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Start Over Descriptor "Systemic Sclerosi" Publisher elsevier b.v.
5 results on '"Systemic Sclerosi"'

1. Imatinib-loaded gold nanoparticles inhibit proliferation of fibroblasts and macrophages from systemic sclerosis patients and ameliorate experimental bleomycin-induced lung fibrosis

Author

Codullo, V, Cova, E, Pandolfi, L, Breda, S, Morosini, M, Frangipane, V, Malatesta, M, Calderan, L, Cagnone, M, Pacini, C, Cavagna, L, Recalde, H, Distler, J, Giustra, M, Prosperi, D, Colombo, M, Meloni, F, Montecucco, C, Codullo V., Cova E., Pandolfi L., Breda S., Morosini M., Frangipane V., Malatesta M., Calderan L., Cagnone M., Pacini C., Cavagna L., Recalde H., Distler J. H. W., Giustra M., Prosperi D., Colombo M., Meloni F., Montecucco C., Codullo, V, Cova, E, Pandolfi, L, Breda, S, Morosini, M, Frangipane, V, Malatesta, M, Calderan, L, Cagnone, M, Pacini, C, Cavagna, L, Recalde, H, Distler, J, Giustra, M, Prosperi, D, Colombo, M, Meloni, F, Montecucco, C, Codullo V., Cova E., Pandolfi L., Breda S., Morosini M., Frangipane V., Malatesta M., Calderan L., Cagnone M., Pacini C., Cavagna L., Recalde H., Distler J. H. W., Giustra M., Prosperi D., Colombo M., Meloni F., and Montecucco C.

Abstract

Interstitial lung involvement in Systemic Sclerosis (SSc-ILD) is a complication with high morbidity and mortality. Specifically, engineered gold nanoparticles (GNPs) are proposed as targeted delivery system increasing efficacy of drugs with antifibrotic effect, such as tyrosine kinases. We aimed to test in vitro and in vivo the activity of targeted Imatinib (Im)-loaded GNP on SSc-ILD patients derived cells and in experimental model of lung fibrosis. GNPs functionalized with anti-CD44 and loaded with Im (GNP-HCIm) were synthesized. Lung fibroblasts (LFs) and alveolar macrophages from bronchoalveolar lavage fluids of SSc-ILD patients were cultured in presence of nanoparticles. GNP-HCIm significantly inhibited proliferation and viability inducing apoptosis of LFs and effectively reduced IL-8 release, viability and M2 polarization in alveolar macrophages. Anti-fibrotic effect of tracheal instilled GNP-HCIm was evaluated on bleomycin lung fibrosis mouse model comparing effect with common route of Im administration. GNP-HCIm were able to reduce significantly lung fibrotic changes and collagen deposition. Finally, electron microscopy revealed the presence of GNPs inside alveolar macrophages. These data support the use of GNPs locally administered in the development of new therapeutic approaches to SSc-ILD.

Published
2019

2. Cardiac troponin T and NT-proBNP as diagnostic and prognostic biomarkers of primary cardiac involvement and disease severity in systemic sclerosis: A prospective study

Author

Bosello, Silvia Laura, De Luca, Giacomo, Berardi, Giorgia, Canestrari, Giovanni, De Waure, Chiara, Gabrielli, Francesca Augusta, Di Mario, Clara, Forni, Franca, Gremese, Elisa, Ferraccioli, Gianfranco, Bosello, Silvia (ORCID:0000-0002-4837-447X), de Waure, Chiara (ORCID:0000-0002-4346-1494), Forni, Franca (ORCID:0000-0002-7208-2047), Gremese, Elisa (ORCID:0000-0002-2248-1058), Ferraccioli, Gianfranco (ORCID:0000-0001-6246-2428), Bosello, Silvia Laura, De Luca, Giacomo, Berardi, Giorgia, Canestrari, Giovanni, De Waure, Chiara, Gabrielli, Francesca Augusta, Di Mario, Clara, Forni, Franca, Gremese, Elisa, Ferraccioli, Gianfranco, Bosello, Silvia (ORCID:0000-0002-4837-447X), de Waure, Chiara (ORCID:0000-0002-4346-1494), Forni, Franca (ORCID:0000-0002-7208-2047), Gremese, Elisa (ORCID:0000-0002-2248-1058), and Ferraccioli, Gianfranco (ORCID:0000-0001-6246-2428)

Abstract

Objectives: The aim of our study was to define the role of high-sensitive cardiac troponin T (hs-cTnT) and NT-proBNP in identifying Systemic Sclerosis (SSc) patients with cardiac involvement and at higher risk of cardiac death. Methods: Plasma hs-cTnT and NT-proBNP concentrations were measured in 245 SSc-patients. Results: hs-cTnT and NT-proBNP levels were higher in SSc-patients than in healthy controls. Hs-cTnT levels were higher than 0.014 ng/ml in 32.3% SSc-patients, while NT-proBNP was above 125 pg/ml in 31.8% of them, irrespective of classical cardiovascular risk factor and of pulmonary arterial hypertension. Elevated hs-cTnT and NT-proBNP were associated with diffuse skin involvement and directly correlated with the skin score. Patients with increased cardiac markers presented a lower left-ventricular ejection fraction (LVEF) and a higher rate of right bundle branch block (RBBB) on electrocardiogram (ECG) compared to patients with normal cardiac enzymes. During the follow-up, 12 SSc-patients experience a disease-related death; 9 of these were directly related to cardiac involvement (sudden cardiac death or heart failure) and the majority of them occurred among patients with increase of at least one cardiac biomarker. Long-term survival was worse in patients with increase of both cardiac biomarkers. Conclusions: Evaluation of hs-cTnT and NT-proBNP levels may provide a tool to screen non-invasively SSc-patients for heart involvement. A higher incidence of impaired systolic function, ECG abnormalities and a poor outcome in SSc-patients with elevated cardiac enzymes suggests that they may be valuable screening biomarkers to detect a cardiac damage at early stages and to improve risk stratification.

Published
2019

3. Free light chains and autoimmunity

Author

Napodano, Cecilia, Pocino, Krizia, Rigante, Donato, Stefanile, Annunziata, Gulli, Francesca, Marino, Mariapaola, Basile, Valerio, Rapaccini, Gian Ludovico, Basile, Umberto, Napodano, Cecilia (ORCID:0000-0002-8720-6284), Pocino, Krizia (ORCID:0000-0003-2456-5308), Rigante, Donato (ORCID:0000-0001-7032-7779), Marino, Mariapaola (ORCID:0000-0001-9155-6378), Rapaccini, Gian Ludovico (ORCID:0000-0002-6467-857X), Napodano, Cecilia, Pocino, Krizia, Rigante, Donato, Stefanile, Annunziata, Gulli, Francesca, Marino, Mariapaola, Basile, Valerio, Rapaccini, Gian Ludovico, Basile, Umberto, Napodano, Cecilia (ORCID:0000-0002-8720-6284), Pocino, Krizia (ORCID:0000-0003-2456-5308), Rigante, Donato (ORCID:0000-0001-7032-7779), Marino, Mariapaola (ORCID:0000-0001-9155-6378), and Rapaccini, Gian Ludovico (ORCID:0000-0002-6467-857X)

Abstract

The study of free light chains (FLCs) has grown as area of enormous interest for many clinicians with the aim of disclosing the exact biological role and potential use of FLCs in the clinical routine. Moreover, the attention given to immunological functions of FLCs has sparked a new light into their pathogenic contribution in different chronic autoimmune-based inflammatory diseases. The release of intracellular antigens following cell death or ineffective clearance of apoptotic debris, modification of self-antigens, and molecular mimicry may trigger the production of immunoglobulins after activation and polyclonal expansion of B cells, by which FLCs are released. The discovery of polyclonal FLCs as potential biomarkers started with the observation of their increased concentrations in a variety of biological fluids related to patients with autoimmune diseases. This review deals with the use of polyclonal FLCs for identifying severity and monitoring outcome after treatment in some autoimmune diseases, namely systemic lupus erythematosus, myasthenia gravis, systemic sclerosis, rheumatoid arthritis and Sjögren's syndrome, as supported by the fact that levels of FLCs correlate with both B cell activation markers and other specific markers of disease activity. In a near future, following the evidence shown, FLCs might probably work as early prognostic markers of severity and also as indicators of response to treatment or early assessment of relapse in selected autoimmune diseases.

Published
2019

4. The Renal Resistive Index in systemic sclerosis: Determinants, prognostic implication and proposal for specific age-adjusted cut-offs

Author

Cosimo Bruni, Edoardo Rosato, Maria Boddi, Jelena Blagojevic, Marco Chiostri, Marco Matucci-Cerinic, Khadija El Aoufy, Silvia Bellando-Randone, Giulia Tesei, Antonietta Gigante, Vanessa Maestripieri, Daniel E. Furst, Serena Guiducci, Amato de Paulis, Alberto Moggi-Pignone, Bruni, C., Rosato, E., Maestripieri, V., Gigante, A., Tesei, G., Bellando-Randone, S., Guiducci, S., Chiostri, M., El Aoufy, K., Blagojevic, J., Moggi-Pignone, A., De Paulis, A., Furst, D. E., Boddi, M., and Matucci-Cerinic, M.

Subjects
Male, Percentile, Fibrosi, Blood Pressure, Vasculopathy, Disease, 030204 cardiovascular system & hematology, Renal artery Doppler ultrasound, Kidney, Kidney Function Tests, Severity of Illness Index, Systemic sclerosi, Renal Artery, 0302 clinical medicine, Fibrosis, 030212 general & internal medicine, education.field_of_study, integumentary system, Interstitial lung disease, Middle Aged, Prognosis, Renal Resistive Index, Systemic sclerosis, medicine.anatomical_structure, Italy, Quartile, Cardiology, Female, Adult, medicine.medical_specialty, Prognosi, Population, Age adjustment, 03 medical and health sciences, Internal medicine, Internal Medicine, medicine, Humans, education, Aged, Retrospective Studies, Scleroderma, Systemic, Lung, business.industry, Ultrasonography, Doppler, medicine.disease, Survival Analysis, Vascular Resistance, business, human activities
Abstract

Background Renal Resistive Index (RRI), reflects changes in both renal vascular and tubular-interstitial compartments and in systemic vascular compliance related to age and comorbidities. Objectives a) To investigate determinants of RRI in SSc population, b) its association with SSc-related features and c) to test its prognostic impact on organ specific worsening or death. Methods 380 SSc patients ≥18 years were enrolled after giving informed consent. Baseline data on RRI, laboratory, instrumental and therapeutic features were retrospectively collected. Age-SSc adjusted cut-offs were created by dividing the population in age quartiles and considering RRI values >75th percentile as pathologic. Clinical follow-up was performed until last available visit or the development/worsening of specific internal organ involvement or death. Results RRI was independently predicted by age and systolic pulmonary arterial pressure on Echo. Therefore, we created Age-SSc adjusted pathologic RRI cut-offs, which were significantly associated with various disease related skin and lung fibrotic manifestations, as well as vasculopathic complications. After a mean follow-up of 3.6 ± 2.6 years, RRI was one of the independent predictors (together with modified Rodnan skin score, interstitial lung disease, presence of dyspnoea and late nailfold-videocapillaroscopy pattern) for mortality, with 0.68 as best cut-off (sensitivity 88.5%, specificity 50.9%). Conclusion If corroborated, Renal Resistive Index cut-offs might be used to evaluate renal and extrarenal involvement in SSc and could serve as predictors of mortality.

Published
2019

5. International consensus: What else can we do to improve diagnosis and therapeutic strategies in patients affected by autoimmune rheumatic diseases (rheumatoid arthritis, spondyloarthritides, systemic sclerosis, systemic lupus erythematosus, antiphospholipid syndrome and Sjogren's syndrome)?: The unmet needs and the clinical grey zone in autoimmune disease management

Author

Pier Luigi Meroni, Serena Vettori, Vasiliki Liakouli, Paola Cipriani, Piero Ruscitti, Laura Massaro, Elisa Tinazzi, Chiara Baldini, Domenico Paolo Emanuele Margiotta, Yehuda Shoenfeld, Mariele Gatto, Saviana Gandolfo, Alberto Cauli, Gabriele Valentini, Francesco Puppo, Onorina Berardicurti, Alexandros A. Drosos, E Bartoloni-Bocci, Alessia Alunno, Francesco Ursini, George Somarakis, Ennio Lubrano, Federico Perosa, Luca Navarini, Jacques-Olivier Pers, Piercarlo Sarzi-Puttini, Francesco Carubbi, Andrea Doria, Luca Quartuccio, Paola Di Benedetto, Amelia Ruffatti, Antonia Minniti, Marcella Prete, Salvatore De Vita, Fabrizio Conti, Ricard Cervera, Athanasios G. Tzioufas, Claudio Lunardi, M. Pendolino, Rik Lories, Francesca Romana Spinelli, Barbara Russo, Rosa Daniela Grembiale, Antonella Afeltra, Francesco Ciccia, Claudio Vitali, Giovanni Triolo, Roberto Giacomelli, Ennio Giulio Favalli, Guido Valesini, Roberta Priori, Giacomelli, R., Afeltra, A., Alunno, A., Baldini, C., Bartoloni-bocci, E., Berardicurti, O., Carubbi, F., Cauli, A., Cervera, R., Ciccia, F., Cipriani, P., Conti, F., De Vita, S., Di Benedetto, P., Doria, A., Drosos, A., Favalli, E., Gandolfo, S., Gatto, M., Grembiale, R., Liakouli, V., Lories, R., Lubrano, E., Lunardi, C., Margiotta, D., Massaro, L., Meroni, P., Minniti, A., Navarini, L., Pendolino, M., Perosa, F., Pers, J., Prete, M., Priori, R., Puppo, F., Quartuccio, L., Ruffatti, A., Ruscitti, P., Russo, B., Sarzi-puttini, P., Shoenfeld, Y., Somarakis, G., Spinelli, F., Tinazzi, E., Triolo, G., Ursini, F., Valentini, G., Valesini, G., Vettori, S., Vitali, C., Tzioufas, A., Division of Rheumatology, Department of Internal Medicine, Università degli studi di Palermo - University of Palermo, Rheumatology Unit (Rheum Unit - PISA), University of Pisa - Università di Pisa, 2nd Chair of Rheumatolog, Department of Medical Sciences, University of Cagliari, Autoinflammatory Diseases Clinical Unit (Department of Autoimmune Diseases Working Group of Adult Rare Diseases), Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Udine and University Hospital of Udine, Department of Clinical and Biological Sciences, Oral Medicine and Oral Oncology Section, University of Turin, Rheumatology Unit, Universita degli Studi di Padova, Laboratory of Tissue Homeostasis and Disease, Laborory of Tissue homeostasis and Disease, Department of Clinical and Experimental Medicine, Università degli Studi di Verona, Allergy, Clinical Immunology and Rheumatology, University of Milan, Lymphocyte B et Auto-immunité (LBAI), Université de Brest (UBO)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut Brestois Santé Agro Matière (IBSAM), Université de Brest (UBO), University of Udine and University Hospital- Udine, Università degli Studi di Udine - University of Udine [Italie], Free University of Bozen-Bolzano, Sackler Faculty of Medicine, Tel Aviv University [Tel Aviv], Cattedra e Unità Operativa di Reumatologia, Policlinico Universitario, Institut de Planétologie et d'Astrophysique de Grenoble (IPAG), Centre National d'Études Spatiales [Toulouse] (CNES)-Université Grenoble Alpes (UGA)-Centre National de la Recherche Scientifique (CNRS)-Institut national des sciences de l'Univers (INSU - CNRS)-Observatoire des Sciences de l'Univers de Grenoble (OSUG), Université Savoie Mont Blanc (USMB [Université de Savoie] [Université de Chambéry])-Institut polytechnique de Grenoble - Grenoble Institute of Technology (Grenoble INP)-Institut national de recherche en sciences et technologies pour l'environnement et l'agriculture (IRSTEA)-Université Joseph Fourier - Grenoble 1 (UJF)-Institut national des sciences de l'Univers (INSU - CNRS)-Centre National de la Recherche Scientifique (CNRS)-Université Grenoble Alpes (UGA)-Université Savoie Mont Blanc (USMB [Université de Savoie] [Université de Chambéry])-Institut polytechnique de Grenoble - Grenoble Institute of Technology (Grenoble INP)-Institut national de recherche en sciences et technologies pour l'environnement et l'agriculture (IRSTEA)-Université Joseph Fourier - Grenoble 1 (UJF)-Centre National de la Recherche Scientifique (CNRS), CNR Istituto di Fotonica e Nanotecnologie [Milano] (IFN), Consiglio Nazionale delle Ricerche [Roma] (CNR), Dipartimento di Fisica, Politecnico di Milano [Milan] (POLIMI), Department of Internal Medicine, Lupus Clinic, Sapienza University [Rome], Sections of Rheumatology (Rheumatol - LECCO), Instituto San Giuseppe, Department of Pathophysiology, Medical School, University of Athens, Giacomelli, Roberto, Afeltra, Antonella, Alunno, Alessia, Baldini, Chiara, Bartoloni Bocci, Elena, Berardicurti, Onorina, Carubbi, Francesco, Cauli, Alberto, Cervera, Ricard, Ciccia, Francesco, Cipriani, Paola, Conti, Fabrizio, De Vita, Salvatore, Di Benedetto, Paola, Doria, Andrea, Drosos, Alexandros A., Favalli, Ennio Giulio, Gandolfo, Saviana, Gatto, Mariele, Grembiale, Rosa Daniela, Liakouli, Vasiliki, Lories, Rik, Lubrano, Ennio, Lunardi, Claudio, Margiotta, Domenico Paolo Emanuele, Massaro, Laura, Meroni, Pierluigi, Minniti, Antonia, Navarini, Luca, Pendolino, Monica, Perosa, Federico, Pers, Jacques Olivier, Prete, Marcella, Priori, Roberta, Puppo, Francesco, Quartuccio, Luca, Ruffatti, Amelia, Ruscitti, Piero, Russo, Barbara, Sarzi Puttini, Piercarlo, Shoenfeld, Yehuda, Somarakis, George A., Spinelli, Francesca Romana, Tinazzi, Elisa, Triolo, Giovanni, Ursini, Francesco, Valentini, Gabriele, Valesini, Guido, Vettori, Serena, Vitali, Claudio, Tzioufas, Athanasios G., University of Verona (UNIVR), Institut national des sciences de l'Univers (INSU - CNRS)-Centre National d'Études Spatiales [Toulouse] (CNES)-Centre National de la Recherche Scientifique (CNRS)-Observatoire des Sciences de l'Univers de Grenoble (OSUG ), Institut polytechnique de Grenoble - Grenoble Institute of Technology (Grenoble INP )-Institut national des sciences de l'Univers (INSU - CNRS)-Institut national de recherche en sciences et technologies pour l'environnement et l'agriculture (IRSTEA)-Université Savoie Mont Blanc (USMB [Université de Savoie] [Université de Chambéry])-Centre National de la Recherche Scientifique (CNRS)-Université Grenoble Alpes [2016-2019] (UGA [2016-2019])-Institut polytechnique de Grenoble - Grenoble Institute of Technology (Grenoble INP )-Institut national de recherche en sciences et technologies pour l'environnement et l'agriculture (IRSTEA)-Université Savoie Mont Blanc (USMB [Université de Savoie] [Université de Chambéry])-Centre National de la Recherche Scientifique (CNRS)-Université Grenoble Alpes [2016-2019] (UGA [2016-2019]), Università degli Studi di Roma 'La Sapienza' = Sapienza University [Rome], Université de Brest (UBO)-Institut Brestois Santé Agro Matière (IBSAM), Université de Brest (UBO)-Institut National de la Santé et de la Recherche Médicale (INSERM), Giacomelli R., Afeltra A., Alunno A., Baldini C., Bartoloni-Bocci E., Berardicurti O., Carubbi F., Cauli A., Cervera R., Ciccia F., Cipriani P., Conti F., De Vita S., Di Benedetto P., Doria A., Drosos A.A., Favalli E.G., Gandolfo S., Gatto M., Grembiale R.D., Liakouli V., Lories R., Lubrano E., Lunardi C., Margiotta D.P.E., Massaro L., Meroni P., Minniti A., Navarini L., Pendolino M., Perosa F., Pers J.-O., Prete M., Priori R., Puppo F., Quartuccio L., Ruffatti A., Ruscitti P., Russo B., Sarzi-Puttini P., Shoenfeld Y., Somarakis G.A., Spinelli F.R., Tinazzi E., Triolo G., Ursini F., Valentini G., Valesini G., Vettori S., Vitali C., and Tzioufas A.G.

Subjects
0301 basic medicine, Quality management, Effectiveness, law.invention, Systemic sclerosi, 0302 clinical medicine, Antiphospholipid syndrome, Biologic drugs treatment, Remission, Rheumatoid arthritis, Sjogren's syndrome, Spondyloarthritides, Systemic lupus erythematosus, Systemic sclerosis, Unmet needs, Immunology and Allergy, Immunology, law, Disease management (health), ComputingMilieux_MISCELLANEOUS, Spondyloarthritide, Clinical Trials as Topic, Effectivene, Disease Management, Quality Improvement, 3. Good health, [SDV.IMM]Life Sciences [q-bio]/Immunology, Unmet need, medicine.medical_specialty, Systemic lupus erythematosu, rheumatoid arthritis, spondyloarthritides, NO, Autoimmune Diseases, 03 medical and health sciences, Internal medicine, Rheumatic Diseases, medicine, Humans, Intensive care medicine, Rheumatoid arthriti, 030203 arthritis & rheumatology, Autoimmune disease, therapy, business.industry, medicine.disease, Rheumatology, 030104 developmental biology, autoimmune rheumatic diseases, Physical therapy, CLARITY, business, Working group
Abstract

Autoimmune diseases are a complex set of diseases characterized by immune system activation and, although many progresses have been done in the last 15 years, several unmet needs in the management of these patients may be still identified. Recently, a panel of international Experts, divided in different working groups according to their clinical and scientific expertise, were asked to identify, debate and formulate a list of key unmet needs within the field of rheumatology, serving as a roadmap for research as well as support for clinicians. After a systematic review of the literature, the results and the discussions from each working group were summarised in different statements. Due to the differences among the diseases and their heterogeneity, a large number of statements was produced and voted by the Experts to reach a consensus in a plenary session. At all the steps of this process, including the initial discussions by the steering committee, the identification of the unmet needs, the expansion of the working group and finally the development of statements, a large agreement was attained. This work confirmed that several unmet needs may be identified and despite the development of new therapeutic strategies as well as a better understanding of the effects of existing therapies, many open questions still remain in this field, suggesting a research agenda for the future and specific clinical suggestions which may allow physicians to better manage those clinical conditions still lacking of scientific clarity.

Published
2017

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