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.
Review
Medicine, General & Internal
Woon Hean Chong, Biplab K. Saha, Scott Beegle
Summary: Systemic sclerosis (SSc) should be considered in patients diagnosed with idiopathic interstitial lung disease (ILD), even without typical scleroderma cutaneous features. Systemic sclerosis sine scleroderma (ssSSc) is a rare subtype that requires the absence of skin thickening but the presence of specific criteria for diagnosis. The radiological and histopathological features of ssSSc-associated ILD are similar to other types of interstitial pneumonia, making diagnosis challenging.
AMERICAN JOURNAL OF THE MEDICAL SCIENCES
(2023)
Review
Medicine, General & Internal
Peter Klein-Weigel, Oliver Sander, Simone Reinhold, Jessica Nielitz, Julia Steindl, Jutta Richter
Summary: Raynaud's phenomenon (RP) is characterized by vasospasm of the fingers or toes triggered by cold or emotional stressors. The most important diagnostic steps include demonstrating a tendency towards vasospasm, excluding occlusions, nail-fold capillaroscopy, and determining autoantibody status. The main treatment options are protection against cold and administration of calcium antagonists.
DEUTSCHES ARZTEBLATT INTERNATIONAL
(2021)
Article
Rheumatology
Kathleen Morrisroe, Dylan Hansen, Wendy Stevens, Laura Ross, Joanne Sahhar, Gene-Siew Ngian, Catherine L. Hill, Lauren Host, Jennifer Walker, Susanna Proudman, Mandana Nikpour
Summary: This study aimed to describe the frequency of progressive pulmonary fibrosis (PPF) in an incident cohort of systemic sclerosis (SSc)-related interstitial lung disease (ILD) and its impact on survival. The results showed that about one-third of the patients experienced PPF, and the presence of PPF was significantly associated with mortality. This suggests that some patients may benefit from earlier introduction of immunosuppressive and/or antifibrotic therapy.
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
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.
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)
Article
Clinical Neurology
Hung-Ling Huang, Wen-Chih Lin, Po-Yu Lin, Meng-Yu Weng, Yuan-Ting Sun
Summary: This study identified factors associated with interstitial lung disease (ILD) in patients with idiopathic inflammatory myopathy (IIM) based on the latest classification and recent advances in autoantibody serology. The presence of mechanic's hand, older age of onset, and specific autoantibodies were significantly associated with ILD in IIM patients. The study results may help physicians in timely screening and monitoring pulmonary function in high-risk groups like those with overlap myositis (OM) and dermatomyositis (DM).
NEUROLOGICAL SCIENCES
(2021)
Article
Rheumatology
Abeer Ghuman, Dinesh Khanna, Celia J. F. Lin, Daniel E. Furst, Ganesh Raghu, Fernando J. Martinez, Mauro Zucchetto, Suiyuan Huang, Angus Jennings, Svetlana Nihtyanova, Christopher P. Denton
Summary: This study investigated prognostic and predictive markers for SSc-associated interstitial lung disease (SSc-ILD). The results showed that male patients were more likely to experience a decline in lung function. In the anti-inflammatory treatment, sex, early disease duration, IL-6 levels, and anti-topoisomerase antibodies were predictive factors for reducing lung function decline.
Review
Medicine, General & Internal
Shire Chaudhry, Lisa Christopher-Stine
Summary: The aim of this review is to analyze and evaluate the clinical significance of myositis specific autoantibodies (MSA) in idiopathic inflammatory myopathies (IIM) and interstitial lung disease (ILD). It reviews literature published from 2005 onwards and provides recommendations for multidisciplinary longitudinal care practices for patients with IIM-ILD, focusing on imaging and other testing. Treatment is not covered.
FRONTIERS IN MEDICINE
(2023)
Review
Medicine, General & Internal
Woon H. Chong, Biplab K. Saha, Scott Beegle
Summary: Antisynthetase syndrome (AS) is a rare disease that often presents with antisynthetase syndrome-associated interstitial lung disease (AS-ILD). The type of myositis-specific antibodies present may influence the frequency of interstitial lung disease (ILD), myopathy, and skin involvement in AS patients. Patients with positive anti-Jo-1 and anti-SSA/RC-52 autoantibodies may have severe lung restriction and may not respond well to immunosuppressive therapies.
AMERICAN JOURNAL OF THE MEDICAL SCIENCES
(2021)
Article
Cell Biology
Espiridion Ramos-Martinez, Angel E. Vega-Sanchez, Gloria Perez-Rubio, Mayra Mejia, Ivette Buendia-Roldan, Montserrat Gonzalez-Perez, Heidegger N. Mateos-Toledo, Warrison A. Andrade, Ramces Falfan-Valencia, Jorge Rojas-Serrano
Summary: Anti-synthetase syndrome (ASSD) is characterized by inflammatory interstitial lung disease (ILD). This study compared the concentrations of cytokines and molecules associated with inflammasome activation in bronchoalveolar lavage (BAL) of ASSD patients and systemic sclerosis (SSc) patients. Higher levels of caspase-1 and LDH activity were observed in ASSD patients, indicating cell death and inflammasome pathway activation. There were significant differences in IL-18 and IFN-gamma levels between the two groups.
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
Rheumatology
Xiaoyang Yue, Junping Yin, Xiaoqing Wang, Harald Heidecke, Alexander Maximilian Hackel, Xiaoru Dong, Brigitte Kasper, Lifang Wen, Liang Zhang, Kai Schulze-Forster, Juliane Junker, Hanna Grasshoff, Antje Mueller, Gerd Wallukat, Ingolf Schimke, Julian Zeiner, Lisa Marie Deckstein, Nicole Mertens, Anja Kerstein-Staehle, Jennifer Elisabeth Hundt, Evi Kostenis, Xinhua Yu, Gabriela Riemekasten, Frank Petersen
Summary: This study successfully induced AT1R antibodies using an immunization strategy and found that these antibodies activate the AT1R, leading to inflammation and fibrosis. These findings suggest that AT1R antibodies could be potential targets for therapies of systemic sclerosis (SSc) and other AT1R antibody-related diseases.
ANNALS OF THE RHEUMATIC DISEASES
(2022)
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)
