Review
Medicine, General & Internal
Adrian Giuca, Carina Mihai, Ciprian Jurcut, Ana Maria Gheorghiu, Laura Groseanu, Alina Dima, Adrian Saftoiu, Ioan Mircea Coman, Bogdan A. Popescu, Ruxandra Jurcut
Summary: Systemic sclerosis (SSc) is a rare disease with unfavorable prognosis, and pulmonary arterial hypertension (PAH) and interstitial lung disease (ILD) are the main causes of morbi-mortality, emphasizing the importance of early detection and treatment for better outcomes.
Article
Rheumatology
Amalia Colalillo, Maria Chiara Grimaldi, Valentina Vaiarello, Chiara Pellicano, Giorgia Leodori, Antonietta Gigante, Antonella Romaniello, Edoardo Rosato
Summary: This study compared the positive predictive value (PPV) of the echocardiography-derived TAPSE/sPAP ratio with that of the DETECT algorithm in screening for PAH in SSc patients. The results showed that TAPSE/sPAP ratio had a higher PPV in identifying SSc patients requiring right heart catheterization (RHC) for confirming PAH diagnosis.
Article
Critical Care Medicine
Hussein J. Hassan, Mario Naranjo, Nour Ayoub, Traci Housten, Steven Hsu, Aparna Balasubramanian, Catherine E. Simpson, Rachel L. Damico, Stephen C. Mathai, Todd M. Kolb, Paul M. Hassoun
Summary: This study compared the characteristics and survival rates of patients with systemic sclerosis-associated pulmonary hypertension (SSc-PH) between the last two decades. The results showed that the transplant-free survival rate has significantly improved for patients with group 1 disease over the past decade, possibly due to earlier detection and better therapeutic management. In contrast, patients with group 2 or 3 disease continue to have a poor prognosis.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2023)
Article
Immunology
Amalia Colalillo, Anna-Maria Hoffmann-Vold, Chiara Pellicano, Antonella Romaniello, Armando Gabrielli, Eric Hachulla, Vanessa Smith, Carmen-Pilar Simeon-Aznar, Ivan Castellvi, Paolo Airo, Marie-Elise Truchetet, Elise Siegert, Oliver Distler, Edoardo Rosato
Summary: This study evaluated the predictive role of the echocardiography-derived TAPSE/sPAP ratio for pulmonary hypertension (PH) diagnosis and mortality. The study found that a TAPSE/sPAP ratio < 0.55 mm/mmHg is a predictive risk factor for PH, and a TAPSE/sPAP ratio <= 0.32 mm/mmHg is a predictive risk marker for all-cause mortality.
AUTOIMMUNITY REVIEWS
(2023)
Review
Medicine, General & Internal
Mario Naranjo, Paul M. Hassoun
Summary: Systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) is a catastrophic complication of autoimmune diseases, causing high mortality rates among patients. Despite advancements in screening methods and therapies, improved outcomes are still needed, urging the search for innovative treatment options.
Review
Medicine, General & Internal
Tijana Tuhy, Paul M. Hassoun
Summary: Systemic sclerosis is an autoimmune disorder that affects the connective tissue and causes skin thickening and complications in the internal organs. Pulmonary involvement, including pulmonary arterial hypertension and interstitial lung disease, is the main cause of morbidity and mortality in scleroderma patients. Current treatment options are not specific to pulmonary involvement in scleroderma, and patients with pulmonary arterial hypertension in this population tend to have worse outcomes and lower response to therapy compared to other forms of Group I pulmonary hypertension.
FRONTIERS IN MEDICINE
(2023)
Article
Respiratory System
Valentine Mismetti, Xavier Delavenne, David Montani, Souad Bezzeghoud, Olivier Delezay, Sophie Hodin, David Launay, Sylvain Marchand-Adam, Hilario Nunes, Edouard Ollier, Martine Reynaud-Gaubert, Jean Pastre, Julie Traclet, Sebastien Quetant, Sabrina Zeghmar, Laurent Bertoletti, Vincent Cottin
Summary: This study conducted a proteomic analysis on patients with SSc-PH-ILD and identified 7 plasma proteins associated with survival. These proteins are involved in the pathways of haemostasis and fibrosis. These potential biomarkers may be useful for predicting patient prognosis.
RESPIRATORY RESEARCH
(2023)
Article
Rheumatology
Maria Vlachou, Hossam Fayed, Adele Dawson, Sally Reddecliffe, Alexander Stevenson, Ross J. Thomson, Benjamin Emmanuel Schreiber, J. Gerry Coghlan
Summary: The study evaluated survival rates and short-term impact of i.v. prostanoids on SSc-PAH patients. Significant improvements in clinical and haemodynamic parameters were observed with i.v. prostanoid therapy. However, survival remains poor and risk scoring at baseline and during therapy can improve prognostication.
Article
Medicine, General & Internal
Loukianos S. Rallidis, Konstantina Papangelopoulou, Georgios Makavos, Christos Varounis, Anastasia Anthi, Stylianos E. Orfanos
Summary: In highly selected SSc patients, low-dose DSE has a satisfactory diagnostic accuracy for the early detection of PAH, helping patients with non-diagnostic resting echocardiography for PH to receive a diagnosis.
JOURNAL OF CLINICAL MEDICINE
(2021)
Article
Critical Care Medicine
Roham T. Zamanian, David Badesch, Lorinda Chung, Robyn T. Domsic, Thomas Medsger, Ashley Pinckney, Lynette Keyes-Elstein, Carla D'Aveta, Meagan Spychala, R. James White, Paul M. Hassoun, Fernando Torres, Andrew J. Sweatt, Jerry A. Molitor, Dinesh Khanna, Holden Maecker, Beverly Welch, Ellen Goldmuntz, Mark R. Nicolls
Summary: The study suggested that B-cell depletion therapy may have potential efficacy and safety for SSc-PAH. Low levels of RF, IL-12, and IL-17 may serve as promising predictors of rituximab response. Despite initially showing no significant difference in the primary analysis, the rituximab group demonstrated a statistically significant improvement in 6MWD at Week 24 when considering data through Week 48.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2021)
Article
Biochemistry & Molecular Biology
Alberto Lo Gullo, Giuseppe Mandraffino, Javier Rodriguez-Carrio, Michele Scuruchi, Davide Sinicropi, Maria Postorino, Carmela Morace, Clemente Giuffrida, Davide Sciortino, Romina Gallizzi, Saverio Loddo, Concetta Zito, Giovanni Squadrito
Summary: Systemic sclerosis (SSc) is characterized by early vasculopathy and fibrosis in various tissues. CD34+ cells and endocan were found to be potential markers for disease status in SSc, with correlations to inflammatory markers and pulmonary arterial pressure. These findings suggest a potential role for CD34+ progenitor cells and endocan in monitoring SSc patients with pulmonary arterial hypertension.
Article
Rheumatology
Dirk M. Wuttge, Anting L. Carlsen, Gabriel Teku, Marie Wildt, Goran Radegran, Mauno Vihinen, Niels H. H. Heegaard, Roger Hesselstrand
Summary: The study found a link between the expression levels of circulating plasma miRNAs and the occurrence of SSc-APAH in female patients with ACA-positive lcSSc.
Article
Biochemistry & Molecular Biology
Mor Zaaroor Levy, Noa Rabinowicz, Maia Yamila Kohon, Avshalom Shalom, Ariel Berl, Tzipi Hornik-Lurie, Liat Drucker, Shelly Tartakover Matalon, Yair Levy
Summary: miR-26 and miR-let-7d may be serological markers for SSc-PAH and are involved in myofibroblast differentiation and complement pathway activation.
Article
Pharmacology & Pharmacy
Xia Fang, Chao He, Xudong Ni, Tianli Zhang, Qianyu Li, Yi Luo, Wei Long, Rui Wu
Summary: The lack of a well-established animal model limits the study of the pathogenesis and treatment of systemic sclerosis with pulmonary hypertension (SSc-PH). In this study, rabbits were injected with monocrotaline (MCT), bleomycin (BLM), or MCT plus BLM to induce SSc-PH. The results showed that co-injection with MCT plus BLM induced persistent fibrosis and progressive pulmonary hypertension, making it a potential study model for SSc-PH.
CLINICAL AND EXPERIMENTAL HYPERTENSION
(2022)
Article
Rheumatology
Rossella De Angelis, Valeria Riccieri, Edoardo Cipolletta, Nicoletta Del Papa, Francesca Ingegnoli, Silvia Bosello, Amelia Spinella, Greta Pellegrino, Marco de Pinto, Silvia Papa, Giuseppe Armentaro, Dilia Giuggioli
Summary: Nailfold videocapillaroscopy findings were compared in SSc patients with and without a diagnosis of pulmonary arterial hypertension (PAH). The results showed that patients with SSc-PAH had more severe abnormalities in nailfold videocapillaroscopy. These findings may be helpful in predicting the diagnosis of PAH.
Article
Cardiac & Cardiovascular Systems
Nicole M. Bhave, Scott H. Visovatti, Brian Kulick, Theodore J. Kolias, Vallerie V. McLaughlin
INTERNATIONAL JOURNAL OF CARDIOVASCULAR IMAGING
(2017)
Letter
Respiratory System
Thomas M. Cascino, Vallerie V. McLaughlin, Caroline R. Richardson, Nilofar Behbahani-Nejad, Victor M. Moles, Scott H. Visovatti, Elizabeth A. Jackson
EUROPEAN RESPIRATORY JOURNAL
(2019)
Article
Rheumatology
Amber Young, Dharshan Vummidi, Scott Visovatti, Kate Homer, Holly Wilhalme, Eric S. White, Kevin Flaherty, Vallerie McLaughlin, Dinesh Khanna
ARTHRITIS & RHEUMATOLOGY
(2019)
Article
Medicine, Research & Experimental
Vinita Yadav, Liguo Chi, Raymond Zhao, Benjamin E. Tourdot, Srilakshmi Yalavarthi, Benjamin N. Jacobs, Alison Banka, Hui Liao, Sharon Koonse, Anuli C. Anyanwu, Scott H. Visovatti, Michael A. Holinstat, J. Michelle Kahlenberg, Jason S. Knight, David J. Pinsky, Yogendra Kanthi
JOURNAL OF CLINICAL INVESTIGATION
(2019)
Article
Respiratory System
Sara Jaafar, Scott Visovatti, Amber Young, Suiyuan Huang, Paul Cronin, Dharshan Vummidi, Vallerie McLaughlin, Dinesh Khanna
EUROPEAN RESPIRATORY JOURNAL
(2019)
Article
Rheumatology
Vivek Nagaraja, Marco Matucci-Cerinic, Daniel E. Furst, Masataka Kuwana, Yannick Allanore, Christopher P. Denton, Ganesh Raghu, Vallerie Mclaughlin, Panduranga S. Rao, James R. Seibold, John D. Pauling, Michael L. Whitfield, Dinesh Khanna
ARTHRITIS & RHEUMATOLOGY
(2020)
Article
Medicine, Research & Experimental
Dinesh Khanna, Cristina Padilla, Lam C. Tsoi, Vivek Nagaraja, Puja P. Khanna, Tracy Tabib, J. Michelle Kahlenberg, Amber Young, Suiyuan Huang, Johann E. Gudjonsson, David A. Fox, Robert Lafyatis
Summary: This study found that the Janus kinase inhibitor tofacitinib was safe and well-tolerated in patients with early diffuse cutaneous systemic sclerosis (SSc). Gene expression analysis showed that tofacitinib inhibited inflammation in specific cell populations of SSc patients, indicating potential therapeutic effects. These findings are important for understanding the pathogenesis of SSc and developing new treatment strategies.
Article
Cardiac & Cardiovascular Systems
Christopher Lewis, Ryan Sanderson, Nektarios Vasilottos, Alexander Zheutlin, Scott Visovatti
HEART FAILURE CLINICS
(2023)
Review
Immunology
Ashima Makol, Vivek Nagaraja, Chiemezie Amadi, Janelle Vu Pugashetti, Elaine Caoili, Dinesh Khanna
Summary: Interstitial lung disease (ILD) is the leading cause of mortality in patients with systemic sclerosis (SSc). Early recognition and intervention are critical with the FDA approval of two medications and a pipeline of novel therapeutics in trials. There is a need to develop and validate more innovative screening modalities to improve the diagnosis of SSc-ILD.
EXPERT REVIEW OF CLINICAL IMMUNOLOGY
(2023)
Article
Cardiac & Cardiovascular Systems
Thomas M. Cascino, Vallerie V. McLaughlin, Caroline R. Richardson, Nilofar Behbahani-Nejad, Victor M. Moles, Scott H. Visovatti, Elizabeth A. Jackson
PULMONARY CIRCULATION
(2019)
Article
Biochemistry & Molecular Biology
Rahul Rattan, Somnath Bhattacharjee, Hong Zong, Corban Swain, Muneeb A. Siddiqui, Scott H. Visovatti, Yogendra Kanthi, Sajani Desai, David J. Pinsky, Sascha N. Goonewardena
BIOORGANIC & MEDICINAL CHEMISTRY
(2017)
Article
Cardiac & Cardiovascular Systems
David M. Cameron, Vallerie V. McLaughlin, Melvyn Rubenfire, Scott Visovatti, David S. Bach
AMERICAN JOURNAL OF CARDIOLOGY
(2017)
Article
Medicine, Research & Experimental
Nadia R. Sutton, Takanori Hayasaki, Matthew C. Hyman, Anuli C. Anyanwu, Hui Liao, Danica Petrovic-Djergovic, Linda Badri, Amy E. Baek, Natalie Walker, Keigo Fukase, Yogendra Kanthi, Scott H. Visovatti, Ellen L. Horste, Jessica J. Ray, Sascha N. Goonewardena, David J. Pinsky