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)
Review
Rheumatology
Michael Hughes, Bashar Kahaleh, Christopher P. Denton, Justin C. Mason, Marco Matucci-Cerinic
Summary: In patients with SSc, the presence of SSc-AAV is associated with a severe disease course, typically presenting with features of microscopic polyangiitis or renal-limited vasculitis. The interaction between SSc and AAV exacerbates vascular disease, with similar patterns of major organ involvement. Current management strategies are based on treating SSc-AAV similar to isolated AAV, highlighting the need for further investigation and therapeutic strategies specific to this rare clinical entity.
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
Michael Hughes, Elisabetta Zanatta, Robert D. Sandler, Jerome Avouac, Yannick Allanore
Summary: This study found that despite the heterogeneity and scarcity of vascular disease in SSc, there have been significant improvements in various vascular complications over time, leading to improved survival rates for patients.
Article
Immunology
Yuyang Han, Ping Tu
Summary: This study retrospectively observed eight patients with refractory livedoid vasculopathy who received treatment with baricitinib. The results showed that baricitinib treatment significantly relieved the signs and symptoms of livedoid vasculopathy and was well tolerated.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Immunology
Isabella M. Atzeni, Yehya Al-Adwi, Berber Doornbos van der Meer, Caroline Roozendaal, Alja Stel, Harry van Goor, C. Tji Gan, Michael Dickinson, Wim Timens, Andries J. Smit, Johanna Westra, Douwe J. Mulder
Summary: This study investigated the levels of soluble RAGE (sRAGE) and its ligand HMGB1 in SSc and their abilities to predict SSc-related pulmonary complications. The results showed that high baseline sRAGE levels may serve as a prospective biomarker for patients with SSc at high risk to develop new onset of PAH, and high sRAGE levels could predict lower survival rates due to PAH in patients with SSc.
FRONTIERS IN IMMUNOLOGY
(2023)
Review
Cardiac & Cardiovascular Systems
Andrew B. Haymet, Nicole Bartnikowski, Emily S. Wood, Michael P. Vallely, Angela McBride, Sophie Yacoub, Scott B. Biering, Eva Harris, Jacky Y. Suen, John F. Fraser
Summary: The glycocalyx layer coating human cells is essential for cell health and function, but it can be easily damaged by various disease conditions. Current research has used different in vitro models to study the glycocalyx, but there is a need for new models that can incorporate multiple features simultaneously.
FRONTIERS IN CARDIOVASCULAR MEDICINE
(2021)
Review
Rheumatology
Chingching Foocharoen, Pantipa Tonsawan, Patnarin Pongkulkiat, Sirirat Anutrakulchai, Ajanee Mahakkanukrauh, Siraphop Suwannaroj
Summary: Scleroderma renal crisis (SRC) is a severe internal organ involvement caused by systemic sclerosis, with high rates of renal replacement therapy and mortality despite various treatments. The optimal treatment for SRC depends on its stage, with angiotensin-converting enzyme inhibitor being the choice. Other vasodilators and complement cascade blockers have been investigated for SRC, but no randomized control trial has been conducted. A new approach for SRC management based on specific clinical features and systemic sclerosis-thrombotic microangiopathy is proposed.
MODERN RHEUMATOLOGY
(2023)
Article
Rheumatology
Maya Malaab, Ludivine Renaud, Naoko Takamura, Kip D. Zimmerman, Willian A. da Silveira, Paula S. Ramos, Sandra Haddad, Marc Peters-Golden, Loka R. Penke, Bethany Wolf, Gary Hardiman, Carl D. Langefeld, Thomas A. Medsger, Carol A. Feghali-Bostwick
Summary: Our study identified distinct molecular features in dermal fibroblasts of systemic sclerosis patients, including dysregulated transcription factors and microRNAs, suggesting a role for epigenetic dysregulation in disease susceptibility. This highlights the potential for using epigenetic modifiers as future therapies in systemic sclerosis.
ANNALS OF THE RHEUMATIC DISEASES
(2022)
Article
Peripheral Vascular Disease
Rosario Cianci, Antonietta Gigante, Domenico Bagordo, Giovanni Pintus, Antonello Giovannetti, Silvia Lai, Sandro Mazzaferro, Edoardo Rosato
Summary: This retrospective observational study found that IgA nephropathy patients have lower RRI values compared to patients with renal scleroderma vasculopathy. Additionally, IgA nephropathy patients showed a higher prevalence of hypertension and were younger in age compared to the renal scleroderma vasculopathy patients.
MICROVASCULAR RESEARCH
(2021)
Article
Rheumatology
Robyn T. Domsic, Thomas A. Medsger, Shiyao Gao, Maureen Laffoon, Suiyuan Huang, Stephen Wisniewski, Cathie Spino, Virginia Steen, Robert Lafyatis, Dinesh Khanna
Summary: By analyzing clinical characteristics and laboratory values, we identified strategies to improve trial design for early diffuse SSc, including skin thickness progression rate, RNA polymerase III antibody positivity, and presence of tendon friction rubs. Applying these strategies to actual trial data can reduce the decline in placebo group skin scores and reveal significant differences in the CRISS score.
Article
Rheumatology
Nina Marijn van Leeuwen, Marc Maurits, Sophie Liem, Jacopo Ciaffi, Nina Ajmone Marsan, Maarten Ninaber, Cornelia Allaart, Henrike Gillet van Dongen, Robbert Goekoop, Tom Huizinga, Rachel Knevel, Jeska De Vries-Bouwstra
Summary: A machine learning model was developed to predict disease progression in systemic sclerosis patients, using data from a prospective cohort. Relevant clinical variables were identified for predicting disease progression.
Article
Cell Biology
Christopher W. Wasson, Begona Caballero-Ruiz, Justin Gillespie, Emma Derrett-Smith, Jamel Mankouri, Christopher P. Denton, Gianluca Canettieri, Natalia A. Riobo-Del Galdo, Francesco Del Galdo
Summary: This study reveals that CLIC4 expression is regulated by the signaling crosstalk between TGF-beta, Wnt-3a, and Hh, and that it strongly correlates with the expression of the pro-fibrotic marker alpha-smooth muscle actin. Further investigations demonstrate that inhibiting CLIC4 can reverse morphogen-dependent fibroblast activation.
Review
Pharmacology & Pharmacy
Thomas H. Thatcher, Margaret A. T. Freeberg, Yu Par Aung Myo, Patricia J. Sime
Summary: Pulmonary fibrotic diseases are characterized by excessive deposition of extracellular matrix proteins due to the proliferation of lung fibroblasts and myofibroblasts. Recent research has shown that specialized pro-resolving mediators (SPMs) can inhibit fibroblast proliferation, myofibroblast differentiation, and the accumulation of excess extracellular matrix. While there are limited reports on SPMs and fibrosis, especially pulmonary fibrosis, their therapeutic implications in fibrosis are promising.
PHARMACOLOGY & THERAPEUTICS
(2023)
Review
Cardiac & Cardiovascular Systems
Antonio Totaro, Cosimo Sacra, Gianluca Testa, Antonio Pierro, Vincenzo Ienco, Eugenio Calvo, Rossella Calabrese, Gaetano Castellano, Ennio Lubrano, Fabio Perrotta, Silvia Scriffignano, Antonio Maria Calafiore
Summary: This is a case report of a rare complication of systemic sclerosis, calcific constrictive pericarditis, treated with pericardiectomy, resulting in significant clinical improvement.
CURRENT PROBLEMS IN CARDIOLOGY
(2023)
