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1

Sepper, Dennis L. "Le forme e il vivente: Morfologia e filosofia della natura in J. W. Goethe. Paola Giacomoni." Isis 85, no. 4 (December 1994): 700–701. http://dx.doi.org/10.1086/357018.

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ABBRI, FERDINANDO. "PAOLA GIACOMONI, Le forme e il vivente. Morfologia e filosofia della natura in J. W. Goethe, Napoli, Guida editori, 1993, 284 pp. ill. Lit. 35.000." Nuncius 9, no. 1 (January 1, 1994): 458–59. http://dx.doi.org/10.1163/221058784x00850.

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TOSCANO, ANNA. "PAOLA GIACOMONI (a cura di), Immagini del corpo in età moderna, Trento, Editrice Università degli Studi di Trento, 1994, 282 pp. («Labirinti» Collana del Dipartimento di Scienze Filologiche e Storiche)." Nuncius 10, no. 1 (January 1, 1995): 359–62. http://dx.doi.org/10.1163/221058785x00264.

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4

Pepper, Simon. "Jacopo Aconcio. Trattato sulle fortificazioni. Edited by, Paola Giacomoni, with the collaboration of, Giovanni Maria Fara and Renato Giacomelli. Translated by, Omar Khalaf. (Studi e Testi, 48.) v + 212 pp., illus., index. Florence: Leo S. Olschki Editore, 2011. €28." Isis 105, no. 2 (June 2014): 426. http://dx.doi.org/10.1086/677993.

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Schwarz, Thomas. "Biophysical and physiological effects of solar radiation on human skin (edited by Paolo Giacomoni)." Archives of Dermatological Research 300, no. 4 (February 22, 2008): 209. http://dx.doi.org/10.1007/s00403-008-0830-9.

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6

Silva, Patrícia Antunes Serieiro. "Frades Pregadores e polêmica anti-herética medieval: novas leituras da Summa contra hereticos (Pseudo Giacomo de Capellis)." Revista de História, no. 181 (November 4, 2022): 1–8. http://dx.doi.org/10.11606/issn.2316-9141.rh.2022.196138.

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O texto é uma resenha da edição crítica comentada da Summa contra hereticos fratris Iacobi de Capellis, um dos tratados mais intrigantes da polêmica anti-herética medieval, publicada na Itália, em 2018. Ele divide-se, basicamente, em duas partes. Na primeira parte, são apresentadas as novas perspectivas sobre autoria, datação e operacionalidade do tratado anônimo, discutidas por Maurizio Ulturale, na introdução do volume. Por fim, na segunda parte do texto, são abordados alguns aspectos do trabalho de reconstrução filológica da Summa feito por Paola Romagnoli.
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Bettiol, A., M. L. Urban, F. Alberici, C. Agostini, C. Baldini, E. Bozzolo, P. Cameli, et al. "OP0148 MEPOLIZUMAB FOR EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS (EGPA): A RETROSPECTIVE REAL-WORLD EUROPEAN STUDY ON 142 PATIENTS." Annals of the Rheumatic Diseases 79, Suppl 1 (June 2020): 94–95. http://dx.doi.org/10.1136/annrheumdis-2020-eular.5544.

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Background:Evidence on the efficacy of Mepolizumab (MEPO) in Eosinophilic Granulomatosis with Polyangiitis (EGPA) is scarce [1].Objectives:To assess the efficacy and safety of MEPO in real-life clinical practice.Methods:We retrospectively included patients diagnosed with EGPA and treated with MEPO (100 or 300 mg/month). MEPO efficacy was evaluated in the first 12 months in terms of systemic disease and asthma control. The occurrence of any adverse event (AE) was recorded.Results:142 patients were included (38% males; median age 46.4 (IQR 36.7-54.4); 110 and 32 on MEPO 100 and 300 mg/month, respectively). General, ear-nose-throat, pulmonary, and neurological symptoms significantly decreased during treatment (table 1). MEPO accounted for a significant reduction in the BVAS (figure 1) and for a steroid sparing effect (figure 2). The proportion of patients with asthma attacks decreased by 90% at 12 months compared to t0, and asthma-related emergency accesses dropped from 17.4% to 2.3%. Overall, 21.1% of patients had a non-serious AE.Table 1.Control of clinical symptomsMEPO beginning (t0)3 monthsp-value(t3 vs t0)6 monthsp-value(t6 vs t0)12 monthsp-value(t12 vs t0)N obsN=142N=135N=123N=89General symptoms40 (28.2%)17 (12.6%)<0.00119 (15.5%)<0.00113 (14.6%)0.002Cutaneous manifestations13 (9.2%)6 (4.4%)0.0085 (4.1%)0.0254 (4.5%)0.180ENT manifestations106 (74.7%)52 (38.5%)<0.00144 (35.8%)<0.00129 (32.6%)<0.001Pulmonary manifestations130 (91.6%)59 (43.7%)<0.00139 (31.7%)<0.00128 (31.5%)<0.001Cardiac manifestations6 (4.2%)2 (1.5%)0.0832 (1.6%)0.08300.157Intestinal manifestations10 (7.0%)1 (0.7%)0.0054 (3.3%)0.0593 (3.4%)0.059Renal manifestations5 (3.5%)3 (2.2%)0.41400.0461 (1.1%)0.317Neurological manifestations36 (25.4%)22 (16.3%)0.01218 (14.6%)0.00310 (11.2%)0.035Figure 1.Changes in BVASFigure 2.Steroid treatmentConclusion:MEPO effectively controlled systemic and respiratory EGPA symptoms in a large European cohort, with no major safety concerns.References:[1]Wechsler et al. MEPO or Placebo for Eosinophilic Granulomatosis with Polyangiitis. NEJM. 2017Disclosure of Interests:Alessandra Bettiol: None declared, Maria Letizia Urban: None declared, Federico Alberici: None declared, Carlo Agostini: None declared, Chiara Baldini: None declared, Enrica Bozzolo: None declared, Paolo Cameli: None declared, Nunzio Crimi: None declared, Stefano Del Giacco: None declared, Allyson Egan: None declared, Georgina Espigol-Frigole Consultant of: Roche and Janssen, Mara Felicetti: None declared, Marco Folci: None declared, Paolo Fraticelli: None declared, Marcello Govoni: None declared, Anna Kernder Grant/research support from: Grant/research support from: GlaxoSmithKline and UCB Pharma for performing the LuLa-study., Carlo Lombardi: None declared, Giuseppe Lopalco: None declared, Claudio Lunardi: None declared, Aladdin J Mohammad Speakers bureau: lecture fees from Roche and Elli Lilly Sweden, PI (GiACTA study), Frank Moosig: None declared, Simone Negrini: None declared, Thomas Neumann: None declared, Pavel Novikov Grant/research support from: This work was supported by the 5-100 Project, Sechenov University, Moscow, Giuseppe Paolazzi: None declared, paola parronchi: None declared, Luca Quartuccio Consultant of: Abbvie, Bristol, Speakers bureau: Abbvie, Pfizer, Vito Racanelli: None declared, Carlo Salvarani: None declared, Maxime Samson: None declared, Jan Schroeder: None declared, Savino Sciascia: None declared, Renato A. Sinico: None declared, Benjamin Terrier: None declared, Paola Toniati: None declared, Domenico Prisco: None declared, Augusto Vaglio: None declared, Giacomo Emmi: None declared
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Tonizzi, Elisabetta M. "Memoria, nostalgia, utopia. Il potere politico dei sentimenti." ITALIA CONTEMPORANEA, no. 263 (December 2011): 255–70. http://dx.doi.org/10.3280/ic2011-263005.

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La discussione si incentra sul libro Nostalgia. Memoria e passaggi tra le sponde dell'Adriatico (Roma, Edizioni di storia e letteratura, 2010, pp. 276), a cura di Rolf Petri, che illustra i contenuti e gli obiettivi scientifici dell'opera. Al dibattito partecipano due discussant, il sociologo Paolo Jedlowski e il filosofo Giacomo Marramao, che affrontano il libro secondo un approccio multidisciplinare, sottolineandone l'appartenenza all'ambito degli ‘studi culturali'. Sviluppano anche la complessa e controversa relazione tra nostalgia, costruzione della nazione e rituali della memoria. Viene inoltre pubblicata una parte dell'ampia discussione suscitata dai loro interventi.
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Granier, Thomas. "Antonella Degl’Innocenti, Paolo Gatti et Christian Giacomozzi (éd.), Le agiografie dei martiri Sisinnio, M." Cahiers de civilisation médiévale, no. 247 (July 1, 2019): 281–83. http://dx.doi.org/10.4000/ccm.4329.

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APFELSTADT, ERIC. "CHRISTOPHER COLUMBUS, PAOLO DAL POZZO TOSCANELLI AND FERNO DE RORIZ: NEW EVIDENCE FOR A FLORENTINE CONNECTION." Nuncius 7, no. 2 (1992): 69–80. http://dx.doi.org/10.1163/182539192x00857.

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Abstracttitle RIASSUNTO /title Si ritiene che Paolo dal Pozzo Toscanelli abbia mandato tre lettere in Portogallo tra il 1474 e il 1482: una, comprendente una mappa, indirizzata ad un canonico di Lisbona chiamato Fernando Martins; un'altra, contenente copie della prima lettera e della carta, a Cristoforo Colombo; e una terza ancora a Colombo. Sebbene l'autenticit delle tre lettere sia stata accettata dalla maggior parte degli studiosi colombiani, l'intera corrispondenza stata negata da alcuni, e le due lettere a Colombo da altri. In giuoco almeno una parte della stima per la scoperta del Mondo Nuovo. Martins stato spesso identificato col canonico portoghese Fernando de Roritz, che stato testimone al testamento di Niccol di Cusa insieme con Paolo dal Pozzo Toscanelli, ma nessun'altra prova della sua conoscenza con Toscanelli stata finora conosciuta. Nuovi documenti adesso confermano la presenza del Roritz a Firenze nel 1459, quando egli ha servito accanto al Toscanelli come medico del cardinale Giacomo di Portogallo ed stato testimone al testamento del cardinale, e di nuovo nel 1466, quando stato testimone all'atto di dote della cappella del cardinale in San Miniato al Monte.
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Trapp, J. B. "Homage to Petrarch as Humanist Saint: Peregrinatio litterarum ergo." Moreana 35 (Number 135-, no. 3-4 (December 1998): 233–43. http://dx.doi.org/10.3366/more.1998.35.3-4.15.

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Francesco Petrarca retired in 1370 to the small country house at Arquà, near Padua, in which he died. The house, its contents, and the great marble sarcophagus erected for his remains outside the parish church brought fame to the village, as Giovanni Boccaccio had prophesied they would. By the fifteenth century literary pilgrims were attracted to the village; in the sixteenth, house and tomb were adomed by Pietro Paolo Valdezocco, and Anton Francesco Doni proposed an elaborate memorial. In the seventeenth, Giacomo Filippo Tomasini described the village and tomb, and by the eighteenth and nineteenth centuries the site had become popular with tourists, Lord Byron included. Among the attractions was the mummified corpse of a cat, said to have belonged to the poet.
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Capizzi, Patrizia, Raffaele Martorana, Alessandro Canzoneri, Alessandra Carollo, and Marco V. Majani. "3D GPR Model in the Military District of San Giacomo Degli Spagnoli (Palermo)." Heritage 6, no. 3 (February 28, 2023): 2601–13. http://dx.doi.org/10.3390/heritage6030137.

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The georadar method was used to try to find some anthropic structures in a large square inside the Carabinieri barracks in the former military complex of San Giacomo degli Spagnoli in Palermo (Italy). These investigations are part of a broader context of a study of the entire area. The purpose of the investigations is to try to understand if under the ground there are the remains of an ancient horse passage that connected the Royal Palace of Palermo with the sea gate of the city. Furthermore, in the Middle Ages, on the site of the present square, there were most likely two churches, which no longer exist, as evidenced by numerous historical testimonies. One of the two, San Giacomo la Mazara, is known to have was placed right in front of the church of San Paolo, the subject of previous investigations. The investigations carried out on the main square of the military district allowed us to reconstruct a 3D georadar model in which numerous anomalies are highlighted. Some superficial anomalies have been attributed to the presence of sub-services, the deeper ones could be caused by the remains of the medieval underground way or those of the no longer existing medieval churches, but identifying their true nature requires further investigations and archaeological tests.
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RGI, Redazione. "Informazione bibliografica." RIVISTA GEOGRAFICA ITALIANA, no. 2 (May 2022): 127–61. http://dx.doi.org/10.3280/rgioa2-2022oa13807.

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Danny Dorling, Rallentare. La fine della grande accelerazione e perch&eacute; &egrave; un bene (Daniele Vignoli) Veronica della Dora, The mantle of the Earth. Genealogies of a geographical metaphor (Laura Lo Presti) Telmo Pievani, Mauro Varotto, Viaggio nell'Italia dell'Antropocene. La geografia visionaria del nostro futuro (Eleonora Guadagno) Enzo Pranzini, Granelli di sabbia. Una guida per camminare sul bordo del mare (Leonardo Rombai) Giada Peterle, La geografia spiegata ai bambini. Le avventure spaziali di Alex e il signor Globo (Marcella Terrusi) Roberta Cevasco, Carlo Alberto Gemignani, Daniela Poli, Luisa Rossi, a cura di, Il pensiero critico fra geografia e scienza del territorio. Scritti su Massimo Quaini (Floriana Galluccio) Michela Lazzeroni, Monica Morazzoni, a cura di, Interpretare la quarta rivoluzione industriale. La geografia in dialogo con le altre discipline (Teresa Graziano) Claudio Calveri, Pier Luigi Sacco, La trasformazione digitale della cultura (Federica Epifani) Silvia E. Piovan, The geohistorical approach. Methods and applications (Arturo Gallia) Franco Cazzola, Uomini e fiumi. Per una storia idraulica ed agraria della bassa pianura del Po (1450-1620) (Matteo Proto) Paolo Molinari, Living in Milan. Housing policies, austerity and urban regeneration (Giacomo Zanolin)
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Armocida, E., A. Turrini, and R. Bianucci. "Genius and psychopathology of the Italian poet Giacomo Leopardi (1798–1837): Paolo Mantegazza’s (1831–1910) criticism of the Lombrosian thought." Neurological Sciences 37, no. 10 (May 14, 2016): 1751–52. http://dx.doi.org/10.1007/s10072-016-2605-y.

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Nayyar, Reshma. "Joshua Stopping the Sun and Ignatius of Loyola at Il Gesù in Rome." Journal of Jesuit Studies 3, no. 2 (March 1, 2016): 211–37. http://dx.doi.org/10.1163/22141332-00302003.

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In 1672, the Jesuit superior general Gian Paolo Oliva commissioned from Baciccio a lavish cycle of ceiling frescoes for Rome’s Il Gesù after earmarking the tribune vault for Giacomo Cortese to decorate with a representation of Joshua Stopping the Sun. Oliva also planned to translate Ignatius of Loyola’s remains to the high altar. Pope Gregory xv had explicitly likened Ignatius to the Old Testament general Joshua during the Jesuit founder’s canonization in 1622, and it may be inferred that Oliva intended to promote a hagiographic connection between the two figures through the prominent juxtaposition of Cortese’s fresco with the saint’s relics. However, the Ignatius-Joshua connection remained uncelebrated: the plan to translate the relics did not come to fruition, Cortese passed away in 1676, and the apse vault was eventually decorated by Baciccio with the Adoration of the Mystic Lamb. Cortese’s demise has heretofore been considered the decisive factor leading to the change in subject of the tribune fresco, but the clandestine correspondence of Lazzero Sorba, S.J., indicates another important factor was at play. These documents evidence an unusually strained relationship between the Society and Pope Innocent xi Odescalchi, elected in 1676. They suggest that the Society’s discomfiture vis-à-vis Innocent xi influenced its decision to replace the self-aggrandizing Joshua Stopping the Sun with the Adoration of the Mystic Lamb.
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Lindgren, Lowell. "Musicians and Librettists in the Correspondence of Gio. Giacomo Zamboni (Oxford, Bodleian Library, Mss Rawlinson Letters 116–138)." Royal Musical Association Research Chronicle 24 (1991): 1–194. http://dx.doi.org/10.1080/14723808.1991.10540945.

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Gio. Giacomo Zamboni, merchant, diplomat and amateur harpsichordist, was born in Florence on 26 July 1683, arrived in London late in 1711 and lived there until his death on 8 April 1753. His career closely parallels that of George Frideric Handel, composer, manager and harpsichordist, who was born in Hanover in 1685, arrived in London late in 1710 and lived there from late 1712 until his death in 1759. When these two men arrived in London, opera in Italian was a novelty at the Queen's Theatre in the Haymarket, which had just begun to employ Italian singers, instrumentalists, composers, librettists and stage designers. During the ensuing decades, there was an unprecedented influx of Italian performers and creators who, like Handel and other ‘outlandish’ personnel at this theatre, found that salaries were higher, working conditions were better and freedom was greater in England than in their own lands. Many therefore stayed as long as possible, and their artistic accomplishments as well as their intricate interactions with British and foreign patrons, diplomats, merchants and musicians are fascinating endeavours that deserve detailed study. At present, the best survey is that in George Dorris, Paolo Rolli and the Italian Circle in London, 1715–44 (The Hague and Paris, 1967), which focuses upon literary accomplishments. The essential base for any such study must, of course, be primary source materials, which include letters and other documents as well as librettos and scores. My hope is that the passages cited below from 458 items, most of which have never before been printed, will significantly broaden our base for study of ‘the Italian circle’ in London between 1716 and 1750.
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Caballero, Juan Luis. "Giacomo PEREGO, Anna Maria PUDELKO, Eustacchio IMPERATO y Tosca FERRANTE (a cura di), Le lettere di Paolo, San Paolo, Cinisello Balsamo (Milano) 2008, 264 pp., 14,5 x 19,5, ISBN 978-88-2156-186-3." Scripta Theologica 41, no. 2 (November 17, 2017): 672. http://dx.doi.org/10.15581/006.41.13289.

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Journals, FrancoAngeli. "Informazione bibliografica." RIVISTA GEOGRAFICA ITALIANA, no. 3 (September 2021): 175–219. http://dx.doi.org/10.3280/rgioa3-2021oa12539.

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L'Informazione bibliografica del numero 3/2021 della &laquo;Rivista Geografica Italiana&raquo; presenta le recensioni dei seguenti testi: &nbsp; &nbsp;Donna Haraway, Chthulucene. Sopravvivere in un pianeta infetto (Michele Bandiera) Cristiano Giorda, a cura di, Geografia e Antropocene. Uomo, ambiente, educazione (Marco Tononi) Paola Piscitelli, a cura di, Atlante delle citt&agrave;. Nove (ri)tratti urbani per un viaggio planetario (Marco Santangelo) Martina Tazzioli, The making of migration: The biopolitics of mobility at Europe's borders (Silvia Aru) Mercedes Bresso, Claude Raffestin, I duecentocinquantamila stadi di Eratostene, al tempo del virus. Dialoghi fra un geografo e una economista ambientale, in giro per il mondo (Alessandro Ricci) Ernesto C. Sferrazza Papa, Le pietre e il potere. Una critica filosofica dei muri (Marcello Tanca) Vincent Berdoulay, Olivier Soubeyran, L'am&eacute;nagement face &agrave; la menace climatique (Angelo Turco) Isabella Giunta, Sara Caria, a cura di, Pasado y presente de la cooperaci&oacute;n internacional: una perspectiva cr&iacute;tica desde las teor&iacute;as del sistema mundo&nbsp;(Mariasole Pepa) Sara Luchetta, Dalla baita al ciliegio. La montagna nella narrativa di Mario Rigoni Stern (Giacomo Zanolin) Edoardo Boria, Storia della cartografia in Italia dall'Unit&agrave; a oggi. Tra scienza, societ&agrave; e progetti di potere (Anna Guarducci) Maria Luisa Sturani, Dividere, governare e rappresentare il territorio in uno Stato di antico regime. La costruzione della maglia amministrativa nel Piemonte Sabaudo (XVI-XVIII sec.) (Anna Guarducci) Egidio Dansero, Davide Marino, Giampiero Mazzocchi e Yota Nicolarea, a cura di,&nbsp;Lo spazio delle politiche locali del cibo: temi, esperienze e prospettive (Chiara Spadaro) Giorgio Osti, Elena Jachia, a cura di, AttivAree. Un disegno di rinascita delle aree interne (Raffaella Coletti) Lucilla Barchetta, La rivolta del verde. Nature e rovine a Torino (Alberto Vanolo) &nbsp; Per leggere i contributi integralmente, cliccare sul quadratino in alto denominato "PDF".
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Palmieri, Giuseppe, Maria Colombino, Milena Casula, Amelia Lissia, Gerardo Botti, Giosuè Scognamiglio, Corrado Caraco, et al. "Mutation analysis of mucosal and cutaneous melanomas during progression." Journal of Clinical Oncology 35, no. 15_suppl (May 20, 2017): e21047-e21047. http://dx.doi.org/10.1200/jco.2017.35.15_suppl.e21047.

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e21047 Background: The prevalence of mutations in driver genes during progression in cutaneous and mucosal melanomas remains inconclusive. We investigated the prevalence and distribution of mutations in main candidate genes involved in melanomagenesis among different melanoma tissues using a next-generation sequencing (NGS) approach. Methods: Forty-eight tumor samples from 36 patients with mucosal melanoma (MM) and fifty-two tumor samples from 34 patients with cutaneous melanoma (CM) were collected, after obtaining patients’ written informed consent for tissue sampling. Genomic DNA was isolated from macrodissected tumor tissues containing at least 80% neoplastic cells and analyzed for mutations in 25 most common melanoma-associated oncogenes and tumor suppressor genes, using the IMI Diagnostic Melanoma Panel on the Ion Torrent platform (Life Technologies, USA). Results: A total of 100 tumor tissues from 70 melanoma patients were analyzed. BRAF mutations were detected in 21/34 (62%) CM patients and 13/36 (36%) mm patients. The second most prevalent mutations were found in K-/N-RAS (6/34; 18%) and cKIT (6/36; 17%) genes among CM and mm patients, respectively. No concomitant mutations of BRAF, RAS, and cKIT genes were detected. Among others, mutations were more frequently found in CCND1 (20%), ARID2 (16%), and NF1(12%) genes, considering the entire series of patients. Vast majority of patients who had paired samples of primary and secondary melanomas showed consistent mutation patterns between primary tumors and metastatic lesions. Similar frequencies of mutations in driver genes were seen across metastatic sites. Conclusions: In the era of targeted therapies, assessment of the spectrum and distribution of mutations in main molecular targets among patients with melanoma is needed. Our findings about the prevalence of mutations in driver genes in paired tumor lesions from patients with cutaneous and mucosal melanoma may be useful in the management of such diseases. The Italian Melanoma Intergroup (IMI) includes the following additional members who participated as investigators in this study: Mario Mandalà, Paola Queirolo, Ignazio Stanganelli, Vanna Chiarion Sileni, Pietro Quaglino, Anna Maria Di Giacomo.
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Bettiol, A., M. L. Urban, F. Bello, D. Fiori, I. Mattioli, G. Lopalco, F. Iannone, et al. "POS0246 SEQUENTIAL RITUXIMAB AND MEPOLIZUMAB IN EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS." Annals of the Rheumatic Diseases 81, Suppl 1 (May 23, 2022): 362.2–363. http://dx.doi.org/10.1136/annrheumdis-2022-eular.4320.

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BackgroundRituximab (RTX) is an effective remission-induction treatment in ANCA-associated vasculitides (AAVs). Some reports have suggested that it might be effective also in Eosinophilic Granulomatosis with Polyangiitis (EGPA), to induce and maintain remission of vasculitic manifestations [1,2]. However, its effects for preventing respiratory relapses seem to be poor. Mepolizumab (Mepo) (both 100 and 300mg/month) is effective in improving respiratory manifestations and lung function, while partially controlling also systemic activity [3,4]. Isolated case reports further indicate that the sequential therapy with RTX and Mepo might be effective [5-7].ObjectivesThe study aimed to investigate the efficacy and safety of a therapeutic regimen based on sequential RTX and Mepo for the control of EGPA.MethodsA multicenter, retrospective, cohort study was conducted on adult patients diagnosed with EGPA according to the ACR classification criteria [8] or MIRRA trial criteria [3]. Only patients who received induction therapy with RTX (any dosage), and subsequent treatment with Mepo (100-300 mg/4 weeks) within 12 months from last RTX administration were included. Patients receiving other induction therapies between RTX and Mepo were excluded. The effectiveness of sequential RTX and Mepo was assessed in terms of disease activity (by the Birmingham Vasculitis Activity Score, BVAS) and daily corticosteroid dosage. Safety data were also collected.ResultsThirty-four EGPA patients treated with sequential RTX and Mepo were included (59% females, median age of 51 years (IQR 40-58); 41% ANCA positive).In most cases (26/34; 76%), RTX was started at the dosage of 1g q2w, and all except two patients had active disease at time of RTX beginning [median BVAS of 9 (IQR 6-14)]. Specifically, most patients started RTX for the control of systemic manifestations (19/34; 56%), or of both systemic and respiratory symptoms (11/34; 32%). All except one patient were receiving oral corticosteroids, at a median dosage of 25 mg/day (10-38).Mepo was started after a median of 14 months (6-23) from RTX initiation and after a median of 5 months (IQR 3-11) from the last RTX administration. Mepo was used at the dosage of 100mg/4 weeks in 32/34 (94%), mostly for the control of respiratory manifestations (25/34, 74%). At the time of starting Mepo, the median BVAS was 4 (2-8), and median prednisolone dose 10 mg/day (7-15). After a median follow-up of 28 months (IQR 23-33) from starting Mepo, the median BVAS decreased to 1.5 (IQR 0-4) and the median corticosteroid dosage to 5 mg/day (2.5-5), with 7/34 (21%) patients being off steroids. At last follow-up, most patients were off-RTX (28/34), typically due to stable disease remission (20/34; 59%).Both RTX and Mepo were well-tolerated; 5 patients had adverse events on RTX (none serious), and 5 on Mepo (including one serious infection).ConclusionSequential use of RTX and Mepo seems to be effective for remission induction and maintenance in EGPA.References[1]Emmi, Ann Rheum Dis, 2018[2]Teixeira, RMD Open, 2019 3. Wechsler, NEJM, 2017[4]Bettiol, Arthritis Rheumatol, 2021[5]Shiroshita, Respir Med Case Rep, 2018[6]Higashitani, Mod Rheumatol Case Rep, 2021[7]Afiari, Cureus 2020[8]Masi, Arthritis Rheum, 1990Table 1.Effectiveness of sequential RTX and Mepo in the 34 patients included in the studyRTX beginningMepo beginningLast follow-upMedian time elapsed (IQR)-14 months (6-23) from RTX beginning28 months (23-33) from Mepo beginningDosage1g q2w (26/34);100mg/4 weeks (32/34)6 patients off Mepo; 28 patients off RTX375mg/m2 for 4 weeks (8/34)300mg/4 weeks (2/34)Reason for treatment beginning (manifestations)Systemic (19/34);Respiratory (25/34);-Systemic + respiratory (11/34);Systemic (4/34);Only respiratory (3/34);Remission maintenance (5/34)Other (1/34)BVAS (median, IQR)9 (6-14)4 (2-8)1.5 (0-4)Prednisolone dosage (median, IQR), mg/day25 (10-38)10 (7-15)5 (2.5-5)Disclosure of InterestsAlessandra Bettiol: None declared, Maria Letizia Urban: None declared, Federica Bello: None declared, Davide Fiori: None declared, Irene Mattioli: None declared, Giuseppe Lopalco: None declared, Florenzo Iannone: None declared, Allyson Egan: None declared, Luca Moroni: None declared, Lorenzo Dagna Consultant of: Consultation honoraria from GSK outside the current work, Marco Caminati: None declared, Simone Negrini: None declared, Paolo Cameli: None declared, Marco Folci: None declared, Paola Toniati: None declared, Roberto Padoan: None declared, Oliver Flossmann: None declared, Roser Solans-Laqué: None declared, Laura Losappio: None declared, Jan Schroeder Consultant of: Advisory Board fees from AstraZeneca and GSK, Marc André: None declared, Laura Moi: None declared, paola parronchi Consultant of: Consultation honoraria from GSK and Novartis, Fabrizio Conti: None declared, Savino Sciascia: None declared, David Jayne Consultant of: Consultant for Astra-Zeneca, Aurinia, BMS, Boehringer-Ingelheim, Chemocentryx, Chugai, CSL, GSK, Infla-RX, Janssen, Novartis, Roche/Genentech, Takeda and Vifor, Augusto Vaglio Consultant of: Consultation honoraria from GSK outside the current work, Giacomo Emmi Consultant of: Consultation honoraria from GSK outside the current work
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Faig, Kenneth W. "Discussion on “Manual and Automated Procedures for Compiling a Very Large Sample of Centenarian Pedigrees,” by Giacomo Nebbia, Lisa Nussbaum, Annie Helmkamp, Stacy Anderson, Thomas Perls, and Paola Sebastiani, Volume 22(4)." North American Actuarial Journal 24, no. 3 (September 6, 2019): 488–90. http://dx.doi.org/10.1080/10920277.2019.1625790.

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Fredi, M., I. Cavazzana, A. Ceribelli, M. G. Lazzaroni, S. Barsotti, M. Benucci, L. Cavagna, et al. "FRI0239 ANTI-NXP2 ANTIBODIES: CLINICAL AND SEROLOGICAL ASSOCIATIONS IN A MULTICENTRIC ITALIAN STUDY." Annals of the Rheumatic Diseases 79, Suppl 1 (June 2020): 703.1–704. http://dx.doi.org/10.1136/annrheumdis-2020-eular.1384.

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Background:anti-NXP2 antibodies is considered a serological marker of dermatomyositis (DM), with calcinosis, severe myositis and, in some series, cancer. Historically, these associations have been detected with immunoprecipitation (IP), but in the last few years commercial lineblot (LB) assay have been released.Objectives:to analyze the clinical features associated to anti-NXP2 antibodies, including the onset of concomitant cancers, both with LB and homemade IPMethods:clinical and serological data from medical charts of 213 patients with a diagnosis of inflammatory miosidites without anti-NXP2 (NXP2-), followed-up by two third-level Centers, and 61 anti-NXP2+ patients from 10 Rheumatological centers were analyzed. Anti-myositis specific (MSA) and anti-myositis associated antibodies (MAA) were detected in single centers by LB (Euroimmun Autoimmune Inflammatory Myopathies 16 antigens). Anti-NXP2 was confirmed by protein and RNA IP, as previously described (1)Results:clinical diagnosis of anti-NXP2+ positive with LB were 42 DM, 11 PM, inclusion body myositis (IBM) 4, necrotizing myositis and overlap (OM) 1 each. Anti-NXP2+ showed a lower age at onset (p<0.0001) more frequent diagnosis of DM (68.8%vs30%,OR5.2) and IBM (6.5%vs0.49%,OR14.8), typical skin manifestations, myositis (93%vs79% OR3.3), concomitant presence of another MSA (12.7%vs2%, OR6.41) and lower rate of features associated with OM or anti-synthetase syndrome. Serum from 49 NXP2+ was available and IP analysis was made with the confirmation of NXP2 in 31 sera (63.2%) with the following diagnosis: DM 27 cases, PM 3, IBM 1. Whilst the majority of the associations were confirmed comparing NXP2LB+/IP+ with the IIM NXP2-, some peculiar associations were found significant only for the double positive patients: dysphagia (53%vs 30%,OR 2.56) and calcinosis (22%vs6.5% OR4) whereas IBM diagnosis and the presence of concomitant MSA antibodies were lost. Survival time from cancer onset is shown in figure.IP did not confirmed anti-NXP2 antibodies in 18 sera: in 4 cases at least one MSA/MAA was identified by IP; these 18 patients did not show differences when compared with 213 anti-NXP2-.Conclusion:Protein IP confirmed anti-NXP2 antibodies in 63% of LB+ sera. Double positive cases showed more typical DM features and rarely occurred in IIM not DM. Anti-NXP2 positivity by LB should be confirmed by other methods in order to correctly diagnose and characterize IIM patients.References:[1]Arthritis Res Ther 2012,30;14:R97Acknowledgments:Forum Italiano per la Ricerca Malattie Autoimmuni (FIRMA)Disclosure of Interests:Micaela Fredi: None declared, Ilaria Cavazzana: None declared, Angela Ceribelli: None declared, Maria Grazia Lazzaroni: None declared, Simone Barsotti: None declared, Maurizio Benucci: None declared, Lorenzo Cavagna: None declared, Ludovico De Stefano: None declared, Andrea Doria Consultant of: GSK, Pfizer, Abbvie, Novartis, Ely Lilly, Speakers bureau: UCB pharma, GSK, Pfizer, Janssen, Abbvie, Novartis, Ely Lilly, BMS, Giacomo Emmi: None declared, Marco Fornaro: None declared, Federica Furini: None declared, Roberto Gerli: None declared, Maria Grazia Giudizi: None declared, Marcello Govoni: None declared, Anna Ghirardello: None declared, Luca Iaccarino Speakers bureau: GSK, Pfizer, Janssen, Novartis, Florenzo Iannone Consultant of: Speaker and consulting fees from AbbVie, Eli Lilly, Novartis, Pfizer, Roche, Sanofi, UCB, MSD, Speakers bureau: Speaker and consulting fees from AbbVie, Eli Lilly, Novartis, Pfizer, Roche, Sanofi, UCB, MSD, Maria Infantino: None declared, Alessandro Mathieu: None declared, Emiliano Marasco: None declared, Paola Migliorini: None declared, Boaz Palterer: None declared, paola parronchi: None declared, Matteo Piga: None declared, Federico Pratesi: None declared, Antonella Radice: None declared, Carlo Selmi: None declared, Valeria Riccieri: None declared, Marilin Tampoia: None declared, Giovanni Zanframundo: None declared, Angela Tincani: None declared, Franco Franceschini: None declared
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Citarella, Armand O. "Le Pergamene Degli Archivi Di Bergamo, a. 740-1000.Maria Luisa Bosco , Patrizia Cancian , Donatella Frioli , Gilda Mantovani , Claudio Leonardi , Mariarosa Cortesi , Gian Giacomo Fissore , Paolo Sambin , Mariarosa Cortesi." Speculum 67, no. 1 (January 1992): 118–19. http://dx.doi.org/10.2307/2863758.

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TCHAROS, STEFANIE. "The Serenata in Early 18th––Century Rome: Sight, Sound, Ritual, and the Signification of Meaning." Journal of Musicology 23, no. 4 (2006): 528–68. http://dx.doi.org/10.1525/jm.2006.23.4.528.

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ABSTRACT By the turn of the 18th century serenatas performed in Rome's urban squares as political or dynastic propaganda were a well-established ritual. In this public forum the effect of sound produced by large instrumental forces was a central feature, yet the serenata was a complex performance in which music was but one element in a series of other displays. Though undoubtedly an important part of the serenata, music's role in this multifaceted performance and its effect on audiences remain unclear. Part of that ambiguity stems from the serenata's ability to service both public and private consumption. Patrons exploited this dual nature, using the more spectacular elements of the serenata to influence the public at large, while also relying on other elements (primarily word and sound) to sway elite audiences. In the context of Rome and its dynastic politics, Giacomo Buonaccorsi's and Pietro Paolo Bencini's serenata Le gare festive in applauso alla Real Casa di Francia (1704) demonstrates how the serenata's dichotomous structures and multiplicity of meaning were deeply linked to larger cultural frameworks and social tensions——in this case, brewing over the War of Spanish Succession. The serenata was both a musical work and a performed event effectively shaped by the genre's ritual practice and by history and politics in late 17th- and early 18th-century Rome. The logic of the serenata's ritual practice shows significant correspondences to the genre's narrative strategies. Within the serenata, allegory served as the catalyst to express layers of meaning to diverse audiences. But more than that, allegory provided the means by which music was contained and its delivery marked. For public audiences, sound was as much visual as it was aural, an immediate and palpable special effect. For privileged listeners, music required reflection as to how spectacular effects acquired deeper meaning when anchored in the significance of the poetic text. Thus music in the serenata was not merely an element in a multifaceted performance but was multidimensional in itself, uniquely straddling both sides of the public/private divide.
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Evelpidou, Niki, and Paolo Pirazzoli. "Comment on «Tidal notches in the Mediterranean Sea: A comprehensive analysis» by Fabrizio Antonioli, Valeria Lo Presti, Alessio Rovere, Luigi Ferranti, Marco Anzidei, Stefano Furlani, Giuseppe Mastronuzzi, Paolo E. Orru, Giovanni Scicchitano, Gianmaria Sannino, Cecilia R. Spampinato, Rossella Pagliarulo, Giacomo Deiana, Eleonora de Sabata, Paolo Sansò, Matteo Vacchi and Antonio Vecchio. Quaternary Science Reviews 119 (2015) 66–84." Quaternary Science Reviews 131 (January 2016): 237–38. http://dx.doi.org/10.1016/j.quascirev.2015.08.023.

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Campochiaro, C., A. Tomelleri, S. Sartorelli, G. De Luca, C. Sembenini, G. Cavalli, P. Mapelli, et al. "AB0466 EFFICACY AND SAFETY OF INFLIXIMAB-BIOSIMILAR IN TAKAYASU ARTERITIS (TAKASIM): A MONOCENTRIC, OBSERVATIONAL, PROSPECTIVE, OPEN-LABEL STUDY." Annals of the Rheumatic Diseases 79, Suppl 1 (June 2020): 1531.2–1531. http://dx.doi.org/10.1136/annrheumdis-2020-eular.3250.

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Background:Treatment of Takayasu arteritis (TA) is mainly based on steroids, but, in approximately 50% of patients, disease-modifying antirheumatic drugs(DMARDs) are required.Objectives:To evaluate efficacy and safety of IFX-biosimilar in TA patients.Methods:Both bDMARD-naïve and IFX-O treated patients were eligible. Primary endpoint was the number of patients with active disease as assessed by magnetic resonance angiography(MRA), 18FDG PET/CT, ITAS2010 and ITAS-ESR/CRP at month 6. Secondary endpoints were safety and tolerability, number of patients with active disease at month 12, quality of life. Non-parametric statistic tests were used.Results:Twenty-three patients(21 female) were recruited. At baseline, mean age was 43.8±14.4 years and mean disease duration 95.5±61.3months. Two patients were IFX-O-naïve. Mean time on IFX-O was 51.5±37.9 months. Four patients had been previously treated with other biologics(tocilizumab, 3; adalimumab, 1). Twenty-one patients(91.3%) were on concomitant steroids(mean dose, 4.8±2.0 mg daily) and 82% on concomitant csDMARDs, kept unchanged throughout the study. At baseline, 4 patients(17%) were classified as active according to ITAS2010, ITAS-ESR, and ITAS-CRP; mean HAQ was 3.48±5.26. Over the study period two patients dropped out the study because of poor disease control (1 at month 3 and 1 at month 6). PET/CT was not available for one patient who was on lactation during the study period and 1 patient refused to undergo imaging re-evaluation. At month 6, MRA was available for 21 patients: it was stable in 11(52%), improved in 5(24%), worsened in 5(24%). PET/CT was available for 20 patients: it was negative in 12(65%), improved although still positive in 3(16%), stable in 1(5%), worsened in 3(16%). At month 6, among 22 patients, 4(18%) were clinically active according to ITAS2010, ITAS-ESR and ITAS-CRP; mean steroid dose was significantly lower compared to baseline(4.2±2.0 mg daily, p=0.009); HAQ didn’t significantly change(mean, 3.35±6.59, p=0.357). At month 12, MRA was available for 20 patients. It was stable in 9 patients(45%), improved in 8 (40%), worsened in 3(15%). PET/CT was available for 19 patients: it was negative in 14(74%), improved although still positive in 2(10%), stable in 3(10%), worsened in 1(5%). At month 12, 3 patients(14%) were active according to ITAS2010 and 2(9%) according to ITAS-ESR and ITAS-CRP; mean steroid dose was significantly lower compared to baseline(3.4±2.56 mg daily, p=0.034); HAQ didn’t significantly change(mean, 3.84±6.34, p=0.919). Nine patients(39%) experienced low-grade side effects related to TNFα-blockade (6, herpes reactivation; 3, urinary tract infection; 1 gastroenteritis). No IFX-B therapy modification was required.Conclusion:Our study suggests that IFX-B is effective and safe both in IFX-O switch and IFX-O naïve TA patients.Disclosure of Interests: :Corrado Campochiaro Speakers bureau: Novartis, Pfizer, Roche, GSK, SOBI, Alessandro Tomelleri: None declared, Silvia Sartorelli: None declared, Giacomo De Luca Speakers bureau: SOBI, Novartis, Celgene, Pfizer, MSD, Camilla Sembenini: None declared, Giulio Cavalli Speakers bureau: SOBI, Novartis, Pfizer, Paola Mapelli: None declared, Maria Picchio: None declared, Maurizio Papa: None declared, Elena Baldissera Speakers bureau: Novartis, Pfizer, Roche, Alpha Sigma, Sanofi, Lorenzo Dagna Grant/research support from: Abbvie, BMS, Celgene, Janssen, MSD, Mundipharma Pharmaceuticals, Novartis, Pfizer, Roche, SG, SOBI, Consultant of: Abbvie, Amgen, Biogen, BMS, Celltrion, Novartis, Pfizer, Roche, SG, and SOBI
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Gatto, M., R. Depascale, A. Tincani, G. Emmi, S. Scarpato, F. Conti, M. Govoni, et al. "AB0441 PREDICTORS OF CLASI RESPONSE OVER TIME IN A MULTICENTRIC REAL LIFE COHORT OF SLE PATIENTS TREATED WITH BELIMUMAB." Annals of the Rheumatic Diseases 81, Suppl 1 (May 23, 2022): 1348.2–1349. http://dx.doi.org/10.1136/annrheumdis-2022-eular.3867.

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BackgroundOver 80% of patients affected with SLE experience skin involvement. The anti-BLyS drug belimumab was shown effective in ameliorating mucocutaneous SLE manifestations in clinical trials and real-life studies. Cutaneous response is quantified through the CLASI (cutaneous lupus erythematosus area and severity index). Clinically relevant improvements are defined as decreases of ≥50% (CLASI50) or 70% (CLASI70) from baseline values.ObjectivesTo assess rates and predictors of CLASI50 and CLASI70 in the Berliss multicentric SLE cohort1 of patients treated with belimumab.MethodsBaseline and ongoing features of patients with baseline active skin involvement (CLASI>0) were assessed in relationship to the chosen outcomes CLASI50 and CLASI70 at 24 and 52 weeks. A subanalysis on patients with CLASI≥5 was as well conducted. Logistic regression was employed to identify predictors of response.Results172 patients displayed skin involvement at baseline (CLASI>0). Of those, 124 displayed at least a 12-month-follow-up and were included in the analysis. Seventy-seven (62.1%) patients reached CLASI50 at 24 weeks and 91 (77.8%) at 52 weeks; 87 (70.2%) reached CLASI70 at 24 and 99 (79.8%) at 52 weeks. Baseline predictors of CLASI50 at 24 weeks were CLASI-damage (CLASI-d) (OR [95%CI], p; 0.79 [0.65-0.98] 0.03) and disease duration (0.93[0.86-0.99], 0.011). No baseline predictors of CLASI70 at 24 weeks emerged, however having achieved a CLASI50 response at 24 weeks portended CLASI50 and 70 response through week 52 (p<0.01, Table 1). In the subgroup of patients with CLASI≥5, longer disease and increased CLASI-d at baseline confirmed as negative predictors of CLASI50 at 24 weeks. In this subset, use of antimalarials and active smoking at baseline predicted CLASI70 at 24 weeks (Table 1).Table 1.Predictors of CLASI-A Response at Week 24 and 52 by Baseline CLASI-A at 50% and 70% Response ThresholdsTimepointOutcomeVariableOR[95%CI] pCLASI>024 weeksCLASI50CLASI-d0.79 [0.65-0.98] 0.030Disease duration0.93[0.86-0.99], 0.011CLASI70CLASI-d0.93 [0.74-1.16], 0.51Disease duration0.97 [0.97-1.02], 0.1852 weeksCLASI50CLASI50 at 24 weeks14.3[4.88-44.42], <0.001CLASI70CLASI50 at 24 weeks6.22 [2.00-19.34], 0.002CLASI≥524 weeksCLASI50CLASI-d0.72 [0.53-0.98], 0.037Disease duration0.93 [0.66-1.00], 0.071CLASI70Antimalarials6.61 [1.20-36.29] 0.032Smoking0.15 [0.03-0.83], 0.03452 weeksCLASI50CLASI50 at 24 weeks22.0 [2.47-196.05], 0.006CLASI70CLASI50 at 24 weeks1.24 [0.06-25.08], 0.88CLASI, cutaneous lupus erythematosus area and severity index; CLASI-d, CLASI damage; CLASI50 and CLASI70: decrease ≥50% or ≥70% in CLASI from baseline. OR and 95%CIs are estimated using a logistic regression model with stratification factors as covariates (SLEDAI-2K at baseline, baseline prednisone dosage).ConclusionEarlier use of belimumab favors achievement of skin response among SLE patients and attainment of a prompt response predicts further response. Use of antimalarials reinforces while smoking hampers a more profound CLASI improvement over time.References:[1]Gatto M, et al. Arthritis Rheumatol. 2020 Aug;72(8):1314-1324Disclosure of InterestsMariele Gatto Speakers bureau: GSK, Grant/research support from: GSK, Roberto Depascale: None declared, Angela Tincani: None declared, Giacomo Emmi: None declared, Salvatore Scarpato: None declared, Fabrizio Conti: None declared, Marcello Govoni: None declared, Marta Mosca: None declared, Maria Gerosa: None declared, Enrica Bozzolo: None declared, Valentina Canti: None declared, Armando Gabrielli: None declared, Elisa Gremese: None declared, Salvatore De Vita: None declared, francesco ciccia: None declared, Carlo Salvarani: None declared, Maurizio Rossini: None declared, Paola Faggioli: None declared, Antonella Laria: None declared, Amato De Paulis: None declared, Roberto Gerli: None declared, Enrico Brunetta: None declared, Alessandro Mathieu: None declared, Carlo Selmi: None declared, Rossella De Angelis: None declared, Simone Negrini: None declared, Margherita Zen: None declared, Andrea Doria Speakers bureau: GSK, Eli Lilly, Roche, Grant/research support from: GSK, Luca Iaccarino Speakers bureau: GSK, Grant/research support from: GSK
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Santaniello, A., C. Bellocchi, L. Bettolini, M. Cassavia, G. Montanelli, A. Severino, M. Caronni, et al. "OP0009 DERIVATION AND VALIDATION OF THE SCLERODERMA LUNG 3-STAGE INDEX (SL3SI), A NEW FUNCTIONAL INDEX FOR INTERSTITIAL LUNG DISEASE WITH PROGNOSTIC IMPLICATIONS." Annals of the Rheumatic Diseases 79, Suppl 1 (June 2020): 7.2–7. http://dx.doi.org/10.1136/annrheumdis-2020-eular.5788.

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Background:The staging of interstitial lung disease (ILD) is important to monitor disease progression and for prognostication. A disease severity scale of Systemic Sclerosis (SSc)-related lung disease has long been proposed (i.e. Medsger’s severity scale). This scale was mostly developed by discussion and consensus and stage thresholds were not computed by a data-driven approach. Hidden Markov models (HMM) are methods to estimate population quantities for chronic diseases with a staged interpretation which are diagnosed by markers measured at irregular intervals.Objectives:To build a SSc-ILD specific disease severity scale with prognostic relevance via HMM modeling.Methods:A total of 358 SSc patients at risk for or with ILD were enrolled in a discovery (207 cases, Milan1) and in a validation (151 cases, Milan2, Pavia and Rome) cohort. Patients were included if satisfied the following criteria: 1) Diagnosis of SSc according to the EULAR/ACR 2013 criteria, 2) absence of anticentromere antibodies, 3) dcSSc subset or 4) other subsets with either 4a) ILD-related antibodies (Scl70, PmScl, Ku) or 4b) evidence of ILD on HRCT, 5) disease duration < 5 years at the time of the first pulmonary function test (PFT). Serial PFTs were retrieved and the time up to the last available visit -if the patient alive-, or to death due to pulmonary complications, was recorded. HMM were used to estimate the threshold of a 3-stage model (SL3SI, Scleroderma Lung 3-Stage Index) based on PFT functional values (normal/mild, moderate, severe involvement) in the discovery cohort. Survival estimates of the SL3SI model were compared to Medsger’s severity classes estimates and their predictive capability evaluated via the explained residual variation (R2) of prediction errors (the higher the better). One-hundred random replicates were generated to simulate the prediction effort in patients with different disease duration and lung severity.Results:Patients characteristics are summarized in the Table. Fifteen-years survival estimates for Mesdger’s classes in the discovery set were: normal=0.88, mild=0.86, moderate=0.84 and severe=0.71. The SL3SI was defined by the following thresholds: normal/mild, FVC and DLco >=75%; moderate FVC or DLco 74-55%; severe, FVC or DLco <55%. SL3SI 15-yrs survival estimates were: normal/mild=0.89, moderate=0.82 and severe=0.63. Prediction analysis showed a higher R2values at 15 yrs for the SL3SI compared to Medsger’s classes, providing evidence for a better predictive capability of the former (discovery: 0.31 vs 0.25; validation: 0.28 vs 0.19).Conclusion:The SL3SI, a simplified 3-stage functional model of SSc-ILD, yields better survival estimates and long-term prognostic information than Medsger’s classes. Its reproducibility and ease of use make it a useful tool for the functional and prognostic evaluation of SSc patients at risk for or with ILD.Table:VariablesDiscovery (n=207)Replication (n=151)DcSSc62 (30%)98 (64%)Age at first PFR48.6±1249.1±14.4Disease duration at first PFR1.7±1.61.3±2.4FVC90.5±18.191.1±20.2DLco70.7±19.861.3±20.1ILD on HRCT179 (86%)125 (80%)Scl70157 (76%)153 (78%)SSA63 (30%)32 (21%)n of visits38571473Follow-up time, yrs11±5.610.6±5.7Deaths27 (13%)23 (15%)Disclosure of Interests:Alessandro Santaniello: None declared, Chiara Bellocchi: None declared, Luca Bettolini: None declared, Marcello Cassavia: None declared, Gaia Montanelli: None declared, Adriana Severino: None declared, Monica Caronni: None declared, Corrado Campochiaro Speakers bureau: Novartis, Pfizer, Roche, GSK, SOBI, Enrico De Lorenzis: None declared, Gerlando Natalello: None declared, Paolo Delvino: None declared, Claudio Tirelli: None declared, Lorenzo Cavagna: None declared, Giacomo De Luca Speakers bureau: SOBI, Novartis, Celgene, Pfizer, MSD, Silvia Laura Bosello: None declared, Lorenzo Beretta Grant/research support from: Pfizer
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Campochiaro, C., G. De Luca, M. G. Lazzaroni, G. Armentaro, A. Spinella, B. Vigone, B. Ruaro, et al. "POS0890 NINTEDANIB REAL-LIFE EFFICACY AND SAFETY IN SYSTEMIC SCLEROSIS (SSc)-INTERTISTIAL LUNG DISEASE (ILD): AN ITALIAN MULTICENTRE PRELIMINARY STUDY." Annals of the Rheumatic Diseases 81, Suppl 1 (May 23, 2022): 741.2–742. http://dx.doi.org/10.1136/annrheumdis-2022-eular.3183.

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BackgroundNintedanib (NTD) has been approved for Systemic Sclerosis (SSc)-Interstitial Lung Disease (ILD) following the positive results of the SENSCIS trial.Objectivesto describe the efficacy and safety of NTD in SSc-ILD in a real-life setting.MethodsThe clinical data of SSc-ILD patients treated with NTD from 10 Italian SSc centres were retrospectively evaluated at baseline, 6 and 12 months: SSc clinical features, NTD tolerability, pulmonary function tests (PFTs) and modified Rodnan skin score (mRSS) were recorded.Results69 SSc-ILD patients (22 males [32%], mean age 60±12 years, disease onset 50±13 years, 4 [6%] anti-centromere, 53 [77%] anti-topoisomerase I, 3 [4%] anti-RNA-polimerase III) were identified. The vast majority (84%) was previously treated with immunosuppressants: 27 (39%) cyclophosphamide, 45 (65%) mycophenolate mofetil, 6 (9%) methotrexate, 9 (13%) azathioprine, 6 (9%) tocilizumab and 22 (32%) rituximab. In 11 (16%) patients, NTD was the first treatment for SSc-ILD. At baseline, 57 patients (83%) were on corticosteroids (mean daily prednisone dose 6±5 mg), 58 (84%) on immunosuppressants, 47 (68%) on mycophenolate mofetil, 14 (20%) on rituximab, 3 (4%) on tocilizumab, 2 on methotrexate (3%) and 1 (1%) on azathioprine. At baseline HRCT showed UIP pattern in 27 (39%) and NSIP pattern in 42 (61%) patients. The modifications of PFTs and mRSS over time are shown in Table 1. Since NTD introduction, gastro-intestinal (GI) side effects were recorded in 34 (49%) patients, with diarrhoea being the most common complaint (35%), followed by nausea/vomiting (23%) and weight loss (16%). In 21 (30%) patients, after a mean time of 2.6±3.4 months, NTD was maintained after dose adjustment. In 5 (7%) patients NTD was stopped after a median time of 5 (1-6) months due to subocclusion and persistent diarrhoea in 3 patients, untreatable nausea and vomiting in one patient and liver toxicity in 1 patient. During the follow-up after a median time of 10 (6 – 33) months, 4 patients died.Table 1.Pulmonary function tests and mRSS at baseline, 6 and 12 months in SSc-ILD on NTD.Baseline6 monthsP valueBaseline12 monthsP valueFVC (% predicted)64 ± 1865 ± 18 (33 pts)0.63870 ± 1969 ± 18 (20 pts)0.586TLC (% predicted)64 ± 1561 ± 14 (27 pts)0.15464 ± 1465 ± 18 (16 pts)0.944DLCO (% predicted)40 ± 1741 ± 18 (29 pts)0.66040 ± 1838 ± 18 (20 pts)0.304mRSS9 ± 68 ± 6 (26 pts)0.0027 ± 48 ± 6 (15 pts)0.334pts= patientsConclusionOur preliminary data confirm that in a real-life clinical scenario NTD, in combination with immunosuppressants, may stabilize PFT. However, despite the fact that GI side effects are frequent, they may be controlled with NTD dose adjustment thus retaining the drug in SSc-ILD patients. The NTD efficacy on skin involvement needs to be thoroughly evaluated on a larger SSc population.Disclosure of InterestsCorrado Campochiaro Speakers bureau: Boeboehringer ingelheim, Giacomo De Luca Speakers bureau: boehringer ingelheim, Maria Grazia Lazzaroni Grant/research support from: boehringer ingelheim, Giuseppe Armentaro: None declared, Amelia Spinella: None declared, Barbara Vigone: None declared, Barbara Ruaro: None declared, Anna Stanziola: None declared, Devis Benfaremo: None declared, Enrico De Lorenzis: None declared, Francesco Benvenuti: None declared, Silvia Laura Bosello Speakers bureau: boehringer ingelheim, Gianluca Moroncini: None declared, Giovanna Cuomo: None declared, Marco Confalonieri: None declared, Lorenzo Beretta: None declared, Elisabetta Zanatta: None declared, Dilia Giuggioli: None declared, Nicoletta Del Papa: None declared, Paolo Airò: None declared, Lorenzo Dagna: None declared, Marco Matucci-Cerinic Speakers bureau: boehringer ingelheim
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Bissell, L. A., D. Furst, S. Johnson, P. Hansen, E. Recalde, D. Khanna, and F. Del Galdo. "POS0868 THE DEVELOPMENT OF THE LINEAR CRISS; A CLINICAL AND PATIENT MEANINGFUL ANCHOR TO THE ACR-CRISS IN SCLERODERMA." Annals of the Rheumatic Diseases 81, Suppl 1 (May 23, 2022): 728. http://dx.doi.org/10.1136/annrheumdis-2022-eular.1896.

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BackgroundThe ACR Composite Response Index in Systemic Sclerosis (ACR-CRISS) is one of the first composite outcome measures in diffuse cutaneous Systemic sclerosis (dcSSc).1 It relies on validated clinical domains selected through a data-driven methodology; however, it only provides a probability of response and is unable to differentiate between patients who do not improve and who worsen respectively.ObjectivesTo improve the clinical interpretation of the ACR-CRISS by creating a continuous ranked score of clinically and patient meaningful changes of its individual measures.MethodsFollowing OmerACT guidelines for outcome measurement development, relevant stakeholders were identified from 5 continents, including 100 physicians with proven experience in managing patients with dcSSc and 100 patients with dcSSc who have participated in at least one clinical trial. An adaptive conjoint analysis survey based on the PAPRIKA method2 and implemented using 1000minds software was administered. Patients and doctors were asked to choose which of two hypothetical patients had a better or worse outcome according to Minimally Clinical Important Differences (MCID) in two domains at a time from FVC, HAQ-DI and mRSS and the presence of organ failure. These pairwise choices were analysed to rank and weight the MCIDs against each other. With patient and public involvement, utilising a ‘think aloud’ approach, a video tutorial was produced explaining the objectives and process of the adaptive survey to the participants.ResultsEighty rheumatologists and 80 patients with dcSSc completed the survey, which ran from June 2020 to January 2021. From the survey, relative weights for the 4 domains, reflecting their relative importance with respect to improving and worsening outcomes, were determined. A continuous composite ranked score reflecting the relative weighting of the individual outcome measures (Ranked Composite Important Difference, RCID) was developed accordingly (Table 1). The score ranges from -1 (worst possible outcome) to 1 (best possible outcome), in patients who experience no organ failure and do not meet any MCID in any of the 3 domains scoring 0.Table 1.The relative median weights of each of the core set measures within the better and worse outcome models, expressed as a score from -1 to 1Worsening weightsImprovement weightsDoctorsPatientsCombinedDoctorsPatientsCombinedOrgan failure-0.355-0.333-0.326N/AN/AN/AFVC-0.324-0.306-0.3160.4630.4520.451mRSS-0.205-0.229-0.2170.3200.3200.324HAQ-DI-0.114-0.115-0.1410.2000.2270.224ConclusionThis collaborative process using a novel, robust methodology and involving both rheumatologists and patients has created a clinically and patient meaningful composite score that can be used as an anchor to the ACR-CRISS, or other clinical outcomes. Performance against the ACR-CRISS and revised CRISS in randomised controlled trials and in observational cohorts will determine the clinical value of the RCID.References[1]Khanna D, et al. A&R 2016;68:299–311[2]Hansen P, Ombler F. Multi-Criteria Decis. Anal 2008;15:87–107AcknowledgementsDK and FDG are recognised as joint senior authors. The authors acknowledge the doctors and patients involved in the Linear Criss working group: Giuseppina Abignano, Paolo Airò, Dina-Marie Aiuto, Yannick Allanore, Shervin Assassi, Jérôme Avouac, Gianluca Bagnato, Alexandra Balbir-Gurman, Silvia Bellando Randone,Lorenzo Beretta, Elana Bernstein, Silvia Laura Bosello, Yolanda Braun Moscovici, Katrina Brown, Maya Buch, Corrado Campochiaro, Patricia Carreira, Lorinda Chung, Julia Coakes, Mary Cox, Giovanna Cuomo, Maurizio Cutolo, Laszlo Czirjak, Lorenzo Dagna, Giacomo De Luca, Nicoletta Del Papa, Christopher Denton, Emma Derrett-Smith, Robyn Domsic, Raluca-Bianca Dumitru, Victoria Flower, Ivan Foeldvari, Armando Gabrielli, Yasir Ghaffar, Roberto Giacomelli, Dilia Giuggioli, Daisy Gonzalez, Jessica Gordon, Yvonne Gouldstone, Marie Hudson, Francesca Ingegnoli, Lorraine Jackson, Sergio Jimenez, Terrance Johnson, Bashar Kahaleh, Robin King, Otylia Kowal-Bielecka, Masataka Kuwana, Maria Lazzaroni, Alain Lescoat, Takashi Matsushita, Marco Matucci Cerinic, Maureen Mayes, Thomas Medsger, Francesca Menegazzi, Tünde Minier, Mandana Nikpour, Chris O’Hora, Emese Paári-Molnár, John Pauling, Jose Antonio Pereira da Silva, Mercè Piñero Vegas, Janet Pope, Susanna Proudman, Ismaila Rafiq, Valeria Riccieri, Tatiana Sofia Rodriguez-Reyna, Tânia Santiago, James Seibold, Richard Silver, Robert Spiera, Tracy Stafford, Virginia Steen, Yossra Atef Suliman. Madelon Vonk, Ian Wright.Disclosure of InterestsLesley-Anne Bissell Speakers bureau: UCB, Abbvie, Galapagos, Daniel Furst: None declared, Sindhu Johnson: None declared, Paul Hansen: None declared, Esmeralda Recalde: None declared, Dinesh Khanna: None declared, Francesco Del Galdo Speakers bureau: Abbvie, AstraZeneca, Boehringer-Ingelheim, Capella Biosciences, Chemomab LTD, Janssen, Kymab LTD, Mitsubishi-Tanabe., Consultant of: Abbvie, AstraZeneca, Boehringer-Ingelheim, Capella Biosciences, Chemomab LTD, Janssen, Kymab LTD, Mitsubishi-Tanabe., Grant/research support from: Abbvie, AstraZeneca, Boehringer-Ingelheim, Capella Biosciences, Chemomab LTD, Janssen, Kymab LTD, Mitsubishi-Tanabe.
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Crisafulli, F., A. Vitale, C. Gaggiano, L. Dagna, G. Cavalli, R. Cimaz, O. Viapiana, et al. "OP0093 RETENTION RATE OF IL-1 INHIBITORS IN PATIENTS WITH SCHNITZLER’S SYNDROME." Annals of the Rheumatic Diseases 80, Suppl 1 (May 19, 2021): 51–52. http://dx.doi.org/10.1136/annrheumdis-2021-eular.3071.

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Background:Schnitzler’s syndrome is an autoinflammatory disease characterized by monoclonal gammopathy and recurrent episodes of urticaria accompanied by clinical and laboratory signs of acute inflammation. Interleukin (IL)-1 inhibitors proved to be useful in the treatment, but data on long-term safety and efficacy of these agents are sparse.Objectives:To evaluate the retention rate of IL-1 inhibitors in patients with Schnitzler’s Syndrome.Methods:Retrospective analysis of an Italian multicenter cohort (9 Centers). All patients fulfilled Strasbourg diagnostic criteria. Data are expressed as median [IQR].Results:We identified 15 patients (8 females, 7 males) who received a total of 24 treatment courses with IL-1 inhibitor treatment (16 anakinra and 8 canakinumab) between January 2001 and December 2019, with a median treatment duration of 19 months [8.5-51.3]. Median age at diagnosis was 64.0 years [56.0-72.5] and median follow up was 5.0 years [2.0-8.0]. Before the biological treatment, all patients were treated with corticosteroids and 11 with at least one conventional synthetic disease-modifying antirheumatic drug (csDMARD): methotrexate (5), colchicine (5), cyclosporine (3), azathioprine (1), mycophenolate mofetil (1), cyclophosphamide (1).Fifteen patients received 16 courses of Anakinra, which was the 1st line biological treatment in 14 patients. Seven patients continued it with benefit, while 7 patients discontinued it: 3 for secondary inefficacy; 3 for adverse events (2 injection site reactions, 1 severe allergic reaction); 1 for secondary inefficacy and leukopenia. Anakinra was used as 2nd line treatment in 1 case (after tocilizumab failure); in 1 patient anakinra was resumed after temporary discontinuation and an attempt with infliximab. One patient died for multiple myeloma progression while on treatment with anakinra. The median duration of the courses with anakinra was 20.0 months [6.0-58.3].Seven patients received 8 courses of canakinumab (150 mg/8weeks in 5 cases and 150 mg/4weeks in 3). In 5 cases the drug was administered as 2nd line biological treatment (after anakinra failure) and in 2 cases as 3rd line treatment (1 after tocilizumab and anakinra failures and 1 after anakinra and adalimumab failure). In 1 patient, it was resumed after temporary discontinuation and an attempt with etanercept. One patient died while on treatment with canakinumab due to a presumably unrelated adverse event. The median duration of canakinumab treatment courses was 19.0 months [13.5-31.0].At last follow-up visit, all patients were on treatment with an IL-1 inhibitor: 8 with anakinra (7 at the dosage of 100 mg/day, 1 at the dosage of 200 mg/day) and 7 with canakinumab (2 at the dosage of 150 mg/8 weeks, 4 at the dosage of 150 mg/4 weeks and 1 at the dosage of 300 mg/4 weeks). Notably, in 3 patients the dosage of canakinumab was increased since the start of the treatment.Among 9 patients who were on treatment with prednisone at the start of the last IL-1 inhibitor, the prednisone median dose was 12.5 mg/day [10.0-18.8] while at the last follow-up visit it was 5.0 mg/day [0-7.5] (p= 0.02).The retention rate of IL-1 inhibitors was 73.4% [SE 9.4] at 1 year and 63.6% [SE 10.4] at 2 years (Figure 1a). There was no significant difference between the retention rate of anakinra (at 1 year: 67.0% [12.2]; at 2 years: 59.6% [12.9]) and canakinumab (at 1 year: 85.7% [13.2]; at 2 years 71.4% [17.1]) (log-rank test: p=0.41) (Figure 1b).Figure 1.a) Retention rate of IL-1 inhibitors (24 courses); b) Retention rate of canakinumab (8 courses) and anakinra (16 courses).Conclusion:In this multicentric cohort of patients affected by Schnitzler’s syndrome, the treatment with IL-1 inhibitors as 1st, 2nd or 3rd line biological treatment permitted a good disease control and corticosteroid reduction in patients who did not respond to csDMARDs and/or to prior other biological DMARDs. The optimal dosage of these drugs needs to be tailored for every patient.Acknowledgements:AIDA NetworkDisclosure of Interests:Francesca Crisafulli: None declared, Antonio Vitale: None declared, Carla Gaggiano: None declared, Lorenzo Dagna: None declared, Giulio Cavalli Speakers bureau: SOBI, Novartis, Paid instructor for: SOBI, Novartis, Consultant of: SOBI, Novartis, Rolando Cimaz: None declared, Ombretta Viapiana: None declared, Florenzo Iannone: None declared, Giuseppe Lopalco: None declared, Roberto Bortolotti: None declared, Masen Abdel Jaber: None declared, Carlomaurizio Montecucco: None declared, Sara Monti: None declared, Silvia Balduzzi: None declared, Giacomo Emmi: None declared, Paolo Airò: None declared, Franco Franceschini: None declared, Luca Cantarini Speakers bureau: SOBI, Novartis, Paid instructor for: SOBI, Grant/research support from: SOBI, Novartis, Micol Frassi: None declared
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Hoffmann-Vold, A. M., C. Brunborg, F. Tirelli, P. Carreira, N. Del Papa, A. Mekinian, M. Vonk, et al. "POS0054 THE IMPACT AND OUTCOME OF COVID-19 ON SYSTEMIC SCLEROSIS PATIENTS FROM THE EUROPEAN SCLERODERMA TRIAL AND RESEARCH GROUP (EUSTAR)." Annals of the Rheumatic Diseases 80, Suppl 1 (May 19, 2021): 232.2–233. http://dx.doi.org/10.1136/annrheumdis-2021-eular.3267.

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Background:Coronavirus disease-19 (COVID-19) has been a major clinical challenge worldwide. Sex, age and comorbidities have been associated with worse outcome in the general population. Systemic sclerosis (SSc) is a severe, autoimmune disease with frequent multi-organ involvement.Objectives:To assess the impact of COVID-19 and to determine factors associated with worse outcome in SSc patients from the European Scleroderma Trial and Research (EUSTAR) database.Methods:SSc patients from the EUSTAR database with COVID-19 were prospectively collected between 15.03.-31.12.2020. Two outcomes were chosen: (1) hospitalization; and (2) severe outcome defined as either non-invasive ventilation, mechanical ventilation/extracorporeal membrane oxygenation (ECMO) or death. General risk factors assessed were sex, age and number of comorbidities. SSc related risk factors were SSc subtype, autoantibodies, disease duration, SSc associated organ manifestations including interstitial lung disease (ILD), pulmonary arterial hypertension (PAH), cardiac, gastrointestinal (GI), and musculoskeletal involvement; digital ulcers (DU), CRP at last visit, renal disease (scleroderma renal crisis and SSc associated renal insufficiency), modified Rodnan skin score (mRSS) and immunosuppressive treatment. Descriptive statistics and logistic regression models were applied.Results:In total, 178 European SSc patients with COVID-19 were registered with a median observation time of 5.5 weeks (Table 1). 95 patients (53%) could recall SAR-Cov-2 contact, while 47 (26%) had no contact. 156 (88%) were symptomatic at COVID-19 onset with fever, cough, malaise and dyspnea being most prevalent. Over the disease course, 63 (36%) developed pneumonia. In total, 67/176 (38%) were hospitalized which were in 84% due to COVID-19. 41/170 (24%) had a severe outcome including 21 (12%) deaths. 128 (72%) recovered completely, while 14 (8%) complained of sequela, with 7 (50%) stating respiratory complications. Age, non-SSc comorbidities, presence of ILD, PAH and SSc associated renal or cardiac disease were numerically associated with hospitalization and severe outcome (Table 1). Univariable logistic analyses for hospitalization and severe outcome are shown in Figure 1. In multivariable logistic regression, age (OR 1.03, 95%CI 1.01-1.07, p=0.019), presence of non-SSc comorbidities (OR 2.52, 95%CI 1.16-5.47, p=0.019) and SSc-related renal disease (predicting success perfectly) were associated with hospitalization and for severe outcome age (OR 1.05, 95%CI 1.01-1.08).Conclusion:SSc patients at older age, with non-SSc comorbidities, SSc related renal disease or ILD are at risk of a more severe outcome and should follow precautions to avoid COVID-19 infections and need careful monitoring in case of COVID-19.Table 1.SSc disease characteristics of COVID-19 patientsAll(n=172)Hospitalized(n=67)Severe outcome(n=41)Age at COVID-19, yrs (SD)57 (14.0)63 (13.8)65 (12.2)Male sex, n (%)38 (21)18 (27)12 (29)≥1 comorbidity, n (%)63/176 (36)37 (55)30 (58)SSc disease duration at COVID, yrs (SD)11.5 (8.8)13.3 (9.7)12.7 (10.2)Diffuse cutaneous SSc, n (%)74 (42)29 (43)19 (46)mRSS, median (IQR)5 (8)5 (9)5 (7)ILD, n (%)90/175 (51)36/65 (55)26/40 (65)PAH, n (%)21/175 (12)11/65 (17)8/40 (20)GI disease, n (%)112/176 (64)45 (67)30 (73)Cardiac disease, n (%)37/166 (22)19/59 (32)16/36 (44)Musculoskeletal disease, n (%)40/175 (23)15/65 (23)6/40 (15)Renal disease, n (%)8/175 (5)7/65 (11)5/40 (13)Ever DU, n (%)69/175 (39)27/65 (42)14/40 (35)CRP, ng/ml (SD)35/177 (20)14 (21)9 (22)Immunosuppressive treatment, n (%)104/177 (59)41/66 (62)26 (63)Figure 1.Univariable logistic analyses for hospitalization and severe outcomeDisclosure of Interests:Anna-Maria Hoffmann-Vold Speakers bureau: Actelion, Boehringer Ingelheim, Roche, Merck Sharp & Dohme, ARXX, Lilly and Medscape, Consultant of: Actelion, Boehringer Ingelheim, Bayer, ARXX, and Medscape, Grant/research support from: Boehringer Ingelheim, Cathrine Brunborg: None declared, Francesca Tirelli: None declared, Patricia Carreira: None declared, Nicoletta Del Papa: None declared, Arsene Mekinian: None declared, Madelon Vonk: None declared, Alessandro Giollo: None declared, Giacomo De Luca: None declared, Maria De Santis: None declared, Corrado Campochiaro: None declared, Carina Mihai: None declared, Paolo Airò Speakers bureau: Bristol Myers Squibb, Bohringer Ingelheim, Consultant of: Bristol Myers Squibb, Bohringer Ingelheim, non-financial support from CSL Behring, SOBI, Janssen, Roche, Sanofi, Pfizer, Maria Grazia Lazzaroni: None declared, Elisabetta Zanatta: None declared, Rosario Foti: None declared, Yannick Allanore: None declared, Daniel Furst: None declared, Marco Matucci-Cerinic: None declared, Armando Gabrielli: None declared, Oliver Distler Speakers bureau: Actelion, Kymera Therapeutics, Mitsubishi Tanabe Pharma, Abbvie, Acceleron, Alexion, Amgen, AnaMar, Arxx Therapeutics, Baecon, Discovery, Blade Therapeutics, Corbus Pharmaceuticals, Drug Development International, Ltd, CSL Behring, Galapagos NV, Glenmark Pharmaceuticals, GSK, Horizon (Curzion) Pharmaceuticals, Inventiva, iQvia, Kymera Therapeutics, Lilly, Novartis, Pfizer, Topadur and UCB, Consultant of: Actelion, Kymera Therapeutics, Mitsubishi Tanabe Pharma, Abbvie, Acceleron, Alexion, Amgen, AnaMar, Arxx Therapeutics, Baecon, Discovery, Blade Therapeutics, Corbus Pharmaceuticals, Drug Development International, Ltd, CSL Behring, Galapagos NV, Glenmark Pharmaceuticals, GSK, Horizon (Curzion) Pharmaceuticals, Inventiva, iQvia, Kymera Therapeutics, Lilly, Novartis, Pfizer, Topadur and UCB, Grant/research support from: Boehringer Ingelheim.
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Matos, Jefferson David Melo de, Leonardo Jiro Nomura Nakano, André Guimarães Rodrigues, Alessandra Dossi Pinto, Mateus Favero Barra Grande, Guilherme da Rocha Scalzer Lopes, and Valdir Cabral Andrade. "Orofacial clefts: treatment based on a multidisciplinary approach." ARCHIVES OF HEALTH INVESTIGATION 9, no. 5 (October 21, 2020): 468–73. http://dx.doi.org/10.21270/archi.v9i5.4804.

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Objective: The present study aims to expose through a literature review the cleft lip and/or cleft palate (CL/CP) and its treatment in a multidisciplinary approach. Methodology: This literature review was conducted by the leading health databases: Pubmed (https://www.ncbi.nlm.nih.gov/pubmed). The keywords for the textual search were: Cleft Lip; Cleft Palate; Dental Staff; Classification; Embryology. The inclusion criteria were: literature on the subject under study, literature of the last years, english language, laboratory and clinical studies and systematic review. Literature Review: Fissures can be defined by a space at the junction between two bones, usually where there would be a suture. Orofacial clefts are part of the congenital facial anomalies resulted from the non-junction of the embryonic facial processes. These changes occur due to an alteration in the migratory velocity of the neural crest cells, in charge of the phenomenon of fusion of the facial prominences between the 6th and 9thweek of embryonic life. Conclusion: The treatment of patients with orofacial clefts requires the approach of a multidisciplinary team that involves physicians in the area of plastic surgery, otorhinolaryngology, pediatrics, geneticists, dentists, prosthetics, nurses and speech pathologists, focusing on patient prevention, recovery and rehabilitation. However, further studies are needed for a better understanding of the subject and the steps that should be applied for each particular case.Descriptors: Cleft Lip; Cleft Palate; Dental Staff; Classification; Embryology.ReferencesShaw WC, Brattström V, Mølsted K, Prahl-Andersen B, Roberts CT, Semb G. The Eurocleft study: intercenter study of treatment outcome in patients with complete cleft lip and palate. Part 5: discussion and conclusions. Cleft Palate Craniofac J. 2005;42(1):93-8. Friede H, Lilja J. The Eurocleft Study: Intercenter study of treatment outcome in patients with complete cleft lip and palate. Cleft Palate Craniofac J. 2005;42(4):453-54.Rosenstein SW, Grasseschi M, Dado D. The Eurocleft Study: Intercenter study of treatment outcome in patients with complete cleft lip and palate. Cleft Palate Craniofac J. 2005;42(4):453.Semb G, Brattström V, Mølsted K, Prahl-Andersen B, Zuurbier P, Rumsey N, Shaw WC. The Eurocleft study: intercenter study of treatment outcome in patients with complete cleft lip and palate. Part 4: relationship among treatment outcome, patient/parent satisfaction, and the burden of care. Cleft Palate Craniofac J. 2005;42(1):83-92. Watkins SE, Meyer RE, Strauss RP, Aylsworth AS. Classification, epidemiology, and genetics of orofacial clefts. Clin Plast Surg. 2014;41(2):149-63. Coleman JR Jr, Sykes JM. The embryology, classification, epidemiology, and genetics of facial clefting. Facial Plast Surg Clin North Am. 2001;9(1):1-13.Pengelly RJ, Arias L, Martínez J, Upstill-Goddard R, Seaby EG, Gibson J, Ennis S, Collins A, Briceño I. Deleterious coding variants in multi-case families with non-syndromic cleft lip and/or palate phenotypes. Sci Rep. 2016;6:30457.Ren Y, Steegman R, Dieters A, Jansma J, Stamatakis H. Bone-anchored maxillary protraction in patients with unilateral complete cleft lip and palate and Class III malocclusion. Clin Oral Investig. 2019;23(5):2429-2441.Alberconi TF, Siqueira GLC, Sathler R, Kelly KA, Garib DG. Assessment of Orthodontic Burden of Care in Patients With Unilateral Complete Cleft Lip and Palate. Cleft Palate Craniofac J. 2018;55(1):74-78.Eriguchi M, Watanabe A, Suga K, Nakano Y, Sakamoto T, Sueishi K, Uchiyama T. Growth of Palate in Unilateral Cleft Lip and Palate Patients Undergoing Two-stage Palatoplasty and Orthodontic Treatment. Bull Tokyo Dent Coll. 2018;59(3):183-91.Smane L, Pilmane M. Evaluation of the presence of MMP-2, TIMP-2, BMP2/4, and TGFβ3 in the facial tissue of children with cleft lip and palate. Acta Med Litu. 2018;25(2):86-94. AlHayyan WA, Pani SC, AlJohar AJ, AlQatami FM. The Effects of Presurgical Nasoalveolar Molding on the Midface Symmetry of Children with Unilateral Cleft Lip and Palate: A Long-term Follow-up Study. Plast Reconstr Surg Glob Open. 2018;6(7):e1764. Thakur S, Singh A, Thakur NS, Diwana VK. Achievement in Nasal Symmetry after Cheiloplasty in Unilateral Cleft Lip and Palate Infants Treated with Presurgical Nasoalveolar Molding. Contemp Clin Dent. 2018;9(3):357-60. Turri de Castro Ribeiro T, Petri Feitosa MC, Almeida Penhavel R, Zanda RS, Janson G, Mazzottini R, Garib DG. Extreme maxillomandibular discrepancy in unilateral cleft lip and palate: Longitudinal follow-up in a patient with mandibular prognathism. Am J Orthod Dentofacial Orthop. 2018;154(2):294-304. Perillo L, Vitale M, d'Apuzzo F, Isola G, Nucera R, Matarese G. Interdisciplinary approach for a patient with unilateral cleft lip and palate. Am J Orthod Dentofacial Orthop. 2018;153(6):883-94. Hoffmannova E, Moslerová V, Dupej J, Borský J, Bejdová Š, Velemínská J. Three-dimensional development of the upper dental arch in unilateral cleft lip and palate patients after early neonatal cheiloplasty. Int J Pediatr Otorhinolaryngol. 2018;109:1-6. Tan ELY, Kuek MC, Wong HC, Ong SAK, Yow M. Secondary Dentition Characteristics in Children With Nonsyndromic Unilateral Cleft Lip and Palate: A Retrospective Study. Cleft Palate Craniofac J. 2018;55(4):582-89. Rodrigues R, Fernandes MH, Monteiro AB, Furfuro R, Sequeira T, Silva CC, Manso MC. SPINA classification of cleft lip and palate: A suggestion for a complement. Arch Pediatr. 2018;25(7):439-41. Ortiz-Posadas MR, Vega-Alvarado L, Maya-Behar J. A new approach to classify cleft lip and palate. Cleft Palate Craniofac J. 2001;38(6):545-50.Spina V, Psillakis JM, Lapa FS, Ferreira MC. Classificação das fissuras lábio-palatinas. Sugestão de modificação [Classification of cleft lip and cleft palate. Suggested changes]. Rev Hosp Clin Fac Med Sao Paulo. 1972;27(1):5-6. Allori AC, Mulliken JB, Meara JG, Shusterman S, Marcus JR. Classification of Cleft Lip/Palate: Then and Now. Cleft Palate Craniofac J. 2017;54(2):175-88. Spina V. A proposed modification for the classification of cleft lip and cleft palate. Cleft Palate J. 1973;10:251-2. Yun-Chia Ku M, Lo LJ, Chen MC, Wen-Ching Ko E. Predicting need for orthognathic surgery in early permanent dentition patients with unilateral cleft lip and palate using receiver operating characteristic analysis. Am J Orthod Dentofacial Orthop. 2018;153(3):405-14. Garib D, Yatabe M, de Souza Faco RA, Gregório L, Cevidanes L, de Clerck H. Bone-anchored maxillary protraction in a patient with complete cleft lip and palate: A case report. Am J Orthod Dentofacial Orthop. 2018;153(2):290-97. De Stefani A, Bruno G, Balasso P, Mazzoleni S, Baciliero U, Gracco A. Teeth agenesis evaluation in an Italian sample of complete unilateral and bilateral cleft lip and palate patients. Minerva Stomatol. 2018;67(4):156-64. Chang SY, Lonic D, Pai BC, Lo LJ. Primary Repair in Patients With Unilateral Complete Cleft of Lip and Primary Palate: Assessment of Outcomes. Ann Plast Surg. 2018;80(2S Suppl 1):S2-6.Vura N, Gaddipati R, Palla Y, Kumar P. An Intraoral Appliance to Retract the Protrusive Premaxilla in Bilateral Cleft Lip Patients Presenting Late for Primary Lip Repair. Cleft Palate Craniofac J. 2018;55(4):622-25.Massie JP, Bruckman K, Rifkin WJ, Runyan CM, Shetye PR, Grayson B, Flores RL. The Effect of Nasoalveolar Molding on Nasal Airway Anatomy: A 9-Year Follow-up of Patients With Unilateral Cleft Lip and Palate. Cleft Palate Craniofac J. 2018;55(4):596-601. Jabbari F, Wiklander L, Reiser E, Thor A, Hakelius M, Nowinski D. Secondary Alveolar Bone Grafting in Patients Born With Unilateral Cleft Lip and Palate: A 20-Year Follow-up. Cleft Palate Craniofac J. 2018;55(2):173-79.Jones CM, Roth B, Mercado AM, Russell KA, Daskalogiannakis J, Samson TD, Hathaway RR, Smith A, Mackay DR, Long RE Jr. The Americleft Project: Comparison of Ratings Using Two-Dimensional Versus Three-Dimensional Images for Evaluation of Nasolabial Appearance in Patients With Unilateral Cleft Lip and Palate. J Craniofac Surg. 2018;29(1):105-8. Gatti GL, Freda N, Giacomina A, Montemagni M, Sisti A. Cleft Lip and Palate Repair. J Craniofac Surg. 2017;28(8):1918-24.
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"Paola Giacomoni;, Luigi Dappiano(Editors). Jacopo Aconcio: Il pensiero scientifico e l’idea di tolleranza. (Editrice Università degli Studi di Trento, Dipartimento di Scienze Filologiche e Storiche.) 305 pp., figs., bibl., index. Trento: Dipartimento di Scienze Filologiche e Storiche, 2005. €15 (paper)." Isis 98, no. 4 (December 2007): 889. http://dx.doi.org/10.1086/529350.

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35

Giuffrida, Milena, Paola Italia, Simone Nieddu, and Desmond Schmidt. "From print to digital: A web edition of Giacomo Leopardi’s Idilli." Digital Scholarship in the Humanities, May 3, 2020. http://dx.doi.org/10.1093/llc/fqaa022.

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Abstract Although most would agree that the future of the scholarly edition lies in the digital medium, it is the print scholarly edition that is still more often cited and read. The production of digital scholarly editions (DSEs) is still seen as an experimental field whose methodology has not yet settled to the extent that a digital editing project can be approached with the same confidence as the making of a print edition. This article describes an experimental conversion of a print scholarly edition—Giacomo Leopardi’s Idilli by Paola Italia (2008)—into a DSE. This posed a challenge due to the complexity of its internal evidence, but was also relatively short and suitable for an experimental edition. Our objective was to assimilate into a web-based DSE all the information contained in the text and apparatus of the print edition. We also sought to discover whether the making of a DSE today that could fully utilize the affordances of the web, would necessarily place a significant technical load on editors who are more accustomed to solving textual problems. We review briefly a number of generic tools for making DSEs and describe two attempts at making our own DSE of Leopardi’s Idilli: a wiki edition whose primary purpose was pedagogical and a DSE based on the software used to make the Charles Harpur Critical Archive (Eggert, 2019, Charles Harpur Critical Archive. http://charles-harpur.org). We compare these experiences and draw conclusions about the prospects of making DSEs today.
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Crisanti, Alice. "Two Indianists and a Commission. Carlo Formichi, Oreste Nazari and the Progress of Sanskrit Studies in Italy." 57 | 2021, no. 1 (June 30, 2021). http://dx.doi.org/10.30687/annor/2385-3042/2021/01/012.

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This paper aims to retrace a piece of the history of Indian studies in Italy between the 19th and 20th centuries. Specifically, it builds on the unpublished documents from the works of the October 1905 Examination Commission – consisting of Michele Kerbaker, Emilio Teza, Fausto Gherardo Fumi, Paolo Emilio Pavolini, and Ernesto Giacomo Parodi – tasked with assessing the scientific and academics titles of Carlo Formichi and Oreste Nazari, who had held temporary Sanskrit chairs in Pisa and Palermo since 1901, in view of promoting them to tenured positions. This is a valuable case-study to understand the dynamics of the development of Indian studies in post-unification Italy, as well as the reciprocal influences between scholars and the reception of contemporary publications in the Orientalistic field.
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Marconi, Gilberto. "La fede e le opere per la giustificazione nella lettera di Giacomo e in Paolo: il motivo ideologico della Riforma." Storicamente 15-16 (June 6, 2020). http://dx.doi.org/10.12977/stor787.

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38

"Gianfranco Cainelli, Paola Galletti, Silvia Pieraccini, Arianna Quintavalla, Daria Giacomini, Gian Piero Spada, "Chiral aldehydes in hydrocarbons: Diastereoselective nucleophilic addition, NMR, and CD spectroscopy reveal dynamic solvation effects,"Chirality(2004) 16(1) 50-56." Chirality 16, no. 4 (2004): 278. http://dx.doi.org/10.1002/chir.20044.

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