Article
Rheumatology
Melani Pestana-Fernandez, Manuel Rubio-Rivas, Carles Tolosa-Vilella, Alfredo Guillen-Del-Castillo, Dolores Colunga-Arguelles, Ana Argibay, Begona Mari-Alfonso, Adela Marin-Ballve, Xavier Pla-Salas, Antonio-J Chamorro, Antoni Castro-Salomo, Ana Belen Madronero-Vuelta, Maria Esther Sanchez-Garcia, Luis Saez-Comet, Cristina Gonzalez-Echavarri, Norberto Ortego-Centeno, Jose Antonio Vargas-Hitos, Jose Antonio Todoli-Parra, Luis Trapiella-Martinez, Gema Maria Lledo, Mayka Freire, Vicent Fonollosa-Pla, Carmen Pilar Simeon-Aznar
Summary: There was no significant difference in the incidence rate of PAH and SRC between patients under ERAs/PDE5i treatment and those without treatment, but treated patients experienced a delayed occurrence of SRC.
Article
Cardiac & Cardiovascular Systems
Ruchika A. Sangani, Justin K. Lui, Kari R. Gillmeyer, Marcin A. Trojanowski, Andreea M. Bujor, Michael P. LaValley, Elizabeth S. Klings
Summary: The study aimed to investigate the clinical features of patients with systemic sclerosis complicated by pulmonary hypertension (SSc-PH) and interstitial lung disease (SSc-PH-ILD), and analyze how the severity of ILD and PH contribute to the mortality rate in SSc patients. The results showed that severe ILD was associated with increased hazards for all-cause mortality in patients with SSc-PH, while mild and moderate ILD had no impact on mortality risk. In patients with SSc-ILD, both moderate and severe PH were associated with increased mortality risk, while mild PH had no impact. The findings suggest that early intervention may benefit disease progression.
PULMONARY CIRCULATION
(2022)
Article
Rheumatology
Hideaki Tsuji, Nobuo Kuramoto, Tsuneo Sasai, Mirei Shirakashi, Hideo Onizawa, Koji Kitagori, Shuji Akizuki, Ran Nakashima, Ryu Watanabe, Akira Onishi, Kosaku Murakami, Hajime Yoshifuji, Masao Tanaka, Motomu Hashimoto, Koichiro Ohmura, Akio Morinobu
Summary: Specific SSc-related autoantibodies are associated with the morbidity and mortality of SRC. Anti-topoisomerase I, anti-RNAPIII, and anti-U1-RNP are associated with the development of SRC, while anti-topoisomerase I is associated with 1-year mortality of SRC.
Article
Radiology, Nuclear Medicine & Medical Imaging
Shivani Ahlawat, Julie Paik, Filippo Del Grande, Elias S. Paris, Parvinder Sujlana, Laura M. Fayad
Summary: In patients with fibrosing systemic sclerosis-associated myopathy, intramuscular and fascial edema are more common on MRI, while patients with non-fibrosing myopathy often exhibit fatty replacement and atrophy.
Article
Multidisciplinary Sciences
Qian Lei, Zhimin Yu, Hang Li, Jun Cheng, Yanggan Wang
Summary: This study reveals the significant role of fatty acid-binding protein 5 (FABP5) in pulmonary hypertension secondary to left heart disease (PH-LHD). Through experiments and in vitro studies, it was demonstrated that inhibiting FABP5 can improve pulmonary vascular remodeling and cardiac function. FABP5 may serve as a potential therapeutic target for PH-LHD.
JOURNAL OF ADVANCED RESEARCH
(2022)
Article
Clinical Neurology
Tugce Yakut, Baran Balcan, Sait Karakurt, Haner Direskeneli, Yasemin Yalcinkaya, Yuksel Peker
Summary: The study suggests that patients with scleroderma who also have obstructive sleep apnea (OSA) have an increased risk for enlarged main pulmonary artery diameter, independent of pulmonary involvement.
SLEEP AND BREATHING
(2021)
Article
Emergency Medicine
Mohammed Uddin, Tanveer Mir, Sarvani Surapaneni, Anupamdeep Mehar, Tawseef Dar, Khalid Changal, Waqas Ullah, Prateek Lohia, Zeenat Bhat, Mujeeb Sheikh, Mark Burket
Summary: This study analyzed the mortality rate of scleroderma renal crisis (SRC) among systemic sclerosis (SSc) patients in US emergency departments. The study found that SRC is a relatively rare medical emergency, and although the incidence rate has increased significantly, the mortality rate remains steady. Compared to non-SRC patients, those with SRC have more complications, particularly congestive heart failure. Therefore, SSc patients with congestive heart failure should be considered for early admission to inpatient services.
AMERICAN JOURNAL OF EMERGENCY MEDICINE
(2022)
Article
Respiratory System
F. Zeba, W. Yanning, J. Melek, F. Duan, M. K. Atalay, M. Jankowich, S. Rounds
Summary: Our study found that PA/AA ratio > 1 is associated with decreased survival in individuals with pulmonary fibrosis, with or without emphysema. Additionally, the study describes markers of cardiac remodeling and the echocardiographic diagnosis of PH in this patient population.
Review
Immunology
Manuel Rubio-Rivas, Narcis A. Homs, Daniel Cuartero, Xavier Corbella
Summary: The overall PAH prevalence found was 6.4% (95%CI 5%-8.3%) and the overall PAH incidence 18.2 cases per 1000 person-years (95%CI 12-27.4). Studies on lcSSc and dcSSc showed different prevalence rates, with lcSSc at 7.7% (95% CI 5.3%-11.1%) and dcSSc at 6.3% (95% CI 4.5%-8.9%). Overall, the incidence of PAH in SSc patients was found to be 18.2 cases per 1000 person-years.
AUTOIMMUNITY REVIEWS
(2021)
Article
Rheumatology
Kimberly Showalter, Laura C. Pinheiro, Deanna Jannat-Khah, Irina Sobol, Jackie Szymonifka, Jackie Finik, Virginia D. Steen, Jessica K. Gordon
Summary: This study identified predictors of hospital readmission among individuals with SSc-associated pulmonary hypertension (SSc-PH). The strongest predictor was the reason for the initial hospitalization being related to PH. Home oxygen use was associated with a lower likelihood of readmission.
Article
Cardiac & Cardiovascular Systems
Andrew Constantine, Konstantinos Dimopoulos
Summary: Pulmonary artery denervation is a novel intervention aimed at reducing sympathetic activity in the pulmonary circulation for the treatment of pulmonary arterial hypertension. Despite some positive effects shown in pre-clinical studies and initial clinical trials, the technique's scope in current practice remains limited due to the lack of well-designed, large-scale, international randomized controlled clinical trials.
TRENDS IN CARDIOVASCULAR MEDICINE
(2021)
Article
Allergy
Alice Cole, Voon H. Ong, Christopher P. Denton
Summary: Scleroderma renal crisis (SRC) is a life-threatening complication of systemic sclerosis (SSc) with a high mortality rate. Although angiotensin-converting enzyme inhibitor therapy has improved renal outcomes, there are currently no preventative measures and patients may rapidly decline. Studies have identified specific phenotypes at higher risk of developing SRC and novel urinary biomarkers may aid in early identification and treatment. Clear guidelines for management have been established to standardize care and promote collaboration between rheumatology and renal physicians. While outcomes following renal transplant have improved, further research is needed to understand the mechanisms and develop new therapies for SRC.
CLINICAL REVIEWS IN ALLERGY & IMMUNOLOGY
(2023)
Review
Rheumatology
George Iliopoulos, Dimitrios Daoussis
Summary: Research has shown limited data about renal impairment in SSc beyond scleroderma renal crisis (SRC). However, studies indicate that renal dysfunction in SSc is not uncommon, with a significant percentage of patients having decreased glomerular filtration rate (GFR). The Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI) formula is suggested as a suitable tool for assessing renal function in SSc patients.
RHEUMATOLOGY INTERNATIONAL
(2021)
Article
Medicine, General & Internal
Yassmin Khadra, Jennifer G. Walker, Paul Hakendorf, Susanna Proudman, Maureen Rischmueller, Catherine L. Hill, Samuel Whittle, Eliza Pontifex, Peter J. Roberts-Thomson
Summary: SRC is a rare but severe complication with a stable incidence of 0.58/million/year in South Australia. Patients have poor survival outcomes, often requiring renal replacement therapy and transplantation. Nailfold capillaroscopy reveals extensive microvascular damage. No improvement in survival was observed over the 34-year study period.
INTERNAL MEDICINE JOURNAL
(2021)
Article
Cardiac & Cardiovascular Systems
Rubin Tan, Qiang You, Dongdong Yu, Chushu Xiao, Joseph Adu-Amankwaah, Jie Cui, Ting Zhang
Summary: In this study, we identified novel common hub genes involved in pulmonary artery remodeling in different PH groups through the analysis of gene expression data. We verified the top 10 candidate genes through animal model validation. This study provides new target genes for further research on the mechanisms and treatment of PH.
FRONTIERS IN CARDIOVASCULAR MEDICINE
(2022)
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
Tatiana Nevskaya, Leonardo M. Calderon, Murray Baron, Janet E. Pope
Summary: Digital ulcers in systemic sclerosis patients lead to increased utilization of healthcare resources, including tests, visits, and aids. Even after adjusting for disease severity in other organ systems, the presence of digital ulcers remains a significant predictor of more frequent physician visits and more tests.
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
Rheumatology
Murray Baron, Ariane Barbacki, Ada Man, J. K. de Vries-Bouwstra, Dylan Johnson, Wendy Stevens, Mohammed Osman, Mianbo Wang, Yuqing Zhang, Joanne Sahhar, Gene-Siew Ngian, Susanna Proudman, Mandana Nikpour
Summary: A prediction model was developed to forecast damage accrual in early SSc patients. The study found that the trajectories of damage accumulation were distinct for limited cutaneous SSc (lcSSc) and diffuse cutaneous SSc (dcSSc). By using baseline damage index (DI) and sex as predictive factors, the model demonstrated excellent performance, with ROC AUC values of 0.9313 for lcSSc and 0.9027 for dcSSc. The predicted "good" and "bad" cases showed clear differences in their actual trajectories in both derivation and validation cohorts.
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.
Article
Rheumatology
Hsin Yen Liu, Gemma Cramarossa, Janet E. Pope
Summary: This study investigated the pattern and risk factors for antimalarial-induced retinopathy in patients with rheumatic diseases. The risk of retinopathy increased after 5 years of antimalarial therapy. SLE diagnosis, high dosages of antimalarial drugs, and long-term use were associated with higher rates of toxicity, and SLE may be an independent risk factor.
ACR OPEN RHEUMATOLOGY
(2023)
Letter
Rheumatology
Janet E. Pope
NATURE REVIEWS RHEUMATOLOGY
(2023)
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)
