Review
Rheumatology
Alain Lescoat, Robert D. Sandler, Francois Zimmermann, David Roofeh, Michael Hughes, John D. Pauling, Susan L. Murphy, Yen T. Chen, Whitney Townsend, Maya H. Buch, Dinesh Khanna
Summary: The aim of this study was to identify instruments used to assess lcSSc, and the results show that lcSSc is underrepresented in the literature.
Article
Health Care Sciences & Services
Chiara Pellicano, Amalia Colalillo, Giuseppina Cusano, Andrea Palladino, Marica Pellegrini, Cinzia Anna Maria Calla, Giorgia Mazzuccato, Valeria Carnazzo, Stefano Pignalosa, Luigi Di Biase, Mariapaola Marino, Umberto Basile, Edoardo Rosato
Summary: This study assessed the serum immunoglobulin G (IgG) subclasses in systemic sclerosis (SSc) patients and their influence on the main complications of the disease. The results showed that SSc patients had lower levels of total IgG, IgG1, and IgG3 compared to healthy controls. Logistic regression analysis found that IgG3 was associated with diffusing capacity of the lung for carbon monoxide (DLco), modified Rodnan skin score (mRSS), and radiological interstitial lung disease (ILD).
JOURNAL OF PERSONALIZED MEDICINE
(2023)
Article
Rheumatology
Alexis F. Guedon, Fabrice Carrat, Luc Mouthon, David Launay, Benjamin Chaigne, Gregory Pugnet, Jean-Christophe Lega, Arnaud Hot, Vincent Cottin, Christian Agard, Yannick Allanore, Anne-Laure Fauchais, Patrick Jego, Robin Dhote, Thomas Papo, Emmanuel Chatelus, Bernard Bonnotte, Jean-Emmanuel Khan, Elisabeth Diot, Boris Bienvenu, Nadine Magy-Bertrand, Viviane Queyrel, Alain Le Quellec, Pierre Kieffer, Zahir Amoura, Jean-Robert Harle, Jean-Baptiste Gaultier, Marie-Helene Balquet, Denis Wahl, Olivier Lidove, Olivier Fain, Arsene Mekinian, Eric Hachulla, Sebastien Riviere
Summary: This study investigated the prevalence and prognostic burden of heart involvement in SSc patients. The results showed that SSc-related cardiac involvement was associated with an increased risk of death, and diffuse SSc subtype and female sex were identified as risk factors. Further analysis should assess the potential impact of treatment on various cardiac outcomes.
Article
Rheumatology
Fulvia Ceccarelli, Carmelo Pirone, Carlo Perricone, Aikaterina Selntigia, Valeria Orefice, Viviana Antonella Pacucci, Simona Truglia, Francesca Romana Spinelli, Paola Galoppi, Cristiano Alessandri, Guido Valesini, Roberto Brunelli, Giuseppina Perrone, Fabrizio Conti
Summary: The study found that pregnancy outcomes of SLE patients were not significantly different from the control group of women without autoimmune diseases, except for a higher occurrence of small for gestational age. Disease flares during pregnancy and postpartum were associated with different factors, highlighting the importance of tailored prevention and management strategies. The study emphasized the positive impact of pre-gestational counseling and a multi-disciplinary approach on pregnancy outcomes for SLE patients.
Review
Immunology
Federica Maritati, Michele Provenzano, Sarah Lerario, Valeria Corradetti, Claudia Bini, Marco Busutti, Valeria Grandinetti, Vania Cuna, Gaetano La Manna, Giorgia Comai
Summary: Systemic sclerosis (SSc) is an immune-mediated rheumatic disease characterized by vascular abnormalities, tissue fibrosis, and inflammation. Renal involvement, specifically scleroderma renal crisis (SRC), is a severe complication of SSc. This review provides an overview of the recent advances in kidney transplantation (KT) for patients with end-stage renal disease (ESRD) due to SSc, with a focus on the risk of disease recurrence post-transplantation and the evolution of other disease manifestations.
FRONTIERS IN IMMUNOLOGY
(2022)
Review
Rheumatology
Robert Lafyatis, Eleanor Valenzi
Summary: The assessment of disease activity in systemic sclerosis (SSc) is challenging, and improved outcome measures are needed. Biomarker studies focused on skin disease may provide opportunities for earlier detection of therapeutic effect. By incorporating high-throughput sequencing and biomarkers, we can gain better understanding of the disease mechanisms in systemic sclerosis.
NATURE REVIEWS RHEUMATOLOGY
(2022)
Article
Medicine, General & Internal
Marco Di Battista, Simone Barsotti, Saverio Vitali, Marco Palma, Giammarco Granieri, Teresa Oranges, Giacomo Aringhieri, Valentina Dini, Alessandra Della Rossa, Emanuele Neri, Marco Romanelli, Marta Mosca
Summary: This study compared the results of modified Rodnan skin score (mRSS), durometry, and ultra-high frequency ultrasound (UHFUS) to evaluate skin involvement in systemic sclerosis (SSc) patients. Durometry showed a positive correlation with mRSS in most regions of interest. UHFUS revealed significant alterations in skin thickness and echogenicity in SSc patients compared to healthy controls (HC), but no correlations were found with mRSS or durometry.
Article
Rheumatology
Valdirene S. Siqueira, Mariely F. S. Helbingen, Ana Cristina Medeiros-Ribeiro, Henrique Carrico da Silva, Renata Miossi, Ana Paula Luppino-Assad, Percival D. Sampaio-Barros
Summary: This study analyzed the early characteristics of SSc in RP patients and identified predictors for the progression to SSc. Combinations of RP + PF + positive ANA + SD-NFC and/or SSc-specific antibody (VEDOSS level 2), as well as RP + PF + positive ANA (VEDOSS level 1; "red flags") were most associated with progression to SSc. Combinations without non-RP clinical symptoms, such as RP + SD-NFC and RP + anticentromere + SD-NFC, were associated with non-progression to SSc.
Article
Medicine, General & Internal
Benjamin Chaigne, Alexandre Bense, Christian Agard, Yannick Allanore, Gregory Pugnet, Eric Hachulla, Jerome Avouac, Boris Bienvenu, Sylvain Palat, Claire Grange, Sabine Berthier, Emmanuel Chatelus, Sebastien Riviere, Marie-Elise Truchetet, Jean-Emmanuel Kahn, Francois Maurier, Elisabeth Diot, Alice Berezne, Luc Mouthon
Summary: This study evaluated mouth opening (MO) in systemic sclerosis (SSc) patients and found that MO can predict disease severity and survival. Most SSc patients had stable MO, but diffuse cutaneous SSc (dcSSc) patients with high but decreasing MO were at risk of poor survival and ILD.
JOURNAL OF INTERNAL MEDICINE
(2023)
Article
Biochemistry & Molecular Biology
Alexandra Nagy, Erik Palmer, Lorinc Polivka, Noemi Eszes, Krisztina Vincze, Eniko Barczi, Aniko Bohacs, Adam Domonkos Tarnoki, David Laszlo Tarnoki, Gyoergy Nagy, Emese Kiss, Pal Maurovich-Horvat, Veronika Mueller
Summary: This study evaluated the clinical characteristics and predictors of lung function changes in SSc-ILD patients treated in a real-world setting. The results showed that untreated patients had the most prominent functional decline, and a normal body mass index was associated with a significant risk of deterioration. The majority of patients improved or were stable during follow-up.
Article
Rheumatology
Takashi Kida, Keiichi Matsuzaki, Isao Yokota, Nozomu Kawase, Masatoshi Kadoya, Hironori Inoue, Yuji Kukida, Shunya Kaneshita, Takuya Inoue, Makoto Wada, Masataka Kohno, Wataru Fukuda, Yutaka Kawahito, Taku Iwami
Summary: This study visualized the trajectories of pulmonary arterial pressure (PAP) in systemic sclerosis (SSc) using latent trajectory modeling and identified distinct clinical phenotypes for each trajectory. The study found that the pattern of PAP changes in SSc can be classified into five trajectories with different clinical characteristics and outcomes.
Article
Rheumatology
Qiang Li, Laura Wallace, Padmaja Patnaik, Margarida Alves, Martina Gahlemann, Veronika Kohlbrenner, Christina Raabe, Jocelyn R. Wang, Elizabeth M. Garry
Summary: The study found low prevalence estimates and incidence rates for SSc and SSc-ILD in the US, with newly diagnosed SSc-ILD patients receiving more immunosuppressive therapy and having more comorbidities compared to newly diagnosed SSc patients.
Article
Critical Care Medicine
Benjamin Thoreau, Marine Eustache, Adele Fievet, Gerard Lasfargues, Laurent Plantier, Elisabeth Diot
Summary: Occupational exposure to toxicants is independently associated with decline in FVC in patients with SSc, regardless of sex. Assessment of occupational exposure may be useful for SSc prognostication.
Article
Critical Care Medicine
Anna -Maria Hoffmann-Vold, Cathrine Brunborg, Paolo Airo, Lidia P. Ananyeva, Laszlo Czirjak, Serena Guiducci, Eric Hachulla, Mengtao Li, Carina Mihai, Gabriela Riemekasten, Petros P. Sfikakis, Gabriele Valentini, Otylia Kowal-Bielecka, Yannick Allanore, Oliver Distler
Summary: This study aimed to test whether enrichment strategies from clinical trials for progressive systemic sclerosis-associated interstitial lung disease (SSc-ILD) affect efficacy, representativeness, and feasibility. The researchers applied the inclusion criteria of major recent SSc-ILD trials to patients with SSc-ILD from the European Scleroderma Trials and Research (EUSTAR) database. The results showed that patient populations that fulfilled different study inclusion criteria significantly differed in various clinical parameters, and the application of enrichment criteria did not have a significant impact on ILD progression.
Article
Rheumatology
Sophie I. E. Liem, Jacopo Ciaffi, Nina Marijn van Leeuwen, Maaike Boonstra, Saad Ahmed, Liesbeth J. J. van de Voorde, Anja Corsel, Talitha Dhondai, Maarten K. Ninaber, J. J. Miranda Geelhoed-Veltman, Marlies E. Heuvers, Maarten E. Tushuizen, Nina Ajmone Marsan, Philippine Kies, Anne A. Schouffoer, Tom W. J. Huizinga, Cornelia F. Allaart, Jeska De Vries-Bouwstra
Summary: The Leiden Combined Care in Systemic Sclerosis (CCISS) cohort in the Netherlands observed a decrease in disease duration at cohort entry, indicating a potentially more timely diagnosis of SSc and opportunities for early interventions. While symptom duration at presentation is longer in females, mortality rate is consistently higher in males, highlighting the need for sex-specific treatment and follow-up.
Article
Rheumatology
Ariane Barbacki, Murray Baron, Mianbo Wang, Yuqing Zhang, Wendy Stevens, Joanne Sahhar, Susanna Proudman, Mandana Nikpour, Ada Man
Summary: This study found distinct trajectories of damage accrual in patients with systemic sclerosis, with age, gender, disease type, tendon friction rubs, and C-reactive protein level being associated with the different trajectories. These findings may be helpful in identifying patients who require early aggressive treatment.
ARTHRITIS CARE & RESEARCH
(2023)
Article
Rheumatology
Paul R. Fortin, Carolyn Neville, Anne-Sophie Julien, Elham Rahme, Vinita Haroun, Jodie Nimigon-Young, Anna-Lisa Morrison, Davy Eng, Christine A. Peschken, Evelyne Vinet, Marie Hudson, Doug Smith, Mark Matsos, Janet E. Pope, Ann E. Clarke, Stephanie Keeling, J. Antonio Avina-Zubieta, Murray Rochon, Deborah Da Costa
Summary: This study aimed to evaluate the effects of the web-based program MyLupusGuide on self-management in SLE patients. The results showed that MyLupusGuide increased self-efficacy but did not significantly improve patient activation. 56% of participants visited the MyLupusGuide site.
ARTHRITIS CARE & RESEARCH
(2023)
Article
Rheumatology
Ernest Choy, Vivian Bykerk, Yvonne C. Lee, Hubert van Hoogstraten, Kerri Ford, Amy Praestgaard, Serge Perrot, Janet Pope, Anthony Sebba
Summary: This study assessed the effects of IL-6 inhibitor sarilumab on disproportionate articular pain (DP) in patients with rheumatoid arthritis (RA). The results showed that about one-quarter of RA patients experienced DP and sarilumab treatment had a positive effect on DP.
Article
Psychiatry
Simone N. Vigod, Natalie Urbach, Andrew Calzavara, Cindy-Lee Dennis, Andrea Gruneir, Brett D. Thombs, Mark Walker, Hilary K. Brown
Summary: A risk index for common postpartum mental health disorders was created and internally validated using health administrative data in Ontario, Canada. The index was developed based on easily collectible variables from hospital birth records and could estimate individual-level risk of developing these disorders.
BRITISH JOURNAL OF PSYCHIATRY
(2023)
Review
Rheumatology
Janet E. E. Pope, Christopher P. P. Denton, Sindhu R. R. Johnson, Andreu Fernandez-Codina, Marie Hudson, Tatiana Nevskaya
Summary: Pope et al. review the current management of organ-based manifestations of systemic sclerosis, including screening and treatment. The treatment for systemic sclerosis has improved, with immunosuppressive agents and autologous haematopoietic stem cell transplantation showing promise. Therapies for interstitial lung disease and pulmonary arterial hypertension have also improved, with mycophenolate mofetil surpassing cyclophosphamide as the initial treatment for SSc-interstitial lung disease. Research is needed to develop targeted and effective treatments for other manifestations and to establish best practices for screening and early intervention.
NATURE REVIEWS RHEUMATOLOGY
(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)
Review
Rheumatology
Laura Ross, Nancy Maltez, Michael Hughes, Jan W. Schoones, Murray Baron, Lorinda Chung, Dilia Giuggioli, Pia Moinzadeh, Yossra A. Suliman, Corrado Campochiaro, Yannick Allanore, Christopher P. Denton, Oliver Distler, Tracy Frech, Daniel E. Furst, Dinesh Khanna, Thomas Krieg, Masataka Kuwana, Marco Matucci-Cerinic, Janet Pope, Alessia Alunno
Summary: A systematic literature review found that intravenous iloprost, phosphodiesterase-5 inhibitors, and atorvastatin are effective for the treatment of SSc digital ulcers. Bosentan can reduce the occurrence of future ulcers. Limited evidence supports the effectiveness of Janus kinase inhibitors, while immunosuppression or anti-platelet agents have insufficient data to support their use. Further research is needed to define the optimal treatment regimen.
Letter
Rheumatology
Janet E. Pope
NATURE REVIEWS RHEUMATOLOGY
(2023)
Letter
Psychiatry
Felix Fischer, Dario Zocholl, Geraldine Rauch, Brooke Levis, Andrea Benedetti, Brett Thombs, Matthias Rose, Polychronis Kostoulas
Article
Psychiatry
Felix Fischer, Dario Zocholl, Geraldine Rauch, Brooke Levis, Andrea Benedetti, Brett Thombs, Matthias Rose, Polychronis Kostoulas
Summary: By re-analyzing the data from the European Health Interview Survey (EHIS) and accounting for the imperfect diagnostic accuracy of the PHQ-8, it was found that the prevalence of major depression in European countries is lower than previously reported.
Article
Rheumatology
Matthew Wong-Pack, Elliot Hepworth, Mohammad Movahedi, Bindee Kuriya, Janet Pope, Edward Keystone, Carter Thorne, Vandana Ahluwalia, Angela Cesta, Carol Mously, Claire Bombardier, Arthur Lau, Sibel Zehra Aydin
Summary: This study examined the impact of the COVID-19 pandemic on RA patients' outcomes and medication profiles, and found that disease activity and PROs remained stable during the pandemic, while there were changes in medication use.
RHEUMATOLOGY ADVANCES IN PRACTICE
(2023)
Article
Rheumatology
E. Rampakakis, C. Thorne, A. Cesta, M. Movahedi, X. Li, C. Mously, V. Ahluwalia, J. Brophy, P. Ciaschini, E. Keystone, A. Lau, G. Major, V. Pavlova, J. Pope, C. Bombardier
Summary: This study examines the prevalence of medical cannabis use and rheumatologists' perceptions among patients attending rheumatology clinics in Ontario. The results showed a high proportion of medical cannabis users, who were younger and had more severe symptoms compared to non-users. Pain, sleep disorders, and anxiety were the most commonly treated symptoms. Most rheumatologists reported being uncomfortable to authorize medical cannabis due to lack of evidence, knowledge, and product standardization.
CLINICAL AND EXPERIMENTAL RHEUMATOLOGY
(2023)
Review
Rheumatology
Arpita Gantayet, Paul Plantinga, Janet E. Pope
Summary: Gynecologic presentations of GPA are rare, especially in the uterus, fallopian tube, ovary, and vagina. Diagnosis of GPA is difficult in the context of negative ANCA and unusual organ presentations. It is important to maintain a clinical suspicion for GPA in the right clinical context.
CURRENT TREATMENT OPTIONS IN RHEUMATOLOGY
(2023)
Article
Rheumatology
R. S. Henry, L. Kwakkenbos, M. -E. Carrier, S. Patten, S. J. Bartlett, L. Mouthon, J. Varga, A. Benedetti, B. D. Thombs
Summary: The study evaluated mental health symptom trajectories in people with systemic sclerosis (SSc) during the COVID-19 pandemic, showing that anxiety symptoms initially increased in early 2020 but quickly returned to pre-COVID-19 levels, while depression symptoms remained stable throughout the pandemic. Fear of COVID-19 was high at first but decreased over time, and loneliness did not significantly change during the pandemic.
CLINICAL AND EXPERIMENTAL RHEUMATOLOGY
(2023)