Article
Multidisciplinary Sciences
Bart Kramer, Claudio Corallo, Angelique van den Heuvel, Justin Crawford, Thomas Olivier, Edo Elstak, Nicola Giordano, Paul Vulto, Henriette L. Lanz, Richard A. J. Janssen, Michela A. Tessari
Summary: The researchers developed an angiogenesis-on-a-chip platform to assess disease parameters related to SSc and other vasculopathies. They demonstrated that their in vitro model is suitable for compound testing and identifying new drugs that can protect microvascular stability. Additionally, they found that sera from SSc patients have an anti-angiogenic effect.
SCIENTIFIC REPORTS
(2022)
Review
Medicine, General & Internal
Kholoud Almaabdi, Zareen Ahmad, Sindhu R. Johnson
Summary: Systemic sclerosis is an autoimmune disease characterized by immune abnormalities, vasculopathy, and fibrosis. Autoantibody testing, including ANA, anti-Scl-70, and anticentromere antibodies, has become increasingly important for diagnosis and prognostication. This review examines the epidemiology, clinical associations, and prognostic value of advanced autoantibody testing in systemic sclerosis.
Review
Immunology
Aurelien Chepy, Louisa Bourel, Vincent Koether, David Launay, Sylvain Dubucquoi, Vincent Sobanski
Summary: Systemic sclerosis (SSc) is a connective tissue disease characterized by extensive fibrosis of the skin and internal organs. Antinuclear antibodies (ANA) are found in almost all SSc patients, but the exact role they play in the disease remains unclear. However, studies have shown that ANA may be associated with disease subtypes, appear before disease onset, correlate with disease activity and severity, and have a response to B-cell targeting therapy.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Hematology
Hannah Kinoshita, Mamatha Mandava, Mariah Jensen-Wachspress, Haili Lang, Elisabeth Joy, Jay Tanna, Chase D. McCann, Samuel O'Brien, Sianna Burnett, Abeer Shibli, Fahmida Hoq, Monica Bhatia, Patrick J. Hanley, Blachy Davila Saldana, Kris M. Mahadeo, Catherine M. Bollard, Michael D. Keller, Allistair Abraham
Summary: Hematopoietic stem cell transplantation (HSCT) is increasingly used to treat sickle cell disease (SCD). However, this treatment increases the risk of viral infection. This study explores the use of virus-specific T-cell (VST) therapies after HSCT to prevent or treat viral infections in SCD patients.
Editorial Material
Medicine, General & Internal
Magda Zanelli, Stefano Ricci, Maurizio Zizzo, Francesca Sanguedolce, Federica De Giorgi, Andrea Palicelli, Giovanni Martino, Stefano Ascani
Summary: A 79-year-old woman presented with peripheral eosinophilia, with a history of colonic polyposis and subsequent right hemicolectomy. Laboratory tests revealed mild macrocitic anaemia, elevated beta 2 microglobulin and serum tryptase levels, and the presence of IgA/kappa monoclonal protein and Bence-Jones protein. Bone marrow biopsy showed two neoplastic components - mast cells and plasma cells, with molecular analysis demonstrating mutations and chromosomal abnormalities. The rare coexistence of systemic mastocytosis and multiple myeloma led to treatment with midostaurin therapy.
Article
Multidisciplinary Sciences
Koen van de Ven, Josien Lanfermeijer, Harry van Dijken, Hiromi Muramatsu, Caroline Vilas Boas de Melo, Stefanie Lenz, Florence Peters, Mitchell B. Beattie, Paulo J. C. Lin, Jose A. Ferreira, Judith van den Brand, Debbie van Baarle, Norbert Pardi, Jorgen de Jonge
Summary: This study evaluated a novel T cell-inducing nucleoside-modified messenger RNA (mRNA) vaccine that encoded conserved proteins of an H1N1 influenza virus, and found that it induced broadly reactive T cells and enhanced protection against potential influenza viruses.
Article
Biochemistry & Molecular Biology
Fangru Chen, Jian Meng, Wenjie Yan, Mengjiao Wang, Yunfei Jiang, Jintao Gao
Summary: This study identifies TK1 as a promising therapeutic target for systemic lupus erythematosus, as silencing TK1 can alleviate inflammation, growth arrest, and senescence in BMSCs of SLE.
FRONTIERS IN MOLECULAR BIOSCIENCES
(2022)
Article
Medicine, General & Internal
Wakar Garra, Yair Levy
Summary: This study aimed to estimate the prevalence of Anti-ARS antibodies among patients with systemic sclerosis and evaluate their clinical associations and risk factors for interstitial lung disease. The results showed a 6% prevalence of Anti-ARS antibodies, which were associated with the development of ILD.
EUROPEAN JOURNAL OF INTERNAL MEDICINE
(2023)
Article
Hematology
Swetha Kambhampati, Ying Sheng, Chiung-Yu Huang, Sophia Bylsma, Mimi Lo, Vanessa Kennedy, Kelsey Natsuhara, Thomas Martin, Jeffrey Wolf, Nina Shah, Sandy W. Wong
Summary: B-cell maturation antigen-targeted chimeric antigen receptor T-cell therapy is an effective treatment for relapsed refractory multiple myeloma. This study analyzed the infection outcomes of 55 MM patients treated with BCMA CAR-T and found that despite multiple risk factors, severe infections were relatively rare.
Article
Dermatology
Yan Huang, Han Zhao, Xiangguang Shi, Jing Liu, Jui-Ming Lin, Qianqian Ma, Shuai Jiang, Weilin Pu, Yanyun Ma, Jianlan Liu, Wenyu Wu, Jiucun Wang, Qingmei Liu
Summary: This study found that GRB2 is highly expressed in endothelial cells of the skin in patients with systemic sclerosis (SSc), and inhibiting GRB2 can effectively attenuate skin fibrosis and endothelial cell apoptosis. Transcriptome and phosphoproteomic analysis revealed the important role of the focal adhesion pathway in the regulation. These findings suggest that GRB2 may be a potential new therapeutic target for SSc.
JOURNAL OF DERMATOLOGICAL SCIENCE
(2023)
Review
Cell Biology
Daniel Levin, Mohammed S. Osman, Caylib Durand, Hyein Kim, Iman Hemmati, Kareem Jamani, Jonathan G. Howlett, Kerri A. Johannson, Jason Weatherald, Matthew Woo, Jason Lee, Jan Storek
Summary: Systemic sclerosis is a complex connective tissue disease with limited efficacy from conventional therapies. Autologous hematopoietic stem cell transplantation shows promise but comes with associated risks. Patient selection, efficacy, and toxicity are vital considerations for treatment.
Article
Medicine, General & Internal
Audrey Benyamine, Daniel Bertin, Noemie Resseguier, Xavier Heim, Julien Bermudez, David Launay, Sylvain Dubucquoi, Adrian Hij, Dominique Farge, Alain Lescoat, Isabelle Bahon-Riedinger, Nouria Benmostefa, Luc Mouthon, Jean-Robert Harle, Gilles Kaplanski, Pascal Rossi, Nathalie Bardin, Brigitte Granel
Summary: The study found that AFAs in SSc patients have important clinical associations, especially when patients are negative for conventional antibodies and exhibit a typical IIF pattern. AFAs have diagnostic value in SSc and may serve as an interesting marker of disease severity.
Review
Rheumatology
Herve Lobbes, Matthieu Mahevas, Sophie Alviset, Lionel Galicier, Nathalie Costedoat-Chalumeau, Zahir Amoura, Laurent Alric, Arnaud Hot, Stephane Durupt, Marc Michel, Bertrand Godeau
Summary: SLE-associated PRCA is a severe condition requiring repeated red blood cell transfusions and various lines of immunosuppressant therapy, with a high risk of severe infectious events and iron overload. Despite sustained response obtained in most patients, the optimal therapeutic strategy remains to be determined.
Article
Medicine, General & Internal
Pawel Zebryk, Piotr Przymuszala, Jan Krzysztof Nowak, Tomasz Piorunek, Tatiana Mularek-Kubzdela, Mariusz Puszczewicz
Summary: This study evaluated the prevalence and clinical significance of SSc-related autoantibodies and compared the sensitivity of two line immunoblot assays. The findings indicate that different autoantibodies are associated with specific characteristics of SSc.
JOURNAL OF CLINICAL MEDICINE
(2023)
Article
Biochemistry & Molecular Biology
Yutong Shi, Caiyun Qi, Ying Bai
Summary: This study analyzed the metabolism of bone marrow cells from patients with multiple sclerosis (MS) at the single-cell level and found activated metabolic pathways and altered cell-cell communication in the plasma cell population. These findings provide insights into the pathogenesis of MS and may contribute to the development of novel immune therapies.
Letter
Rheumatology
Clodoveo Ferri, Dilia Giuggioli, Vincenzo Raimondo, Poupak Fallahi, Alessandro Antonelli
ANNALS OF THE RHEUMATIC DISEASES
(2023)
Meeting Abstract
Rheumatology
C. Ferri, V. Raimondo, L. Gragnani, D. Giuggioli, L. Dagna, A. Tavoni, F. Ursini, M. L'andolina, F. Caso, P. Ruscitti, M. Caminiti, R. Foti, V. Riccieri, S. Guiducci, R. Pellegrini, E. Zanatta, G. Varcasia, D. Olivo, P. Gigliotti, G. Cuomo, G. Murdaca, R. Cecchetti, R. De Angelis, N. Romeo, F. Ingegnoli, F. Cozzi, V. Codullo, I. Cavazzana, M. Colaci, G. Abignano, M. De Santis, E. Lubrano, E. Fusaro, A. Spinella, F. Lumetti, G. De Luca, S. Bellando Randone, E. Visalli, Y. Dal Bosco, G. Amato, D. Giannini, S. Bilia, F. Masini, G. Pellegrino, E. Pigatto, E. Generali, G. Pagano Mariano, G. Pettiti, G. Zanframundo, R. Brittelli, V. Aiello, R. Caminiti, D. Scorpiniti, T. Ferrari, C. Campochiaro, V. Brusi, M. Fredi, L. Moschetti, F. Cacciapaglia, S. M. Ferrari, I. Di Cola, M. Vadacca, S. Lorusso, M. Monti, S. Lorini, S. R. Paparo, F. Ragusa, G. Elia, V. Mazzi, M. L. Aprile, M. Tasso, M. Miccoli, S. L. Bosello, S. D'angelo, A. Doria, F. Franceschini, R. Meliconi, M. Matucci-Cerinic, F. Iannone, R. Giacomelli, C. Salvarani, A. L. Zignego, P. Fallahi, A. Antonelli
ANNALS OF THE RHEUMATIC DISEASES
(2022)
Article
Pharmacology & Pharmacy
Clodoveo Ferri, Vincenzo Raimondo, Laura Gragnani, Dilia Giuggioli, Lorenzo Dagna, Antonio Tavoni, Francesco Ursini, Massimo L'Andolina, Francesco Caso, Piero Ruscitti, Maurizio Caminiti, Rosario Foti, Valeria Riccieri, Serena Guiducci, Roberta Pellegrini, Elisabetta Zanatta, Giuseppe Varcasia, Domenico Olivo, Pietro Gigliotti, Giovanna Cuomo, Giuseppe Murdaca, Riccardo Cecchetti, Rossella De Angelis, Nicoletta Romeo, Francesca Ingegnoli, Franco Cozzi, Veronica Codullo, Ilaria Cavazzana, Michele Colaci, Giuseppina Abignano, Maria De Santis, Ennio Lubrano, Enrico Fusaro, Amelia Spinella, Federica Lumetti, Giacomo De Luca, Silvia Bellando-Randone, Elisa Visalli, Ylenia Dal Bosco, Giorgio Amato, Daiana Giannini, Silvia Bilia, Francesco Masini, Greta Pellegrino, Erika Pigatto, Elena Generali, Giuseppa Pagano Mariano, Giorgio Pettiti, Giovanni Zanframundo, Raffaele Brittelli, Vincenzo Aiello, Rodolfo Caminiti, Daniela Scorpiniti, Tommaso Ferrari, Corrado Campochiaro, Veronica Brusi, Micaela Fredi, Liala Moschetti, Fabio Cacciapaglia, Sabrina Rosaria Paparo, Francesca Ragusa, Valeria Mazzi, Giusy Elia, Silvia Martina Ferrari, Ilenia Di Cola, Marta Vadacca, Sebastiano Lorusso, Monica Monti, Serena Lorini, Maria Letizia Aprile, Marco Tasso, Mario Miccoli, Silvia Bosello, Salvatore D'Angelo, Andrea Doria, Franco Franceschini, Riccardo Meliconi, Marco Matucci-Cerinic, Florenzo Iannone, Roberto Giacomelli, Carlo Salvarani, Anna Linda Zignego, Poupak Fallahi, Alessandro Antonelli
Summary: This Italian study investigated the prevalence, prognostic factors, and outcomes of COVID-19 in autoimmune systemic diseases (ASD) patients. The study found that ASD patients had a higher prevalence of COVID-19 compared to the general population but a comparable death rate. Among ASD patients, systemic sclerosis (SSc) patients had a significantly higher COVID-19-related death rate. Older age, male gender, SSc, pre-existing lung involvement, and long-term steroid treatment were identified as major adverse prognostic factors for developing COVID-19. Patients treated with conventional synthetic disease-modifying antirheumatic drugs (csDMARDs) and SSc patients treated with low dose aspirin had a significantly lower prevalence of COVID-19.
CURRENT PHARMACEUTICAL DESIGN
(2022)
Article
Medicine, General & Internal
Michele Colaci, Luca Zanoli, Alberto Lo Gullo, Domenico Sambataro, Gianluca Sambataro, Maria Letizia Aprile, Pietro Castellino, Lorenzo Malatino
Summary: Systemic sclerosis (SSc) patients showed increased arterial stiffness compared to healthy controls, as indicated by higher carotid-femoral PWV, carotid-radial PWV, and intima-media thickness in SSc. The positive correlation between PWV and age was observed in SSc, and younger SSc patients had lower carotid elasticity compared to age-matched controls.
JOURNAL OF CLINICAL MEDICINE
(2022)
Review
Hematology
Jurgen Thiele, Hans Michael Kvasnicka, Attilio Orazi, Umberto Gianelli, Naseema Gangat, Alessandro M. Vannucchi, Tiziano Barbui, Daniel A. Arber, Ayalew Tefferi
Summary: A group of international experts met to update the World Health Organization classification system for hematopoietic tumors and introduced the new International Consensus Classification (ICC) for Myeloid Neoplasms and Acute Leukemias. The focus of this review is on the ICC-2022 category of JAK2 mutation-prevalent myeloproliferative neoplasms (MPNs) and the importance of bone marrow morphology and genetic markers in disease classification and diagnostics.
AMERICAN JOURNAL OF HEMATOLOGY
(2023)
Article
Biochemistry & Molecular Biology
Giuseppe Argentino, Alessandro Barbieri, Ruggero Beri, Caterina Bason, Andrea Ruzzenente, Oliviero Olivieri, Elisa Tinazzi, Antonio Puccetti, Claudio Vitali, Nicoletta Del Papa, Simonetta Friso, Claudio Lunardi
Summary: This study found that endothelin-1 (ET-1) indirectly induces the production of reactive oxygen species (ROS) by stimulating aldosterone production in fibroblasts, which may play a pivotal role in the pathogenesis of systemic sclerosis (SSc) and pulmonary arterial hypertension (PAH).
Meeting Abstract
Hematology
Valentina Bellani, Daniele Cattaneo, Cristina Bucelli, Silvia Artuso, Umberto Gianelli, Francesco Onida, Alessandra Iurlo
Review
Pathology
Carlo Pescia, Gianluca Lopez, Umberto Gianelli, Giorgio Alberto Croci
Summary: Fibroblastic reticulum cell tumor (FRCT) is a rare dendritic neoplasm that usually occurs in lymph nodes but can also be found in the spleen and soft tissues. These tumors exhibit peculiar cytokeratin expression. The clinical presentation can range from very slow-growing to aggressive disease with features of malignancy. FRCT is challenging to diagnose due to its rarity and deceptive cytokeratin expression.
Article
Medicine, General & Internal
Michele Colaci, Jessika Dichiara, Maria Letizia Aprile, Massimo Ippolito, Claudia Schinocca, Giuliana Guggino, Lorenzo Malatino
Summary: Polymyalgia rheumatica (PMR) is an inflammatory disease that primarily affects older adults, causing pain and stiffness in the shoulder and pelvic girdles. 18F-FDG-PET/CT is a useful diagnostic technique for detecting potential giant cell arteritis (GCA), as well as persistent active PMR or other misdiagnosed inflammatory diseases.
FRONTIERS IN MEDICINE
(2022)
Review
Biology
Michele Colaci, Maria Letizia Aprile, Domenico Sambataro, Gianluca Sambataro, Lorenzo Malatino
Summary: The presence of liver involvement in systemic sclerosis (SSc) is considered atypical, besides the possible coexistence of other autoimmune hepatic disorders. However, the occurrence of portal hypertension and, more specifically, of the syndromes called idiopathic portal hypertension (IPH) and regenerative nodular hyperplasia (RNH) have been anecdotally reported in the literature for SSc patients. No specific SSc pattern linked to IPH emerged, even though reports from the literature often described the limited skin subset. Coexistence of prothrombotic states and overlap with other hepatic diseases could facilitate IPH onset. Besides being a rare condition, the onset of IPH in SSc patients is an occurrence that should be taken into account.
Article
Medicine, General & Internal
Matteo Regolo, Alessandra Sorce, Mauro Vaccaro, Michele Colaci, Benedetta Stancanelli, Giuseppe Natoli, Massimo Motta, Ivan Isaia, Federica Castelletti, Federica Giangreco, Daniela Fichera, Paola Aparo, Alessandra Lanzafame, Mario Russo, Nicola Santangelo, Paola Noto, Lorenzo Malatino
Summary: Severe cases of SARS-CoV-2 infections often progress to ARDS, leading to a bad outcome. Respiratory symptoms in COVID-19 patients do not necessarily correspond to disease progression. NLR and CRP levels show different trends in relation to mortality and ICU admission.
JOURNAL OF CLINICAL MEDICINE
(2023)
Review
Medicine, General & Internal
Gaetano La Rocca, Francesco Ferro, Chiara Baldini, Alessandro Libra, Domenico Sambataro, Michele Colaci, Lorenzo Malatino, Stefano Palmucci, Carlo Vancheri, Gianluca Sambataro
Summary: In recent years, the role of innate immunity, specifically interferon (IFN) and Interleukin 6, in idiopathic inflammatory myopathies (IIM) has gained increased attention. Both of these molecules transmit their signals through JAK/STAT pathway. This review focuses on evaluating the involvement of the JAK/STAT pathway in IIM, and discusses the potential therapeutic role of JAK inhibitors in diseases with strong IFN signature, such as dermatomyositis and antisynthetase syndrome.
FRONTIERS IN MEDICINE
(2023)
Review
Cell Biology
Leonie Saft, Hans M. Kvasnicka, Ludmila Boudova, Umberto Gianelli, Stefano Lazzi, Maria Rozman
Summary: Myeloid/lymphoid neoplasms with eosinophilia and tyrosine kinase gene fusions are rare hematopoietic tumors with diverse clinical and morphological presentations. Diagnosis of specific TK gene fusions may require additional evaluation with advanced molecular genetic techniques. The addition of new entities and genetic alterations involving TK genes in the latest classifications highlights the importance of studying M/LN-eo with TK gene fusions.
Article
Rheumatology
Rossella De Angelis, Valeria Riccieri, Edoardo Cipolletta, Nicoletta Del Papa, Francesca Ingegnoli, Silvia Bosello, Amelia Spinella, Greta Pellegrino, Marco de Pinto, Silvia Papa, Giuseppe Armentaro, Dilia Giuggioli
Summary: Nailfold videocapillaroscopy findings were compared in SSc patients with and without a diagnosis of pulmonary arterial hypertension (PAH). The results showed that patients with SSc-PAH had more severe abnormalities in nailfold videocapillaroscopy. These findings may be helpful in predicting the diagnosis of PAH.
Article
Rheumatology
Elisabeth Deibel, Patricia E. Carreira, Madelon Vonk, Nicoletta del Papa, Radim Becvar, Alfredo Guillen-Del-Castillo, Corrado Campochiaro, Hadi Poormoghim, Sophie Liem, Maria-Grazia Lazzaroni, Alessandro Giollo, Arsene Mekinian, Jeska de Vries-Bouwstra, Maria De Santis, Alexandra Balbir-Gurman, Carina Mihai, Giacomo De Luca, Sergey Moiseev, Elisabetta Zanatta, Rosario Foti, Simona Rednic, Christopher Denton, Maurizio Cutolo, Laura Belloli, Paolo Airo, Liudmila Garzanova, Gianluca Moroncini, Murat Inanc, Stylianos Panopoulos, Jose-Luis Tandaipan, Emmanuel Chatelus, Edoardo Rosato, Masataka Kuwana, Sule Yavuz, Juan J. Alegre-Sancho, Vanessa Smith, Gabriella Szucs, Joerg Henes, Ignasi Rodriguez-Pinto, Fabiola Atzeni, Julia Spierings, Marie-Elise Truchetet, Marcin Milchert, Daniel Brito de Araujo, Gabriela Riemekasten, Vera Bernardino, Thierry Martin, Francesco del Galdo, Alessandra Vacca, Fabian Mendoza, Oyvind Midtvedt, Giuseppe Murdaca, Tania Santiago, Veronica Codullo, Fabio Cacciapaglia, Ulrich Walker, Cathrine Brunborg, Francesca Tirelli, Yannick Allanore, Daniel E. Furst, Marco Matucci, Armando Gabrielli, Oliver Distler, Anna-Maria Hoffmann-Vold
Summary: The outcome of patients with both SSc and COVID-19 significantly improved over time due to intrinsic and extrinsic factors. Patients in waves 2 and 3 had a significantly lower rate of severe COVID-19, hospitalization, ventilatory support, and death compared to wave 1. These patients were younger, more likely to be male, and had lower rates of arterial hypertension and SSc cardiac involvement.
ARTHRITIS CARE & RESEARCH
(2023)