicine, General & Internal
Agata Sebastian, Marta Madej, Pawel Gajdanowicz, Maciej Sebastian, Anna Luczak, Magdalena Zemelka-Wiacek, Marek Jutel, Piotr Wiland
Summary: Primary Sjogren syndrome (pSS) is a complex multifactorial disease, involving various cytokines including interferon. This study aimed to quantify specific cytokines involved in the pathomechanism of pSS and determine their clinical correlation. Results showed that IFN gamma was the most frequently detected cytokine, and was present along with other specific cytokines in most patients. However, no clinical correlation was observed among the three most common cytokines, IL6, IFN gamma, and TNF alpha. These preliminary research findings suggest the possibility of exploring new treatments for pSS.
JOURNAL OF CLINICAL MEDICINE
(2022)
Article
Biochemistry & Molecular Biology
Yuriko Tanaka, Mayu Onozato, Tetuo Mikami, Terumi Kohwi-Shigematsu, Takeshi Fukushima, Motonari Kondo
Summary: The study found that early signs of Sjogren's Syndrome were observed in SATB1cKO mice, with IFN-dependent IDO expression induction being a crucial early event in the disease. Serum L-KYN could potentially be used as a marker for early SS diagnosis before autoantibodies become detectable.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Review
Immunology
Qipeng Zhan, Jianan Zhang, Yubin Lin, Wenjing Chen, Xinzou Fan, Dunfang Zhang
Summary: Sjogren's syndrome (SS) is a chronic autoimmune disease with dryness of the mouth and eyes as the main manifestations and systemic complications. IFNs, Th17 cell-related cytokines, and B cell-related cytokines are crucial for the pathogenesis of SS. Experimental treatments strategies include targeting Treg/Th17, mesenchymal stem cell treatment, targeting BAFF, inhibiting JAK pathway, etc.
FRONTIERS IN IMMUNOLOGY
(2023)
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
Immunology
Erika Huijser, Cornelia G. van Helden-Meeuwsen, Dwin G. B. Grashof, Jessica R. Tarn, Zana Brkic, Josje M. A. Huisman, M. Javad Wahadat, Harmen J. G. van de Werken, Ana P. Lopes, Joel A. G. van Roon, Paul L. A. van Daele, Sylvia Kamphuis, Wan-Fai Ng, Siroon Bekkering, Leo A. B. Joosten, Willem A. Dik, Marjan A. Versnel
Summary: Trained immunity is a long-term reprogramming of innate immune cells that is linked to atherosclerosis. This study investigated the link between type I interferons and trained immunity in patients with primary Sjogren's syndrome. The findings suggest that type I interferon induces a trained immunity phenotype in monocytes, while trained immunity also affects the production of type I interferons.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Medicine, General & Internal
Agata Sebastian, Marta Madej, Maciej Sebastian, Anna Luczak, Pawel Gajdanowicz, Magdalena Zemelka-Wiacek, Piotr Wiland
Summary: The upregulation of IFN pathways and their stimulated genes is associated with primary Sjogren's syndrome (pSS). The study found that patients with positive IFN gamma were younger and had higher levels of RF and ESSDAI compared to patients without IFN gamma.
JOURNAL OF CLINICAL MEDICINE
(2022)
Review
Biochemistry & Molecular Biology
Julie Sarrand, Laurie Baglione, Dorian Parisis, Muhammad Soyfoo
Summary: Sjogren's syndrome is a chronic autoimmune disease that primarily affects exocrine glands. It is characterized by lymphoplasmacytic infiltration and dysfunction of the glandular tissues. In addition to dry eyes and dry mouth, patients commonly experience fatigue, pain, and other systemic symptoms.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
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
Alexis Collins, Dennis Lendrem, James Wason, Jessica Tarn, Nadia Howard-Tripp, Iris Bodewes, Marjan A. Versnel, Jacques-Eric Gottenberg, Raphaele Seror, Xavier Mariette, Wan-Fai Ng
Summary: By stratifying patients with primary Sjogren's syndrome into different pathobiological subgroups using the Newcastle Sjogren's Stratification Tool and re-analysing the clinical outcomes and interferon activity data, it was found that both high symptom burden and low symptom burden subgroups may respond positively to hydroxychloroquine, but the improvement may not be achieved through reducing interferon activities.
RHEUMATOLOGY INTERNATIONAL
(2021)
Review
Medicine, General & Internal
Erika Huijser, Marjan A. Versnel
Summary: Primary Sjogren's syndrome (pSS) is a systemic autoimmune rheumatic disease characterized by dryness of the eyes and mucous membranes, with majority of patients exhibiting persistent systemic activation of the type I interferon (IFN) system. Dysregulated detection of endogenous nucleic acids (NAs) is widely implicated in the pathogenesis of systemic autoimmune diseases. Stimulation of endosomal Toll-like receptors by NA-containing immune complexes contributes to the systemic type I IFN activation.
JOURNAL OF CLINICAL MEDICINE
(2021)
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
Samuel Lara-Reyna, James A. Poulter, Elton J. R. Vasconcelos, Mark Kacar, Michael F. McDermott, Reuben Tooze, Rainer Doffinger, Sinisa Savic
Summary: Biallelic mutations in SLC29A3 cause H syndrome, a complex disorder with 25% of patients developing autoinflammatory complications. Transcriptomic analysis of monocytes and macrophages in H syndrome patients showed similarities with SAIDs and a unique upregulation of IFN gamma signature. These findings may lead to better therapeutic options for this rare disorder.
JOURNAL OF CLINICAL IMMUNOLOGY
(2021)
Review
Immunology
Fabiola Atzeni, Francesco Gozza, Giacomo Cafaro, Carlo Perricone, Elena Bartoloni
Summary: Sjogren Syndrome (SS) is associated with an increased risk of cardiovascular and cerebrovascular events, which represent a major burden for SS patients. The risk of CV events is influenced by multiple factors, including traditional risk factors and disease-related mechanisms. The impact of drugs used for treating SS on CV events is still lacking sufficient data.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Endocrinology & Metabolism
Maaike E. Straat, Borja Martinez-Tellez, Huub J. van Eyk, Maurice B. Bizino, Suzanne van Veen, Eleonora Vianello, Rinke Stienstra, Tom H. M. Ottenhoff, Hildo J. Lamb, Johannes W. A. Smit, Ingrid M. Jazet, Patrick C. N. Rensen, Mariette R. Boon
Summary: There are differences in immune gene expression between South Asian and Europid individuals with T2D, with a more activated IFN-signaling pathway in South Asians, particularly in women.
JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM
(2022)
Article
Immunology
Kathryn C. Fitzgerald, Christopher A. Mecoli, Morgan Douglas, Samantha Harris, Berna Aravidis, Jemima Albayda, Elias S. Sotirchos, Ahmet Hoke, Ana-Maria Orbai, Michelle Petri, Lisa Christopher-Stine, Alan N. Baer, Julie J. Paik, Brittany L. Adler, Eleni Tiniakou, Homa Timlin, Pavan Bhargava, Scott D. Newsome, Arun Venkatesan, Vinay Chaudhry, Thomas E. Lloyd, Carlos A. Pardo, Barney J. Stern, Mark Lazarev, Brindusa Truta, Shiv Saidha, Edward S. Chen, Michelle Sharp, Nisha Gilotra, Edward K. Kasper, Allan C. Gelber, Clifton O. Bingham, Ami A. Shah, Ellen M. Mowry
Summary: The study shows that glucocorticoid exposure may increase the risk of COVID-19 in people with autoimmune or inflammatory conditions. Those with pandemic-related reduced income may be more vulnerable to disruptions in healthcare services.
CLINICAL INFECTIOUS DISEASES
(2022)
Letter
Rheumatology
Caoilfhionn M. Connolly, Teresa Po-Yu Chiang, Brian J. Boyarsky, Jake A. Ruddy, Mayan Teles, Jennifer L. Alejo, Allan Massie, William A. Werbel, Ami A. Shah, Lisa Christopher-Stine, Jacqueline Garonzik-Wang, Dorry L. Segev, Julie J. Paik
ANNALS OF THE RHEUMATIC DISEASES
(2022)
Letter
Rheumatology
Rachel Wallwork, Livia Casciola-Rosen, Ami A. Shah
ANNALS OF THE RHEUMATIC DISEASES
(2022)
Letter
Rheumatology
Mayan Teles, Caoilfhionn M. Connolly, Sarah Frey, Teresa Po-Yu Chiang, Jennifer J. Alejo, Brian J. Boyarsky, Ami A. Shah, Jemima Albayda, Lisa Christopher-Stine, William A. Werbel, Dorry L. Segev, Julie J. Paik
ANNALS OF THE RHEUMATIC DISEASES
(2022)
Article
Integrative & Complementary Medicine
Dana D. DiRenzo, Carly Hunt, Erica M. Sibinga, Neda F. Gould, Ami A. Shah, Susan J. Bartlett, Clifton O. Bingham
Summary: This study explored the feasibility of using a meditation and stress-reduction app to improve the quality of life and symptoms of patients with rheumatic disease. The results showed that using the app for 30 days led to significant improvements in fatigue and potential improvements in other symptoms. Further research on app use and its effects is needed.
EXPLORE-THE JOURNAL OF SCIENCE AND HEALING
(2022)
Article
Rheumatology
Christopher A. Mecoli, Tak Igusa, Mengkun Chen, XingYao Wang, Jemima Albayda, Julie J. Paik, Eleni Tiniakou, Brittany Adler, Carrie Richardson, Will Kelly, Sonye Danoff, Andrew L. Mammen, Elizabeth A. Platz, Antony Rosen, Lisa Christopher-Stine, Livia Casciola-Rosen, Ami A. Shah
Summary: This study investigated the cancer risk in patients with idiopathic inflammatory myopathy (IIM) compared to the general population. The results showed that IIM patients had a 1.43-fold increased risk of developing cancer. Specific autoantibodies, such as anti-transcription intermediary factor 1 gamma (anti-TIF1 gamma), were associated with an increased risk of ovarian and breast cancer.
ARTHRITIS & RHEUMATOLOGY
(2023)
Article
Rheumatology
Lan Yu, Robyn T. Domsic, Lesley-Ann Saketkoo, Jane Withey, Tracy M. Frech, Ariane L. Herrick, Laura K. Hummers, Ami A. Shah, Christopher P. Denton, Dinesh Khanna, John D. Pauling
Summary: A novel patient-reported outcome instrument, the ASRAP questionnaire, has been developed and scored to assess the severity and impact of RP in SSc. The questionnaire was developed with extensive patient input and went through qualitative assessment and linguistic testing. A long-form and short-form version of the ASRAP questionnaire have been calibrated and scored for formal validation.
ARTHRITIS CARE & RESEARCH
(2023)
Article
Rheumatology
Ana E. E. Tucker, Jamie Perin, Elizabeth R. R. Volkmann, Tsion Abdi, Ami A. A. Shah, John Pandolfino, Richard M. M. Silver, Zsuzsanna H. H. McMahan
Summary: This study aimed to investigate whether patterns of esophageal dysfunction in patients with systemic sclerosis (SSc) are associated with specific clinical phenotypes. The results showed that absent contractility (AC) is closely associated with the clinical manifestations of SSc, while ineffective esophageal motility (IEM) may be associated with a milder phenotype.
ARTHRITIS CARE & RESEARCH
(2023)
Article
Rheumatology
Anne R. Bass, Noha Abdel-Wahab, Pankti D. Reid, Jeffrey A. Sparks, Cassandra Calabrese, Deanna P. Jannat-Khah, Nilasha Ghosh, Divya Rajesh, Carlos Andres Aude, Lydia Gedmintas, Lindsey MacFarlane, Senada Arabelovic, Adewunmi Falohun, Komal Mushtaq, Farah Al Haj, Adi Diab, Ami A. Shah, Clifton O. Bingham, Karmela Kim Chan, Laura C. Cappelli
Summary: This study compares the safety and effectiveness of biologic and conventional DMARDs for ICI-IA. The results show that biologic DMARDs can achieve faster arthritis control than MTX, but may be associated with a shorter time to cancer progression.
ANNALS OF THE RHEUMATIC DISEASES
(2023)
Article
Rheumatology
Leonardo Martin Calderon, Robyn T. Domsic, Ami A. Shah, Janet E. Pope
Summary: A preventative care approach is crucial to reduce the impact of SSc-related complications, such as OP and malignancies. Clinicians should ensure vitamin D levels are normal in SSc patients, as some may still be deficient despite supplementation. Nutritional screening should be conducted, with appropriate interventions for at-risk patients. OP guidelines should be followed for bone density testing and treatment for moderate to high-risk patients. Regular screening for malignancies based on individual risk factors is also recommended.
RHEUMATIC DISEASE CLINICS OF NORTH AMERICA
(2023)
Article
Rheumatology
Leonardo Martin Calderon, Janet E. Pope, Ami A. Shah, Robyn T. Domsic
Summary: There are complications associated with SSc that can be prevented or reduced through proper screening and treatment, such as preventing infections with routine vaccinations. Patients with SSc may experience decreased seroconversion due to immunosuppressants, and temporary DMARD suspension may be necessary for vaccinations. Additionally, a prime-boost approach to influenza vaccination may benefit patients.
RHEUMATIC DISEASE CLINICS OF NORTH AMERICA
(2023)
Article
Rheumatology
Christopher A. Mecoli, Brant Chee, Mengkun Chen, XingYao Wang, Jemima Albayda, Julie J. Paik, Eleni Tiniakou, Brittany Adler, Will Kelly, Andrew L. Mammen, Elizabeth A. Platz, Livia Casciola-Rosen, Lisa Christopher-Stine, Ami A. Shah
Summary: This study evaluated the diagnostic yield of computed tomography (CT) imaging for cancer screening/surveillance in patients with idiopathic inflammatory myopathy (IIM). The results showed that dermatomyositis patients had the highest diagnostic yields on CT scans of the chest and abdomen/pelvis, while patients under 40 years old had lower diagnostic yields.
ARTHRITIS CARE & RESEARCH
(2023)
Article
Rheumatology
David Fiorentino, Christopher A. Mecoli, Tak Igusa, Jemima Albayda, Julie J. Paik, Eleni Tiniakou, Brittany Adler, Andrew L. Mammen, Ami A. Shah, Antony Rosen, Lisa Christopher-Stine, Livia Casciola-Rosen
Summary: This study demonstrates the disease specificity and clinical significance of anti-CCAR1 autoantibodies in dermatomyositis (DM) patients. It also reveals a lower risk of cancer in DM patients with anti-TIF1 gamma positive and anti-CCAR1 positive status.
ARTHRITIS & RHEUMATOLOGY
(2023)
Article
Rheumatology
Robyn T. Domsic, Robin Pokrzywinski, Larissa Stassek, Wade W. Benton, Christa-Lynn Vampola, Daniel E. Furst, Lorinda Chung, Virginia Steen, Maureen D. Mayes, Ami A. Shah, Jerry A. Molitor, Kelly Oliver, Vivek Nagaraja, Dinesh Khanna
Summary: The study aimed to understand the symptoms and impacts of Raynaud phenomenon (RP) in patients with systemic sclerosis (SSc) and evaluate the content validity and usability of the Raynaud Diary, a new electronic patient-reported outcome (PRO) measure for RP. Participants reported frequent RP symptoms such as color change, numbness, tingling, pain, and discomfort. Common triggers for RP attacks included temperature-related factors and stress. The participants demonstrated understanding of the Raynaud Diary instructions and indicated that they could use it to record the worst severity of individual RP symptoms.
ACR OPEN RHEUMATOLOGY
(2023)
Article
Rheumatology
Elana J. Bernstein, Shervin Assassi, Flavia Castelino, Lorinda Chung, Chase Correia, Luke B. Evnin, Tracy M. Frech, Jessica K. Gordon, Brian A. Skaug, Faye N. Hant, Laura K. Hummers, Nora Sandorfi, Ami A. Shah, Victoria K. Shanmugam, Virginia D. Steen, Dinesh Khanna
Summary: This study investigated the HRCT ordering practices at SSc centers in the United States and identified patient characteristics associated with HRCT performance. The majority of patients with SSc underwent HRCT, and a positive anti-centromere antibody was inversely associated with HRCT performance.
ACR OPEN RHEUMATOLOGY
(2022)