Review
Biochemistry & Molecular Biology
Beatrice Moccaldi, Laura De Michieli, Marco Binda, Giulia Famoso, Roberto Depascale, Martina Perazzolo Marra, Andrea Doria, Elisabetta Zanatta
Summary: CTD-PAH is a life-threatening complication of connective tissue diseases characterized by increased pulmonary arterial pressure and resistance. Due to non-specific symptoms and lack of consensus on screening strategies, CTD-PAH is often diagnosed at an advanced stage. Non-invasive tools, particularly novel serum biomarkers, may improve early diagnosis and disease monitoring of CTD-PAH.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Pharmacology & Pharmacy
Jiang-Shan Tan, Song Hu, Ting-Ting Guo, Lu Hua, Xiao-Jian Wang
Summary: By text mining and functional enrichment analysis, we identified potential drugs that may have therapeutic effects on connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH).
FRONTIERS IN PHARMACOLOGY
(2022)
Review
Medicine, General & Internal
Weizhen Xiang, Rongrong Dong, Meiqi Li, Baocheng Liu, Zhenzhen Ma, Qingrui Yang
Summary: Connective tissue disease (CTD) patients with positive anti-U1 ribonucleoprotein (RNP) antibodies are at high risk for pulmonary arterial hypertension (PAH), and these antibodies are also associated with decreased mortality in CTD-PAH patients.
JOURNAL OF CLINICAL MEDICINE
(2023)
Article
Rheumatology
Y. Zhang, Q. Jin, Z. Zhao, Q. Zhao, X. Yu, L. Yan, X. Li, C. An, X. Ma, C. Xiong, Q. Luo, Z. Liu
Summary: CTD-PAH patients had higher rest heart rate, and lower values of various physiological parameters during exercise compared to IPAH patients. Even after adjustment, CTD-PAH still demonstrated poorer exercise tolerance and cardiovascular function than IPAH.
CLINICAL AND EXPERIMENTAL RHEUMATOLOGY
(2021)
Review
Rheumatology
Yufang Ding, Junyan Qian, Shangzhu Zhang, Dong Xu, Xiaomei Leng, Jiuliang Zhao, Qian Wang, Wen Zhang, Xinping Tian, Mengtao Li, Xiaofeng Zeng
Summary: This systematic review evaluates the efficacy of immunosuppressive therapy in patients with connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) and finds that immunosuppressive therapy has a certain efficacy in CTD-PAH, especially in SLE-PAH patients. However, further research is needed on biological agents and the therapeutic effect of different immunosuppressants.
INTERNATIONAL JOURNAL OF RHEUMATIC DISEASES
(2022)
Article
Respiratory System
Xingbei Dong, Yue Shi, Ying Xia, Xiao Zhang, Junyan Qian, Jiuliang Zhao, Jinmin Peng, Qian Wang, Li Weng, Mengtao Li, Bin Du, Xiaofeng Zeng
Summary: This study investigates the diversity of hemodynamic types of connective tissue disease associated pulmonary hypertension (CTD-PH), their differences in clinical characteristics and outcomes. The results show that different types of CTD-PH present different clinical phenotypes and outcomes, highlighting the importance of phenotyping PH in CTD-PH patients.
BMC PULMONARY MEDICINE
(2022)
Article
Medicine, General & Internal
Mattia Bellan, Francesco Murano, Federico Ceruti, Cristina Piccinino, Stelvio Tonello, Rosalba Minisini, Ailia Giubertoni, Daniele Sola, Roberta Pedrazzoli, Veronica Maglione, Giulia Francesca Manfredi, Antonio Acquaviva, Roberto Piffero, Giuseppe Patti, Mario Pirisi, Pier Paolo Sainaghi
Summary: This study found that the concentrations of ICOS and ICOS-L in the serum of patients with pulmonary arterial hypertension (PAH) were higher, regardless of the underlying cause. When compared to CTD patients, CTD-PAH patients had higher concentrations of ICOS and ICOS-L. Logistic regression analysis showed that ICOS and ICOS-L were associated with a diagnosis of PAH. ICOS and ICOS-L may serve as promising candidate biomarkers for the diagnostic screening of PAH among CTD patients.
Article
Health Care Sciences & Services
Chun-Hsin Wu, Chun-Yu Lin, Chih-Hsin Hsu, Sheng-Hsiang Lin, Chia-Tse Weng
Summary: The study explored the differences in clinical features and outcomes between idiopathic PAH (iPAH) and connective tissue disease-related PAH (CTD-PAH) in Taiwanese patients. It was found that CTD-PAH patients had higher levels of NT-proBNP and lower DLCO compared to iPAH patients. The mortality rates were similar between CTD-PAH and iPAH, with a higher mean pulmonary arterial pressure (mPAP) being a predictor of PAH-induced mortality.
Article
Medicine, General & Internal
Chao Qu, Wei Feng, Qi Zhao, Qi Liu, Xing Luo, Gang Wang, Meng Sun, Zhibo Yao, Yufei Sun, Shenglong Hou, Chunyang Zhao, Ruoxi Zhang, Xiufen Qu
Summary: This study examined the effect of levosimendan on acute decompensated right heart failure (RHF) in patients with connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH). The results showed that levosimendan treatment effectively improved the condition of patients and had positive effects on survival in hospital.
FRONTIERS IN MEDICINE
(2022)
Article
Health Care Sciences & Services
Tsung-Yuan Yang, Yong-Hsin Chen, Wun-Zhih Siao, Gwo-Ping Jong
Summary: This article reports on a 58-year-old male patient who developed pulmonary arterial hypertension (PAH) confirmed by echocardiography and right cardiac catheterization 32 years after the diagnosis of ankylosing spondylitis (AS). This is the first detailed case description of AS associated with PAH. Physicians are recommended to be aware of this rare comorbidity in AS patients, and early echocardiographic screening is necessary for symptomatic patients.
JOURNAL OF PERSONALIZED MEDICINE
(2023)
Review
Pharmacology & Pharmacy
Mithum Kularatne, Athenais Boucly, Laurent Savale, Sabina Solinas, Celine Cheron, Anne Roche, Mitja Jevnikar, Xavier Jais, David Montani, Marc Humbert, Olivier Sitbon
Summary: Despite advances in treatment, patients with CTD-associated PAH still have poorer survival compared to those with idiopathic PAH. This review discusses the factors contributing to disparate outcomes and explores potential improvements for increasing quality of life and survival in CTD-associated PAH patients.
EXPERT OPINION ON PHARMACOTHERAPY
(2023)
Article
Chemistry, Medicinal
Mattia Bellan, Cristina Piccinino, Stelvio Tonello, Rosalba Minisini, Ailia Giubertoni, Daniele Sola, Roberta Pedrazzoli, Ileana Gagliardi, Erika Zecca, Elisa Calzaducca, Federica Mazzoleni, Roberto Piffero, Giuseppe Patti, Mario Pirisi, Pier Paolo Sainaghi
Summary: This study aimed to evaluate the role of plasma osteopontin (OPN) as a candidate biomarker of PAH in CTD patients, and found that OPN was significantly associated with PAH among CTD patients, suggesting it may have potential as a non-invasive disease biomarker for PAH.
Article
Rheumatology
Yasuyuki Todoroki, Minoru Satoh, Satoshi Kubo, Shunpei Kosaka, Shunsuke Fukuyo, Keisuke Nakatsuka, Kazuyoshi Saito, Shin Tanaka, Shingo Nakayamada, Yoshiya Tanaka
Summary: This study evaluated the clinical relevance of anti-survival motor neuron (SMN) complex antibodies in patients with mixed connective tissue disease (MCTD). The results showed that the detection rate of anti-SMN complex antibodies was significantly higher in MCTD patients compared to those with systemic lupus erythematosus (SLE) and systemic sclerosis (SSc). Furthermore, MCTD patients positive for anti-SMN complex antibodies had a higher prevalence of pulmonary arterial hypertension (PAH) and interstitial lung disease (ILD).
Article
Cardiac & Cardiovascular Systems
Megan M. Lowery, Nicholas S. Hill, Lu Wang, Erika B. Rosenzweig, Aparna Bhat, Serpil Erzurum, J. Emanuel Finet, Christine L. Jellis, Sunjeet Kaur, Deborah H. Kwon, Rawan Nawabit, Milena Radeva, Gerald J. Beck, Robert P. Frantz, Paul M. Hassoun, Anna R. Hemnes, Evelyn M. Horn, Jane A. Leopold, Franz P. Rischard, Reena Mehra
Summary: In this study, the association between sleep-related hypoxia and apnea-hypopnea index (AHI) with right ventricular (RV) function and survival in patients with group 1 pulmonary arterial hypertension (PAH) was prospectively evaluated. The results showed that sleep-related hypoxia was more strongly associated than AHI with measures of RV dysfunction, death, or transplantation overall and in group 1 non-CTD-associated PAH but only with RV dysfunction in CTD-associated PAH.
JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY
(2023)
Article
Cardiac & Cardiovascular Systems
Kellen Hirsch, Stephanie Nolley, David D. Ralph, Ying Zheng, William A. Altemeier, Christopher J. Rhodes, Nicholas W. Morrell, Martin R. Wilkins, Peter J. Leary, Samuel G. Rayner
Summary: This study compared the differences in inflammatory and angiogenic biomarker profiles among patients with different etiologies of pulmonary arterial hypertension (PAH) and their association with clinical outcomes. The results showed that patients with connective tissue disease-associated PAH (CTD-PAH) had significantly elevated levels of inflammatory and angiogenic biomarkers. Six biomarkers were associated with mortality in the entire PAH cohort, with IL-6 and NT-proBNP remaining significant after controlling for multiple comparisons.
JOURNAL OF HEART AND LUNG TRANSPLANTATION
(2023)
Article
Education, Scientific Disciplines
Brendan W. Munzer, Max Griffith, Whitney A. Townsend, Jesse Burk-Rafel
Article
Rheumatology
Amber Young, Vivek Nagaraja, Mark Basilious, Mirette Habib, Whitney Townsend, Heather Gladue, David Badesch, J. Simon R. Gibbs, Deepa Gopalan, Alessandra Manes, Ronald Oudiz, Toru Satoh, Adam Torbicki, Fernando Torres, Vallerie McLaughlin, Dinesh Khanna
SEMINARS IN ARTHRITIS AND RHEUMATISM
(2019)
Review
Oncology
Nauzley C. Abedini, Rachel K. Hechtman, Achintya D. Singh, Rafina Khateeb, Jason Mann, Whitney Townsend, Vineet Chopra
Review
Health Care Sciences & Services
Matthew R. Carey, Valerie M. Vaughn, Jason Mann, Whitney Townsend, Vineet Chopra, Payal K. Patel
JOURNAL OF GENERAL INTERNAL MEDICINE
(2020)
Review
Medicine, General & Internal
Alain Lescoat, David Roofeh, Whitney Townsend, Michael Hughes, Robert D. Sandler, Francois Zimmermann, John D. Pauling, Maya H. Buch, Dinesh Khanna
Summary: Limited cutaneous systemic sclerosis (lcSSc) is the most frequent subset of systemic sclerosis, yet lacks focus in clinical studies. The objective of this scoping review is to identify the outcome measures used to assess lcSSc.
Review
Cardiac & Cardiovascular Systems
Carmel Ashur, Thomas M. Cascino, Christopher Lewis, Whitney Townsend, Ananda Sen, Dorothy Pekmezi, Caroline R. Richardson, Elizabeth A. Jackson
Summary: The study found that the use of activity trackers among cardiac rehabilitation participants led to significant increases in daily step count and aerobic capacity compared to controls. Larger trials are needed to further assess the effectiveness of activity trackers in cardiac rehabilitation programs.
JOURNAL OF CARDIOPULMONARY REHABILITATION AND PREVENTION
(2021)
Review
Gastroenterology & Hepatology
Emily Harding-Theobald, Jeremy Louissaint, Bharat Maraj, Edward Cuaresma, Whitney Townsend, Mishal Mendiratta-Lala, Amit G. Singal, Grace L. Su, Anna S. Lok, Neehar D. Parikh
Summary: Studies have shown that radiomic features have good discriminatory performance in differentiating HCC from other solid lesions, predicting microvascular invasion, early recurrence after hepatectomy, and prognosis after therapies. However, the overall quality of the included studies was low due to common deficiencies in internal and external validation, standardized imaging segmentation, and comparison to a gold standard. Therefore, standardization of protocols and outcome measurements, sharing of algorithms and analytic methods, and external validation are necessary before widespread application of radiomics in HCC diagnosis and prognosis in clinical practice.
ALIMENTARY PHARMACOLOGY & THERAPEUTICS
(2021)
Review
Rheumatology
David Roofeh, Shaney L. Barratt, Athol U. Wells, Leticia Kawano-Dourado, Donald Tashkin, Vibeke Strand, James Seibold, Susanna Proudman, Kevin K. Brown, Paul F. Dellaripa, Tracy Doyle, Thomas Leonard, Eric L. Matteson, Chester Oddis, Joshua J. Solomon, Jeffrey A. Sparks, Robert Vassallo, Lara Maxwell, Dorcas Beaton, Robin Christensen, Whitney Townsend, Dinesh Khanna
Summary: The OMERACT organization focuses on improving health care outcomes for patients with autoimmune and musculoskeletal diseases. The CTD-ILD Working Group within OMERACT aims to identify outcome measures for studies of patients with CTD-ILD. The study summarized evidence from various sources to determine the use of lung physiology measures in SSc-ILD studies.
SEMINARS IN ARTHRITIS AND RHEUMATISM
(2021)
Review
Cardiac & Cardiovascular Systems
Michael J. Pienta, Supriya Shore, Tessa M. F. Watt, Gardner Yost, Whitney Townsend, Lourdes Cabrera, Michael D. Fetters, Carol Chenoweth, Keith Aaronson, Francis D. Pagani, Donald S. Likosky
Summary: This review summarizes 31 studies that examined patient-related factors associated with infection after LVAD implantation, showing that patient-related comorbidities, especially body composition and diabetes, were most commonly evaluated, but there was no consistent association with infections in LVAD recipients.
JOURNAL OF HEART AND LUNG TRANSPLANTATION
(2022)
Review
Cardiac & Cardiovascular Systems
Supriya Shore, Michael J. Pienta, Tessa M. F. Watt, Gardner Yost, Whitney A. Townsend, Lourdes Cabrera, Michael D. Fetters, Carol Chenoweth, Keith D. Aaronson, Francis D. Pagani, Donald S. Likosky
Summary: This study analyzed factors related to infection in LVAD recipients, revealing that only increasing center experience and establishing a silicone-skin interface at the driveline exit site were consistently associated with lower infection risk.
JOURNAL OF HEART AND LUNG TRANSPLANTATION
(2022)
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.
Review
Medicine, General & Internal
Jawad Al-Khafaji, Ryan F. Townsend, Whitney Townsend, Vineet Chopra, Ashwin Gupta
Summary: This study aimed to apply a human factors framework to analyze the effectiveness of clinical checklists in reducing diagnostic errors. The study found that most checklists have limited human factors components, and checklists that focus on tasks are more likely to be associated with error reduction.
Article
Oncology
Andrew M. Brown, Ihab Kassab, Marco Massani, Whitney Townsend, Amit G. Singal, Cigdem Soydal, Laura Moreno-Luna, Lewis R. Roberts, Vincent L. Chen, Neehar D. Parikh
Summary: This study conducted an overall and individual patient data (IPD) meta-analysis of transarterial radioembolization (TARE) and transarterial chemoembolization (TACE) for the treatment of hepatocellular carcinoma (HCC). The results showed that TARE had a significantly longer time to progression (TTP) compared to TACE, but there was no significant difference in overall survival (OS) between the two modalities. The limitations of the current data include inconsistent length of follow-up and response criteria. Therefore, prospective studies comparing these treatments are needed.
Review
Rheumatology
Alain Lescoat, Susan L. Murphy, David Roofeh, John D. Pauling, Michael Hughes, Robert Sandler, Francois Zimmermann, Rachel Wessel, Whitney Townsend, Lorinda Chung, Christopher P. Denton, Peter A. Merkel, Virginia Steen, Yannick Allanore, Francesco Del Galdo, Dominique Godard, David Cella, Sue Farrington, Maya H. Buch, Dinesh Khanna
Summary: Systemic sclerosis presents with a diverse range of clinical manifestations and is classified into limited and diffuse cutaneous subgroups. While diffuse cutaneous systemic sclerosis has been the focus of clinical trials, limited cutaneous systemic sclerosis also has significant morbidity and impact on quality of life. The lack of interventional studies for limited cutaneous systemic sclerosis is partly due to a lack of outcome measures, but a proposed composite index specifically for this subgroup could improve clinical trial representation and response to outcomes.
JOURNAL OF SCLERODERMA AND RELATED DISORDERS
(2021)
Review
Rheumatology
Sohail Farshad, Alexandra Halalau, Whitney Townsend, Elena Schiopu
ACR OPEN RHEUMATOLOGY
(2020)
Article
Rheumatology
Walter P. Maksymowych, Nele Herregods, Nisha Varma, Arthur B. Meyers, Jennifer Stimec, Andrea S. Doria, Nikolay Tzaribachev, Tarimobo M. Otobo, Marion A. van Rossum, Joel Paschke, Stephanie Wichuk, Robert G. Lambert
Summary: This study aimed to investigate whether systematic calibration improves the scoring proficiency of JAMRIS-SIJ and whether contrast-enhancement enhances its performance. The results showed that calibrated readers achieved greater reliability in scoring specific inflammatory and structural lesions. Sensitivity and reliability for scoring inflammatory lesions were higher on fluid-sensitive sequences compared to contrast-enhanced sequences. Therefore, systematic calibration should be implemented before using JAMRIS-SIJ in clinical trials, and it is unlikely that contrast-enhanced MRI will improve the performance of this method.
SEMINARS IN ARTHRITIS AND RHEUMATISM
(2024)
Article
Rheumatology
L. van Ouwerkerk, S. A. Bergstra, T. D. Maarseveen, T. W. J. Huizinga, R. Knevel, C. F. Allaart
Summary: This study evaluated whether the initial use of glucocorticoid (GC) bridging in RA patients leads to a higher probability of long-term GC and bDMARD use. The results showed that patients who initially started GC had a higher risk of later GC use, but the risk of bDMARD use was not significantly increased.
SEMINARS IN ARTHRITIS AND RHEUMATISM
(2024)
Article
Rheumatology
ShuangHua Liu, YiMei Tan, WeiDong Huang, HongSheng Luo, BingCheng Pan, Shuan Wu
Summary: This study assessed the cardiovascular safety of zoledronic acid in the treatment of primary osteoporosis. The results showed that in women with primary osteoporosis, zoledronic acid may increase the risk of atrial fibrillation and arrhythmias, but the cardiovascular risk in men with osteoporosis is uncertain.
SEMINARS IN ARTHRITIS AND RHEUMATISM
(2024)