Article
Rheumatology
Sabrina Hoa, Selma Lazizi, Murray Baron, Mianbo Wang, Marvin J. Fritzler, Marie Hudson
Summary: There is an association between cancer and specific autoantibodies in SSc patients, with increased cancer risk in patients positive for anti-topoisomerase I and anti-U1-RNP antibodies. However, synchronous cancer is rare in this cohort of Canadian SSc patients.
Article
Biology
Sevdalina Nikolova Lambova, Ekaterina Krasimirova Kurteva, Sanie Syuleymanova Dzhambazova, Georgi Hristov Vasilev, Dobroslav Stanimirov Kyurkchiev, Mariela Gencheva Geneva-Popova
Summary: In this pilot study involving 19 patients with SSc, a correlation between more advanced capillaroscopic changes and the presence of anti-Scl-70 autoantibodies was confirmed. Additionally, positive anti-RNAP III-155 antibodies were found in SSc patients with or without early microangiopathy.
Article
Rheumatology
Kristina Elizabeth Neergaard Clark, Corrado Campochiaro, Eszter Csomor, Adam Taylor, Katherine Nevin, Nicholas Galwey, Mary A. Morse, Jennifer Singh, Yee Voan Teo, Voon H. Ong, Emma Derrett-Smith, Nicolas Wisniacki, Shaun M. Flint, Christopher P. Denton
Summary: Clinical heterogeneity is a key feature of systemic sclerosis (SSc), with hallmark SSc autoantibodies playing a central role in diagnosis and associating with distinct patterns of complications. This study used high-dimensional transcriptional and proteomic analysis to reveal a molecular spectrum of SSc based on skin gene expression and serum protein analysis, showing differences in fibrosis markers and gene expression profiles between different autoantibody specificities. Individual patient pathway analysis identified overlapping and distinct disease processes within SSc subgroups.
ANNALS OF THE RHEUMATIC DISEASES
(2021)
Article
Rheumatology
Lea Lopez, Thomas Barnetche, Gael Galli, Julien Seneschal, Elodie Blanchard, Emilie Shipley, Jean-Luc Pellegrin, Estibaliz Lazaro, Joel Constans, Pierre Duffau, Thierry Schaeverbeke, Christophe Richez, Marie Kostine, Marie-Elise Truchetet
Summary: This study observed an increased risk of cancer in systemic sclerosis (SSc) patients with RNA polymerase III antibodies. Other risk factors identified were age, time of SSc diagnosis, interstitial lung disease, and anti-topoisomerase antibodies. Regular cancer screening should be considered for these patients.
Article
Rheumatology
Burcu Ayoglu, Michele Donato, Daniel E. Furst, Leslie J. Crofford, Ellen Goldmuntz, Lynette Keyes-Elstein, Judith James, Susan Macwana, Maureen D. Mayes, Peter McSweeney, Richard A. Nash, Keith M. Sullivan, Beverly Welch, Ashley Pinckney, Rong Mao, Lorinda Chung, Purvesh Khatri, Paul J. Utz
Summary: Results from the SCOT clinical trial showed that HSCT had significant benefits over CTX in patients with systemic sclerosis. The objective of this study was to test the hypothesis that transplantation stabilizes the autoantibody repertoire in patients with favorable clinical outcomes. Analysis of autoantibody profiles revealed significant differences between HSCT and CTX-treated patients, suggesting that HSCT alters the autoantibody repertoire while CTX treatment does not.
ANNALS OF THE RHEUMATIC DISEASES
(2023)
Review
Immunology
Claire F. Beesley, Nina R. Goldman, Taher E. Taher, Christopher P. Denton, David J. Abraham, Rizgar A. Mageed, Voon H. Ong
Summary: Systemic sclerosis (SSc) is an immune-mediated rheumatic disease characterized by excessive extracellular matrix deposition. B cells play a fundamental role in the pathogenesis and development of SSc, as they infiltrate lesional sites and produce profibrotic cytokines. B cell counts are increased in SSc patients and show differences in various B cell compartments. B cell signaling is impaired in SSc patients, and B cell depletion therapy has shown therapeutic benefits.
FRONTIERS IN IMMUNOLOGY
(2023)
Article
Immunology
Elopy N. Sibanda, Yvonne Dube, Mazvita Chakawa, Takafira Mduluza, Francisca Mutapi
Summary: This study investigated the clinical and laboratory characteristics of Zimbabwean patients with SSc specific autoantibodies, revealing racial variations in the expression of these antibodies. Most patients did not meet the classification criteria for SSc but still exhibited significant symptoms. Respiratory and skin symptoms were among the most common in the patients.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Immunology
Jean -Baptiste Vulsteke, Vanessa Smith, Carolien Bonroy, Rita Derua, Daniel Blockmans, Petra De Haes, Steven Vanderschueren, Jan L. Lenaerts, Kristl G. Claeys, Wim A. Wuyts, Patrick Verschueren, Gilles Vanhandsaeme, Yves Piette, Ellen De Langhe, Xavier Bossuyt
Summary: The study identified 8 telomere-and telomerase-associated autoantigens in SSc patients using unlabeled protein immunoprecipitation combined with gel-free liquid chromatography-tandem mass spectrometry. These findings provide new insights into the pathogenesis of SSc.
JOURNAL OF AUTOIMMUNITY
(2023)
Review
Biochemistry & Molecular Biology
Hanna Grasshoff, Konstantinos Fourlakis, Sara Comduhr, Gabriela Riemekasten
Summary: Systemic sclerosis is a rare connective tissue disorder characterized by immune dysregulation, inflammation, fibrosis and vasculopathy. Alterations in the B-cell compartment, including polyclonal B-cell hyperreactivity and autoantibody production, are important in the pathogenesis of the disease. Autoantibodies serve as biomarkers and contribute to pathological mechanisms. Therapeutic approaches targeting B cells and autoantibodies may hold promise in the treatment of systemic sclerosis.
Article
Allergy
Gemma Lepri, Martina Catalano, Silvia Bellando-Randone, Serena Pillozzi, Elisa Giommoni, Roberta Giorgione, Cristina Botteri, Marco Matucci-Cerinic, Lorenzo Antonuzzo, Serena Guiducci
Summary: There is a well-known association between systemic sclerosis (SSc) and cancer, suggesting shared genetic and environmental risk factors. The use of immunosuppressive drugs in SSc may increase the risk of cancer occurrence, and certain serological features such as anti-RNA polymerase III and anti-topoisomerase I autoantibodies appear to be associated with increased cancer frequency in SSc patients. Additionally, lung fibrosis, long-standing SSc pulmonary involvement, and specific medication exposures have been linked to different types of cancer.
CLINICAL REVIEWS IN ALLERGY & IMMUNOLOGY
(2022)
Article
Medicine, General & Internal
Pawel Zebryk, Piotr Przymuszala, Jan Krzysztof Nowak, Tomasz Piorunek, Tatiana Mularek-Kubzdela, Mariusz Puszczewicz
Summary: This study evaluated the prevalence and clinical significance of SSc-related autoantibodies and compared the sensitivity of two line immunoblot assays. The findings indicate that different autoantibodies are associated with specific characteristics of SSc.
JOURNAL OF CLINICAL MEDICINE
(2023)
Article
Immunology
Aurelien Chepy, Solange Vivier, Fabrice Bray, Camille Ternynck, Jean-Pascal Meneboo, Martin Figeac, Alexandre Filiot, Lucile Guilbert, Manel Jendoubi, Christian Rolando, David Launay, Sylvain Dubucquoi, Guillemette Marot, Vincent Sobanski
Summary: This study investigated the effect of purified immunoglobulin G (IgG) from systemic sclerosis (SSc) patients on protein and mRNA expression of dermal fibroblasts (FBs) using a multi-omics approach. The results showed that SSc sera and purified IgG induced unique protein profile patterns, and this effect depended on the serotype of the patient.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Medicine, General & Internal
Fabian A. Mendoza, Anthony DiMarino, Sidney Cohen, Christopher Adkins, Shady Abdelbaki, Satish Rattan, Christopher Cao, Susie Denuna-Rivera, Sergio A. Jimenez
Summary: This article describes the successful treatment of two patients with SSc-associated swallowing dysfunction using IVIG, resulting in significant symptom improvement and improved esophageal motility.
JOURNAL OF CLINICAL MEDICINE
(2022)
Article
Medicine, General & Internal
Mariko Hara, Rumi Ueha, Taku Sato, Takao Goto, Ayumi Yoshizaki, Hayakazu Sumida, Shinichi Sato, Tatsuya Yamasoba
Summary: Systemic sclerosis is often associated with dysphagia and esophageal dysmotility. This study evaluated the association between dysphagia and esophageal dysmotility in patients with systemic sclerosis and identified risk factors. The results showed that dysphagia was associated with autoantibody positivity and older age, while there were no identified risk factors for esophageal dysmotility. No correlation was found between dysphagia and esophageal dysmotility.
JOURNAL OF CLINICAL MEDICINE
(2023)
Article
Rheumatology
Nina M. van Leeuwen, Corrie M. Wortel, Cynthia M. Fehres, Jaap A. Bakker, Hans U. Scherer, Rene E. M. Toes, Tom W. J. Huizinga, Jeska K. de Vries-Bouwstra
Summary: The study found an association between ACA and ATA autoimmune antibody responses and the degree of microangiopathy in SSc, indicating that the breadth of autoimmune response and microvascular damage interact in the pathophysiology of SSc.
JOURNAL OF RHEUMATOLOGY
(2021)
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)