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.
Review
Biochemistry & Molecular Biology
Joe E. Mouawad, Carol Feghali-Bostwick
Summary: Systemic sclerosis, also known as scleroderma, is an autoimmune disorder that affects the connective tissues and has a high mortality rate. Fibrosis, particularly in the lungs, is a hallmark of the disease and is currently the leading cause of death. Understanding the molecular mechanisms involved in lung fibrosis is essential for developing potential therapies to improve patient outcomes and quality of life.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Review
Chemistry, Medicinal
Martina Orlandi, Laura Antonia Meliante, Arianna Damiani, Lorenzo Tofani, Cosimo Bruni, Serena Guiducci, Marco Matucci-Cerinic, Silvia Bellando-Randone, Sara Tomassetti
Summary: The use of bronchoalveolar lavage (BAL) in the evaluation of systemic sclerosis (SSc) interstitial lung disease (ILD) remains controversial. Studies have found a positive correlation between BAL cytology and lung function, as well as high-resolution computed tomography (HRCT) findings. Cytokines, chemokines, growth factors, coagulation factors, and eicosanoids have been shown to be present in higher quantities in SSc-ILD patients and may be related to more severe pulmonary disease. However, there is no consensus regarding the role of BAL cellularity as a predictor of mortality.
Article
Medicine, Research & Experimental
Masataka Kuwana, Aiko Saito, Wataru Sakamoto, Christina Raabe, Kumiko Saito
Summary: This study estimated the incidence and prevalence rates of systemic sclerosis (SSc) and SSc-associated interstitial lung disease (SSc-ILD) in Japan using medical databases. The results showed that approximately 30% of SSc patients had ILD, and the utilization of immunomodulatory medications was low.
ADVANCES IN THERAPY
(2022)
Review
Chemistry, Medicinal
Barbara Ruaro, Marco Confalonieri, Marco Matucci-Cerinic, Francesco Salton, Paola Confalonieri, Mario Santagiuliana, Gloria Maria Citton, Elisa Baratella, Cosimo Bruni
Summary: Systemic sclerosis patients often suffer from interstitial lung disease, with early diagnosis playing a pivotal role in improving prognosis. Treatment strategies typically involve a combination of immunosuppressants and targeted biological therapies based on disease severity and progression risk. Hematopoietic autologous stem cell transplantation has shown benefits for progressive SSc patients, while lung transplantation is considered for refractory cases of SSc-ILD.
Review
Rheumatology
Kristine E. Konopka, Jeffrey L. Myers
Summary: Interstitial lung disease is a common manifestation of systemic sclerosis, with fibrotic nonspecific interstitial pneumonia being the most common form followed by usual interstitial pneumonia. Lung biopsy may not be necessary for confirming the presence of interstitial lung disease in the right clinical setting, with surgical lung biopsies often reserved for atypical presentations. This review discusses histological findings in SSc-ILD and other changes sometimes seen in lung biopsies from systemic sclerosis patients.
THERAPEUTIC ADVANCES IN MUSCULOSKELETAL DISEASE
(2021)
Article
Medicine, General & Internal
Kelly Morgan, Charlotte Woollard, Dylan Beinart, Lauren Host, Janet Roddy
Summary: The study aimed to investigate whether rituximab could stabilize or improve pulmonary function in patients with SSc-ILD. A retrospective analysis of patients who received rituximab despite progressing on conventional therapy at two tertiary centers was conducted. Results showed that all patients demonstrated stable pulmonary function testing at 1-year post-rituximab, indicating a potential role for rituximab in the treatment of SSc-ILD.
INTERNAL MEDICINE JOURNAL
(2023)
Article
Medicine, General & Internal
Yuichiro Shirai, Ryosuke Fukue, Yuko Kaneko, Masataka Kuwana
Summary: Serum KL-6 levels fluctuate in SSc patients with ILD, especially in those with extensive disease, and are associated with various factors related to the severity of the disease.
Review
Rheumatology
Rudra P. Goswami, Animesh Ray, Moumita Chatterjee, Arindam Mukherjee, Geetabali Sircar, Parasar Ghosh
Summary: Treatment with RTX in SSc-ILD resulted in a significant improvement in both FVC and DLCO during the first year of treatment, with lower rates of infection compared to controls.
Article
Rheumatology
Alain Lescoat, Doerte Huscher, Nils Schoof, Paolo Airo, Jeska De Vries-Bouwstra, Gabriela Riemekasten, Eric Hachulla, Andrea Doria, Edoardo Rosato, Nicolas Hunzelmann, Carlomaurizio Montecucco, Armando Gabrielli, Anna-Maria Hoffmann-Vold, Oliver Distler, Jennifer Ben Shimol, Maurizio Cutolo, Yannick Allanore
Summary: The prevalence and characteristics of SSc-associated interstitial lung disease (SSc-ILD) vary between geographical regions. This study aimed to explore the differences in prevalence, phenotype, treatment, and prognosis in patients with SSc-ILD from different regions in the EUSTAR database. The results showed significant variations in clinical characteristics, survival rates, and treatment approaches among different regions.
Article
Rheumatology
David Roofeh, Kevin K. Brown, Ella A. Kazerooni, Donald Tashkin, Shervin Assassi, Fernando Martinez, Athol U. Wells, Ganesh Raghu, Christopher P. Denton, Lorinda Chung, Anna-Maria Hoffmann-Vold, Oliver Distler, Kerri A. Johannson, Yannick Allanore, Eric L. Matteson, Leticia Kawano-Dourado, John D. Pauling, James R. Seibold, Elizabeth R. Volkmann, Simon L. F. Walsh, Chester Oddis, Eric S. White, Shaney L. Barratt, Elana J. Bernstein, Robyn T. Domsic, Paul F. Dellaripa, Richard Conway, Ivan Rosas, Nitin Bhatt, Vivien Hsu, Francesca Ingegnoli, Bashar Kahaleh, Puneet Garcha, Nishant Gupta, Surabhi Khanna, Peter Korsten, Celia Lin, Stephen C. Mathai, Vibeke Strand, Tracy J. Doyle, Virginia Steen, Donald F. Zoz, Juan Ovalles-Bonilla, Ignasi Rodriguez-Pinto, Padmanabha D. Shenoy, Andrew Lewandoski, Elizabeth Belloli, Alain Lescoat, Vivek Nagaraja, Wen Ye, Suiyuan Huang, Toby Maher, Dinesh Khanna
Summary: This study aimed to establish a framework by which experts define disease subsets in systemic sclerosis associated interstitial lung disease (SSc-ILD). The results showed that using the proposed conceptual framework, international experts were able to achieve a consensus on classifying SSc-ILD patients along the dimensions of disease severity, risk of progression and progression over time.
Review
Medicine, General & Internal
Nicholas Landini, Martina Orlandi, Cosimo Bruni, Edoardo Carlesi, Cosimo Nardi, Linda Calistri, Giovanni Morana, Sara Tomassetti, Stefano Colagrande, Marco Matucci-Cerinic
Summary: The extent and severity of ILD can predict mortality in SSc-ILD patients on CT, as well as densitometric analysis parameters, fibrotic extent, and reticulation extent. Extensive ILD is also associated with death, need for supplemental oxygen, or lung transplantation. Honeycombing extent is a predictor for respiratory mortality.
FRONTIERS IN MEDICINE
(2022)
Review
Rheumatology
Martin Aringer, Gabriela Riemekasten
Summary: Systemic sclerosis is an autoimmune disease that causes skin and tissue fibrosis. Autologous stem cell transplantation has shown efficacy in systemic sclerosis and may also have benefits for interstitial lung disease. Some drugs have been approved for treating systemic sclerosis-associated interstitial lung disease.
BEST PRACTICE & RESEARCH IN CLINICAL RHEUMATOLOGY
(2021)
Article
Immunology
Bo Broens, Conny J. van der Laken, Gerben J. C. Zwezerijnen, Esther J. Nossent, Lilian J. Meijboom, Julia Spierings, Jeska K. de Vries-Bouwstra, Jacob M. van Laar, Alexandre E. Voskuyl
Summary: Positron emission tomography (PET) is a promising technique for evaluating systemic sclerosis associated interstitial lung disease (SSc-ILD), particularly in severe diffuse cutaneous SSc (dcSSc) patients eligible for autologous hematopoietic stem cell transplantation (aHSCT). This article discusses the potential benefits of using PET in early severe dcSSc and ILD patients in the context of aHSCT, as well as the potential value of other PET tracers in ILD assessment and understanding the mechanisms of aHSCT in the lung.
FRONTIERS IN IMMUNOLOGY
(2022)
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
Surgery
N. K. R. Sehgal, C. Scallan, C. Sullivan, M. Cedeno, J. Pencak, J. Kirkland, K. Scott, J. D. Thornton
AMERICAN JOURNAL OF TRANSPLANTATION
(2016)
Article
Immunology
N. K. R. Sehgal, C. Sullivan, C. Scallan, M. Figueroa, J. A. Pencak, J. Kirkland, K. Scott, J. D. Thornton
TRANSPLANTATION PROCEEDINGS
(2016)
Article
Pharmacology & Pharmacy
Ciaran Scallan, Robert J. Doonan, Stella S. Daskalopoulou
CLINICAL AND EXPERIMENTAL HYPERTENSION
(2010)
Review
Peripheral Vascular Disease
Robert J. Doonan, Anais Hausvater, Ciaran Scallan, Dimitri P. Mikhailidis, Louise Pilote, Stella S. Daskalopoulou
HYPERTENSION RESEARCH
(2010)
Review
Respiratory System
Amornpun Wongkarnjana, Ciaran Scallan, Martin R. J. Kolb
CURRENT OPINION IN PULMONARY MEDICINE
(2020)
Article
Cardiac & Cardiovascular Systems
Terence Ho, Ciaran Scallan, Nahid Rezaee, Nathan Hambly, P. Gerard Cox, Martin Kolb, Parameswaran Nair
RESPIRATORY MEDICINE
(2020)
Article
Respiratory System
Ciaran Scallan, Lauren Strand, Jennifer Hayes, Suha Kadura, Bridget Collins, Lawrence Ho, Carolyn Spada, Will Canestaro, Martin Kolb, Ganesh Raghu
Summary: This study developed a novel and simple tool for assessing health-related quality of life (HRQoL) in patients with IPF, which demonstrated good reliability and validity. The tool was compared with two other validated tools and showed potential for objectively measuring HRQoL in routine clinical practice for IPF patients.
EUROPEAN RESPIRATORY JOURNAL
(2022)
Article
Biochemistry & Molecular Biology
Toyoshi Yanagihara, Kazuya Tsubouchi, Mahsa Gholiof, Sy Giin Chong, Kenneth E. Lipson, Quan Zhou, Ciaran Scallan, Chandak Upagupta, Jussi Tikkanen, Shaf Keshavjee, Kjetil Ask, Martin R. J. Kolb
Summary: Idiopathic pulmonary fibrosis is a fatal lung disease characterized by the accumulation of myofibroblasts. The study found that connective-tissue growth factor (CTGF) plays a key role in pulmonary fibrosis. Increased levels of CTGF exacerbate lung fibrosis and CTGF expression is upregulated in patients with idiopathic pulmonary fibrosis. Different cells contribute to the maintenance of the fibrotic phenotype during fibrogenesis.
AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY
(2022)
Review
Pharmacology & Pharmacy
Toyoshi Yanagihara, Ciaran Scallan, Kjetil Ask, Martin R. J. Kolb
Summary: IPF is a chronic progressive lung disease with high mortality. Despite recent therapeutic advancements, there is a major interest in identifying new therapeutic targets and precision approaches to therapy. Novel technologies such as transcriptomics and serum biomarkers will play a key role in guiding future drug development and therapeutic decision making.
EXPERT OPINION ON THERAPEUTIC TARGETS
(2021)
Article
Critical Care Medicine
Toyoshi Yanagihara, Kazuya Tsubouchi, Quan Zhou, Michael Chong, Kohei Otsubo, Takuma Isshiki, Jonas C. Schupp, Seidai Sato, Ciaran Scallan, Chandak Upagupta, Spencer Revill, Anmar Ayoub, Sy Giin Chong, Anna Dvorkin-Gheva, Naftali Kaminski, Jussi Tikkanen, Shaf Keshavjee, Guillaume Pare, Christophe Guignabert, Kjetil Ask, Martin R. J. Kolb
Summary: This study investigated the role of vascular remodeling in fibrotic lungs and its contribution to the progression of fibrosis. It was found that endothelial dysfunction and activation of vascular smooth muscle cells enhance fibrogenesis through impaired BMPR2 signaling. Tacrolimus may have therapeutic potential in treating advanced IPF and concomitant PH. Additionally, genetic abnormalities may determine the development of PH in advanced IPF.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2023)
Article
Respiratory System
S. G. Chong, R. Alsilmi, C. Scallan, P. G. Cox, M. R. J. Kolb, N. Hambly, A. Naqvi
Summary: Diffuse pulmonary ossification is a rare pathological condition characterized by bone formation in the lung parenchyma. It is increasingly recognized with the use of high-resolution computed tomography (HRCT) and surgical lung biopsy. Concurrent diagnosis of diffuse pulmonary ossification and idiopathic pulmonary fibrosis may have different clinical presentations, pathologic features, and outcomes. In certain cases, diffuse pulmonary ossification may obviate the need for a surgical lung biopsy.
CANADIAN JOURNAL OF RESPIRATORY CRITICAL CARE AND SLEEP MEDICINE
(2022)
Letter
Respiratory System
Ciaran Scallan, Aida Venado, Lisa Han, Haodong Xu, Michael Mulligan, Ganesh Raghu
ANNALS OF THE AMERICAN THORACIC SOCIETY
(2020)
Article
Respiratory System
Muhammad Nouman Iqbal, Emily Stott, Anne M. Huml, Vidya Krishnan, Ciaran Joseph Scallan, Jawid Darvesh, Karthik Kode, Chloe Castro, Naveen Turlapati, Clare Landefeld, Julie Pencak, Maria Cedeno, William Baughman, Catherine Sullivan, J. Daryl Thornton
ANNALS OF THE AMERICAN THORACIC SOCIETY
(2016)
Article
Clinical Neurology
Joy Liao, Zahira Khalid, Ciaran Scallan, Carlos Morillo, Martin O'Donnell
