367 results on '"del Papa, Nicoletta"'
Search Results
102. Autologous Fat Grafting in the Treatment of Fibrotic Perioral Changes in Patients with Systemic Sclerosis
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Del Papa, Nicoletta, primary, Caviggioli, Fabio, additional, Sambataro, Domenico, additional, Zaccara, Eleonora, additional, Vinci, Valeriano, additional, Di Luca, Gabriele, additional, Parafioriti, Antonina, additional, Armiraglio, Elisabetta, additional, Maglione, Wanda, additional, Polosa, Riccardo, additional, Klinger, Francesco, additional, and Klinger, Marco, additional
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- 2015
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103. Low-dose oral imatinib in the treatment of systemic sclerosis interstitial lung disease unresponsive to cyclophosphamide: a phase II pilot study
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Fraticelli, Paolo, Gabrielli, Barbara, Pomponio, Giovanni, Valentini, Gabriele, Bosello, Silvia Laura, Riboldi, Piersandro, Gerosa, Maria, Faggioli, Paola, Giacomelli, Roberto, Del Papa, Nicoletta, Gerli, Roberto, Lunardi, Claudio, Bombardieri, Stefano, Malorni, Walter, Corvetta, Angelo, Moroncini, Gianluca, Gabrielli, Armando, Bosello, Silvia (ORCID:0000-0002-4837-447X), Fraticelli, Paolo, Gabrielli, Barbara, Pomponio, Giovanni, Valentini, Gabriele, Bosello, Silvia Laura, Riboldi, Piersandro, Gerosa, Maria, Faggioli, Paola, Giacomelli, Roberto, Del Papa, Nicoletta, Gerli, Roberto, Lunardi, Claudio, Bombardieri, Stefano, Malorni, Walter, Corvetta, Angelo, Moroncini, Gianluca, Gabrielli, Armando, and Bosello, Silvia (ORCID:0000-0002-4837-447X)
- Abstract
Introduction: Pulmonary involvement represents a major cause of death of systemic sclerosis (SSc) patients. Recent data suggest that tyrosine kinase inhibitors, such as imatinib, may be a therapeutic option for SSc patients. However, preliminary published clinical trials were inconclusive about imatinib efficacy and showed side effects. The purpose of this study was to verify efficacy and tolerability of low-dose imatinib on interstitial lung disease in a cohort of SSc patients unresponsive to cyclophosphamide therapy.Methods: Thirty consecutive SSc patients with active pulmonary involvement, unresponsive to cyclophosphamide, were treated with imatinib 200 mg/day for 6 months followed by a 6-month follow-up. A "good response" was defined as an increase of forced vital capacity (FVC) by more of 15% and/or increase of diffusing capacity of carbon monoxide (DLCO) > 15% and PaO2 > 90% of initial value and high-resolution computed tomography (HRCT)-scan pattern unchanged or improved.Results: Twenty-six patients completed the study. Three patients died and one patient was lost to follow-up. Four patients (15.32%) had a good response, 7 worsened and 15 had a stabilized lung disease. Overall, 19 (73.07%) patients had an improved or stabilized lung disease. After a 6-month follow-up, 12 (54.5%) of the 22 patients showed an improved or stabilized lung disease.Conclusions: Lung function was stabilized in a large proportion of patients unresponsive to cyclophosphamide therapy and a beneficial outcome emerged from the analysis of HRCT lung scans. There was no significant improvement of skin involvement, and the low dose was well tolerated. These data provide useful suggestions to design future randomized clinical trials for SSc therapeutics.
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- 2014
104. Does Ex Vivo CD34+ Cell Selection Change the Outcome of Systemic Sclerosis Patients Treated with Autologous Hematopoietic Stem Cell Transplantation (AHSCT), an Adwp EBMT Study?
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Oliveira, Maria Carolina, primary, Labopin, Myriam, additional, Henes, Jörg, additional, Moore, John, additional, Del Papa, Nicoletta, additional, Stanciu, Ricca, additional, Sakellari, Ioanna, additional, Schroers, Roland, additional, Scherer, Hans Ulrich, additional, Kyrcz-Krzemien, Slawomira, additional, Daikeler, Thomas, additional, Alexander, Tobias, additional, Finke, Jürgen, additional, Badoglio, Manuela, additional, Snowden, John A, additional, and Farge, Dominique, additional
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- 2014
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105. Sjogren's Syndrome Disease Damage Index (SjSDDI) and Disease Activity Measurement (SjSDAM): two scoring systems for the assessment of DD and DA in SjS, derived by the analysis of a cohort of Italian patients
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Valesini, Guido, Priori, Roberta, Orefice, Maddalena, CARLOMAURIZIO MONTECUCCO, Migliaresi, Sergio, Maglione, Wanda, Maddali-Bongi, Susanna, Govoni, Marcello, Gerli, Roberto, Franceschini, Franco, Epis, Oscar, Vita, Salvatore, Del Papa, Nicoletta, Covelli, Michele, Bombardieri, Stefano, Benucci, Maurizio, Baldini, Chiara, Palombi, Gianluigi, and Vitali, Claudio
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- 2006
106. Antiendothelial Cell Antibodies in Primary Vasculitides
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Meroni, Pier Luigi, primary, Del Papa, Nicoletta, additional, Sinico, Alberto, additional, Tincani, Angela, additional, Barcellini, Wilma, additional, Borghi, Maria Orietta, additional, Radice, Antonella, additional, Federici, Augusto B., additional, and Balestrieri, Genesio, additional
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107. Nailfold videocapillaroscopy micro-haemorrhage and giant capillary counting as an accurate approach for a steady state definition of disease activity in systemic sclerosis
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Sambataro, Domenico, primary, Sambataro, Gianluca, additional, Zaccara, Eleonora, additional, Maglione, Wanda, additional, Polosa, Riccardo, additional, Afeltra, Antonella MV, additional, Vitali, Claudio, additional, and Del Papa, Nicoletta, additional
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- 2014
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108. Autologous Hematopoietic Stem Cell Transplantation Versus Intravenous Pulse Therapy Cyclophosphamide for Severe or Rapidly Progressive Systemic Sclerosis, the Astis Trial
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Dominique, Farge, primary, Van Laar, Jaap, additional, Sont, Jacob K, additional, Naraghi, Kamran, additional, Marjanovic, Zora, additional, Largero, Jerome, additional, Schuerwegh, Annemie, additional, Marijt, Erik W.A., additional, Vonk, Madelon, additional, Schattenberg, Anton, additional, Matucci, Marco, additional, Miniati, Irene, additional, Voskuyl, Alexandre, additional, Van de Loosdrecht, Arjan A., additional, Daikeler, Thomas, additional, Koetter, Ina, additional, Schmarlzing, Marc, additional, Martin, Thierry, additional, Weiner, Stefan, additional, Deligny, Christophe, additional, Durand, jean Marc, additional, Emery, Paul, additional, Machold, klaus P, additional, Sarrot-Reynauld, Françoise, additional, Warnatz, Klaus, additional, Adoue, Daniel, additional, Constans, Joel, additional, Tony, Hans-Peter, additional, Del Papa, Nicoletta, additional, Fassas, Athanasios, additional, Himsel, Andrea, additional, Launay, David, additional, Monaco, Andrea Lo, additional, Philippe, Pierre, additional, Quere, Isabelle, additional, Rich, Eric, additional, Westhovens, Rene, additional, Griffiths, Bridget, additional, Saccardi, Riccardo, additional, Van Den Hoogen, Frank H, additional, Fibbe, Willem E., additional, Socie, Gerard, additional, Gratwohl, Alois, additional, and Tyndall, Alan, additional
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- 2012
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109. Antiendothelial Cell Antibodies Induce Apoptosis of Bone Marrow Endothelial Progenitors in Systemic Sclerosis
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DEL PAPA, NICOLETTA, primary, QUIRICI, NADIA, additional, SCAVULLO, CINZIA, additional, GIANELLI, UMBERTO, additional, CORTI, LAURA, additional, VITALI, CLAUDIO, additional, FERRI, CLODOVEO, additional, GIUGGIOLI, DILIA, additional, MANFREDI, ANDREINA, additional, MAGLIONE, WANDA, additional, ONIDA, FRANCESCO, additional, COLACI, MICHELE, additional, BOSARI, SILVANO, additional, and LAMBERTENGHI DELILIERS, GIORGIO, additional
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- 2010
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110. Management of antiphospholipid syndrome
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Del Papa, Nicoletta, primary and Vaso, Nikoleta, additional
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- 2010
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111. Corneal Involvement in Rheumatoid Arthritis: An In Vivo Confocal Study
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Villani, Edoardo, primary, Galimberti, Daniela, additional, Viola, Francesco, additional, Mapelli, Chiara, additional, Del Papa, Nicoletta, additional, and Ratiglia, Roberto, additional
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- 2008
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112. Pulmonary Arterial Hypertension in Idiopathic Myelofibrosis Is Associated with an Alterated Angiogenic Status.
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Cortelezzi, Agostino, primary, Del Papa, Nicoletta, primary, Calori, Rossella, primary, Pasquini, Maria Cristina, primary, Cortiana, Michela, primary, Silvestris, Ilaria, primary, Gritti, Giuseppe, primary, Sozzi, Fabiola B., primary, Bianchi, Paola, primary, and Deliliers, Giorgio Lambertenghi, primary
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- 2006
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113. Stem Cells Defects in Systemic Sclerosis.
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Quirici, Nadia, primary, Del Papa, Nicoletta, primary, Scavullo, Cinzia, primary, Cortiana, Michela, primary, Borsotti, Chiara, primary, Maglione, Wanda, primary, Comina, Denise, primary, Cortelezzi, Agostino, primary, Deliliers, Giorgio Lambertenghi, primary, and Soligo, Davide, primary
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- 2005
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114. Role of antimalarials in COVID-19: observational data from a cohort of rheumatic patients.
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Favalli, Ennio Giulio, De Lucia, Orazio, Biggioggero, Martina, Del Papa, Nicoletta, and Caporali, Roberto
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- 2021
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115. Reply
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Tedeschi, Alberto, primary, Meroni, Pier Luigi, additional, Del Papa, Nicoletta, additional, Boschetti, Carla, additional, and Miadonna, Antonio, additional
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- 1999
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116. Introduction
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Meroni, Pier Luigi, primary and Del Papa, Nicoletta, additional
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- 1997
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117. Anti-endothelial cell IgG antibodies from patients with Wegener's granulomatosis bind to human endothelial cells in vitro and induce adhesion molecule expression and cytokine secretion
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del Papa, Nicoletta, primary, Guidali, Luisa, additional, Sironi, Marina, additional, Shoenfeld, Yehuda, additional, Mantovani, Alberto, additional, Tincani, Angela, additional, Balestrieri, Genesio, additional, Radice, Antonella, additional, Sinico, R. Alberto, additional, and Meroni, Pier Luigi, additional
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- 1996
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118. Anti-beta 2-glycoprotein I antibodies: a marker of antiphospholipid syndrome?
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Balestrieri, Genesio, primary, Tincani, Angela, additional, Spatola, Laura, additional, Allegri, Flavio, additional, Prati, Enrica, additional, Cattaneo, Roberto, additional, Valesini, Guido, additional, Del Papa, Nicoletta, additional, and Meroni, Pierluigi, additional
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- 1995
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119. Antiphospholipid Antibodies and Endothelial Cells
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Meroni, Pier Luigi, primary, Del Papa, Nicoletta, additional, Gambini, Donatella, additional, Tincani, Angela, additional, and Balestrieri, Genesio, additional
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- 1994
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120. Simvastatin Reduces Endothelial Activation and Damage But Is Partially Ineffective in Inducing Endothelial Repair in Systemic Sclerosis
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Del Papa, Nicoletta, Cortiana, Michela, Vitali, Claudio, Silvestris, Ilaria, Maglione, Wanda, Comina, Denise, Lucchi, Tiziano, and Cortelezzi, Agostino
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OBJECTIVE: To investigate whether statins may improve endothelial function in systemic sclerosis (SSc) by evaluating the effects of simvastatin on vasculogenesis [indicated by the expansion of circulating endothelial progenitor cells (EPC)] and the markers of vascular injury in the peripheral blood of patients with SSc. METHODS: Twenty SSc patients with normal cholesterol concentrations and 20 hypercholesterolemic subjects were allocated to receive 20 mg/day simvastatin for 12 weeks. Peripheral blood samples were collected before and 12 weeks after initiation of treatment, and 4 weeks after discontinuation. Five-parameter, 3-color flow cytometry was performed with a FacScan to enumerate EPC and mature circulating endothelial cells (CEC). Levels of soluble E-selectin, intercellular adhesion molecule-1, vascular cell adhesion molecule-1, interleukin 6, and endothelin-1 were assessed by commercial ELISA. RESULTS: Simvastatin treatment significantly increased EPC in the hypercholesterolemic group, but failed to improve the EPC levels in the SSc patients, mainly in patients with late disease. Baseline levels of CEC were significantly higher in SSc patients compared with controls and at the end of the treatment they were significantly decreased. Regarding other markers of endothelial activation, we found that all the cytokine levels decreased in a statistically significant manner in the treated patients. CONCLUSION: Treatment with simvastatin results in rapid and significant improvement of measures of endothelial activation, suggesting a potential role of statins in the treatment of peripheral vascular disease in SSc. The lack of effect on increase of EPC confirms our previous findings of a defective endothelial stem cell recruitment in the bone marrow of SSc patients. This could indicate that the potential effectiveness of statins in SSc could mainly be ascribed to their effectiveness in modulating endothelial activation mechanisms.
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- 2008
121. NEMO score in nailfold videocapillaroscopy is a good tool to assess both steady state levels and overtime changes of disease activity in patients with systemic sclerosis: a comparison with the proposed composite indices for this disease status entity.
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Pignataro, Francesca, Maglione, Wanda, Minniti, Antonina, Sambataro, Domenico, Sambataro, Gianluca, Campanaro, Francesco, Valentini, Gabriele, Vitali, Claudio, and Del Papa, Nicoletta
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- 2019
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122. Comparison of performance of the 2016 ACR-EULAR classification criteria for primary Sjögren's syndrome with other sets of criteria in Japanese patients.
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Vitali, Claudio, Del Papa, Nicoletta, Tsuboi, Hiroto, Hagiwara, Shinya, Asashima, Hiromitsu, Takahashi, Hiroyuki, Hirota, Tomoya, Noma, Hisashi, Umehara, Hisanori, Kawakami, Atsushi, Nakamura, Hideki, Sano, Hajime, Tsubota, Kazuo, Ogawa, Yoko, Takamura, Etsuko, Saito, Ichiro, Inoue, Hiroko, Nakamura, Seiji, Moriyama, Masafumi, and Takeuchi, Tsutomu
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SJOGREN'S syndrome diagnosis ,DIFFERENTIAL diagnosis ,MEDICAL protocols ,RHEUMATOLOGY ,SJOGREN'S syndrome - Abstract
Objectives: To compare the performance of the new 2016 American College of Rheumatology (ACR)-European League Against Rheumatism (EULAR) classification criteria for primary Sjögren's syndrome (SS) with 1999 revised Japanese Ministry of Health criteria for diagnosis of SS (JPN), 2002 American-European Consensus Group classification criteria for SS (AECG) and 2012 ACR classification criteria for SS (ACR) in Japanese patients.Methods: The study subjects were 499 patients with primary SS (pSS) or suspected pSS who were followed up in June 2012 at 10 hospitals in Japan. All patients had been assessed for all four criteria of JPN (pathology, oral, ocular, anti-SS-A/SS-B antibodies). The clinical diagnosis by the physician in charge was set as the 'gold standard'.Results: pSS was diagnosed in 302 patients and ruled out in 197 patients by the physician in charge. The sensitivity of the ACR-EULAR criteria in the diagnosis of pSS (95.4%) was higher than those of the JPN, AECG and ACR (82.1%, 89.4% and 79.1%, respectively), while the specificity of the ACR-EULAR (72.1%) was lower than those of the three sets (90.9%, 84.3% and 84.8%, respectively). The differences of sensitivities and specificities between the ACR-EULAR and other three sets of criteria were statistically significant (p<0.001). Eight out of 302 patients with pSS and 11 cases out of 197 non-pSS cases satisfied only the ACR-EULAR criteria, compared with none of the other three sets.Conclusions: The ACR-EULAR criteria had significantly higher sensitivity and lower specificity in diagnosis of pSS, compared with the currently available three sets of criteria. [ABSTRACT FROM AUTHOR]- Published
- 2017
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123. Toupet fundoplication to prevent progression of scleroderma-associated interstitial lung damage: a study protocol.
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Aiolfi, Alberto, Bona, Davide, Manara, Michele, Ogliari, Cristina, Baldessari, Chiara, Resta, Marco, Del Papa, Nicoletta, Cirri, Silvia, Baisi, Alessandro, Bonitta, Gianluca, Sconfienza, Luca, Harari, Sergio, Nosotti, Mario, Vecchi, Maurizio, Schoppmann, Sebastian F., and Bonavina, Luigi
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- 2024
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124. Autologous stem cell transplantation in scleroderma
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Farge, Dominique, Ait Abdallah, Nassim, Marjanovic, Zora, and Del Papa, Nicoletta
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Patients with severe rapidly progressive systemic sclerosis (SSc) have a poor prognosis.
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- 2021
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125. High NEMO score values in nailfold videocapillaroscopy are associated with the subsequent development of ischaemic digital ulcers in patients with systemic sclerosis
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Del Papa, Nicoletta, Pignataro, Francesca, Maglione, Wanda, Minniti, Antonina, Sambataro, Domenico, Sambataro, Gianluca, Valentini, Gabriele, Caporali, Roberto, and Vitali, Claudio
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Background: Nailfold videocapillaroscopy (NVC) is a feasible method that allows the observation of the microvascular changes that mark the course of systemic sclerosis (SSc). In previous studies, we demonstrated that the NEMO score, i.e. the cumulative number of microhaemorrhages and microthromboses, is a good indicator of the steady-state level and overtime changes of disease activity (DA) in SSc. Objectives: To verify whether high NEMO scores, which mirror a very active microvascular derangement in the fingers, may be associated with the subsequent development of ischaemic digital ulcers (IDUs). Methods: The NEMO score was assessed at baseline (T0) in 98 patients with SSc, all classified according to the ACR-EULAR criteria. Of them, 90 were females, 48 had the limited and 50 had the diffuse cutaneous variant of SSc. Afterwards, the patients were closely followed up for 2 years, and the appearance of new IDUs recorded at any time of the follow-up. The T0-NEMO score values of patients who developed IDUs were compared to those of patients who did not. A receiver operating curve (ROC) was constructed, and the area under the curve (AUC) calculated by plotting the sensitivity and 1-specificity of the different NEMO score values in predicting the subsequent development of IDUs. Results: During the follow-up, 38 out of 98 patients developed one or more IDUs. The NEMO score at T0 was significantly higher in those who developed IDUs with respect to those who did not [median 14.5 (95% CI 11.0–21.5) and 4.5 (95% CI 4.0–6.0), respectively, p< 0.0001]. The ROC curve derived from different T0-NEMO score values had an AUC of 0.79 (95% CI 0.69–0.86, p< 0.0001). A NEMO score of ≥ 12 had a sensitivity of 83.3% (95% CI 71.5–91.7) and a specificity of 63.2% (95% CI 46.0–78.2), with positive (P) and negative (N) predictive (PV) values of 58.9% (95% CI 44.7–72.2) and 85.6% (71.8–94.4), respectively. A NEMO score of ≥ 16 had a sensitivity of 95.0% (95% CI 86.1–99.0) and a NPV of 93.4% (77.5–99.2). Conclusions: Being a valid tool to measure DA levels in SSc, the NEMO score also appears to be closely related to the subsequent development of IDUs in this disease.
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- 2020
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126. Long Non-Coding RNAs Modulate Sjögren's Syndrome Associated Gene Expression and Are Involved in the Pathogenesis of the Disease.
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Dolcino, Marzia, Tinazzi, Elisa, Vitali, Claudio, Del Papa, Nicoletta, Puccetti, Antonio, and Lunardi, Claudio
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NON-coding RNA ,GENE expression ,TYPE I interferons ,EXOCRINE glands ,MOLECULAR interactions - Abstract
Primary Sjögren's syndrome (pSjS) is a chronic systemic autoimmune disorder, primarily affecting exocrine glands; its pathogenesis is still unclear. Long non-coding RNAs (lncRNAs) are thought to play a role in the pathogenesis of autoimmune diseases and a comprehensive analysis of lncRNAs expression in pSjS is still lacking. To this aim, the expression of more than 540,000 human transcripts, including those ascribed to more than 50,000 lncRNAs is profiled at the same time, in a cohort of 16 peripheral blood mononuclear cells PBMCs samples (eight pSjS and eight healthy subjects). A complex network analysis is carried out on the global set of molecular interactions among modulated genes and lncRNAs, leading to the identification of reliable lncRNA-miRNA-gene functional interactions. Taking this approach, a few lncRNAs are identified as targeting highly connected genes in the pSjS transcriptome, since they have a major impact on gene modulation in the disease. Such genes are involved in biological processes and molecular pathways crucial in the pathogenesis of pSjS, including immune response, B cell development and function, inflammation, apoptosis, type I and gamma interferon, epithelial cell adhesion and polarization. The identification of deregulated lncRNAs that modulate genes involved in the typical features of the disease provides insight in disease pathogenesis and opens avenues for the design of novel therapeutic strategies. [ABSTRACT FROM AUTHOR]
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- 2019
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127. Simvastatin reduces endothelial activation and damage but is partially ineffective in inducing endothelial repair in systemic sclerosis
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Del Papa, Nicoletta, Cortiana, Michela, Vitali, Claudio, Silvestris, Ilaria, Maglione, Wanda, Comina, Denise P., Tiziano Lucchi, and Cortelezzi, Agostino
128. Short-term simvastatin treatment improves endothelial function and markers of endothelial damage in patients with systemic sclerosis
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Del Papa, Nicoletta, Cortiana, Michela, Silvestris, Ilaria, Maglione, Wanda, Comina, Denise P., Manara, Maria, Tiziano Lucchi, and Cortelezzi, Agostino
129. Does Ex VivoCD34+ Cell Selection Change the Outcome of Systemic Sclerosis Patients Treated with Autologous Hematopoietic Stem Cell Transplantation (AHSCT), an Adwp EBMT Study?
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Oliveira, Maria Carolina, Labopin, Myriam, Henes, Jörg, Moore, John, Del Papa, Nicoletta, Stanciu, Ricca, Sakellari, Ioanna, Schroers, Roland, Scherer, Hans Ulrich, Kyrcz-Krzemien, Slawomira, Daikeler, Thomas, Alexander, Tobias, Finke, Jürgen, Badoglio, Manuela, Snowden, John A, and Farge, Dominique
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Introduction
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- 2014
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130. Bosentan Improves Functional Class, Pulmonary Artery Systolic Pressure, and DLCO in Scleroderma Patients with Pulmonary Hypertension: A Possible Synergy with Iloprost
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BERETTA, LORENZO, SCORZA, RAFFAELLA, DEL PAPA, NICOLETTA, and MAZZONE, ANTONIO
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- 2006
131. Response to antifibrotic therapy and decrease of circulating BAG3 protein levels in systemic sclerosis patients with reduced forced vital capacity.
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De Marco, Margot, Basile, Anna, Cammarota, Anna Lisa, Iannone, Claudia, Falco, Antonia, Marzullo, Liberato, Rosati, Alessandra, Caporali, Roberto, Turco, Maria Caterina, and Del Papa, Nicoletta
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VITAL capacity (Respiration) , *SYSTEMIC scleroderma , *PROTEINS - Published
- 2024
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132. Surgical treatment of recalcitrant gastroesophageal reflux disease in patients with systemic sclerosis: a systematic review.
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Aiolfi, Alberto, Nosotti, Mario, Matsushima, Kazuhide, Perali, Carolina, Ogliari, Cristina, Del Papa, Nicoletta, Bonitta, Gianluca, and Bona, Davide
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FUNDOPLICATION , *GASTROESOPHAGEAL reflux , *SYSTEMIC scleroderma , *HEARTBURN , *BARRETT'S esophagus , *OPERATIVE surgery , *GASTRIC bypass - Abstract
Introduction: Gastroesophageal reflux disease (GERD) is frequently seen in patients with systemic sclerosis (SSc). Long-standing GERD may cause esophagitis, long-segment strictures, and Barrett's esophagus and may worsen pre-existing pulmonary fibrosis with an increased risk of end-stage lung disease. Surgical treatment of recalcitrant GERD remains controversial. The purpose of this systematic review was to summarize the current data on surgical treatment of recalcitrant GERD in SSc patients. Materials and methods: A systematic literature review according to PRISMA and MOOSE guidelines. PubMed, EMBASE, and Web of Science databases were consulted. Results: A total of 101 patients were included from 7 studies. The age ranged from 34 to 61 years and the majority were females (73.5%). Commonly reported symptoms were heartburn (92%), regurgitation (77%), and dysphagia (74%). Concurrent pulmonary disease was diagnosed in 58% of patients. Overall, 63 patients (62.4%) underwent open fundoplication, 17 (16.8%) laparoscopic fundoplication, 15 (14.9%) Roux en-Y gastric bypass (RYGB), and 6 (5.9%) esophagectomy. The postoperative follow-up ranged from 12 to 65 months. Recurrent symptoms were described in up to 70% and 30% of patients undergoing fundoplication and RYGB, respectively. Various symptoms were reported postoperatively depending on the type of surgical procedures, anatomy of the valve, need for esophageal lengthening, and follow-up. Conclusions: The treatment of recalcitrant GERD in SSc patients is challenging. Esophagectomy should be reserved to selected patients. Minimally invasive RYGB appears feasible and safe with promising preliminary short-term results. Current evidence is scarce while a definitive indication about the most appropriate surgical treatment is lacking. [ABSTRACT FROM AUTHOR]
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- 2021
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133. Phenotype of limited cutaneous systemic sclerosis patients with positive anti-topoisomerase I antibodies: data from the EUSTAR cohort
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Elisabetta Zanatta 1, Dörte Huscher 2, Augusta Ortolan 1, Jérôme Avouac 3, Paolo Airò 4, Alexandra Balbir-Gurman 5, Elise Siegert 6, Marco Matucci Cerinic 7, Franco Cozzi 8, Gabriela Riemekasten 9, Anna-Maria Hoffmann-Vold 10, Oliver Distler 11, Armando Gabrielli 12, Stefan Heitmann 13, Nicolas Hunzelmann 14, Carlomaurizio Montecucco 15, Jadranka Morovic-Vergles 16, Camillo Ribi 17, Andrea Doria 1, Yannick Allanore 3, EUSTAR collaborators, Giovanna Cuomo, Gianluca Moroncini, Jiri Stork, Fiorenzo Iannone, Ulrich Walker, Eugenia Bertoldo, Dorota Krasowska, Maria João Salvador, Mohammed Tikly, Eric Hachulla, Valeria Riccieri, Ami Sha, Ana Maria Gheorghiu, Cord Sunderkötter, Francesca Ingegnoli, Luc Mouthon, Vanessa Smith, Francesco Paolo Cantatore, Kilian Eyerich, Piotr Wiland, Marie Vanthuyne, Branimir Anic, Maria Üprus, Brigitte Granel, Alessandra Vacca, Cristina-Mihaela Tanaseanu, Paloma García de la Peña Lefebvre, Jean Sibilia, Ira Litinsky, Lesley Ann Saketkoo, Eduardo Kerzberg, Massimiliano Limonta, Doron Rimar, Petros Sfikakis, Maurizio Cutolo, Patricia E Carreira, Rosario Foti, Srdan Novak, Michele Iudici, Mislav Radic, Raffaele Pellerito, Carlo Francesco Selmi, Lidia P Ananieva, Gabriela Szücs, Carlos de la Puente, Ruxandra Maria Ionescu, Jörg Distler, Maria Rosa Pozzi, Juan Jose Alegre-Sancho, Kristine Herrmann, Ellen De Langhe, Sule Yavuz, Carolina de Souza Müller, Svetlana Agachi, Douglas Veale, Esthela Loyo, Mengtao Li, Edoardo Rosato, Britta Maurer, Ivan Castellví, François Spertini, Kamal Solanki, Nicoletta Del Papa, Gerard Espinosa, László Czirják, Bernard Coleiro, Dominique Farge Bancel, Christopher Denton, Nemanja Damjanov, Jörg Henes, Vera Ortiz Santamaria, Michaela Kohm, Bojana Stamenkovic, 1, Elisabetta Zanatta, 2, Dörte Huscher, 1, Augusta Ortolan, 3, Jérôme Avouac, 4, Paolo Airò, 5, Alexandra Balbir-Gurman, 6, Elise Siegert, 7, Marco Matucci Cerinic, 8, Franco Cozzi, 9, Gabriela Riemekasten, Hoffmann-Vold 10, Anna-Maria, Distler 11, Oliver, Gabrielli 12, Armando, Heitmann 13, Stefan, Hunzelmann 14, Nicola, Montecucco 15, Carlomaurizio, Morovic-Vergles 16, Jadranka, Ribi 17, Camillo, 1, Andrea Doria, 3, Yannick Allanore, Collaborators, Eustar, Cuomo, Giovanna, Moroncini, Gianluca, Stork, Jiri, Iannone, Fiorenzo, Walker, Ulrich, Bertoldo, Eugenia, Krasowska, Dorota, João Salvador, Maria, Tikly, Mohammed, Hachulla, Eric, Riccieri, Valeria, Sha, Ami, Maria Gheorghiu, Ana, Sunderkötter, Cord, Ingegnoli, Francesca, Mouthon, Luc, Smith, Vanessa, Paolo Cantatore, Francesco, Eyerich, Kilian, Wiland, Piotr, Vanthuyne, Marie, Anic, Branimir, Üprus, Maria, Granel, Brigitte, Vacca, Alessandra, Tanaseanu, Cristina-Mihaela, García de la Peña Lefebvre, Paloma, Sibilia, Jean, Litinsky, Ira, Ann Saketkoo, Lesley, Kerzberg, Eduardo, Limonta, Massimiliano, Rimar, Doron, Sfikakis, Petro, Cutolo, Maurizio, E Carreira, Patricia, Foti, Rosario, Novak, Srdan, Iudici, Michele, Radic, Mislav, Pellerito, Raffaele, Francesco Selmi, Carlo, P Ananieva, Lidia, Szücs, Gabriela, de la Puente, Carlo, Maria Ionescu, Ruxandra, Distler, Jörg, Rosa Pozzi, Maria, Jose Alegre-Sancho, Juan, Herrmann, Kristine, De Langhe, Ellen, Yavuz, Sule, de Souza Müller, Carolina, Agachi, Svetlana, Veale, Dougla, Loyo, Esthela, Li, Mengtao, Rosato, Edoardo, Maurer, Britta, Castellví, Ivan, Spertini, Françoi, Solanki, Kamal, Del Papa, Nicoletta, Espinosa, Gerard, Czirják, László, Coleiro, Bernard, Farge Bancel, Dominique, Denton, Christopher, Damjanov, Nemanja, Henes, Jörg, Ortiz Santamaria, Vera, Kohm, Michaela, and Stamenkovic, Bojana
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interstitial lung disease ,Scleroderma, Systemic ,Hypertension, Pulmonary ,disease subset ,anti-topoisomerase I ,Scleroderma ,Systemic sclerosis ,cutaneous form ,outcome ,Phenotype ,Rheumatology ,Scleroderma, Limited ,Antibodies, Antinuclear ,Scleroderma, Diffuse ,Humans ,Pharmacology (medical) ,Lung Diseases, Interstitial - Abstract
Objectives To characterize patients with positive anti-topoisomerase I (ATA) in lcSSc. Methods SSc patients enrolled in the EUSTAR cohort with a disease duration of ≤3 years at database entry were considered. We assessed the risk of major organ involvement in the following groups: ATA-lcSSc vs ACA-lcSSc and vs ANA without specificity (ANA)-lcSSc, and ATA-lcSSc vs ATA-dcSSc. Cox regression models with time-dependent covariates were performed with the following outcomes: new-onset interstitial lung disease (ILD), ILD progression [forced vital capacity (FVC) decline ≥10% and ≥5% vs values at ILD diagnosis), primary myocardial involvement (PMI), pulmonary hypertension (PH), any organ involvement and all-cause mortality. Results We included 1252 patients [194 ATA-lcSSc (15.5%)], with 7.7 years (s.d. 3.5) of follow-up. ILD risk was higher in ATA-lcSSc vs ACA- and ANA-lcSSc and similar to ATA-dcSSc, although with less frequent restrictive lung disease. The risk of FVC decline ≥10% (35% of ATA-lcSSc) was lower in ATA-lcSSc than in ATA-dcSSc, whereas FVC decline ≥5% occurs similarly between ATA-lcSSc (58% of patients) and other SSc subsets, including ATA-dcSSc. The risk of PMI was similar in ATA-lcSSc and ANA-lcSSc but lower than in ACA-lcSSc; no difference in PH and mortality risk was observed among lcSSc subsets. The risk of any organ involvement, PMI and PH was lower and the mortality tended to be lower in ATA-lcSSc vs ATA-dcSSc. Conclusion ATA-lcSSc patients have a high risk of ILD, albeit with a lower risk of progression compared with ATA-dcSSc, supporting careful screening for ILD in this subgroup.
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- 2022
134. Taking care of systemic sclerosis patients during COVID-19 pandemic: rethink the clinical activity.
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Minniti, Antonina, Maglione, Wanda, Pignataro, Francesca, Cappadona, Carmela, Caporali, Roberto, and Del Papa, Nicoletta
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COVID-19 pandemic , *SYSTEMIC scleroderma , *INFUSION therapy , *COVID-19 , *SARS-CoV-2 - Abstract
COVID-19 outbreak has quickly spread worldwide, causing a high pressure on the health-care system. In Italy, from March 8, 2020, all the deferrable clinical activities have been suspended to increase the health care offer for COVID-19 patients. The hospital organization has been modified also in order to assure non-COVID-19 patients assistance. The Scleroderma Unit of ASST Pini-CTO Hospital, in Milan, in the region mostly hit by SARS-CoV-2 in Italy, follows more than 600 patients affected by systemic sclerosis (SSc). Patients with SSc need a close follow-up with a regular screening of organ involvement and frequent intravenous treatments. All SSc patients have been educated about ministerial directives to limit COVID-19 spread. The organization of our Scleroderma Unit has been quickly rethought to assure SSc patients assistance in safety for them and for health-care workers during urgent visits or infusion therapies. Using electronic way of communication with frequent virtual contact and guarantying home deliveries of some therapies, we allowed a continuity of care also outside the Hospital. [ABSTRACT FROM AUTHOR]
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- 2020
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135. In vitro and ex vivo effect of tiaprofenic acid on human peripheral blood mononuclear cells
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Barcellini, Wilma, Borghi, Maria Orietta, Fain, Cristina, Del Papa, Nicoletta, Favini, Patrizia, and Meroni, Pier Luigi
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- 1992
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136. Glucocorticoids prescribing practices in systemic sclerosis: an analysis of the EUSTAR database
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Iudici, Michele, Mongin, Denis, Siegert, Elise, Carreira, Patricia E, Distler, Jörg, Henes, Jörg, Zanatta, Elisabetta, Hachulla, Eric, De Luca, Giacomo, Müller, Carolina de Souza, Santiago, Tânia, Tandaipan, José-Luis, Bianchi, Breno Valdetaro, De Santis, Maria, Hoffmann-Vold, Anna-Maria, Gabrielli, Armando, Distler, Oliver, Courvoisier, Delphine Sophie, Giovanna Cuomo, Gianluca Moroncini, Jiri Stork, Fiorenzo Iannone, Ulrich Walker, Eugenia Bertoldo, Dorota Krasowska, Maria João Salvador, Mohammed Tikly, Valeria Riccieri, Ami Sha, Ana Maria Gheorghiu, Cord Sunderkötter, Francesca Ingegnoli, Luc Mouthon, Vanessa Smith, Francesco Paolo Cantatore, Kilian Eyerich, Piotr Wiland, Marie Vanthuyne, Branimir Anic, Maria Üprus, Brigitte Granel, Alessandra Vacca, Cristina-Mihaela Tanaseanu, Paloma García de la Peña Lefebvre, Jean Sibilia, Ira Litinsky, Lesley Ann Saketkoo, Eduardo Kerzberg, Massimiliano Limonta, Doron Rimar, Petros Sfikakis, Maurizio Cutolo, Rosario Foti, Srdan Novak, Mislav Radic, Raffaele Pellerito, Carlo Francesco Selmi Rozzano, Lidia P Ananieva, Gabriela Szűcs, Carlos de la Puente, Ruxandra Maria Ionescu, Maria Rosa Pozzi, Juan Jose Alegre-Sancho, Kristine Herrmann, Ellen De Langhe, Sule Yavuz Altunizade, Svetlana Agachi, Douglas Veale, Esthela Loyo, Mengtao Li, Edoardo Rosato, Britta Maurer, Iván Castellví, François Spertini, Kamal Solanki, Nicoletta Del Papa, Gerard Espinosa, László Czirják, Bernard Coleiro, Dominique Farge Bancel, Christopher Denton, Nemanja Damjanov, Vera Ortiz Santamaria Granollers, Michaela Kohm, Bojana Stamenkovic, Yannick Allanore, Paolo Airo, Alexandra Balbir-Gurman, Marco Matucci Cerinic, Gabriela Riemekasten, Stefan Heitmann, Nicolas Hunzelmann, Carlomaurizio Montecucco, Jadranka Morovic-Vergles, Camillo Ribi, Michele, Iudici, Denis, Mongin, Elise, Siegert, Patricia E, Carreira, Jörg, Distler, Jörg, Hene, Elisabetta, Zanatta, Eric, Hachulla, Giacomo, De Luca, Carolina de Souza, Müller, Tânia, Santiago, José-Luis, Tandaipan, Breno Valdetaro, Bianchi, Maria, De Santi, Anna-Maria, Hoffmann-Vold, Armando, Gabrielli, Oliver, Distler, Courvoisier, Sophie, Delphine, Cuomo, Giovanna, Moroncini, Gianluca, Stork, Jiri, Iannone, Fiorenzo, Walker, Ulrich, Bertoldo, Eugenia, Krasowska, Dorota, João Salvador, Maria, Tikly, Mohammed, Riccieri, Valeria, Sha, Ami, Maria Gheorghiu, Ana, Sunderkötter, Cord, Ingegnoli, Francesca, Mouthon, Luc, Smith, Vanessa, Paolo Cantatore, Francesco, Eyerich, Kilian, Wiland, Piotr, Vanthuyne, Marie, Anic, Branimir, Üprus, Maria, Granel, Brigitte, Vacca, Alessandra, Tanaseanu, Cristina-Mihaela, García de la Peña Lefebvre, Paloma, Sibilia, Jean, Litinsky, Ira, Ann Saketkoo, Lesley, Kerzberg, Eduardo, Limonta, Massimiliano, Rimar, Doron, Sfikakis, Petro, Cutolo, Maurizio, Foti, Rosario, Novak, Srdan, Radic, Mislav, Pellerito, Raffaele, Francesco Selmi Rozzano, Carlo, P Ananieva, Lidia, Szűcs, Gabriela, de la Puente, Carlo, Maria Ionescu, Ruxandra, Rosa Pozzi, Maria, Jose Alegre-Sancho, Juan, Herrmann, Kristine, De Langhe, Ellen, Yavuz Altunizade, Sule, Agachi, Svetlana, Veale, Dougla, Loyo, Esthela, Li, Mengtao, Rosato, Edoardo, Maurer, Britta, Castellví, Iván, Spertini, Françoi, Solanki, Kamal, Del Papa, Nicoletta, Espinosa, Gerard, Czirják, László, Coleiro, Bernard, Farge Bancel, Dominique, Denton, Christopher, Damjanov, Nemanja, Ortiz Santamaria Granollers, Vera, Kohm, Michaela, Stamenkovic, Bojana, Allanore, Yannick, Airo, Paolo, Balbir-Gurman, Alexandra, Matucci Cerinic, Marco, Riemekasten, Gabriela, Heitmann, Stefan, Hunzelmann, Nicola, Montecucco, Carlomaurizio, Morovic-Vergles, Jadranka, and Ribi, Camillo
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Rheumatology ,systemic sclerosis ,Pharmacology (medical) ,epidemiology ,glucocorticoid - Abstract
Objectives To estimate the prevalence of long-term exposure to glucocorticoids (GCs) and to identify factors associated with, and variations in prescribing practices over time and across recruiting countries. Methods We included patients with SSc having a visit recorded in the EUSTAR database from January 2013 onward. We analysed the prevalence and the main features of GCs users, their exposure to GCs over time, and their GCs dosages. Multivariable linear regression was used to analyse the factors identified as associated with GCs intake duration. Time trends, and variations in GCs utilization across recruiting countries were explored. Missing data were imputed using multiple imputation with chained equations. Results The 9819 patients included were mostly females (85%), the majority had lcSSc (73%), and the median age was 58 years. At baseline, 34% of patients (n = 2769/8109) (48% dcSSc vs 29% lcSSc) were on GCs, and the median dose was 7.5 mg/day. GCs users were more frequently males and anti-Scl70 positive, and more commonly had dcSSc and more severe disease. On average, GCs users spent 25% of their follow-up time (median 33.2 months) on GCs, with no significant between-subsets difference. Notably, 33% (n = 971/2959) and 22% (n = 647/2959) of patients followed up for >1 year had received GCs for >6 and >12 months, respectively. Multivariable analysis showed that patient and disease characteristics poorly explained the variability in GCs exposure (adjusted-R2 = 0.06, P Conclusions GCs are widely and long-term prescribed in SSc, with significant between-countries and within-country differences. A gradual decrease in their utilization has been observed.
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- 2022
137. The Model for Early COvid-19 Recognition (MECOR) Score: A Proof-of-Concept for a Simple and Low-Cost Tool to Recognize a Possible Viral Etiology in Community-Acquired Pneumonia Patients during COVID-19 Outbreak
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Valentina Zuccaro, Mauro Giuffrè, Cristina Maurel, Michele Colaci, Andrea Palermo, Francesca Pignataro, Gianluca Sambataro, Giovanna Vignigni, Lorenzo Cavagna, Nunzio Crimi, Massimiliano Fabbiani, Sebastiano Emanuele Torrisi, Domenico Sambataro, Roberto Cesareo, Chiara Cassol, Nicoletta Del Papa, Erik Roman-Pognuz, Carlo Vancheri, Francesca Montagnani, Stefano Di Bella, Verena Zerbato, Roberto Luzzati, Lorenzo Malatino, Sambataro, Gianluca, Giuffrè, Mauro, Sambataro, Domenico, Palermo, Andrea, Vignigni, Giovanna, Cesareo, Roberto, Crimi, Nunzio, Torrisi, Sebastiano Emanuele, Vancheri, Carlo, Malatino, Lorenzo, Colaci, Michele, Del Papa, Nicoletta, Pignataro, Francesca, Roman-Pognuz, Erik, Fabbiani, Massimiliano, Montagnani, Francesca, Cassol, Chiara, Cavagna, Lorenzo, Zuccaro, Valentina, Zerbato, Verena, Maurel, Cristina, Luzzati, Roberto, and Di Bella, Stefano
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0301 basic medicine ,medicine.medical_specialty ,COVID-19 ,SARS-CoV-2 ,blood cell count ,coronavirus ,diagnosis ,interstitial lung disease ,neutrophils ,platelets ,pneumonia ,triage ,Clinical Biochemistry ,Disease ,Blood cell count ,Coronavirus ,Diagnosis ,Interstitial lung disease ,Neutrophils ,Platelets ,Pneumonia ,Triage ,Chest pain ,Article ,03 medical and health sciences ,0302 clinical medicine ,Community-acquired pneumonia ,Internal medicine ,medicine ,Peripheral blood cell ,Prospective cohort study ,platelet ,lcsh:R5-920 ,business.industry ,neutrophil ,medicine.disease ,coronaviru ,diagnosi ,030104 developmental biology ,030220 oncology & carcinogenesis ,medicine.symptom ,business ,lcsh:Medicine (General) - Abstract
This study aims to assess the peripheral blood cell count &ldquo, signature&rdquo, of Severe Acute Respiratory Syndrome-Coronavirus 2 (SARS-CoV-2) to discriminate promptly between COronaVIrus Disease 19 (COVID-19) and community-acquired pneumonia (CAP). We designed a retrospective case-control study, enrolling 525 patients (283 COVID-19 and 242 with CAP). All patients had a fever and at least one of the following signs: cough, chest pain, or dyspnea. We excluded patients treated with immunosuppressants, steroids, or affected by diseases known to modify blood cell count. COVID-19 patients showed a significant reduction in white blood cells (neutrophils, lymphocytes, monocytes, eosinophils) and platelets. We studied these parameters univariately, combined the significant ones in a multivariate model (AUROC 0.86, Nagelkerke PSEUDO-R2 0.5, Hosmer&ndash, Lemeshow p-value 0.9) and examined its discriminative performance in an internally-randomized validation cohort (AUROC 0.84). The cut-off selected according to Youden&rsquo, s Index (&minus, 0.13) showed a sensitivity of 84% and a specificity of 72% in the training cohort, and a sensitivity of 88% and a specificity of 73% in the validation cohort. In addition, we determined the probability of having COVID-19 pneumonia for each Model for possible Early COvid-19 Recognition (MECOR) Score value. In conclusion, our model could provide a simple, rapid, and cheap tool for prompt COVID-19 diagnostic triage in patients with CAP. The actual effectiveness should be evaluated in further, prospective studies also involving COVID-19 patients with negative nasopharyngeal swabs.
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- 2020
138. The cumulative number of micro-haemorrhages and micro-thromboses in nailfold videocapillaroscopy is a good indicator of disease activity in systemic sclerosis: A validation study of the NEMO score
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Wanda Maglione, Claudio Vitali, Nicoletta Del Papa, Francesca Pignataro, R. Andracco, Roberta Ferrara, Eleonora Zaccara, Gianluca Sambataro, Domenico Sambataro, Antonella Riccardi, Gabriele Valentini, Serena Vettori, Rosaria Irace, Andracco, Romina, Irace, Rosaria, Zaccara, Eleonora, Vettori, Serena, Maglione, Wanda, Riccardi, Antonella, Pignataro, Francesca, Ferrara, Roberta, Sambataro, Domenico, Sambataro, Gianluca, Vitali, Claudio, Valentini, Gabriele, and Del Papa, Nicoletta
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Adult ,Male ,0301 basic medicine ,medicine.medical_specialty ,Validation study ,lcsh:Diseases of the musculoskeletal system ,Adolescent ,Immunology ,Nailfold videocapillaroscopy ,Hemorrhage ,Logistic regression ,Microscopic Angioscopy ,Cohort Studies ,Fingers ,Disease activity ,Young Adult ,03 medical and health sciences ,Systemic sclerosi ,0302 clinical medicine ,Rheumatology ,Internal medicine ,medicine ,Humans ,Immunology and Allergy ,In patient ,skin and connective tissue diseases ,Aged ,Aged, 80 and over ,030203 arthritis & rheumatology ,Scleroderma, Systemic ,business.industry ,Curve analysis ,Reproducibility of Results ,Thrombosis ,Middle Aged ,Surgery ,030104 developmental biology ,Nails ,Cohort ,Systemic sclerosis ,Female ,lcsh:RC925-935 ,business ,Research Article - Abstract
Background Some abnormalities in nailfold videocapillaroscopy (NVC), such as the presence of micro-haemorrhages (MHEs), micro-thromboses (MTs), giant capillaries (GCs) and reduction in the number of capillaries (nCs), suggest a disease activity (DA) phase in systemic sclerosis (SSc). In a previous paper, we showed that the number of micro-haemorrhages and micro-thromboses (the so-called NEMO score) was the NVC feature more closely associated with DA. The present study was aimed at validating the NEMO score as a measure of DA in patients with SSc. Methods Two cohorts of 122 and 97 patients with SSc who were referred to two different rheumatology units, one in Milan and one in Naples, respectively, constituted the validation cohorts. The NEMO score, the total number of GCs and the mean nCs per digit were the parameters defined in each patient by eight-finger NVC. An expert operator analysed the NVCs in each of the participating units. The European Scleroderma Study Group (ESSG) index was used to define the DA level in each patient at the time of NVC examination. Results The NEMO score was the NVC parameter more strictly correlated with the ESSG score in both the Milan and Naples cohorts (p
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- 2017
139. Long-term retention rate, adverse event temporal patterns and rescue treatment strategies of mycophenolate mofetil in systemic sclerosis: insights from real-life.
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De Lorenzis E, Natalello G, Pellegrino G, Verardi L, Batani V, Lepri G, Stano S, Armentano G, De Pinto M, Motta F, Di Donato S, Kakkar V, Fiore S, Bisconti I, Campochiaro C, Cometi L, Tonutti A, Spinella A, Truglia S, Cavalli S, De Santis M, Giuggioli D, Del Papa N, Guiducci S, Cacciapaglia F, De Luca G, Iannone F, Ricceri V, Matucci Cerinic M, D'Agostino MA, Del Galdo F, and Bosello SL
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Background: Mycophenolate mofetil (MMF) is a mainstay for the treatment of systemic sclerosis (SSc). The occurrence and implications of MMF-related adverse events on drug retention rates in real life remain poorly defined. We aimed to determine the MMF retention rate and to investigate the causes and patterns of discontinuation, adverse events (AEs) and treatment options used after discontinuation., Methods: SSc patients who started MMF treatment underwent a retrospective longitudinal assessment for up to 5 years. We documented the incidence, predictors, and impacts of MMF treatment on gastrointestinal intolerance, infections, laboratory abnormalities, and cancer. Rescue strategies implemented after MMF discontinuation were recorded., Results: The 5-year MMF retention rate of 554 patients stood at 70.7% and 19.6% of them stopped MMF due to AEs. One out of every four patients experienced a dose reduction or discontinuation of MMF due to AEs, with gastrointestinal intolerance being the predominant cause. The 5-year cumulative incidence rates for gastrointestinal intolerance, cancer, severe infections, and laboratory toxicity leading to MMF discontinuation were 6.4%, 4.1%, 3.1%, and 2.1%, respectively. Lower respiratory tract was the most affected, with bacteria being the predominant causative agent. Intestinal and pulmonary circulation involvement were tied to elevated AE rates and MMF discontinuation. The most common approaches post-MMF cessation were "watch and wait" and switch to rituximab., Conclusions: MMF use in SSc appears to be limited by the occurrence of AEs, both in terms of persistence and dosing of the drug. Rescue options after MMF discontinuation are limited and many patients remain without immunosuppressant., (© The Author(s) 2024. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)
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- 2024
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140. Pulmonary and extra-pulmonary effects of lung transplantation in an Italian cohort of patients with systemic sclerosis.
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Iannone C, Pellico MR, Morlacchi LC, Rossetti V, Vicenzi M, Beretta L, Severino A, Airò P, Cacciapaglia F, Codullo V, Faggioli P, Iagnocco A, Meloni F, Mercante L, Saracco M, Stano S, Zaccara E, Minniti A, Cavalli S, Trignani G, Blasi F, Nosotti M, Boffini M, Caporali R, and Del Papa N
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Objectives: Lung transplantation (LuTx) is a life-saving intervention for Systemic Sclerosis (SSc) patients with end-stage lung disease. The aim of this study was to evaluate patients' survival and LuTx outcomes on systemic disease manifestations., Methods: A retrospective evaluation was conducted on SSc patients who underwent LuTx between 2010 and 2021. Outcomes assessed at baseline, 6, 12, and 24 months post-LuTx included skin involvement by modified Rodnan skin score (mRSS), global disease activity using a modified EUSTAR index (0-9 scale). Lung function rescue was evaluated by forced vital capacity (FVC). Patient survival was assessed by Kaplan-Meier analysis., Results: 13 SSc patients were included, with a male/female ratio 9/4 and a median age of 48.7 years. Nine patients were affected by diffuse cutaneous scleroderma (dcSSc) and four by limited cutaneous scleroderma (lcSSc). FVC significantly increased from 56% of the predicted value at baseline to 78% at 2 years (p= 0.003). mRSS decreased from 7.4 ± 3.8-3.3 ± 2.5 in patients with dcSSc (p= 0.02). The modified EUSTAR index score decreased from 2.54 ± 1.8 at baseline to 0.49 ± 0.5 at 2 years (p= 0.02). Survival rate was 92.3% at 2 years, and 76.9% at 5 years. No unexpected adverse events were observed., Conclusions: In SSc patients undergoing LuTx, an excellent 2-year survival was observed, without any disease-related adverse events. Our study supports LuTx as a viable option in SSc patients with end-stage lung disease. Apart from expected recovery of lung function, LuTx was associated with improvement of mRSS and global systemic disease activity., (© The Author(s) 2024. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)
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- 2024
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141. Overexpression of BAG3 (Bcl2-associated athanogene 3) in serum and skin of patients with systemic sclerosis.
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De Marco M, Armentaro G, Falco A, Minniti A, Cammarota AL, Iannone C, Basile A, D'Ardia A, Zeppa P, Marzullo L, Rosati A, Vitali C, Caporali R, and Del Papa N
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- Humans, Female, Male, Middle Aged, Adult, Case-Control Studies, Fibrosis, Aged, Up-Regulation, Scleroderma, Diffuse blood, Scleroderma, Limited blood, Scleroderma, Limited diagnosis, Biopsy, Scleroderma, Systemic blood, Scleroderma, Systemic pathology, Scleroderma, Systemic metabolism, Apoptosis Regulatory Proteins metabolism, Apoptosis Regulatory Proteins blood, Adaptor Proteins, Signal Transducing, Skin pathology, Skin metabolism, Biomarkers blood
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Objectives: BAG3 (Bcl2-associated athanogene3) is able to induce the transformation of cancer-associated fibroblasts to alpha smooth muscle actin (a-SMA) positive (+) myofibroblasts. In systemic sclerosis (SSc), a-SMA+ myofibroblasts also play an important role in the progression of fibrosis in the skin and involved internal organs. The aim of the study was to investigate whether BAG3 is overexpressed in SSc and may be a biomarker of fibrogenesis., Methods: BAG3 serum levels were measured in 106 patients with SSc, 47 with the limited (lc) and 59 the diffuse (dc) SSc, and in age- and sex-matched healthy controls (HC). BAG3 levels were then compared according to their clinical subset, nailfold video-capillaroscopic (NVC) patterns, interstitial lung disease (ILD, and correlated with modified Rodnan skin score (mRSS) and global disease activity. BAG3 expression was also investigated in skin biopsies of 8 dcSSc patients., Results: BAG3 serum levels were significantly higher in dcSSc (143.3 pg/mL, 95%CI 78-208.5) than in HC (0.68 pg/mL, 95%CI 0.13-1.23), and were significantly higher in patients with late NVC pattern and ILD but did not correlate with disease activity and mRSS. Of note, BAG3 was strongly expressed in the skin biopsies of dcSSc patients., Conclusions: BAG3 is overexpressed in dcSSc patients and may contribute to skin and organ fibrosis by prompting the transition of fibroblasts into myofibroblasts and increasing their survival. Thus, BAG3 may play an important role in SSc fibrotic pathogenesis and be a potential biomarker of fibrosis. Further research on its role as a therapeutic target is warranted.
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- 2024
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142. Dysregulation of circulating collagen turnover markers in very early systemic sclerosis.
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Dobrota R, Jordan S, Juhl P, Del Papa N, Maurer B, Becker M, Mihai C, Bay-Jensen AC, Karsdal MA, Siebuhr AS, and Distler O
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- Humans, Female, Male, Middle Aged, Adult, Extracellular Matrix metabolism, Collagen metabolism, Case-Control Studies, Cross-Sectional Studies, ROC Curve, Aged, Biglycan blood, Biglycan metabolism, Collagen Type III blood, Collagen Type III metabolism, Scleroderma, Systemic blood, Scleroderma, Systemic diagnosis, Biomarkers blood
- Abstract
Objective: Clinical observation suggests that vascular activation and autoimmunity precede remodelling of the extracellular matrix (ECM) in systemic sclerosis (SSc). We challenge this paradigm by hypothesising that ECM biomarkers are already disturbed in patients with very early SSc (veSSc) when fibrosis is not yet clinically detectable., Methods: 42 patients with veSSc, defined as the presence of Raynaud's phenomenon and at least one of puffy fingers, positive antinuclear antibodies or pathological nailfold capillaroscopy, not meeting the 2013 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for SSc, were compared with healthy controls (HCs, n=29). ECM degradation (BGM, C3M, C4M and C6M) and ECM formation biomarkers (PRO-C3, PRO-C4 and PRO-C5) were measured in serum using ELISAs. A cross-sectional analysis at baseline and a longitudinal analysis was performed., Results: Compared with HC, veSSc patients showed a strongly dysregulated turnover of type III and IV collagens (higher C3M, C4M, both p<0.0001 and PRO-C3, p=0.004, lower turnover ratios PRO-C3/C3M and PRO-C4/C4M, both p<0.0001). The biglycan degradation biomarker BGM was higher in veSSc than in HC (p=0.006), whereas the degradation biomarker for type VI collagen, C6M, was lower (p=0.002). In an ROC analysis, biomarkers of type III and IV collagen excellently distinguished between veSSc and HC: C3M, AUC=0.95, p<0.0001; C4M, AUC=0.97, p<0.0001; turnover ratios PRO-C3/C3M, AUC=0.80, p<0.0001; PRO-C4/C4M, AUC=0.97; p<0.0001., Conclusion: These findings indicate ECM remodelling as a very early phenomenon of SSc occurring in parallel with microvascular and autoimmune changes. Biomarkers of type III and IV collagens distinguished between veSSc patients and HC, indicating them as potential biomarkers for the detection of veSSc., Competing Interests: Competing interests: RD: Research support: Actelion, Pfizer (Articulum fellowship), eular training bursary, Iten-Kohaut foundation; consultancy: Boehringer-Ingelheim; speakers’ buro: Actelion; congress support: Amgen, Otsuka. SJ: None. PJ: was employed at Nordic Bioscience. BM: Research support: AbbVie, Protagen, Novartis Biomedical; lectures: Boehringer-Ingelheim, GSK, Novartis, Otsuka; consultancy: Novartis, Boehringer Ingelheim, Janssen-Cilag, GSK; congress support: Medtalk, Pfizer, Roche, Actelion, Mepha, MSD; patent mir-29 for the treatment of systemic sclerosis issued (US8247389). MB: GSK, Vifor. Bayer, Amgen, MSD outside of this work. CM: speakers bureau for Boehringer-Ingelheim, medbase Switzerland, MED Talks Switzerland, Mepha and PlayToKnow AG, advisory board for Boehringer-Ingelheim, consulting fees and/or honoraria from Boehringer Ingelheim and Janssen, and congress support from Boehringer Ingelheim and Roche. A-CB-J and MAK are full-time employees, part of senior management and shareholders at Nordic Bioscience. ASS was employed as a scientist at Nordic Bioscience at the time of conduction of the study, however is not longer working within the field. OD has/had consultancy relationship with and/or has received research funding from and/or has served as a speaker for the following companies in the area of potential treatments for systemic sclerosis and its complications in the last three calendar years: 4P-Pharma, Abbvie, Acceleron, Alcimed, Altavant, Amgen, AnaMar, Arxx, AstraZeneca, Blade, Bayer, Boehringer Ingelheim, Corbus, CSL Behring, Galderma, Galapagos, Glenmark, Gossamer, Horizon, Janssen, Kymera, Lupin, Medscape, Merck, Miltenyi Biotec, Mitsubishi Tanabe, Novartis, Prometheus, Redxpharma, Roivant and Topadur. Patent issued 'mir-29 for the treatment of systemic sclerosis' (US8247389, EP2331143). OD is a member of the editorial board of RMD open., (© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)
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- 2024
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143. Rituximab retention rate in systemic sclerosis: a long term real-life multicenter study.
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De Luca G, De Lorenzis E, Campochiaro C, Cacciapaglia F, Del Papa N, Zanatta E, Airò P, Lazzaroni MG, Giuggioli D, De Santis M, Alonzi G, Stano S, Binda M, Moccaldi B, Tonutti A, Cavalli S, Batani V, Natalello G, Iannone F, D'Agostino MA, Dagna L, Matucci-Cerinic M, and Bosello SL
- Abstract
Objectives: to report real-life data on rituximab retention-rate as indicator of safety and efficacy in a multicentric national cohort of systemic sclerosis patients., Methods: SSc patients treated with rituximab and followed for at least 36 months were included, clinically characterized, and longitudinally monitored. A competing risk analysis with sub-Hazard Ratio(sHR) definition was performed to explore the clinical variables linked to specific cause of rituximab discontinuation., Results: One-hundred-fifty-two SSc-patients (mean age 47.3 ± 12.3 years; females 79.6%; diffuse disease 77.6%; anti-topoisomerase-I positivity 63.2%) were evaluated over a median(IQR) time of 3.3(1.7-5.0) years. The primary indication for rituximab were interstitial lung disease (ILD)(38.8%), worsening skin fibrosis(36.8%), and arthritis(13.8%); 138 patients(90.8%) received more than one rituximab course. The 5-years rituximab retention rate was 59.9%(44.6-64.7%). Clinical response was the most common reason for rituximab discontinuation[5.7(3.7-8.4) per 100 patient-year] and was associated with a shorter disease duration[sHR 0.8(0.7-0.9)], anti-topoisomerase-I negativity[sHR 0.4(0.2-0.9)], previous digital ulcers[sHR 2.6(1.1-6.2] and no history of arthritis[sHR 0.3 (0.1-0.8)]. Treatment failure was the second cause of rituximab discontinuation[3.7(2.2-6.0) per 100 patient-year] and was associated with anti-centromere antibody positivity[sHR 2.8(1.1-7.4)] and anti-topoisomerase-I negativity[sHR 0.2(0.1-0.6)]. Adverse events(AEs) were the less common cause of discontinuation[3.1(1.7-5.2) per 100 patient-year], associated with limited cutaneous subset[sHR 3.4(1.2-9.7)] and previous mycophenolate mofetil treatment[sHR 4.5(1.2-16.3)]., Conclusion: rituximab is a safe and effective treatment in SSc: clinical response emerged as the primary reason for rituximab discontinuation, and AEs had a limited impact on treatment persistence. The identification of specific disease features associated with a response to rituximab will be useful in the management of SSc-patients., (© The Author(s) 2024. Published by Oxford University Press on behalf of the British Society for Rheumatology.)
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- 2024
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144. Significant nailfold capillary loss and late capillaroscopic pattern are associated with pulmonary arterial hypertension in systemic sclerosis.
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De Angelis R, Riccieri V, Cipolletta E, Del Papa N, Ingegnoli F, Bosello S, Spinella A, Pellegrino G, de Pinto M, Papa S, Armentaro G, and Giuggioli D
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- Humans, Female, Male, Middle Aged, Cross-Sectional Studies, Case-Control Studies, Aged, Hypertension, Pulmonary etiology, Hypertension, Pulmonary diagnostic imaging, Hypertension, Pulmonary physiopathology, Adult, Pulmonary Arterial Hypertension physiopathology, Pulmonary Arterial Hypertension diagnostic imaging, Pulmonary Arterial Hypertension etiology, Microvascular Density, Microscopic Angioscopy methods, Scleroderma, Systemic complications, Scleroderma, Systemic physiopathology, Scleroderma, Systemic pathology, Scleroderma, Systemic diagnostic imaging, Capillaries pathology, Capillaries diagnostic imaging, Nails blood supply
- Abstract
Objective: To evaluate differences in nailfold videocapillaroscopy (NVC) findings between SSc patients with and without a diagnosis of pulmonary arterial hypertension (PAH)., Methods: One hundred and ten SSc patients were enrolled in this cross-sectional, case-control, multicentre study. Patients were divided into cases (SSc-PAH confirmed by right heart catheterization) and controls (SSc-nonPAH with low probability of PAH). NVC patterns (early, active and late) and morphological parameters (microvascular density, non-specific abnormalities, giant capillaries, micro-haemorrhages, avascular areas) were considered using a semiquantitative scoring system., Results: SSc-PAH patients showed higher frequencies of late pattern (P < 0.01), non-specific abnormalities (P < 0.01), lower capillary density (P < 0.01), higher avascular areas (P < 0.01) and a higher mean NVC score (P < 0.01). Contrarily, the early/active pattern (P < 0.01) and a higher rate of micro-haemorrhages (P = 0.04) were more frequent in non-PAH patients. By a multivariate analysis, SSc-PAH patients, compared with non-PAH, had more non-specific abnormalities [27/55, 49.1% vs 10/55, 18.2%; adjusted odd ratio (OR) 16.89; 95% CI: 3.06, 93.16], a lower capillary density (grade 3, 20/55, 36.4% vs 5/55, 9.1%; adjusted OR 38.33; 95% CI: 2.34, 367.80) and avascular areas (18/55, 32.7% vs 10/55, 18.2%; adjusted OR 16.90; 95% CI: 2.64, 44.35). A correlation was found between the mean pulmonary arterial pressure and avascular areas (P < 0.01), capillary density (P < 0.01) and non-specific abnormalities (P < 0.01). A clinical model including the NVC variables may be able to predict a diagnosis of PAH., Conclusion: Our results indicate that the distinctive peripheral microcirculatory injury of SSc, i.e. capillary loss and morphological abnormalities, appear more severe and pronounced in patients with SSc-PAH., (© The Author(s) 2023. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)
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- 2024
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145. Innovative cellular therapies for autoimmune diseases: expert-based position statement and clinical practice recommendations from the EBMT practice harmonization and guidelines committee.
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Greco R, Alexander T, Del Papa N, Müller F, Saccardi R, Sanchez-Guijo F, Schett G, Sharrack B, Snowden JA, Tarte K, Onida F, Sánchez-Ortega I, Burman J, Castilla Llorente C, Cervera R, Ciceri F, Doria A, Henes J, Lindsay J, Mackensen A, Muraro PA, Ricart E, Rovira M, Zuckerman T, Yakoub-Agha I, and Farge D
- Abstract
Autoimmune diseases (ADs) are characterized by loss of immune tolerance, high chronicity, with substantial morbidity and mortality, despite conventional immunosuppression (IS) or targeted disease modifying therapies (DMTs), which usually require repeated administration. Recently, novel cellular therapies (CT), including mesenchymal stromal cells (MSC), Chimeric Antigen Receptors T cells (CART) and regulatory T cells (Tregs), have been successfully adopted in ADs. An international expert panel of the European Society for Blood and Marrow Transplantation and the International Society for the Cell and Gene Therapy, reviewed all available evidence, based on the current literature and expert practices, on use of MSC, CART and Tregs, in AD patients with rheumatological, neurological, and gastroenterological indications. Expert-based consensus and recommendations for best practice and quality of patient care were developed to support clinicians, scientists, and their multidisciplinary teams, as well as patients and care providers and will be regularly updated., Competing Interests: RG discloses speaking honoraria from Biotest, Pfizer, Medac, Neovii and Magenta. TA received study support from Amgen, Janssen and honoraria from Neovii, GSK, Astra-Zeneca, Abbvie. FM received honoraria & travel support from BMS, Janssen, Gilead, Miltenyi, Novartis, Astra-Zeneca, Biontech, received research support from Gilead, and discloses advisory board from Biontech. JAS discloses consultancy for Vertex, Medac and Jazz, and advisory board from Kiadis. PA discloses study support by NIHR, payment for expert testimony by Pinsent Mason and Bugge Valentin and consulting to Cellerys AG. RS discloses speaking honoraria from Novartis and Gilead. CCL received travel support for attending meeting by Gilead and discloses consultancy for Nektar Therapeutics and Gilead. FSG received study support from Novartis and Gilead, speaking honoraria from Astra-Zeneca and travel support from Abbvie, Gilead and Pierre-Fabre. FO discloses speaking honoraria from Takeda, Medac, Kyowa Kirin, Menarini-Stemline, and travel support from Medac, Jazz and Janssen. JOL received study support from Abbvie, Gilead, Takeda, consultancy & honoraria from AbbVie, BMS, Celgene, Celtrion, Engytix, Ferring, Galapagos, Gilead, GSK, Janssen, Lilly, MSD, Pfizer, Shire, Takeda, travel support by Abbvie, Takeda, Celltrion. AM received study support from Kyverna, Miltenyi and honoraria from Miltenyi, and participated to advisory board from Century Therapeutics. RC discloses speaking honoraria from GSK, AstraZeneca, Werfen, Rubió, Eli-Lilly, Pfizer. IYA discloses speaking honoraria from Kite, Novartis and BMS. None of the mentioned conflicts of interest were related to financing of the content of this manuscript. The remaining authors have nothing to declare., (© 2024 The Author(s).)
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- 2024
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146. Does the Impact of COVID-19 on Patients With Systemic Sclerosis Change Over Time?
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Deibel E, Carreira PE, Vonk M, Del Papa N, Bečvář R, Guillén-Del-Castillo A, Campochiaro C, Poormoghim H, Liem S, Lazzaroni MG, Giollo A, Mekinian A, de Vries-Bouwstra J, De Santis M, Balbir-Gurman A, Mihai C, De Luca G, Moiseev S, Zanatta E, Foti R, Rednic S, Denton C, Cutolo M, Belloli L, Airo P, Garzanova L, Moroncini G, İnanç M, Panopoulos S, Tandaipan JL, Chatelus E, Rosato E, Kuwana M, Yavuz S, Alegre-Sancho JJ, Smith V, Szűcs G, Henes J, Rodríguez-Pintó I, Atzeni F, Spierings J, Truchetet ME, Milchert M, Brito de Araujo D, Riemekasten G, Bernardino V, Martin T, Del Galdo F, Vacca A, Mendoza F, Midtvedt Ø, Murdaca G, Santiago T, Codullo V, Cacciapaglia F, Walker U, Brunborg C, Tirelli F, Allanore Y, Furst DE, Matucci M, Gabrielli A, Distler O, and Hoffmann-Vold AM
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- Male, Humans, COVID-19 Testing, COVID-19, Scleroderma, Systemic complications, Scleroderma, Systemic diagnosis, Scleroderma, Systemic epidemiology, Scleroderma, Localized, Hypertension
- Abstract
Objective: The outcome of patients with COVID-19 improved over the pandemic, including patients with systemic rheumatic diseases. However, data on patients with systemic sclerosis (SSc) are lacking. This study aimed to assess the outcome of patients with both SSc and COVID-19 over several waves., Methods: Patients with both SSc and COVID-19 who were registered in the European Scleroderma Trials and Research group (EUSTAR) were collected between April 2020 and April 2021. Patients were assigned to waves 1, 2, or 3 depending on the date of their COVID-19 diagnosis. Primary endpoints were death, intensive care unit stay, or ventilatory support (severe outcome). Subgroup analyses of patients who were hospitalized or died were conducted. General and SSc-specific characteristics and treatment were compared over the waves. Descriptive statistics and multivariate logistic regression were applied., Results: A total of 333 patients were included; 57 patients (17%) had a severe outcome, and 30 patients (9%) died. Compared to wave 1, significantly fewer patients with SSc suffered from severe COVID-19 in waves 2 and 3 (28.2% vs 9.8% and 12.7%; P < 0.001), fewer patients required hospitalization (46.7% vs 19.6% and 25.5%; P < 0.001) or ventilatory support (24.0% vs 8.7% and 10.9%; P = 0.001), and fewer patients died (15.7% vs 5.0% and 7.5%; P = 0.011). Patients were significantly younger, more often men, had less frequent arterial hypertension, and less SSc cardiac involvement over waves 1 to 3. Patients received significantly less medium to high doses of corticosteroids as they did SSc treatment., Conclusion: The outcome of patients with both SSc and COVID-19 improved significantly over time because of intrinsic and extrinsic factors., (© 2023 The Authors. Arthritis Care & Research published by Wiley Periodicals LLC on behalf of American College of Rheumatology.)
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- 2024
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147. Herpes zoster in lupus nephritis: experience on 292 patients followed up for 15 years.
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Reggiani F, Cardi S, Tumminello F, Calatroni M, Locatelli L, Gerosa M, Del Papa N, and Moroni G
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- Humans, Immunosuppressive Agents adverse effects, Azathioprine adverse effects, Retrospective Studies, Treatment Outcome, Mycophenolic Acid, Methylprednisolone therapeutic use, Lupus Nephritis drug therapy, Lupus Nephritis epidemiology, Lupus Nephritis chemically induced, Herpes Zoster epidemiology
- Abstract
Objectives: To evaluate the prevalence, incidence, and predictors of herpes zoster (HZ) development in lupus nephritis (LN)., Methods: This retrospective study included 292 LN patients to determine HZ incidence during the last decades and its correlation with LN activity. LN patients with HZ were matched with LN patients without HZ in a 1:2 ratio based on sex, age, year of LN diagnosis, and LN histological class at kidney biopsy to assess HZ risk factors. Statistical tests included t-test, U-test, and Fisher's test. Univariate and multivariate logistic regression analyses were conducted to identify potential risk factors., Results: HZ occurred after LN diagnosis in 66 patients (prevalence 22.6%) with an average of 8.7 years (range 0.2-28.4 years). Although with the potential limitations of the retrospective nature and the extensive duration of the study, the incidence of HZ was 15.6/1,000 person-years, increasing from 6.9 before 1980 to 16.0 in the 1990s and 43.9 after 2010. HZ onset was unrelated to LN activity. LN was active in 43% of cases and quiescent in the other 57% of cases at HZ diagnosis. The percentage of patients who developed lupus flares during the year after HZ (18.9%) was not different from that which occurred during the year before HZ (17.2%, p = 0.804). After excluding confounding factors through matching, the univariate analysis suggested that cyclosporin during induction therapy (p = 0.011) and higher cumulative doses of glucocorticoids (GCs; >50 g, p = 0.004), cyclophosphamide (CYC; >5 g, p = 0.001), and mycophenolate mofetil (MMF > 1,000 g, p = 0.007) predisposed patients to HZ. Univariate and multivariate analyses revealed a protective role of azathioprine (p = 0.008) and methylprednisolone pulses (p = 0.010) during induction therapy., Conclusions: HZ occurs unpredictably throughout the course of LN, underscoring the importance of continuous monitoring for these patients. In addition, the incidence of HZ seems to have increased in recent decades. Induction therapy with azathioprine and methylprednisolone pulses appears to provide protection, while higher cumulative doses of GCs, CYC, and MMF increase susceptibility., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. The reviewer EB declared a shared parent affiliation with the authors MG and NP to the handling editor at the time of review. The author(s) declared that they were an editorial board member of Frontiers, at the time of submission. This had no impact on the peer review process and the final decision., (Copyright © 2023 Reggiani, Cardi, Tumminello, Calatroni, Locatelli, Gerosa, Del Papa and Moroni.)
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- 2023
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148. Higher risk of short term COVID-19 vaccine adverse events in myositis patients with autoimmune comorbidities: results from the COVAD study.
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Dey M, R N, Nikiphorou E, Sen P, Saha S, Lilleker JB, Agarwal V, Kardes S, Day J, Milchert M, Joshi M, Gheita T, Salim B, Velikova T, Gracia-Ramos AE, Parodis I, Selva O'Callaghan A, Kim M, Chatterjee T, Tan AL, Makol A, Nune A, Cavagna L, Saavedra MA, Shinjo SK, Ziade N, Knitza J, Kuwana M, Distler O, Barman B, Singh YP, Ranjan R, Jain A, Pandya SC, Pilania RK, Sharma A, Manoj MM, Gupta V, Kavadichanda CG, Patro PS, Ajmani S, Phatak S, Goswami RP, Chowdhury AC, Mathew AJ, Shenoy P, Asranna A, Bommakanti KT, Shukla A, Pande AR, Chandwar K, Pauling JD, Wincup C, Üsküdar Cansu D, Zamora Tehozol EA, Rojas Serrano J, García-De La Torre I, Del Papa N, Sambataro G, Fabiola A, Govoni M, Parisi S, Bartoloni Bocci E, Sebastiani GD, Fusaro E, Sebastiani M, Quartuccio L, Franceschini F, Sainaghi PP, Orsolini G, De Angelis R, Danielli MG, Venerito V, Traboco LS, Hoff LS, Kusumo Wibowo SA, Tomaras S, Langguth D, Limaye V, Needham M, Srivastav N, Yoshida A, Nakashima R, Sato S, Kimura N, Kaneko Y, Loarce-Martos J, Prieto-González S, Gil-Vila A, Gonzalez RA, Chinoy H, Agarwal V, Aggarwal R, and Gupta L
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- Humans, COVID-19 Vaccines, Vaccination, COVID-19, Myositis, Autoimmune Diseases
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- 2023
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149. Long-term kidney outcome of patients with rheumatological diseases and antineutrophil cytoplasmic antibody-glomerulonephritis: comparison with a primitive ANCA-glomerulonephritis cohort.
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Locatelli L, Calatroni M, Reggiani F, Bonelli GD, Gerosa M, Argolini LM, Trezzi B, Del Papa N, Angelini C, Pozzi MR, Sinico RA, and Moroni G
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- Humans, Antibodies, Antineutrophil Cytoplasmic, Hematuria etiology, Retrospective Studies, Kidney pathology, Glomerulonephritis, Rheumatic Diseases, Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis complications, Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis diagnosis, Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis drug therapy
- Abstract
Objectives: Antineutrophil cytoplasmic antibody (ANCA) may appear in the course of rheumatic diseases (RD) but the kidney involvement is very rare and the prognosis poorly defined., Methods: We retrospectively identified patients with RD among 153 patients with ANCA glomerulonephritis (ANCA-GN). Their clinical/histological presentation and outcome were compared with that of primitive ANCA-GN patients (1:4) matched for sex, age, ANCA type and follow-up., Results: Nine patients (5.9%) were included: three had rheumatoid arthritis, two systemic sclerosis, two psoriatic arthritis, one ankylosing spondylitis and one seronegative spondylarthritis. Seven patients were MPO positive, two PR3 positive. ANCA-GN developed 74 months after RD with microscopic haematuria and acute kidney dysfunction in all but two patients. After 68-month follow-up, four patients (44.4%) achieved response to therapy defined as eGFR >60/min/1,73 m2 or stable, no microscopic haematuria and negative ANCA. At ANCA-GN diagnosis, serum creatinine and C-reactive protein were significantly lower in RD-ANCA-GN (2.38 vs. 3.34mg/dl, p=0.05 and 2.3mg/dl vs. 7.2mg/dl; p=0.05, respectively) while haemoglobin was higher (12.3g/dl vs. 9.3g/dl p<0.01) than in the 36 primitive ANCA-GN patients of control group. At kidney biopsy, focal forms were more frequent in RD patients (44.45% vs. 18.75%, p=0.11). The treatment between the two groups was not significantly different. At last observation, the percentage of patients with ESKD was lower in RD than in controls (11.1%vs. 30.5%; p=0.23)., Conclusions: Patients with RD seem to develop ANCA-GN with less severe clinical/histological kidney involvement, and better long-term kidney survival than primitive ANCA-GN. This is probably due to the strict monitoring of RD patients that allows a prompter ANCA-GN diagnosis and treatment.
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- 2023
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150. "Usual" interstitial pneumonia with autoimmune features: a prospective study on a cohort of idiopathic pulmonary fibrosis patients.
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Sambataro G, Ferrara CA, Torrisi SE, Spadaro C, Vignigni G, Vancheri A, Del Papa N, Orlandi M, Colaci M, Malatino L, Palmucci S, Cavagna L, Sambataro D, and Vancheri C
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- Humans, Prospective Studies, Retrospective Studies, Tomography, X-Ray Computed, Autoimmune Diseases complications, Autoimmune Diseases diagnostic imaging, Idiopathic Pulmonary Fibrosis diagnosis, Idiopathic Pulmonary Fibrosis diagnostic imaging, Lung Diseases, Interstitial diagnostic imaging, Lung Diseases, Interstitial etiology
- Abstract
Objectives: The classification interstitial pneumonia with autoimmune features (IPAF) includes patients with interstitial lung disease (ILD) associated with autoimmune characteristics insufficient to reach classification criteria for a specific autoimmune disease (SAD). These criteria are divided into three domains: clinical, serological and morphological. The latter domain does not include the usual interstitial pneumonia (UIP) pattern, which is deemed not to be significantly associated with SAD. Therefore, the enrolment of these patients is more difficult, requiring at least one item from both of the other domains. The objective of this study is to evaluate the rate of progression towards SAD of a cohort of UIP patients satisfying only one IPAF domain (we called this group "UIPAF") compared with classic idiopathic pulmonary fibrosis (IPF)., Methods: We prospectively enrolled IPF patients with radiologic and/or histologic UIP pattern, followed jointly by rheumatologists and pulmonologists from January 2017 to January 2021, with a minimum follow-up of 12 months., Results: We enrolled 190 IPF patients, 38 (20%) of whom were classified as UIPAF. IPF and UIPAF patients were similar for general characteristics, severity and prognosis, at presentation and at annual check-up. However, 28.9% of UIPAF patients progressed towards SAD, compared with 2% of IPF patients (χ2=30.4, p≤0.0001)., Conclusions: The association between a single clinical or serological domain of IPAF and UIP pattern is predictive for the development of a SAD if compared with isolated UIP. ILD can be the first manifestation of SAD, even with a UIP pattern, therefore, the morphological domain of IPAF criteria could be removed.
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- 2022
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