Review
Rheumatology
Jakob Hoeppner, Cosimo Bruni, Oliver Distler, Simon C. Robson, Gerd R. Burmester, Elise Siegert, Jorg H. W. Distler
Summary: SSc is a chronic autoimmune rheumatic disease with histopathological hallmarks including vasculopathy, fibrosis, and autoimmune phenomena. Purinergic signalling pathway may play a role in the pathophysiology of SSc, exacerbating vasculopathy and immune dysregulation, while also promoting organ and tissue fibrosis. Targeting purinergic signalling could offer new therapeutic options for SSc.
Review
Biology
Angelica Mandujano, Melissa Golubov
Summary: Systemic sclerosis is an autoimmune disease characterized by inflammation, vasculopathy, and fibrosis. Despite the development of various animal models, none fully replicate all aspects of the disease. Nailfold capillaroscopy, widely used in humans, has potential applications in animal models for studying vasculopathy and developing novel treatment options.
Article
Immunology
Benjamin Chaigne, Sarah Leonard-Louis, Luc Mouthon
Summary: Scleroderma associated myopathy (SScAM) is a heterogeneous musculoskeletal manifestation of systemic sclerosis (SSc) that lacks a clear definition. However, SScAM is associated with disability, poor quality of life, and mortality. This review provides an overview of the most up-to-date literature on SScAM, including clinical and antibody associations, recent histopathological findings, prognosis, and treatment.
AUTOIMMUNITY REVIEWS
(2023)
Review
Biochemistry & Molecular Biology
Junsuk Ko, Maria Noviani, Vasuki Ranjani Chellamuthu, Salvatore Albani, Andrea Hsiu Ling Low
Summary: Systemic sclerosis is an autoimmune disease with unclear pathophysiology, but vascular abnormalities play a significant role in its development.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Review
Rheumatology
Xiongjie Bi, Tingting Mills, Minghua Wu
Summary: This review provides an updated overview of the existing SSc animal models and the potentially relevant pathways to SSc pathogenesis, focusing on the most recently generated and investigated animal models that delve into novel pathways beyond existing models or employ genetic technologies to gain a deeper understanding of SSc pathogenesis including activation of early type I interferon (IFN) signaling pathway, immune cell function, and pulmonary artery hypertension (PAH).
CURRENT OPINION IN RHEUMATOLOGY
(2023)
Review
Medicine, Research & Experimental
Hao Ren, Licong Liu, Yangfan Xiao, Yaqian Shi, Zhuotong Zeng, Yan Ding, Puyu Zou, Rong Xiao
Summary: Systemic sclerosis is an autoimmune disease characterized by immune dysfunction, vascular system dysfunction, and tissue fibrosis. Early diagnosis and rational treatment are crucial for improving patient survival and quality of life.
BIOMEDICINE & PHARMACOTHERAPY
(2023)
Review
Dermatology
Carmen Bobeica, Elena Niculet, Mihaela Craescu, Elena-Laura Parapiru, Carmina Liana Musat, Ciprian Dinu, Iulia Chiscop, Luiza Nechita, Victorita Stefanescu, Loana Anca Stefanopol, Ana Maria Pelin, Alexandru Nechifor, Gabriela Balan, Alin Laurentiu Tatu
Summary: This paper presents data from the medical literature that have identified vestibular and auditory symptoms among patients with SSc, associating the clinical case presentation of a patient suffering from SSc, which is associated with hearing loss. The need for additional studies on larger groups of patients is underlined, in order to clarify the impact of vasculopathy and fibrosis on the acoustic and vestibular analyzer in patients with SSc.
CLINICAL COSMETIC AND INVESTIGATIONAL DERMATOLOGY
(2022)
Article
Biochemistry & Molecular Biology
Anna Wajda, Marcela Walczyk, Ewa Dudek, Barbara Stypinska, Aleksandra Lewandowska, Katarzyna Romanowska-Prochnicka, Marek Chojnowski, Marzena Olesinska, Agnieszka Paradowska-Gorycka
Summary: Systemic sclerosis (SSc) is a rare multisystem chronic disease with autoimmune features. In this study, the expression profile of selected microRNAs, including miR-126, -132, -143, -145, -155, -181a, -29a and -3148, was investigated in SSc patients compared to healthy controls. The results showed that the expression of these microRNAs was significantly altered in SSc patients, with miR-181a downregulated and miR-132, miR-143, miR-145 and miR-155 overexpressed. These findings suggest that these microRNAs may serve as potential biomarkers for SSc diagnosis and disease progression.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Medicine, General & Internal
Cosimo Bruni, Laura Cometi, Antonietta Gigante, Edoardo Rosato, Marco Matucci-Cerinic
Summary: The study found that arthritis and dyspnea are independent predictors of major vascular complications in patients with Systemic Sclerosis. While vasoactive/vasodilating drugs did not have a primary preventive effect on MVCs development, certain medications were able to slow disease progression and delay the onset of major vascular complications.
EUROPEAN JOURNAL OF INTERNAL MEDICINE
(2021)
Review
Biochemistry & Molecular Biology
Bianca Saveria Fioretto, Irene Rosa, Marco Matucci-Cerinic, Eloisa Romano, Mirko Manetti
Summary: Systemic sclerosis (SSc, scleroderma) is a rare connective tissue disease characterized by immune dysregulation, small vessel vasculopathy, impaired angiogenesis, and fibrosis. Microvascular impairment is the initial event of the disease and is linked to disabling and life-threatening clinical manifestations. Despite available treatment options, managing SSc-related vascular disease remains challenging. Vascular biomarkers have shown promise in assessing disease progression, predicting prognosis, and guiding therapy.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Endocrinology & Metabolism
Margarita Barriga, Raquel Benitez, Gema Robledo, Marta Caro, Francisco O'Valle, Jenny Campos-Salinas, Mario Delgado
Summary: This study investigates the role of the neuropeptide cortistatin in scleroderma. The findings suggest that cortistatin acts as an endogenous regulator of skin inflammation and fibrosis. Deficiency in cortistatin may indicate a poor prognosis for scleroderma and its associated complications. Cortistatin-based therapies may be potential treatments for severe systemic sclerosis and for managing fibrosis-related side effects in oncology patients receiving bleomycin chemotherapy.
NEUROENDOCRINOLOGY
(2022)
Article
Biology
Petros P. Sfikakis, Nikolaos Vlachogiannis, Panagiotis A. Ntouros, Sophie Mavrogeni, Thomas G. Maris, Apostolos H. Karantanas, Vassilis L. Souliotis
Summary: Microvascular wall abnormalities in systemic sclerosis can lead to iron microhemorrhages, which may induce fibrosis. Iron induces endothelial-to-mesenchymal transition, which can be reversed by reactive oxygen species scavengers. In vitro and in vivo studies show that iron induces oxidative stress and DNA damage in fibroblasts and mononuclear cells. Magnetic resonance imaging also reveals increased iron deposition in the fingers of a patient with early systemic sclerosis. This study suggests that iron may be a pathogenetic link between microvasculopathy and fibrosis, as well as a mechanism for increased oxidative stress in systemic sclerosis.
Review
Medicine, General & Internal
Maria Martin-Lopez, Patricia E. Carreira
Summary: Systemic sclerosis (SSc) is an autoimmune connective tissue disease characterized by immune dysregulation and progressive fibrosis. Interstitial lung disease (ILD) is the leading cause of death in patients with SSc. The management of SSc-ILD has shifted to include targeted biologic and antifibrotic therapies.
JOURNAL OF CLINICAL MEDICINE
(2023)
Review
Rheumatology
Tracy M. Frech
Summary: This review discusses the imaging techniques for vascular assessment in systemic sclerosis (SSc), including nailfold and oral capillaroscopy, laser speckle contrast analysis, ultrasound techniques, and mobile infrared thermography and optical coherence tomography. The equipment cost, technical training, data standardization, and invasiveness of these techniques are critical for their implementation in SSc clinical trials and practice to understand progressive vasculopathy.
CURRENT OPINION IN RHEUMATOLOGY
(2022)
Article
Medicine, General & Internal
Giorgia Leodori, Chiara Pellicano, Antonietta Gigante, Edoardo Rosato
Summary: This study evaluated the role of renal resistance indices and estimated glomerular filtration rate as predictive markers of mortality in systemic sclerosis patients over a 10-year follow-up. It was found that eGFR was a predictive marker of mortality due to SSc and all causes, while RRI was a predictive marker of mortality due to all causes.
EUROPEAN JOURNAL OF INTERNAL MEDICINE
(2021)
Article
Rheumatology
Elisabeth Blaja, Suzana Jordan, Carmen-Marina Mihai, Rucsandra Dobrota, Mike Oliver Becker, Britta Maurer, Marco Matucci-Cerinic, Oliver Distler
Summary: The study demonstrated that very early systemic sclerosis (SSc) patients are a heterogeneous group with mild or early disease, showing diverse clinical presentations. Some patients may have long-standing mild disease, and there was no difference in clinical features at initial presentation between patients with potentially progressive disease and those with long-standing mild disease. These findings highlight the importance of considering the heterogeneity of very early SSc patients for risk stratification and study design.
JOURNAL OF RHEUMATOLOGY
(2021)
Letter
Rheumatology
Dinesh Khanna, Oliver Distler
ANNALS OF THE RHEUMATIC DISEASES
(2022)
Review
Rheumatology
Paolo Spagnolo, Oliver Distler, Christopher J. Ryerson, Argyris Tzouvelekis, Joyce S. Lee, Francesco Bonella, Demosthenes Bouros, Anna-Maria Hoffmann-Vold, Bruno Crestani, Eric L. Matteson
Summary: This study discusses the different types of interstitial lung diseases (ILDs) and their association with autoimmune and connective tissue diseases (CTDs), as well as introduces drug treatment studies for some fibrosing ILDs. It emphasizes the importance of fibrosis in ILDs.
ANNALS OF THE RHEUMATIC DISEASES
(2021)
Article
Clinical Neurology
Andrea M. Aegerter, Manja Deforth, Venerina Johnston, Gisela Sjogaard, Thomas Volken, Hannu Luomajoki, Julia Dratva, Holger Dressel, Oliver Distler, Achim Elfering, Markus Melloh
Summary: Working from home during the COVID-19 pandemic did not show clinically relevant changes in neck pain intensity and disability. The number of work breaks and hours spent at the computer were found to have a greater effect on neck pain than the place of work or workstation ergonomics. Further research should investigate the impact of social and psychological factors on neck pain among office workers.
EUROPEAN SPINE JOURNAL
(2021)
Article
Immunology
Monika Krosel, Marcel Gabathuler, Malgorzata Maciukiewicz, Larissa Moser, Gideon Isaac Lee, Miriam Marks, Matija Tomsic, Oliver Distler, Caroline Ospelt, Kerstin Klein
Summary: In rheumatoid arthritis, chromatin remodeling and histone 3 lysine 27 acetylation are associated with sustained inflammatory responses in synovial fibroblasts. Writer proteins CBP and p300 have overlapping but distinct regulatory functions in controlling gene expression, with p300 being the major HAT for H3K27ac. Inhibition of CBP/p300 mirrors effects seen with p300 silencing, suggesting that inhibitors targeting these proteins may not be sufficient as anti-inflammatory drugs.
JOURNAL OF AUTOIMMUNITY
(2021)
Review
Rheumatology
Jakob Hoeppner, Cosimo Bruni, Oliver Distler, Simon C. Robson, Gerd R. Burmester, Elise Siegert, Jorg H. W. Distler
Summary: SSc is a chronic autoimmune rheumatic disease with histopathological hallmarks including vasculopathy, fibrosis, and autoimmune phenomena. Purinergic signalling pathway may play a role in the pathophysiology of SSc, exacerbating vasculopathy and immune dysregulation, while also promoting organ and tissue fibrosis. Targeting purinergic signalling could offer new therapeutic options for SSc.
Review
Rheumatology
Diana Andronic, Octavian Andronic, Astrid Juengel, Martin C. Berli, Oliver Distler, Florian Brunner
Summary: Despite ongoing research, the exact underlying processes of Complex Regional Pain Syndrome (CRPS) are still not well understood. This systematic review examines potential cutaneous biomarkers in CRPS and identifies the implications for various pathophysiological processes and skin morphology. The findings suggest involvement of inflammation, vascular dysregulation, small fiber neuropathy, and hypersensitivity. Additionally, skin morphology changes, including neurite loss, disturbed mast cell migration, and increased expression of alpha 1-adrenoceptors on keratinocytes, were observed. These results provide a foundation for future developments in diagnosis and potential therapeutic targets for CRPS Type I patients.
RHEUMATOLOGY INTERNATIONAL
(2022)
Article
Rheumatology
Eric L. Matteson, Clive Kelly, Joerg H. W. Distler, Anna-Maria Hoffmann-Vold, James R. Seibold, Shikha Mittoo, Paul F. Dellaripa, Martin Aringer, Janet Pope, Oliver Distler, Alexandra James, Rozsa Schlenker-Herceg, Susanne Stowasser, Manuel Quaresma, Kevin R. Flaherty
Summary: In fibrosing autoimmune disease-related interstitial lung diseases (ILDs) with a progressive phenotype, nintedanib demonstrated efficacy in slowing the rate of decline in forced vital capacity (FVC), with manageable adverse events.
ARTHRITIS & RHEUMATOLOGY
(2022)
Article
Rheumatology
Yannick Allanore, Madelon C. Vonk, Oliver Distler, Arata Azuma, Maureen D. Mayes, Martina Gahlemann, Alexandra James, Veronika Kohlbrenner, Margarida Alves, Dinesh Khanna, Kristin B. Highland
Summary: Nintedanib can reduce the decline in forced vital capacity (FVC) in patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD) and has consistent safety profile and FVC outcomes in both short-term and long-term use.
ANNALS OF THE RHEUMATIC DISEASES
(2022)
Letter
Rheumatology
Raphael Micheroli, Chantal Pauli, Kristina Buerki, Philipp Rossbach, Oliver Distler, Caroline Ospelt, Adrian Ciurea
Article
Rheumatology
Raphael Micheroli, Seraphina Kissling, Kristina Buerki, Pascale Exer, Rene Braem, Michael J. Nissen, Burkhard Moeller, Michael Andor, Oliver Distler, Almut Scherer, Adrian Ciurea
Summary: The study findings suggest that TNFi can decelerate the radiographic progression of SIJ in patients with axSpA, especially when used for at least 1 year.
Article
Rehabilitation
Andrea Martina Aegerter, Manja Deforth, Thomas Volken, Venerina Johnston, Hannu Luomajoki, Holger Dressel, Julia Dratva, Markus Josef Ernst, Oliver Distler, Beatrice Brunner, Gisela Sjogaard, Markus Melloh, Achim Elfering
Summary: This study investigated the effect of a multi-component intervention on neck pain-related work productivity loss among Swiss office workers. The results showed that the intervention had a significant reduction in neck pain-related work productivity loss, with implications for employers, employees, and policy makers.
JOURNAL OF OCCUPATIONAL REHABILITATION
(2023)
Article
Rheumatology
Alexandru Garaiman, Farhad Nooralahzadeh, Carina Mihai, Nicolas Perez Gonzalez, Nikitas Gkikopoulos, Mike Oliver Becker, Oliver Distler, Michael Krauthammer, Britta Maurer
Summary: The study aims to evaluate the performance of a vision transformer-based deep-learning model in identifying microangiopathy in NFC images of SSc patients and compare it with the performance of rheumatologists. The results show that the ViT performs well in identifying different microangiopathic changes, but there is still room for improvement in terms of reliability compared to rheumatologists.
Article
Rheumatology
Tea Gegenava, Federico Fortuni, Nina Marijn van Leeuwen, Anders H. Tennoe, Anna-Maria Hoffmann-Vold, Ruxandra Jurcut, Adrian Giuca, Laura Groseanu, Felix Tanner, Oliver Distler, Jeroen J. Bax, Jeska De Vries-Bouwstra, Nina Ajmone Marsan
Summary: This study aimed to assess the impact of sex differences on cardiovascular outcomes in patients with systemic sclerosis (SSc). The results showed that male SSc patients had a higher incidence of cardiovascular events, and the adjusted left ventricular global longitudinal strain may explain this gender difference in outcomes.
Article
Rheumatology
Caroline Evers, Suzana Jordan, Britta Maurer, Mike Oliver Becker, Carina Mihai, Rucsandra Dobrota, Petra Hoederath, Oliver Distler
Summary: Pain assessment is important in systemic sclerosis (SSc) patients, with hand and lower back being common pain locations. Pain chronification is associated with pain location, psychological issues, and well-being, emphasizing the need to prevent chronic pain to improve quality of life.
ARTHRITIS RESEARCH & THERAPY
(2021)
