Article
Medicine, General & Internal
Loukianos S. Rallidis, Konstantina Papangelopoulou, Anastasia Anthi, Iraklis Tsangaris, Christos Varounis, Georgios Makavos, Dimitrios Konstantonis, Panagiotis Vlachoyiannopoulos, Stylianos E. Orfanos, Efstathios K. Iliodromitis
Summary: This study investigated the diagnostic accuracy of exercise Doppler echocardiography for PAH in systemic sclerosis patients, revealing that post-exercise TR velocity >3.4 m/s had high sensitivity and accuracy in detecting PAH, and was positively correlated with RV contractile reserve.
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
Rheumatology
Justin K. Lui, Kari R. Gillmeyer, Ruchika A. Sangani, Robert J. Smyth, Deepa M. Gopal, Marcin A. Trojanowski, Andreea M. Bujor, Renda Soylemez Wiener, Michael P. LaValley, Elizabeth S. Klings
Summary: We developed a predictive model for pulmonary hypertension (PH) in systemic sclerosis (SSc) patients to minimize the risk of potential complications. The random forest model showed the best performance in diagnosing PH, with high sensitivity and specificity, allowing for better patient selection for right heart catheterization (RHC) and reducing patient risk.
ARTHRITIS CARE & RESEARCH
(2023)
Article
Rheumatology
Jinzhi Lai, Xiaoxiao Guo, Jiuliang Zhao, Hui Wang, Zhuang Tian, Qian Wang, Mengtao Li, Quan Fang, Ligang Fang, Yongtai Liu, Xiaofeng Zeng
Summary: This study aimed to investigate the impact of interventricular systolic asynchrony (IVSA) on the prognosis of patients with systemic sclerosis-associated pulmonary arterial hypertension (PAH). The results showed that IVSA time difference was significantly associated with clinical worsening and mortality, indicating that IVSA analysis could be a noninvasive and easy-to-use approach for assessing the prognosis of patients with SSc-associated PAH.
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.
Review
Medicine, General & Internal
Weronika Topyla-Putowska, Michal Tomaszewski, Andrzej Wysokinski, Andrzej Tomaszewski
Summary: Pulmonary arterial hypertension (PAH) is a rare, progressive disease characterized by abnormally increased pulmonary artery pressure. The diagnosis of PAH requires a multistep process, with hemodynamic testing considered the gold standard. Echocardiography plays a crucial role in the diagnostic algorithm of PAH, allowing assessment of right heart structure and function, pressure estimation, and exclusion of other causes of elevated pulmonary bed pressure.
JOURNAL OF CLINICAL MEDICINE
(2021)
Article
Cardiac & Cardiovascular Systems
Dilia Giuggioli, Valeria Riccieri, Edoardo Cipolletta, Nicoletta Del Papa, Francesca Ingegnoli, Amelia Spinella, Greta Pellegrino, Anna Maria Risa, Marco de Pinto, Silvia Papa, Giuseppe Armentaro, Rossella De Angelis
Summary: Systemic sclerosis (SSc) is a connective tissue disease characterized by immune-system alterations, fibrosis, and microangiopathy. Pulmonary arterial hypertension (PAH) is a severe complication affecting about 10-15% of SSc patients, and appropriate screening programs are necessary. Nail fold videocapillaroscopy (NVC) studies show more severe microvascular dysfunction in SSc patients with PAH, suggesting a correlation with pulmonary microangiopathy.
FRONTIERS IN CARDIOVASCULAR MEDICINE
(2022)
Article
Cardiac & Cardiovascular Systems
Jinzhi Lai, Jiuliang Zhao, Kaiwen Li, Xiaohan Qin, Hui Wang, Zhuang Tian, Qian Wang, Mengtao Li, Xiaoxiao Guo, Yongtai Liu, Xiaofeng Zeng
Summary: The study suggests that the ratio of tricuspid annular plane systolic excursion (TAPSE) to pulmonary artery systolic pressure (PASP) accurately assesses the risk status and predicts prognosis in patients with systemic sclerosis-associated pulmonary arterial hypertension (PAH). A higher TAPSE/PASP ratio is associated with better overall survival.
FRONTIERS IN CARDIOVASCULAR MEDICINE
(2022)
Article
Biology
Mohammadbagher Sharifkazemi, Mohammadali Nazarinia, Alireza Arjangzade, Mohamad Goldust, Zahra Hooshanginezhad
Summary: Systemic Sclerosis (SSc) is a chronic connective tissue disorder with cardiac involvement being a predictor of poor prognosis. This study evaluated the mechanical performance of all cardiac chambers in SSc patients using two-dimensional speckle tracking echocardiography (2D-STE) and compared it with normal individuals. The results showed that 2D-STE can diagnose impaired chamber mechanics and function in subclinical stages.
Review
Biology
Mojca Skafar, Jana Ambrozic, Janez Toplisek, Marta Cvijic
Summary: Resting and exercise right heart catheterisation is the gold standard method for diagnosing and differentiating types of pulmonary hypertension (PH). However, non-invasive exercise stress echocardiography may be an alternative, as it can unmask exercise PH and detect early stages of left ventricular diastolic dysfunction. Resting echocardiography can detect overt right ventricle dysfunction, but measures of occult RV dysfunction are lacking. Exercise echocardiography shows promising results in assessing RV contractile reserve and providing prognostic information.
Article
Rheumatology
Suparaporn Wangkaew, Panupong Pota, Narawudt Prasertwittayakij, Arintaya Phrommintikul
Summary: In this study, the majority of patients had early DcSSc, and the incidence of PH was low, with all cases developing concurrently with ILD or left heart disease, leading to poor survival rates. Higher NYHA class, telangiectasia, and enlarged LA diameter were identified as predictors of PH, while Raynaud's phenomenon and high oxygen saturation were protective factors.
CLINICAL RHEUMATOLOGY
(2021)
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
Agriculture, Dairy & Animal Science
Jorge Isidoro Matos, Sara Nieves Garcia-Rodriguez, Noelia Costa-Rodriguez, Alicia Caro-Vadillo, Elena Carreton, Jose Alberto Montoya-Alonso
Summary: This study assessed the diagnostic value of echocardiography tissue Doppler imaging (TDI) in determining the presence of pulmonary hypertension (PH) in dogs with heartworm disease. The TDI measurements showed significant differences between dogs with and without PH, and cut-off values with excellent sensitivity and specificity were found. The TDI mode may be a useful adjunct diagnostic method for PH in dogs with Dirofilaria immitis.
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)
Article
Cardiac & Cardiovascular Systems
Stefano Ghio, Valentina Mercurio, Andrea Attanasio, Gaetano Asile, Carlo Gabriele Tocchetti, Stefania Paolillo
Summary: There is a strong bidirectional relationship between diabetes mellitus (DM) and heart failure (HF), with DM activating multiple molecular and pathophysiological mechanisms that can damage the heart in the long term. However, the prognostic role of DM in chronic and acute HF is still unclear, with gaps in evidence due to the heterogeneity of patient phenotypes and the necessity to better characterize individual patients and their comorbidities. This review aims to summarize the negative impact of DM on HF and analyze available data on the prognostic implications in both chronic and acute HF.
HEART FAILURE REVIEWS
(2023)
Article
Rheumatology
Cosimo Bruni, Mariaelena Occhipinti, Michael Pienn, Gianna Camiciottoli, Maurizio Bartolucci, Silvia Laura Bosello, Christian Payer, Zoltan Balint, Anna Rita Larici, Alessandra Tottoli, Lorenzo Tofani, Enrico De Lorenzis, Gemma Lepri, Silvia Bellando-Randone, Amelia Spinella, Dilia Giuggioli, Francesco Masini, Giovanna Cuomo, Federico Lavorini, Stefano Colagrande, Horst Olschewski, Marco Matucci-Cerinic
Summary: Recent advancements have made it possible to quantitatively assess lung vascular and parenchymal changes in thoracic CT images using automated software tools. In this study, the researchers investigated the vessel parameters of patients with SSc and correlated them with interstitial lung disease (ILD) features. The results showed that the CT vessel parameters increased in parallel with ILD extent and functional impairment, suggesting that they may serve as a biomarker of SSc-ILD severity.
Article
Rheumatology
Michele Iudici, Denis Mongin, Elise Siegert, Patricia E. Carreira, Joerg Distler, Joerg Henes, Elisabetta Zanatta, Eric Hachulla, Giacomo De Luca, Carolina de Souza Mueller, Tania Santiago, Jose-Luis Tandaipan, Breno Valdetaro Bianchi, Maria De Santis, Anna-Maria Hoffmann-Vold, Armando Gabrielli, Oliver Distler, Delphine Sophie Courvoisier
Summary: The study aimed to estimate the prevalence of long-term exposure to glucocorticoids (GCs) among SSc patients and identify factors associated with prescribing practices over time and across countries. The results showed that the utilization of GCs is widespread and long-term in SSc patients, with significant variations between countries and within-country. However, there has been a gradual decrease in their utilization.
Article
Cardiac & Cardiovascular Systems
Stefano Ghio, Mauro Acquaro, Piergiuseppe Agostoni, Giuseppe Ambrosio, Erberto Carluccio, Vincenzo Castiglione, Davide Colombo, Michele D'Alto, Santo Delle Grottaglie, Frank Lloyd Dini, Michele Emdin, Martino Fortunato, Andrea Igoren Guaricci, Francesca Jacoangeli, Alberto M. Marra, Stefania Paolillo, Sivia Papa, Luca Vicini Scajola, Michele Correale, Alberto Palazzuoli
Summary: For a long time, cardiologists have underestimated the role of the right heart in heart failure caused by left heart disease. However, the importance of evaluating right ventricular structure and function in left heart failure is now well recognized and emphasized in the latest heart failure guidelines. Yet, there are still unanswered questions about which imaging techniques and parameters should be used to assess the severity of RV dysfunction, and whether less commonly used diagnostic tools have a role in this field.
HEART FAILURE REVIEWS
(2023)
Article
Cardiac & Cardiovascular Systems
Andrea M. D. 'Armini, Maurizio Pin, Anna Celentano, Leslie J. Te Masiglat, Ermelinda Borrelli, Benedetta Vanini, Catherine Klersy, Giuseppe Silvaggio, Cristian Monterosso, Alessia Alloni, Carlo Pellegrini, Stefano Ghio
Summary: This study showed that in patients with chronic thromboembolic pulmonary hypertension undergoing pulmonary endarterectomy, a higher number of treated pulmonary artery branches is associated with better hemodynamic and clinical outcomes after surgery.
INTERNATIONAL JOURNAL OF CARDIOLOGY
(2023)
Review
Cardiac & Cardiovascular Systems
Manuel J. Richter, Federico Fortuni, Fawaz Alenezi, Michele D'Alto, Roberto Badagliacca, Nathan W. Brunner, Arie P. van Dijk, Philipp Douschan, Henning Gall, Stefano Ghio, Francesco Lo Giudice, Ekkehard Grunig, Francois Haddad, Luke Howard, Sudarshan Rajagopal, Niels Stens, Davide Stolfo, Dick H. J. Thijssen, Carmine Dario Vizza, Roham T. Zamanian, Liang Zhong, Werner Seeger, Hossein A. Ghofrani, Khodr Tello
Summary: A meta-analysis of 35 studies found that right atrial (RA) imaging assessment is a relevant prognostic marker in patients with pulmonary hypertension (PH). RA area, RA indexed area, RA peak longitudinal strain, and RA total emptying fraction were significantly associated with combined end-points including death, clinical worsening, and/or lung transplantation. RA volume and volume index showed marginal significant associations.
JOURNAL OF HEART AND LUNG TRANSPLANTATION
(2023)
Article
Biology
Maria Anifanti, Andriana Teloudi, Alexandros Mitropoulos, Niki Syrakou, Eleni Pagkopoulou, Eva Triantafyllidou, Carina Bostrom, Louise Pyndt Diederichsen, Giovanna Cuomo, Theodoros Dimitroulas, Markos Klonizakis, Evangelia Kouidi
Summary: This study aimed to investigate the effects of a three-month combined exercise program on right ventricular morphology and function in SSc patients. The results showed that the exercise program improved cardiorespiratory efficiency and right ventricular systolic function in SSc patients, as assessed by 2DSTE.
Article
Pediatrics
Pier Paolo Bassareo, Paola Argiento, Colin Joseph McMahon, Esme Dunne, Kevin Patrick Walsh, Maria Giovanna Russo, Michele D'Alto
Summary: Paediatric pulmonary hypertension (PH) is a complex illness with high morbidity and mortality if left untreated. Limited long-term data are available for the subtype idiopathic pulmonary arterial hypertension (IPAH) in children. This study evaluated the clinical features and outcomes of 10 paediatric patients with IPAH over a 20-year period. The results indicate the need for lung transplantation and significant mortality in this population.
Article
Rheumatology
Corrado Campochiaro, Giacomo De Luca, Maria-Grazia Lazzaroni, Giuseppe Armentaro, Amelia Spinella, Barbara Vigone, Barbara Ruaro, Anna Stanziola, Devis Benfaremo, Enrico De Lorenzis, Beatrice Moccaldi, Silvia Laura Bosello, Giovanna Cuomo, Lorenzo Beretta, Elisabetta Zanatta, Dilia Giuggioli, Nicoletta Del Papa, Paolo Airo, Marco Confalonieri, Gianluca Moroncini, Lorenzo Dagna, Marco Matucci-Cerinic
Summary: In this real-life study, the efficacy and safety of Nintedanib in treating SSc-ILD were evaluated. The results showed that Nintedanib, in combination with immunosuppressants, could stabilize lung function and reduce lung progression. However, gastrointestinal side effects were observed frequently.
Article
Rheumatology
Rossella De Angelis, Clodoveo Ferri, Dilia Giuggioli, Gianluigi Bajocchi, Lorenzo Dagna, Silvia Bellando-Randone, Giovanni Zanframundo, Rosario Foti, Fabio Cacciapaglia, Giovanna Cuomo, Alarico Ariani, Edoardo Rosato, Gemma Lepri, Francesco Girelli, Valeria Riccieri, Elisabetta Zanatta, Silvia Laura Bosello, Ilaria Cavazzana, Francesca Ingegnoli, Maria De Santis, Giuseppe Murdaca, Giuseppina Abignano, Nicoletta Romeo, Alessandra Della Rossa, Maurizio Caminiti, Anna Maria Iuliano, Giovanni Ciano, Lorenzo Beretta, Gianluca Bagnato, Ennio Lubrano, Ilenia De Andres, Alessandro Giollo, Marta Saracco, Cecilia Agnes, Edoardo Cipolletta, Federica Lumetti, Amelia Spinella, Luca Magnani, Corrado Campochiaro, Giacomo De Luca, Veronica Codullo, Elisa Visalli, Claudio Di Vico, Antonietta Gigante, Greta Pellagrino, Erika Pigatto, Maria-Grazia Lazzaroni, Franco Franceschini, Elena Generali, Gianna Mennillo, Simone Barsotti, Giuseppa Pagano Mariano, Federica Furini, Licia Vultaggio, Simone Parisi, Clara Lisa Peroni, Davide Rozza, Anna Zanetti, Greta Carrara, Gianpiero Landolfi, Carlo Alberto Scire, Gerolamo Bianchi, Enrico Fusaro, Gian Domenico Sebastiani, Marcello Govoni, Salvatore D'Angelo, Franco Cozzi, Serena Guiducci, Andrea Doria, Carlo Salvarani, Florenzo Iannone, Marco Matucci-Cerinic
Summary: This study aimed to describe the demographic, clinical, and laboratory features of systemic sclerosis sine scleroderma (ssSSc) within a large SSc cohort. The results showed that ssSSc has longer duration of RP, milder disease course, and higher positivity of anticentromere antibodies compared to lcSSc and dcSSc.
Article
Medicine, General & Internal
Giovanna Cuomo, Carlo Iandoli, Raffaele Galiero, Alfredo Caturano, Claudio Di Vico, Danilo Perretta, Pier Vincenzo Adamo, Roberta Ferrara, Luca Rinaldi, Ciro Romano, Ferdinando Carlo Sasso
Summary: This study assessed hepatic fibrosis and steatosis in systemic sclerosis (SSc) patients. The results showed that only 3.4% of patients had marked liver fibrosis, while 5.1% had severe liver steatosis. Therefore, liver fibrosis did not appear to be a significant concern in SSc patients, but moderate fibrosis could still be detected in a significant proportion of subjects. Long-term follow-up may clarify whether liver fibrosis in SSc patients may continue to progress.
Review
Medicine, General & Internal
Gaetano Rea, Nicola Sverzellati, Marialuisa Bocchino, Roberta Lieto, Gianluca Milanese, Michele D'Alto, Giorgio Bocchini, Mauro Maniscalco, Tullio Valente, Giacomo Sica
Summary: Diffuse lung disorders (DLDs) and interstitial lung diseases (ILDs) are pathological conditions affecting the lung parenchyma and interstitial network. Radiologists play a crucial role in diagnosing and monitoring ILDs using high-resolution computed tomography (HRCT). HRCT provides detailed imaging of lung tissue and subtle morphological changes, while quantitative software and artificial intelligence (AI) enhance analysis and provide comprehensive evaluations beyond human capabilities.
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
E. Gremese, Francesco Ciccia, Carlo Selmi, Giovanna Cuomo, Rosario Foti, Marco Matucci-Cerinic, Fabrizio Conti, Enrico Fusaro, Giuliana Guggino, Florenzo Iannone, Andrea Delle Sedie, Roberto Perricone, Luca Idolazzi, Paolo Moscato, Elke Theander, Wim Noel, Paul Bergmans, Silvia Marelli, Laure Gossec, Josef S. Smolen
Summary: This study compared the real-world persistence, effectiveness, and tolerability of ustekinumab and TNFi in PsA patients. The results showed that ustekinumab had better persistence than TNFi, overall and in specific subgroups. After one year of treatment, both ustekinumab and TNFi showed similar effectiveness.
CLINICAL AND EXPERIMENTAL RHEUMATOLOGY
(2023)