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1. Healthcare utilization and unmet needs of patients with antisynthetase syndrome: An international patient survey

Author: Weiss, M., Holzer, M. T., Muehlensiepen, F., Ignatyev, Y., Fiehn, C., Bauhammer, J., Schmidt, J., Schlüter, S., Dihkan, A., Scheibner, D., Schneider, U., Valor-Mendez, L., Corte, G., Gupta, L., Chinoy, H., Lundberg, I., Cavagna, L., Distler, J. H. W., Schett, G., and Knitza, J.
Published: 2023
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2. The added value of a European Reference Network on rare and complex connective tissue and musculoskeletal diseases: insights after the first 5 years of the ERN ReCONNET

Author: Talarico, R, Aguilera, S, Alexander, T, Amoura, Z, Andersen, J, Arnaud, L, Avcin, T, Marsal Barril, S, Beretta, L, Bombardieri, S, Bortoluzzi, A, Bouillot, C, Bulina, I, Burmester, G, Cannizzo, S, Cavagna, L, Chaigne, B, Cornet, A, Corti, P, Costedoat-Chalumeau, N, Davidsone, Z, Doria, A, Fenech, C, Ferraris, A, Fischer-Betz, R, Fonseca, J, Frank, C, Gaglioti, A, Galetti, I, Guimaraes, V, Hachulla, E, Holmner, M, Houssiau, F, Iaccarino, L, Jacobsen, S, Limper, M, Malfait, F, Mariette, X, Marinello, D, Martin, T, Matthews, L, Matucci-Cerinic, M, Meyer, A, Milas-Ahic, J, Moinzadeh, P, Montecucco, C, Mouthon, L, Muller-Ladner, U, Nagy, G, Patarata, E, Pileckyte, M, Pruunsild, C, Rednic, S, Romao, V, Schneider, M, Scire, C, Smith, V, Sulli, A, Tamirou, F, Tani, C, Taruscio, D, Taulaigo, A, Tincani, A, Ticciati, S, Turchetti, G, van Hagen, P, van Laar, J, Vieira, A, de Vries-Bouwstra, J, Zschocke, J, Cutolo, M, Mosca, M, Talarico R., Aguilera S., Alexander T., Amoura Z., Andersen J., Arnaud L., Avcin T., Marsal Barril S., Beretta L., Bombardieri S., Bortoluzzi A., Bouillot C., Bulina I., Burmester G. R., Cannizzo S., Cavagna L., Chaigne B., Cornet A., Corti P., Costedoat-Chalumeau N., Davidsone Z., Doria A., Fenech C., Ferraris A., Fischer-Betz R., Fonseca J. E., Frank C., Gaglioti A., Galetti I., Guimaraes V., Hachulla E., Holmner M., Houssiau F., Iaccarino L., Jacobsen S., Limper M., Malfait F., Mariette X., Marinello D., Martin T., Matthews L., Matucci-Cerinic M., Meyer A., Milas-Ahic J., Moinzadeh P., Montecucco C., Mouthon L., Muller-Ladner U., Nagy G., Patarata E., Pileckyte M., Pruunsild C., Rednic S., Romao V. C., Schneider M., Scire C. A., Smith V., Sulli A., Tamirou F., Tani C., Taruscio D., Taulaigo A. V., Tincani A., Ticciati S., Turchetti G., van Hagen P. M., van Laar J. M., Vieira A., de Vries-Bouwstra J. K., Zschocke J., Cutolo M., Mosca M., Talarico, R, Aguilera, S, Alexander, T, Amoura, Z, Andersen, J, Arnaud, L, Avcin, T, Marsal Barril, S, Beretta, L, Bombardieri, S, Bortoluzzi, A, Bouillot, C, Bulina, I, Burmester, G, Cannizzo, S, Cavagna, L, Chaigne, B, Cornet, A, Corti, P, Costedoat-Chalumeau, N, Davidsone, Z, Doria, A, Fenech, C, Ferraris, A, Fischer-Betz, R, Fonseca, J, Frank, C, Gaglioti, A, Galetti, I, Guimaraes, V, Hachulla, E, Holmner, M, Houssiau, F, Iaccarino, L, Jacobsen, S, Limper, M, Malfait, F, Mariette, X, Marinello, D, Martin, T, Matthews, L, Matucci-Cerinic, M, Meyer, A, Milas-Ahic, J, Moinzadeh, P, Montecucco, C, Mouthon, L, Muller-Ladner, U, Nagy, G, Patarata, E, Pileckyte, M, Pruunsild, C, Rednic, S, Romao, V, Schneider, M, Scire, C, Smith, V, Sulli, A, Tamirou, F, Tani, C, Taruscio, D, Taulaigo, A, Tincani, A, Ticciati, S, Turchetti, G, van Hagen, P, van Laar, J, Vieira, A, de Vries-Bouwstra, J, Zschocke, J, Cutolo, M, Mosca, M, Talarico R., Aguilera S., Alexander T., Amoura Z., Andersen J., Arnaud L., Avcin T., Marsal Barril S., Beretta L., Bombardieri S., Bortoluzzi A., Bouillot C., Bulina I., Burmester G. R., Cannizzo S., Cavagna L., Chaigne B., Cornet A., Corti P., Costedoat-Chalumeau N., Davidsone Z., Doria A., Fenech C., Ferraris A., Fischer-Betz R., Fonseca J. E., Frank C., Gaglioti A., Galetti I., Guimaraes V., Hachulla E., Holmner M., Houssiau F., Iaccarino L., Jacobsen S., Limper M., Malfait F., Mariette X., Marinello D., Martin T., Matthews L., Matucci-Cerinic M., Meyer A., Milas-Ahic J., Moinzadeh P., Montecucco C., Mouthon L., Muller-Ladner U., Nagy G., Patarata E., Pileckyte M., Pruunsild C., Rednic S., Romao V. C., Schneider M., Scire C. A., Smith V., Sulli A., Tamirou F., Tani C., Taruscio D., Taulaigo A. V., Tincani A., Ticciati S., Turchetti G., van Hagen P. M., van Laar J. M., Vieira A., de Vries-Bouwstra J. K., Zschocke J., Cutolo M., and Mosca M.
Abstract: In order to address the main challenges related to the rare diseases (RDs) the European Commission launched the European Reference Networks (ERNs), virtual networks involving healthcare providers (HCPs) across Europe. The mission of the ERNs is to tackle low prevalence and RDs that require highly specialised treatment and a concentration of knowledge and resources. In fact, ERNs offer the potential to give patients and healthcare professionals across the EU access to the best expertise and timely exchange of lifesaving knowledge, trying to make the knowledge travelling more than patients. For this reason, ERNs were established as concrete European infrastructures, and this is particularly crucial in the framework of rare and complex diseases in which no country alone has the whole knowledge and capacity to treat all types of patients. It has been five years since their kick-off launch in Vilnius in 2017. The 24 ERNs have been intensively working on different transversal areas, including patient management, education, clinical practice guidelines, patients' care pathways and many other fundamental topics. The present work is therefore aimed not only at reporting a summary of the main activities and milestones reached so far, but also at celebrating the first 5 years of the ERN on Rare and Complex Connective Tissue and Musculo-skeletal Diseases (ReCONNET), in which the members of the network built together one of the 24 infrastructures that are hopefully going to change the scenario of rare diseases across the EU.
Published: 2022

3. Defining anti-synthetase syndrome: a systematic literature review

Author: Zanframundo, G, Faghihi-Kashani, S, Scire, C, Bonella, F, Corte, T, Doyle, T, Fiorentino, D, Gonzalez-Gay, M, Hudson, M, Kuwana, M, Lundberg, I, Mammen, A, Mchugh, N, Miller, F, Monteccucco, C, Oddis, C, Rojas-Serrano, J, Schmidt, J, Selva-O'Callaghan, A, Werth, V, Sakellariou, G, Aggarwal, R, Cavagna, L, Zanframundo G., Faghihi-Kashani S., Scire C. A., Bonella F., Corte T. J., Doyle T. J., Fiorentino D., Gonzalez-Gay M. A., Hudson M., Kuwana M., Lundberg I. E., Mammen A., McHugh N., Miller F. W., Monteccucco C., Oddis C. V., Rojas-Serrano J., Schmidt J., Selva-O'Callaghan A., Werth V. P., Sakellariou G., Aggarwal R., Cavagna L., Zanframundo, G, Faghihi-Kashani, S, Scire, C, Bonella, F, Corte, T, Doyle, T, Fiorentino, D, Gonzalez-Gay, M, Hudson, M, Kuwana, M, Lundberg, I, Mammen, A, Mchugh, N, Miller, F, Monteccucco, C, Oddis, C, Rojas-Serrano, J, Schmidt, J, Selva-O'Callaghan, A, Werth, V, Sakellariou, G, Aggarwal, R, Cavagna, L, Zanframundo G., Faghihi-Kashani S., Scire C. A., Bonella F., Corte T. J., Doyle T. J., Fiorentino D., Gonzalez-Gay M. A., Hudson M., Kuwana M., Lundberg I. E., Mammen A., McHugh N., Miller F. W., Monteccucco C., Oddis C. V., Rojas-Serrano J., Schmidt J., Selva-O'Callaghan A., Werth V. P., Sakellariou G., Aggarwal R., and Cavagna L.
Abstract: Anti-synthetase syndrome (ASSD) is a heterogeneous autoimmune disease characterised by multi-system involvement with a wide variety of manifestations. Validated classification criteria are necessary to improve recognition and prevent misclassification, especially given the lack of reliable and standardised autoantibody testing. We systematically reviewed the literature to analyse proposed ASSD criteria, characteristics, and diagnostic performance. Methods We searched PubMed and Embase databases (01/01/1984 to 06/11/2018) and the ACR and EULAR meeting abstracts (2017-2018). Sensitivities, specificities, positive, negative likelihood ratios and risk of bias were calculated for ASSD criteria and key variables reported in the literature. We performed meta-analysis when appropriate. Results We retrieved 4,358 studies. We found 85 proposed ASSD criteria from a total of 82 studies. All but one study included anti-synthetase autoantibody (ARS) positivity in the ASSD criteria. Most studies required only one ASSD feature plus anti-ARS to define ASSD (n=64, 78%), whereas 16 studies required more than one ASSD variable plus anti-ARS. The only criteria not including anti-ARS positivity required 5 ASSD clinical features. We found limited data and wide variability in the diagnostic performance of each variable and definition proposed in the literature. Given these limitations we only meta-analysed the performance of individual muscle biopsy and clinical variables in diagnosing ASSD, which performed poorly. Conclusion The current ASSD criteria include a variety of serological, clinical, and histological features with wide variability amongst proposed definitions and the performance of these definitions has not been tested. This systematic literature review suggests the need for additional data and consensus-driven classification criteria for ASSD.
Published: 2022

4. Clinical spectrum time course in non-Asian patients positive for anti-MDA5 antibodies

Author: Cavagna, L, Meloni, F, Meyer, A, Sambataro, G, Belliato, M, De Langhe, E, Cavazzana, I, Pipitone, N, Triantafyllias, K, Mosca, M, Barsotti, S, Zampogna, G, Biglia, A, Emmi, G, De Visser, M, Van Der Kooi, A, Parronchi, P, Hirschi, S, da Silva, J, Scire, C, Furini, F, Giannini, M, Martinez Gonzalez, O, Damian, L, Piette, Y, Smith, V, Mera-Valera, A, Bachiller-Corral, J, Cabezas Rodriguez, I, Brandy-Garcia, A, Maurier, F, Perrin, J, Gonzalez-Moreno, J, Drott, U, Delbruck, C, Schwarting, A, Arrigoni, E, Sebastiani, G, Iuliano, A, Nannini, C, Quartuccio, L, Rodriguez Cambron, A, Blazquez Canamero, M, Villa Blanco, I, Cagnotto, G, Pesci, A, Luppi, F, Dei, G, Romero Bueno, F, Franceschini, F, Chiapparoli, I, Zanframundo, G, Lettieri, S, De Stefano, L, Cutolo, M, Mathieu, A, Piga, M, Prieto-Gonzalez, S, Moraes-Fontes, M, Fonseca, J, Jovani, V, Riccieri, V, Santaniello, A, Montfort, S, Bilocca, D, Erre, G, Bartoloni, E, Gerli, R, Monti, M, Lorenz, H, Sambataro, D, Bellando Randone, S, Schneider, U, Valenzuela, C, Lopez-Mejias, R, Cifrian, J, Mejia, M, Gonzalez Perez, M, Wendel, S, Fornaro, M, De Luca, G, Orsolini, G, Rossini, M, Dieude, P, Knitza, J, Castaneda, S, Voll, R, Rojas-Serrano, J, Valentini, A, Vancheri, C, Matucci-Cerinic, M, Feist, E, Codullo, V, Iannone, F, Distler, J, Montecucco, C, Gonzalez-Gay, M, Cavagna L., Meloni F., Meyer A., Sambataro G., Belliato M., De Langhe E., Cavazzana I., Pipitone N., Triantafyllias K., Mosca M., Barsotti S., Zampogna G., Biglia A., Emmi G., De Visser M., Van Der Kooi A., Parronchi P., Hirschi S., da Silva J. A. P., Scire C. A., Furini F., Giannini M., Martinez Gonzalez O., Damian L., Piette Y., Smith V., Mera-Valera A., Bachiller-Corral J., Cabezas Rodriguez I., Brandy-Garcia A. M., Maurier F., Perrin J., Gonzalez-Moreno J., Drott U., Delbruck C., Schwarting A., Arrigoni E., Sebastiani G. D., Iuliano A., Nannini C., Quartuccio L., Rodriguez Cambron A. B., Blazquez Canamero M. A., Villa Blanco I., Cagnotto G., Pesci A., Luppi F., Dei G., Romero Bueno F. I., Franceschini F., Chiapparoli I., Zanframundo G., Lettieri S., De Stefano L., Cutolo M., Mathieu A., Piga M., Prieto-Gonzalez S., Moraes-Fontes M. F., Fonseca J. E., Jovani V., Riccieri V., Santaniello A., Montfort S., Bilocca D., Erre G. L., Bartoloni E., Gerli R., Monti M. C., Lorenz H. M., Sambataro D., Bellando Randone S., Schneider U., Valenzuela C., Lopez-Mejias R., Cifrian J., Mejia M., Gonzalez Perez M. -I., Wendel S., Fornaro M., De Luca G., Orsolini G., Rossini M., Dieude P., Knitza J., Castaneda S., Voll R. E., Rojas-Serrano J., Valentini A., Vancheri C., Matucci-Cerinic M., Feist E., Codullo V., Iannone F., Distler J. H., Montecucco C., Gonzalez-Gay M. A., Cavagna, L, Meloni, F, Meyer, A, Sambataro, G, Belliato, M, De Langhe, E, Cavazzana, I, Pipitone, N, Triantafyllias, K, Mosca, M, Barsotti, S, Zampogna, G, Biglia, A, Emmi, G, De Visser, M, Van Der Kooi, A, Parronchi, P, Hirschi, S, da Silva, J, Scire, C, Furini, F, Giannini, M, Martinez Gonzalez, O, Damian, L, Piette, Y, Smith, V, Mera-Valera, A, Bachiller-Corral, J, Cabezas Rodriguez, I, Brandy-Garcia, A, Maurier, F, Perrin, J, Gonzalez-Moreno, J, Drott, U, Delbruck, C, Schwarting, A, Arrigoni, E, Sebastiani, G, Iuliano, A, Nannini, C, Quartuccio, L, Rodriguez Cambron, A, Blazquez Canamero, M, Villa Blanco, I, Cagnotto, G, Pesci, A, Luppi, F, Dei, G, Romero Bueno, F, Franceschini, F, Chiapparoli, I, Zanframundo, G, Lettieri, S, De Stefano, L, Cutolo, M, Mathieu, A, Piga, M, Prieto-Gonzalez, S, Moraes-Fontes, M, Fonseca, J, Jovani, V, Riccieri, V, Santaniello, A, Montfort, S, Bilocca, D, Erre, G, Bartoloni, E, Gerli, R, Monti, M, Lorenz, H, Sambataro, D, Bellando Randone, S, Schneider, U, Valenzuela, C, Lopez-Mejias, R, Cifrian, J, Mejia, M, Gonzalez Perez, M, Wendel, S, Fornaro, M, De Luca, G, Orsolini, G, Rossini, M, Dieude, P, Knitza, J, Castaneda, S, Voll, R, Rojas-Serrano, J, Valentini, A, Vancheri, C, Matucci-Cerinic, M, Feist, E, Codullo, V, Iannone, F, Distler, J, Montecucco, C, Gonzalez-Gay, M, Cavagna L., Meloni F., Meyer A., Sambataro G., Belliato M., De Langhe E., Cavazzana I., Pipitone N., Triantafyllias K., Mosca M., Barsotti S., Zampogna G., Biglia A., Emmi G., De Visser M., Van Der Kooi A., Parronchi P., Hirschi S., da Silva J. A. P., Scire C. A., Furini F., Giannini M., Martinez Gonzalez O., Damian L., Piette Y., Smith V., Mera-Valera A., Bachiller-Corral J., Cabezas Rodriguez I., Brandy-Garcia A. M., Maurier F., Perrin J., Gonzalez-Moreno J., Drott U., Delbruck C., Schwarting A., Arrigoni E., Sebastiani G. D., Iuliano A., Nannini C., Quartuccio L., Rodriguez Cambron A. B., Blazquez Canamero M. A., Villa Blanco I., Cagnotto G., Pesci A., Luppi F., Dei G., Romero Bueno F. I., Franceschini F., Chiapparoli I., Zanframundo G., Lettieri S., De Stefano L., Cutolo M., Mathieu A., Piga M., Prieto-Gonzalez S., Moraes-Fontes M. F., Fonseca J. E., Jovani V., Riccieri V., Santaniello A., Montfort S., Bilocca D., Erre G. L., Bartoloni E., Gerli R., Monti M. C., Lorenz H. M., Sambataro D., Bellando Randone S., Schneider U., Valenzuela C., Lopez-Mejias R., Cifrian J., Mejia M., Gonzalez Perez M. -I., Wendel S., Fornaro M., De Luca G., Orsolini G., Rossini M., Dieude P., Knitza J., Castaneda S., Voll R. E., Rojas-Serrano J., Valentini A., Vancheri C., Matucci-Cerinic M., Feist E., Codullo V., Iannone F., Distler J. H., Montecucco C., and Gonzalez-Gay M. A.
Abstract: Objective To define the clinical spectrum time-course and prognosis of non-Asian patients positive for anti-MDA5 antibodies. Methods We conducted a multicentre, international, retrospective cohort study. Results 149 anti-MDA5 positive patients (median onset age 53 years, median disease duration 18 months), mainly females (100, 67%), were included. Dermatomyositis (64, 43%) and amyopathic dermatomyositis (47, 31%), were the main diagnosis; 15 patients (10%) were classified as interstitial pneumonia with autoimmune features (IPAF) and 7 (5%) as rheumatoid arthritis. The main clinical findings observed were myositis (84, 56%), interstitial lung disease (ILD) (108, 78%), skin lesions (111, 74%), and arthritis (76, 51%). The onset of these manifestations was not concomitant in 74 cases (50%). Of note, 32 (21.5%) patients were admitted to the intensive care unit for rapidly progressive-ILD, which occurred in median 2 months from lung involvement detection, in the majority of cases (28, 19%) despite previous immunosuppressive treatment. One-third of patients (47, 32% each) was ANA and anti-ENA antibodies negative and a similar percentage was anti-Ro52 kDa antibodies positive. Non-specific interstitial pneumonia (65, 60%), organising pneumonia (23, 21%), and usual interstitial pneumonia-like pattern (14, 13%) were the main ILD patterns observed. Twenty-six patients died (17%), 19 (13%) had a rapidly progressive-ILD. Conclusion The clinical spectrum of the anti-MDA5 antibodies-related disease is heterogeneous. Rapidly-progressive ILD deeply impacts the prognosis also in non-Asian patients, occurring early during the disease course. Anti-MDA5 antibody positivity should be considered even when baseline autoimmune screening is negative, anti-Ro52 kDa antibodies are positive, and radiology findings show a NSIP pattern.
Published: 2022

5. POS1216 MULTIMORBIDITY AND PROMIS HEALTH OUTCOMES IN PATIENTS WITH IDIOPATHIC INFLAMMATORY MYOPATHIES: DATA FROM A LARGE, GLOBAL E-SURVEY (COVAD STUDY)

Author: Fornaro, M., primary, Venerito, V., additional, Iannone, F., additional, Ravichandran, N., additional, Nikiphorou, E., additional, Joshi, M., additional, Tan, A. L., additional, Saha, S., additional, Katsuyuki Shinjo, S., additional, Agarwal, V., additional, Ziade, N., additional, Velikova, T., additional, Jagtap, K., additional, Milchert, M., additional, Parodis, I., additional, Gracia-Ramos, A. E., additional, Cavagna, L., additional, Kuwana, M., additional, Knitza, J., additional, Makol, A., additional, Dzifa, D., additional, Toro Gutierrez, C. E., additional, Vinicio Caballero, C., additional, Distler, O., additional, Day, J., additional, Study, C., additional, Chinoy, H., additional, Aggarwal, R., additional, and Gupta, L., additional
Published: 2023
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6. OP0081 PREVALENCE, CHARACTERISTICS, AND PREDICTORS OF BREAKTHROUGH COVID-19 INFECTIONS IN PATIENTS WITH RHEUMATOID ARTHRITIS: DATA FROM THE COVID-19 VACCINATION IN AUTOIMMUNE DISEASE (COVAD) STUDY

Author: Ravichandran, N., primary, Parodis, I., additional, Gupta, L., additional, Katsuyuki Shinjo, S., additional, Ziade, N., additional, Milchert, M., additional, Cavagna, L., additional, Tan, A. L., additional, Lilleker, J. B., additional, Nune, A., additional, Pauling, J., additional, Wincup, C., additional, Katchamart, W., additional, Goo, P. A., additional, Study, C., additional, Chinoy, H., additional, Aggarwal, R., additional, Agarwal, V., additional, and Nikiphorou, E., additional
Published: 2023
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7. POS0573 COMORBIDITIES, COMPLEX MULTIMORBIDITY AND PROMIS HEALTH OUTCOMES AMONGAUTOIMMUNE RHEUMATIC DISEASES: DATA FROM THE COVAD STUDY

Author: Gupta, L., primary, Ravichandran, N., additional, Venerito, V., additional, Dey, M., additional, Sen, P., additional, Saha, S., additional, Tan, A. L., additional, Katsuyuki Shinjo, S., additional, Agarwal, V., additional, Joshi, M., additional, Ziade, N., additional, Velikova, T., additional, Jagtap, K., additional, Milchert, M., additional, Parodis, I., additional, Gracia-Ramos, A. E., additional, Cavagna, L., additional, Kuwana, M., additional, Knitza, J., additional, Makol, A., additional, Dzifa, D., additional, Toro Gutierrez, C. E., additional, Vinicio Caballero, C., additional, Distler, O., additional, Day, J., additional, Chinoy, H., additional, Aggarwal, R., additional, and Nikiphorou, E., additional
Published: 2023
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8. POS0273 POST COVID-19 SYNDROME IN PATIENTS WITH AUTOIMMUNE RHEUMATIC DISEASES: RESULTS FROM THE COVAD STUDY

Author: Sen, P., primary, Ravichandran, N., additional, Joshi, M., additional, Saha, S., additional, Jagtap, K., additional, Agarwal, V., additional, Distler, O., additional, Nikiphorou, E., additional, Tan, A. L., additional, Katsuyuki Shinjo, S., additional, Ziade, N., additional, Velikova, T., additional, Milchert, M., additional, Parodis, I., additional, Gracia-Ramos, A. E., additional, Cavagna, L., additional, Kuwana, M., additional, Knitza, J., additional, Makol, A., additional, Patel, A., additional, Pauling, J., additional, Wincup, C., additional, Barman, B., additional, Zamora-Tehozol, E. A., additional, Rojas-Serrano, J., additional, Garcia-De La Torre, I., additional, Colunga-Pedraza, I. J., additional, Merayo-Chalico, J., additional, Katchamart, W., additional, Goo, P. A., additional, Chen, Y. M., additional, Hoff, L. S., additional, Kibbi, L., additional, Halabi, H., additional, Vaidya, B., additional, Shaharir, S. S., additional, Shumnalieva, R., additional, Hasan, A. T. M. T., additional, Dzifa, D., additional, Toro Gutierrez, C. E., additional, Vinicio Caballero, C., additional, Lilleker, J. B., additional, Salim, B., additional, Gheita, T. A., additional, Chatterjee, T., additional, Nune, A., additional, Saavedra, M. A., additional, Day, J., additional, Chinoy, H., additional, Aggarwal, R., additional, and Gupta, L., additional
Published: 2023
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9. POS1254 REDUCED HEALTH-RELATED QUALITY OF LIFE USING PROMIS IN PATIENTS WITH SYSTEMIC SCLEROSIS: A CROSS-SECTIONAL ANALYSIS FROM THE INTERNATIONAL COVAD-2 E-SURVEY

Author: Yomono, K., primary, Li, Y., additional, Maroufy, V., additional, Ravichandran, N., additional, Yoshida, A., additional, Jagtap, K., additional, Velikova, T., additional, Sen, P., additional, Cavagna, L., additional, Agarwal, V., additional, Knitza, J., additional, Makol, A., additional, Dzifa, D., additional, Toro Gutierrez, C. E., additional, Chatterjee, T., additional, Patel, A., additional, Aggarwal, R., additional, Gupta, L., additional, and Kuwana, M., additional
Published: 2023
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10. POS0948 PATTERNS OF DISEASE PROGRESSION IN SYSTEMIC SCLEROSIS PATIENTS WITH INTERSTITIAL LUNG DISEASE

Author: Santaniello, A., primary, Bellocchi, C., additional, Bosello, S. L., additional, De Lorenzis, E., additional, Natalello, G., additional, Del Papa, N., additional, Cavalli, S., additional, Benfaremo, D., additional, De Luca, G., additional, Campochiaro, C., additional, Cavagna, L., additional, Codullo, V., additional, Montanelli, G., additional, Severino, A., additional, Caronni, M., additional, Vigone, B., additional, Montecucco, C., additional, Dagna, L., additional, Moroncini, G., additional, Caporali, R., additional, and Beretta, L., additional
Published: 2023
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11. POS1234 CEREBROVASCULAR AND CARDIOVASCULAR RISK OF PATIENTS WITH IDIOPATHIC INFLAMMATORY MYOPATHIES IS INCREASED AND CAN BE PREDICTED BY ENA POSITIVITY

Author: Triantafyllias, K., primary, Gauch, S., additional, Cavagna, L., additional, and Schwarting, A., additional
Published: 2023
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12. POS1231 IMPAIRED HEALTH-RELATED QUALITY OF LIFE IN PATIENTS WITH IDIOPATHIC INFLAMMATORY MYOPATHIES: A CROSS-SECTIONAL ANALYSIS FROM AN INTERNATIONAL E-SURVEY

Author: Yoshida, A., primary, LI, Y., additional, Maroufy, V., additional, Kuwana, M., additional, Ravichandran, N., additional, Makol, A., additional, Sen, P., additional, Lilleker, J. B., additional, Agarwal, V., additional, Kardes, S., additional, Day, J., additional, Milchert, M., additional, Joshi, M., additional, Gheita, T. A., additional, Salim, B., additional, Velikova, T., additional, Gracia-Ramos, A. E., additional, Parodis, I., additional, Nikiphorou, E., additional, Tan, A. L., additional, Nune, A., additional, Cavagna, L., additional, Saavedra, M. A., additional, Katsuyuki Shinjo, S., additional, Ziade, N., additional, Knitza, J., additional, Distler, O., additional, Chinoy, H., additional, Aggarwal, R., additional, and Gupta, L., additional
Published: 2023
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13. POS0124 SURVIVAL ANALYSIS IN CONNECTIVE TISSUE DISEASES WITH INTERSTITIAL LUNG DISEASE COMPARED TO IDIOPATHIC PULMONARY FIBROSIS: MULTICENTRE ITALIAN STUDY

Author: Fornaro, M., primary, Cacciapaglia, F., additional, Giotta, M., additional, Zanframundo, G., additional, Lazzaroni, M. G., additional, Bartoletti, A., additional, Pedretti, E., additional, Sambataro, G., additional, Orlandi, M., additional, De Pace, C. C., additional, Colella, S., additional, Lacedonia, D., additional, Vanchieri, C., additional, Airo’, P., additional, Cavagna, L., additional, Guiducci, S., additional, and Iannone, F., additional
Published: 2023
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14. LB0006 COVID-19 severity, breakthrough infections and anti-SARS-CoV-2 vaccine safety in young people with rheumatic and non-rheumatic autoimmune diseases: results from the COVAD1 and COVAD2 projects

Author: Alunno, A., primary, Carubbi, F., additional, Tan, A. L., additional, Sen, P., additional, Cavagna, L., additional, Joshi, M., additional, Day, J., additional, Jagtap, K., additional, Saha, S., additional, Toro Gutierrez, C. E., additional, Vinicio Caballero, C., additional, Distler, O., additional, Chinoy, H., additional, Aggarwal, R., additional, Agarwal, V., additional, and Gupta, L., additional
Published: 2023
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15. POS0880 COMPARATIVE DISEASE BURDEN IN PATIENTS WITH RHEUMATOID ARTHRITIS, PSORIATIC ARTHRITIS OR ANKYLOSING SPONDYLITIS: DATA FROM COVAD PATIENT-REPORTED E-SURVEY

Author: Venerito, V., primary, Fornaro, M., additional, Iannone, F., additional, Cavagna, L., additional, Kuwana, M., additional, Agarwal, V., additional, Ravichandran, N., additional, Day, J., additional, Joshi, M., additional, Saha, S., additional, Shaharir, S. S., additional, Katchamart, W., additional, Goo, P. A., additional, Traboco, L., additional, Chen, Y. M., additional, Sen, P., additional, Lilleker, J. B., additional, Nune, A., additional, Pauling, J., additional, Wincup, C., additional, Tan, A. L., additional, Ziade, N., additional, Milchert, M., additional, Gracia-Ramos, A. E., additional, Vinicio Caballero, C., additional, Study, C., additional, Aggarwal, R., additional, and Gupta, L., additional
Published: 2023
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16. OP0082 COVID-19 VACCINE SAFETY DURING PREGNANCY AND BREASTFEEDING IN WOMEN WITH AUTOIMMUNE DISEASES: RESULTS FROM THE COVAD STUDY

Author: Andreoli, L., primary, Lini, D., additional, Schreiber, K., additional, Sen, P., additional, Ravichandran, N., additional, Parodis, I., additional, Toro Gutierrez, C. E., additional, Katchamart, W., additional, Goo, P. A., additional, Shumnalieva, R., additional, Chibuzo, O. C., additional, Velikova, T., additional, Day, J., additional, Joshi, M., additional, Katsuyuki Shinjo, S., additional, Gracia-Ramos, A. E., additional, Cavagna, L., additional, Kuwana, M., additional, Knitza, J., additional, Makol, A., additional, Chen, Y. M., additional, Study, C., additional, Chinoy, H., additional, Agarwal, V., additional, Aggarwal, R., additional, and Gupta, L., additional
Published: 2023
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17. COVID-19 VACCINE SAFETY DURING PREGNANCY AND BREASTFEEDING IN WOMEN WITH AUTOIMMUNE DISEASES : RESULTS FROM THE COVAD STUDY

Author: Andreoli, L., Lini, D., Schreiber, K., Sen, P., Ravichandran, N., Parodis, Ioannis, Toro Gutierrez, C. E., Katchamart, W., Goo, P. A., Shumnalieva, R., Chibuzo, O. C., Velikova, T., Day, J., Joshi, M., Katsuyuki Shinjo, S., Gracia-Ramos, A. E., Cavagna, L., Kuwana, M., Knitza, J., Makol, A., Chen, Y. M., Chinoy, H., Agarwal, V., Aggarwal, R., Gupta, L., Andreoli, L., Lini, D., Schreiber, K., Sen, P., Ravichandran, N., Parodis, Ioannis, Toro Gutierrez, C. E., Katchamart, W., Goo, P. A., Shumnalieva, R., Chibuzo, O. C., Velikova, T., Day, J., Joshi, M., Katsuyuki Shinjo, S., Gracia-Ramos, A. E., Cavagna, L., Kuwana, M., Knitza, J., Makol, A., Chen, Y. M., Chinoy, H., Agarwal, V., Aggarwal, R., and Gupta, L.
Abstract: Background: COVID-19 vaccine hesitancy among pregnant and breastfeeding women with autoimmune diseases (AID) is often attributed to the fear of adverse events (AE) and disease flares (DF). No data are available regarding COVID-19 vaccine safety in this population. Objectives: We aimed at describing delayed-onset (>7 days) vaccine-related AE (minor and major), DF, and related AID treatment modifications from the COVID-19 Vaccination in Autoimmune Diseases (COVAD) study. Methods: Among complete responses from 9201 participants as of June 21, 2022, 6787 (73.8%) were women. Six subgroups were identified upon diagnosis of AID vs healthy controls (HC) and their pregnancy/breastfeeding status at the time of any dose of vaccine (Figure 1). Results: Forty pregnant and 52 breastfeeding AID patients were identified and their vaccination rates (at least one dose) was 100% and 96.2%, respectively (Table 1). Overall AE, minor AE, and major AE were reported significantly more frequently by pregnant than non-pregnant patients (45% vs. 26%, p=0.01; 40% vs. 25.9%, p=0.03; 17.5% vs. 4.6%, p<0.01), but no difference was found in comparison with pregnant HC. No difference was observed between breastfeeding patients and HC. Post-vaccination DF were reported by 17.5% of pregnant and 20% of breastfeeding patients, and by 18% of age- and disease-matched control patients (n=2315). All DF in pregnant/breastfeeding patients were managed with glucocorticoids and a fifth of them required initiation or change in immunosuppressive treatment. Conclusion: This study provides the first insights into the safety of COVID-19 vaccination during the antenatal period in women with AID. While AEs were more commonly reported by pregnant patients with AID, these were no higher than among pregnant healthy controls without AID. These observations are reassuring, likely to strengthen physician-patient communication and overcome hesitancy as the benefits for the mother and fetus by passive immunization are
Published: 2023
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18. PREVALENCE, CHARACTERISTICS, AND PREDICTORS OF BREAKTHROUGH COVID-19 INFECTIONS IN PATIENTS WITH RHEUMATOID ARTHRITIS : DATA FROM THE COVID-19 VACCINATION IN AUTOIMMUNE DISEASE (COVAD) STUDY

Author: Ravichandran, N., Parodis, Ioannis, Gupta, L., Katsuyuki Shinjo, S., Ziade, N., Milchert, M., Cavagna, L., Tan, A. L., Lilleker, J. B., Nune, A., Pauling, J., Wincup, C., Katchamart, W., Goo, P. A., Study, C., Chinoy, H., Aggarwal, R., Agarwal, V., Nikiphorou, E., Ravichandran, N., Parodis, Ioannis, Gupta, L., Katsuyuki Shinjo, S., Ziade, N., Milchert, M., Cavagna, L., Tan, A. L., Lilleker, J. B., Nune, A., Pauling, J., Wincup, C., Katchamart, W., Goo, P. A., Study, C., Chinoy, H., Aggarwal, R., Agarwal, V., and Nikiphorou, E.
Abstract: Background: Global data on COVID-19 breakthrough infections (BI) following COVID-19 vaccination among autoimmune rheumatic diseases (AIRDs) and especially rheumatoid arthritis (RA) is scarce. Objectives: This study aimed to examine the characteristics of COVID-19 BI among patients with RA and compare them with AIRDs and healthy controls (HCs). Methods: A global e-survey, January-May 2022, collected data on COVID-19 vaccination, and BI in patients with RA, AIRDs, non-rheumatic autoimmune disease (nrAIDs), and HCs. BI was defined as infection after both primary or booster vaccine doses. Severe BI was defined as the need for hospitalization, including intensive unit care, oxygen therapy, or advanced treatment in the form of monoclonal antibodies. Results: Of the 9595 vaccinated respondents of the e-survey, 3224 (33.6%) reported COVID-19. One BI was reported in 323/1802 (17.9%) patients with RA, 584/3869 (15.0%) patients with other AIRDs, and 467/3435 (13.5%) HCs. Similarly, second BI was reported by 280 (8.6%); 42 (2.3%) among RA, 90 (2.3%) among other AIRDs, and 124 (3.6%) among HCs. The prevalence of first BI in patients with RA was higher than that in those with AIRDs (OR=1.2; 95%CI=1.1-1.4; p=0.001) and HCs (OR=1.4; 95%CI=1.2-1.6; p<0.001), but similar to nrAIDs (p=0.783). The prevalence of second BI was lower in patients with RA than in HCs (OR=0.6; 95%CI=0.4-0.9; p=0.012) and nrAIDs (OR=0.4; 95%CI=0.2-0.7; p=0.004), but similar to AIRDs (p=0.991). When compared with HCs, patients with RA reported significantly higher joint pain, hospitalizations, and need for advanced treatment at first BI. Patients with RA from very high HDI countries had lower hazard of first BI than those from high HDI countries (HR=0.026; 95%CI=0.001-0.6; p=0.027). Rituximab use predicted more frequent hospitalization (OR=3.4; 95%CI=1.3-11.4; p=0.045) and severe BI (OR=3.0; 95%CI=1.2-7.3; p=0.014). Conclusion: Nearly one in five patients with RA reported BI. BI prevalence was higher in pat
Published: 2023
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19. IMPAIRED HEALTH-RELATED QUALITY OF LIFE IN PATIENTS WITH IDIOPATHIC INFLAMMATORY MYOPATHIES : A CROSS-SECTIONAL ANALYSIS FROM AN INTERNATIONAL SURVEY

Author: Yoshida, A., Li, Y., Maroufy, V., Kuwana, M., Ravichandran, N., Makol, A., Sen, P., Lilleker, J. B., Agarwal, V., Kardes, S., Day, J., Milchert, M., Joshi, M., Gheita, T. A., Salim, B., Velikova, T., Gracia-Ramos, A. E., Parodis, Ioannis, Nikiphorou, E., Tan, A. L., Nune, A., Cavagna, L., Saavedra, M. A., Katsuyuki Shinjo, S., Ziade, N., Knitza, J., Distler, O., Chinoy, H., Aggarwal, R., Gupta, L., Yoshida, A., Li, Y., Maroufy, V., Kuwana, M., Ravichandran, N., Makol, A., Sen, P., Lilleker, J. B., Agarwal, V., Kardes, S., Day, J., Milchert, M., Joshi, M., Gheita, T. A., Salim, B., Velikova, T., Gracia-Ramos, A. E., Parodis, Ioannis, Nikiphorou, E., Tan, A. L., Nune, A., Cavagna, L., Saavedra, M. A., Katsuyuki Shinjo, S., Ziade, N., Knitza, J., Distler, O., Chinoy, H., Aggarwal, R., and Gupta, L.
Abstract: Background: Comprehensive and large-scale assessment of health-related quality of life in patients with idiopathic inflammatory myopathies (IIMs) worldwide is lacking. The second COVID-19 vaccination in autoimmune disease (COVAD-2) study [1] is an international, multicentre, self-reported e-survey assessing several aspects of COVID-19 infection and vaccination as well as validated patient-reported outcome measures (PROMs) to outline patient experience in various autoimmune diseases (AIDs), with a particular focus on IIMs. Objectives: To investigate physical and mental health in a global cohort of IIM patients compared to those with non-IIM autoimmune inflammatory rheumatic diseases (AIRDs), non-rheumatic AIDs (NRAIDs), and those without AIDs (controls), using Patient-Reported Outcome Measurement Information System (PROMIS) global health data obtained from the COVAD-2 survey. Methods: Demographics, AID diagnoses, comorbidities, disease activity, treatments, and PROMs were extracted from the COVAD-2 database. The primary outcomes were PROMIS Global Physical Health (GPH) and Global Mental Health (GMH) scores. Secondary outcomes included PROMIS physical function short form-10a (PROMIS PF-10a), pain visual analogue scale (VAS), and PROMIS Fatigue-4a scores. Each outcome was compared between IIMs, non-IIM AIRDs, NRAIDs, and controls. Factors affecting GPH and GMH scores in IIMs were identified using multivariable regression analysis. Results: A total of 10,502 complete responses from 1582 IIMs, 4700 non-IIM AIRDs, 545 NRAIDs, and 3675 controls, which accrued as of May 2022, were analysed. Patients with IIMs were older [59±14 (IIMs) vs. 48±14 (non-IIM AIRDs) vs. 45±14 (NRAIDs) vs. 40±14 (controls) years, p<0.001] and more likely to be Caucasian [82.7% (IIMs) vs. 53.2% (non-IIM AIRDs) vs. 62.4% (NRAIDs) vs. 34.5% (controls), p<0.001]. Among IIMs, dermatomyositis (DM) and juvenile DM were the most common (31.4%), followed by inclusion body myositis (IBM) (24.9%). Patie
Published: 2023
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20. COMORBIDITIES, COMPLEX MULTIMORBIDITY AND PROMIS HEALTH OUTCOMES AMONGAUTOIMMUNE RHEUMATIC DISEASES : DATA FROM THE COVAD STUDY

Author: Gupta, L., Ravichandran, N., Venerito, V., Dey, M., Sen, P., Saha, S., Tan, A. L., Shinjo, S. Katsuyuki, Agarwal, V., Joshi, M., Ziade, N., Velikova, T., Jagtap, K., Milchert, M., Parodis, Ioannis, Gracia-Ramos, A. E., Cavagna, L., Kuwana, M., Knitza, J., Makol, A., Dzifa, D., Gutierrez, C. E. Toro, Caballero, C. Vinicio, Distler, O., Day, J., Chinoy, H., Aggarwal, R., Nikiphorou, E., Gupta, L., Ravichandran, N., Venerito, V., Dey, M., Sen, P., Saha, S., Tan, A. L., Shinjo, S. Katsuyuki, Agarwal, V., Joshi, M., Ziade, N., Velikova, T., Jagtap, K., Milchert, M., Parodis, Ioannis, Gracia-Ramos, A. E., Cavagna, L., Kuwana, M., Knitza, J., Makol, A., Dzifa, D., Gutierrez, C. E. Toro, Caballero, C. Vinicio, Distler, O., Day, J., Chinoy, H., Aggarwal, R., and Nikiphorou, E.
Abstract: Background: Comorbidities have a profound impact on the QoL of patients living with autoimmune rheumatic diseases (AIRDs). Unfortunately, global data on the burden of comorbidities and its impact on health outcomes in this vulnerable group is scarce. Objectives: We studied the prevalence, distribution and clustering of comorbidities and multimorbidity among patients with AIRDs and healthy controls (HCs) and its impact on health outcomes, utilizing data from the ongoing 2nd COVAD study. Methods: The COVAD study is a global e-survey that embodies patient voice while empowering collaborators and young researchers. The study group of 157 physicians across 106 countries from February-June 2022 captured details of AIRDs, autoimmune and non-autoimmune comorbidities, and validated patient reported outcomes. Human Development Index (UNDP 2021-22) of country of residence was taken as a surrogate marker for socioeconomic status (SES). Basic multimorbidity (BM), Complex multimorbidity (CM), Autoimmune multimorbidity (AM) are defined as the co-occurrence of ≥2 non-rheumatic comorbidities, ≥3 non-rheumatic chronic conditions affecting ≥3 different organ systems [1] and ≥3 autoimmune diseases (AIDs) in an individual respectively. PROMIS global physical health (PGP), mental health (PGM), fatigue 4a (F4a) and physical function short form (SF10) scores were calculated for the different groups and compared using descriptive statistics, linear regression and cluster analysis (hierarchical followed by K means). Results: Of 17,612 total respondents, 6149 (62.7%) had underlying AIRDs and 3652 (37.3%) were HCs, with female (80.8%) and Caucasian (53.9%) predominance in the former. All types of multimorbidity were more frequent in AIRDs than HCs, including any comorbidity (77.1% versus 25.0%; OR: 2.9; 2.7-3.2), BM (21.0% vs 6.2%; 4.0; 3.4-4.6), and CM (3.1% vs 0.5%; 6.4; 3.9-10.4), and with prevalence increasing with age (p<0.001) (Figure 1A, B). Comorbidity prevalence was the highest amo
Published: 2023
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21. MULTIMORBIDITY AND PROMIS HEALTH OUTCOMES IN PATIENTS WITH IDIOPATHIC INFLAMMATORY MYOPATHIES : DATA FROM A LARGE, GLOBAL E-SURVEY (COVAD STUDY)

Author: Fornaro, M., Venerito, V., Iannone, F., Ravichandran, N., Nikiphorou, E., Joshi, M., Tan, A. L., Saha, S., Shinjo, S. Katsuyuki, Agarwal, V., Ziade, N., Velikova, T., Jagtap, K., Milchert, M., Parodis, Ioannis, Gracia-Ramos, A. E., Cavagna, L., Kuwana, M., Knitza, J., Makol, A., Dzifa, D., Toro Gutierrez, C. E., Vinicio Caballero, C., Distler, O., Day, J., Chinoy, H., Aggarwal, R., Gupta, L., Fornaro, M., Venerito, V., Iannone, F., Ravichandran, N., Nikiphorou, E., Joshi, M., Tan, A. L., Saha, S., Shinjo, S. Katsuyuki, Agarwal, V., Ziade, N., Velikova, T., Jagtap, K., Milchert, M., Parodis, Ioannis, Gracia-Ramos, A. E., Cavagna, L., Kuwana, M., Knitza, J., Makol, A., Dzifa, D., Toro Gutierrez, C. E., Vinicio Caballero, C., Distler, O., Day, J., Chinoy, H., Aggarwal, R., and Gupta, L.
Abstract: Background: Prevalence of comorbidities and their impact on health outcomes in Idiopathic inflammatory myopathies (IIMs) is limited. Objectives: This study aimed to explore the prevalence of multimorbidity in patients with IIMs, other autoimmune rheumatic diseases (AIRDs) and Healthy controls (HCs). We further explore the impact of comorbidities on patients’ physical, mental, and social health assessed by the Patient-Reported Outcome Measurement Information System (PROMIS instruments). Methods: Data for this study were acquired from the COVAD 2 e-survey hosted by a study group consisting of 167 collaborators in 110 countries. Basic multimorbidity (BM) was defined as the co-occurrence of two or more comorbidities in an individual, while complex multimorbidity (CM) signified the co-occurrence of 3 or more chronic conditions affecting 3 or more different organ systems. PROMIS global physical health (PGP), mental health (PGM), fatigue 4a (F4a) and physical function short form (SF10) were analysed using descriptive statistics and linear regression models. Hierarchical Clustering on Principal Components was performed to outline the grouping. Results: Of 10740 complete respondents, 1558 IIMs, 4591 AIRDs and 3652 HCs were analysed. Individuals with IIMs exhibited high burden of any comorbidity (OR: 1.62 vs AIRDs and 2.95 vs HCs,p<0.01), BM (OR 1.66 vs AIRDs and 3.52 vs HCs,p<0.01), CM (OR: 1.69 vs AIRDs and 6.23 vs HCs,p<0.01), and mental health disorders (MHDs) (OR 1.33 vs AIRDs and 2.63 vs HCs,p<0.01). IIM patients with comorbidities (and MHDs) had worse physical function (low PGP, PGM, SF10 and higher F4a scores, all p<0.001). Worse physical function (PGP) was predicted by age (0.35; 0.030), active disease (-1.51; <0.001), BM (-1.11; <0.001), and MHDs (-1.47; <0.001). PGM was impacted by age (0.51; 0.004), active disease (-1.34, <0.001), BM (-0.75; 0.001) and MHDs (-2.22; <0.001). Determinants of SF10a were age (-3.86; <0.001), active dis
Published: 2023
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22. Systemic sclerosis and COVID-19 vaccine safety: short-term insights from the global COVID-19 vaccination in autoimmune disease (COVAD) survey

Author: Naveen, R., Thakare, D. R., Kuwana, M., Pauling, J. D., Day, J., Joshi, M., Parodis, I., Sen, P., Jagtap, K., Nikiphorou, E., Saha, S., Agarwal, V., Chatterjee, T., Lilleker, J. B., Kardes, S., Milchert, M., Gheita, T., Salim, B., Velikova, T., Gracia-Ramos, A. E., Tan, A. L., Nune, A., Cavagna, L., Saavedra, M. A., Shinjo, S. K., Ziade, N., Knitza, J., Distler, O., Chinoy, H., Barman, B., Singh, Y. P., Ranjan, R., Jain, A., Pandya, S. C., Pilania, R. K., Sharma, A., Manesh Manoj, M., Gupta, V., Kavadichanda, C. G., Patro, P. S., Ajmani, S., Phatak, S., Goswami, R. P., Chowdhury, A. C., Mathew, A. J., Shenoy, P., Asranna, A., Bommakanti, K. T., Shukla, A., Pande, A. R., Chandwar, K., Cansu, D. U., Wincup, C., Makol, A., Del Papa, N., Sambataro, G., Fabiola, A., Govoni, M., Parisi, S., Bocci, E. B., Sebastiani, G. D., Fusaro, E., Sebastiani, M., Quartuccio, L., Franceschini, F., Sainaghi, P. P., Orsolini, G., De Angelis, R., Danielli, M. G., Venerito, V., Traboco, L. S., Wibowo, S. A. K., Serrano, J. R., La Torre, I. G. -D., Tehozol, E. A. Z., Loarce-Martos, J., Prieto-Gonzalez, S., Gonzalez, R. A., Yoshida, A., Nakashima, R., Sato, S., Kimura, N., Kaneko, Y., Tomaras, S., Gromova, M. A., Aharonov, O., Hmamouchi, I., Hoff, L. S., Giannini, M., Maurier, F., Campagne, J., Meyer, A., Nagy-Vincze, M., Langguth, D., Limaye, V., Needham, M., Srivastav, N., Hudson, M., Landon-Cardinal, O., Shaharir, S. S., Zuleta, W. G. R., Silva, J. A. P., Fonseca, J. E., Zimba, O., Aggarwal, R., and Gupta, L.
Subjects: Adverse events, Autoimmune diseases, Vaccination, COVID-19, Systemic sclerosis
Published: 2023

23. Vaccine hesitancy decreases in rheumatic diseases, long-term concerns remain in myositis: a comparative analysis of the COVAD surveys

Author: Sen, P, Naveen, R, Houshmand, N, Kia, Sm, Joshi, M, Saha, S, Jagtap, K, Agarwal, V, Nune, A, Nikiphorou, E, Tan, Al, Shinjo, Sk, Ziade, N, Velikova, T, Milchert, M, Parodis, I, Gracia-Ramos, Ae, Cavagna, L, Kuwana, M, Knitza, J, Makol, A, Patel, A, Pauling, Jd, Wincup, C, Barman, B, Tehozol, Eaz, Serrano, Jr, de la Torre, I, Colunga-Pedraza, Ij, Merayo-Chalico, J, Chibuzo, Oc, Katchamart, W, Goo, Pa, Shumnalieva, R, Chen, Ym, Hoff, Ls, El Kibbi, L, Halabi, H, Vaidya, B, Shaharir, Ss, Hasan, Atmt, Dey, D, Gutierrez, Cet, Caballero-Uribe, Cv, Lilleker, Jb, Salim, B, Gheita, T, Chatterjee, T, Distler, O, Saavedra, Ma, Day, J, Chinoy, H, COVAD Study Grp, Kardes, S, Kardes, Sa, Andreoli, L, Lini, D, Screiber, K, Vince, Mn, 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, Ghodke, A, Boro, H, Fazal, Zz, Cansu, Du, Yildirim, R, Gasparyan, Ay, Del Papa, N, Sambataro, G, Fabiola, A, Govoni, M, Parisi, S, Bocci, Eb, Sebastiani, Gd, Fusaro, E, Sebastiani, M, Quartuccio, L, Franceschini, F, Sainaghi, Pp, Orsolini, G, De Angelis, R, Danielli, Mg, Venerito, V, Grignaschi, S, Giollo, A, Alluno, A, Ioannone, F, Fornaro, M, Traboco, Ls, Wibowo, Sak, Loarce-Martos, J, Prieto-Gonzalez, S, Gonzalez, Ra, Yoshida, A, Nakashima, R, Sato, S, Kimura, N, Kaneko, Y, Gono, T, Tomaras, S, Proft, Fn, Holzer, Mt, Gromova, Ma, Aharonov, O, Griger, Z, Hmamouchi, I, El Bouchti, I, Baba, Z, Giannini, M, Maurier, F, Campagne, J, Meyer, A, Langguth, D, Limaye, V, Needham, M, Srivastav, N, Hudson, M, Landon-Cardinal, O, Zuleta, Wgr, Arbelaez, A, Cajas, J, Silva, Jap, Fonseca, Je, Zimba, O, Bohdana, D, Ima-Edomwonyi, U, Dedeke, I, Airenakho, E, Madu, Nh, Yerima, A, Olaosebikan, H, Becky, A, Koussougbo, Od, Palalane, E, So, H, Ugarte-Gil, Mf, Chinchay, L, Bernaola, Jp, Pimentel, V, Fathi, Hm, Mohammed, Rha, Harifi, G, Fuentes-Silva, Y, Cabriza, K, Losanto, J, Colaman, N, Cachafeiro-Vilar, A, Bautista, Gg, Ejg, Ho, Gonzalez, R, Nunez, Ls, Vergara, Mc, Baez, Jt, Alonzo, H, Pastelin, Cbs, Salinas, Rg, Obiols, Aq, Chavez, N, Ordonez, Ab, Argueta, S, Llerena, Gar, Sierra-Zorita, R, Arrieta, D, Hidalgo, Er, Saenz, R, Escalante, Mi, Morales, R, Calapaqui, W, Quezada, I, Arredondo, G, Aggarwal, R, and Gupta, L
Subjects: Rheumatology, idiopathic inflammatory myopathies, COVID-19 vaccines, vaccine hesitancy, registries, Pharmacology (medical), autoimmune disease
Abstract: ObjectiveCOVID-19 vaccines have a favorable safety profile in patients with autoimmune rheumatic diseases (AIRDs) such as idiopathic inflammatory myopathies (IIMs); however, hesitancy continues to persist among these patients. Therefore, we studied the prevalence, predictors and reasons for hesitancy in patients with IIMs, other AIRDs, non-rheumatic autoimmune diseases (nrAIDs) and healthy controls (HCs), using data from the two international COVID-19 Vaccination in Autoimmune Diseases (COVAD) e-surveys.MethodsThe first and second COVAD patient self-reported e-surveys were circulated from March to December 2021, and February to June 2022 (ongoing). We collected data on demographics, comorbidities, COVID-19 infection and vaccination history, reasons for hesitancy, and patient reported outcomes. Predictors of hesitancy were analysed using regression models in different groups.ResultsWe analysed data from 18 882 (COVAD-1) and 7666 (COVAD-2) respondents. Reassuringly, hesitancy decreased from 2021 (16.5%) to 2022 (5.1%) (OR: 0.26; 95% CI: 0.24, 0.30, P ConclusionVaccine hesitancy has decreased from 2021 to 2022, long-term safety concerns remain among patients with IIMs, particularly in Caucasians and those with poor physical function.
Published: 2023

24. Anti-Ro52 antibodies positivity in antisynthetase syndrome: a single centre cohort study

Author: Lettieri, S, primary, Bozzalla-Cassione, E, additional, Zanframundo, G, additional, Vertui, V, additional, Della Zoppa, M, additional, Chino, V, additional, Bertuccio, F, additional, Cavagna, L, additional, and Meloni, F, additional
Published: 2022
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25. Cobalt hip prosthesis intoxication mimicking an autoimmune disease

Author: Biglia, A, Morandi, V, Monti, S, Delvino, P, Cavagna, L, Montecucco, C, Biglia A., Morandi V., Monti S., Delvino P., Cavagna L., Montecucco C., Biglia, A, Morandi, V, Monti, S, Delvino, P, Cavagna, L, Montecucco, C, Biglia A., Morandi V., Monti S., Delvino P., Cavagna L., and Montecucco C.
Abstract: Les prothèses de hanche à couple de frottement métal-métal contenant du cobalt peuvent être à l'origine d'une toxicité systémique provoquée par le relargage d'ions cobalt dans la circulation sanguine. Des taux élevés de cobalt dans le sang peuvent provoquer diverses manifestations cliniques imitant d'autres maladies, notamment auto-immunes, hématologiques et infectieuses. Nous rapportons un cas d'intoxication au cobalt à partir d'une prothèse de hanche, imitant une maladie auto-immune avec des signes d'inflammation systémique, des douleurs articulaires et musculaires sans synovite et la positivité de plusieurs auto-anticorps. Une femme de 69 ans présentait depuis un an une douleur de hanche droite, une fièvre récurrente, des arthralgies et myalgies, des adénopathies médiastinale et iliaque droite. Elle avait une prothèse de hanche depuis sept ans. L'examen physique était normal, à l'exception d' une douleur de la hanche droite. Les analyses biologiques ont révélé une nette élévation des marqueurs de l'inflammation et tous les tests microbiologiques étaient négatifs. Une ponction articulaire guidée par échographie de la hanche droite a permis de prélever un liquide limpide dont la mise en culture s'est avérée négative. Des taux élevés de cobalt dans le plasma et les urines indiquaient la présence d'une intoxication à ce métal. L'imagerie par résonance magnétique en séquence de réduction des artéfacts métalliques (MARS) a confirmé la présence d'une masse périprothétique caractéristique d'une réaction à des débris métalliques. Les manifestations cliniques et les taux de cobalt se sont normalisés après le remplacement de la prothèse.
Published: 2021

26. Combined interventional sialendoscopy and intraductal steroid therapy for recurrent sialadenitis in Sjögrenʼs syndrome: Results of a pilot monocentric trial

Author: Capaccio, P., Canzi, P., Torretta, S., Rossi, V., Benazzo, M., Bossi, A., Vitali, C., Cavagna, L., and Pignataro, L.
Published: 2018
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27. COVID‐19 Vaccination In Autoimmune Diseases (COVAD) Study: Vaccine Safety In Idiopathic Inflammatory Myopathies

Author: Gil‐Vila, A, Naveen, R, Selva‐O'Callaghan, A, Sen, P, Nune, A, Gaur, PS, Gonzalez, RA, Lilleker, JB, Joshi, M, Agarwal, V, Kardes, S, Kim, M, Day, J, Makol, A, Milchert, M, Gheita, T, Salim, B, Velikova, T, Gracia‐Ramos, AE, Parodis, I, Nikiphorou, E, Tan, AL, Chatterjee, T, Cavagna, L, Saavedra, MA, Shinjo, SK, Ziade, N, Knitza, J, Kuwana, M, Distler, O, Chinoy, H, Aggarwal, R, Gupta, L, and COVAD Study Group
Abstract: Introduction/Aims We studied COVID-19 vaccination-related adverse events (ADEs) 7-days post-vaccination in patients with idiopathic inflammatory myopathies (IIMs) and other systemic autoimmune and inflammatory disorders (SAIDs). Methods 7-day vaccine ADEs were collected in an international patient self-reported e-survey. Descriptive statistics and multivariable regression were performed. Results 10,900 respondents [1227 IIMs; 4640 SAIDs; 5033 healthy controls (HCs), median age 42 (IQR 30-55) years, 74% female, 45% Caucasian, 69% completely vaccinated] were analysed. 76.3% IIMs patients reported minor and 4.6% major ADEs. Patients with active IIMs reported more frequent major [OR 2.7 (1.04-7.3)] and minor [OR 1.5 (1.1-2.2)] ADEs than inactive IIMs. Rashes were more frequent in IIMs [OR-2.3(1.2-4.2)] than HCs. ADEs were not impacted by steroid dose, although hydroxychloroquine and intravenous/subcutaneous immunoglobulins were associated with a higher risk of minor ADEs [OR 1.9 (1.1-3.3), OR 2.2 (1.1-4.3)]. Overall, ADEs were less frequent in inclusion body myositis (IBM) and BNT162b2 (Pfizer) vaccine recipients Discussion 7-day post-vaccination ADEs were comparable in patients with IIMs, SAIDs, and HCs, except for a higher risk of rashes in IIMs. Patients with DM, active disease may be at higher risk, and IBM patients at lower risk of specific ADEs. Overall, the benefit of preventing severe COVID-19 through vaccination likely outweighs the risk of vaccine-related ADEs Our results may inform future guidelines regarding COVID-19 vaccination in patients with SAIDs, and specifically in IIMs. Studies to evaluate long-term outcomes and disease flares are needed to shed more light on developing future COVID-19 vaccination guidelines.
Published: 2022

28. Multidisciplinary Approach in the Early Detection of Undiagnosed Connective Tissue Diseases in Patients With Interstitial Lung Disease: A Retrospective Cohort Study

Author: Tirelli, C, Morandi, V, Valentini, A, La Carrubba, C, Dore, R, Zanframundo, G, Morbini, P, Grignaschi, S, Franconeri, A, Oggionni, T, Marasco, E, De Stefano, L, Kadija, Z, Mariani, F, Codullo, V, Alpini, C, Scire, C, Montecucco, C, Meloni, F, Cavagna, L, Tirelli C., Morandi V., Valentini A., La Carrubba C., Dore R., Zanframundo G., Morbini P., Grignaschi S., Franconeri A., Oggionni T., Marasco E., De Stefano L., Kadija Z., Mariani F., Codullo V., Alpini C., Scire CA., Montecucco C., Meloni F., Cavagna L., Tirelli, C, Morandi, V, Valentini, A, La Carrubba, C, Dore, R, Zanframundo, G, Morbini, P, Grignaschi, S, Franconeri, A, Oggionni, T, Marasco, E, De Stefano, L, Kadija, Z, Mariani, F, Codullo, V, Alpini, C, Scire, C, Montecucco, C, Meloni, F, Cavagna, L, Tirelli C., Morandi V., Valentini A., La Carrubba C., Dore R., Zanframundo G., Morbini P., Grignaschi S., Franconeri A., Oggionni T., Marasco E., De Stefano L., Kadija Z., Mariani F., Codullo V., Alpini C., Scire CA., Montecucco C., Meloni F., and Cavagna L.
Abstract: Interstitial lung disease (ILD) encompasses a wide range of parenchymal lung pathologies with different clinical, histological, radiological, and serological features. Follow-up, treatment, and prognosis are strongly influenced by the underlying pathogenesis. Considering that an ILD may complicate the course of any connective tissue disease (CTD) and that CTD's signs are not always easily identifiable, it could be useful to screen every ILD patient for a possible CTD. The recent definition of interstitial pneumonia with autoimmune features is a further confirmation of the close relationship between CTD and ILD. In this context, the multidisciplinary approach is assuming a growing and accepted role in the correct diagnosis and follow-up, to as early as possible define the best therapeutic strategy. However, despite clinical advantages, until now, the pathways of the multidisciplinary approach in ILD patients are largely heterogeneous across different centers and the best strategy to apply is still to be established and validated. Aims of this article are to describe the organization of our multidisciplinary group for ILD, which is mainly focused on the early identification and management of CTD in patients with ILD and to show our results in a 1 year period of observation. We found that 15% of patients referred for ILD had an underlying CTD, 33% had interstitial pneumonia with autoimmune feature, and 52% had ILD without detectable CTD. Furthermore, we demonstrated that the adoption of a standardized strategy consisting of a screening questionnaire, specific laboratory tests, and nailfold videocapillaroscopy in all incident ILD proved useful in making the right diagnosis.
Published: 2020

29. Cobalt hip prosthesis intoxication mimicking an autoimmune disease

Author: Biglia, A, Morandi, V, Monti, S, Delvino, P, Cavagna, L, Montecucco, C, Biglia A., Morandi V., Monti S., Delvino P., Cavagna L., Montecucco C., Biglia, A, Morandi, V, Monti, S, Delvino, P, Cavagna, L, Montecucco, C, Biglia A., Morandi V., Monti S., Delvino P., Cavagna L., and Montecucco C.
Abstract: Cobalt-containing hip prosthesis may cause systemic toxicity due to the release of cobalt from metal-on-metal (MoM) joint arthroplasty into the bloodstream. High cobalt blood levels can lead to a variety of clinical manifestations, mimicking other disorders, especially autoimmune, hematologic, and infectious diseases. Our purpose is to describe a clinical case of cobalt hip prosthesis intoxication mimicking an autoimmune disease, with systemic inflammation signs, arthro-myalgias unrelated to overt synovitis, and multiple autoantibody positivity. A 69-years-old woman presented with a 1-year history of right coxalgia, recurrent fever, arthro-myalgias, mediastinal and right iliac reactive lymphadenopathy. She underwent hip replacement surgery seven years earlier. The physical examination was unremarkable except for right hip pain. Laboratory tests showed markedly increased inflammatory indices and microbiological tests were all negative. Ultrasound-guided arthrocentesis of right hip yielded limpid fluid with negative cultures. Increased cobalt levels in plasma and urine showed metal intoxication. Magnetic resonance imaging with metal artifact reduction sequence (MARS) confirmed a periprosthetic mass as usually seen in reaction to metal debris. Prosthesis substitution was performed with a resolution of the clinical picture and normalization of cobalt levels.
Published: 2020

30. Successful treatment with baricitinib in a patient with refractory cutaneous dermatomyositis

Author: Delvino, P, Bartoletti, A, Monti, S, Biglia, A, Montecucco, C, Carducci, M, Ripamonti, G, Cavagna, L, Delvino P., Bartoletti A., Monti S., Biglia A., Montecucco C., Carducci M., Ripamonti G., Cavagna L., Delvino, P, Bartoletti, A, Monti, S, Biglia, A, Montecucco, C, Carducci, M, Ripamonti, G, Cavagna, L, Delvino P., Bartoletti A., Monti S., Biglia A., Montecucco C., Carducci M., Ripamonti G., and Cavagna L.
Published: 2020

31. COVAD survey 2 long-term outcomes: unmet need and protocol

Author: Fazal, Z.Z., Sen, P., Joshi, M., Ravichandran, N., Lilleker, J.B., Agarwal, V., Kardes, S., Kim, M., Day, J., Makol, A., Milchert, M., Gheita, T., Salim, B., Velikova, T., Gracia-Ramos, A.E., Parodis, I., Nikiphorou, E., Tan, A.L., Chatterjee, T., Cavagna, L., Saavedra, M.A., Shinjo, S.K., Ziade, N., Selva-O’Callaghan, A., Nune, A., Knitza, J., Kuwana, M., Gutiérrez, C-E.T., Caballero-Uribe, C.V., Dey, D., Distler, O., Chinoy, H., Aggarwal, R., Gupta, L., Barman, B., Singh, Y.P., Ranjan, R., Jain, A., Pandya, S.C., Pilania, R.K., Sharma, A., Manoj, M.M., Gupta, V., Kavadichanda, C.G., Patro, Pr.S., Ajmani, S., Phatak, S., Goswami, R.P., Chowdhury, A.C., Mathew, A.J., Shenoy, P., Asranna, A., Bommakanti, K.T., Shukla, A., Pandey, A.K.R., Gaur, P.S., Mamadapur, M., Ghodke, A., Chandwar, K., Jagtap, K., Cansu, D.Ü., Yıldırım, R., Patel, A., Pauling, J.D., Wincup, C., Giannini, M., Maurier, F., Campagne, J., Meyer, A., Del Papa, N., Sambataro, G., Fabiola, A., Govoni, M., Parisi, S., Bocci, E.B., Sebastiani, G.D., Fusaro, E., Sebastiani, M., Quartuccio, L., Franceschini, F., Sainaghi, P.P., Orsolini, G., De Angelis, R., Danielli, M.G., Venerito, V., Grignaschi, S., Giollo, A., Traboco, L.S., Shaharir, S.S., Wibowo, S.A.K., Tehozol, E.A.Z., Serrano, J.R., De La Torre, I.G., Colunga‑Pedraza, I.J., Merayo-Chalico, J., Loarce-Martos, J., Prieto-González, S., Gil-Vila, A., Aranega, R., Hoff, L.S., Nakashima, R., Sato, S., Kimura, N., Kaneko, Y., Tomaras, S., Proft, F.N., Holzer, M-T, Gromova, M.A., Aharonov, O., Nagy-Vincze, M., Griger, Z., Hmamouchi, I., El bouchti, P.I., Baba, Z., Ima-Edomwonyi, U., Dedeke, I., Airenakho, E., Madu, N.H., Yerima, A., Olaosebikan, H., Chibuzo, O.C., Becky, A., Koussougbo, O.D., Palalane, E., Langguth, D., Limaye, V., Needham, M., Srivastava, N., Hudson, M., Landon-Cardinal, O., Zuleta, W.G.R., Arbeláez, Á., Cajas, J., Silva, J.A.P., Fonseca, J.E., Zimba, O., Bohdana, D., So, H., Ugarte-Gil, M.F., Chinchay, L., Bernaola, J.P., Pimentel, V., Tanveer Hasan, A.T.M., Saha, S., Vaidya, B., Fathi, H.M., Mohammed, R.H.A., Chen, Y-M, Harifi, G., El Kibbi, L., Halabi, H.M., Akawatcharangura, P., Katchamart, W., Fuentes-Silva, Y., Cabriza, K., Losanto, J., Colaman, N., Cachafeiro-Vilar, A., Bautista, G.G., Ho, E.J.G., González, R.A., Nunez, L.S., Vergara, C.M., Báez, J.T., Alonzo, H., Pastelin, C.B.S., Salinas, R.G., Obiols, A.Q., Chávez, N., Ordóñez, A.B., Argueta, S., Quijivix, D., Llerena, G.A.R., Sierra-Zorita, R., Arrieta, D., Hidalgo, E.R., Saenz, R., M., I.E., Morales, R., Calapaqui, W., Quezada, I., Arredondo, G., Fazal, Z.Z., Sen, P., Joshi, M., Ravichandran, N., Lilleker, J.B., Agarwal, V., Kardes, S., Kim, M., Day, J., Makol, A., Milchert, M., Gheita, T., Salim, B., Velikova, T., Gracia-Ramos, A.E., Parodis, I., Nikiphorou, E., Tan, A.L., Chatterjee, T., Cavagna, L., Saavedra, M.A., Shinjo, S.K., Ziade, N., Selva-O’Callaghan, A., Nune, A., Knitza, J., Kuwana, M., Gutiérrez, C-E.T., Caballero-Uribe, C.V., Dey, D., Distler, O., Chinoy, H., Aggarwal, R., Gupta, L., Barman, B., Singh, Y.P., Ranjan, R., Jain, A., Pandya, S.C., Pilania, R.K., Sharma, A., Manoj, M.M., Gupta, V., Kavadichanda, C.G., Patro, Pr.S., Ajmani, S., Phatak, S., Goswami, R.P., Chowdhury, A.C., Mathew, A.J., Shenoy, P., Asranna, A., Bommakanti, K.T., Shukla, A., Pandey, A.K.R., Gaur, P.S., Mamadapur, M., Ghodke, A., Chandwar, K., Jagtap, K., Cansu, D.Ü., Yıldırım, R., Patel, A., Pauling, J.D., Wincup, C., Giannini, M., Maurier, F., Campagne, J., Meyer, A., Del Papa, N., Sambataro, G., Fabiola, A., Govoni, M., Parisi, S., Bocci, E.B., Sebastiani, G.D., Fusaro, E., Sebastiani, M., Quartuccio, L., Franceschini, F., Sainaghi, P.P., Orsolini, G., De Angelis, R., Danielli, M.G., Venerito, V., Grignaschi, S., Giollo, A., Traboco, L.S., Shaharir, S.S., Wibowo, S.A.K., Tehozol, E.A.Z., Serrano, J.R., De La Torre, I.G., Colunga‑Pedraza, I.J., Merayo-Chalico, J., Loarce-Martos, J., Prieto-González, S., Gil-Vila, A., Aranega, R., Hoff, L.S., Nakashima, R., Sato, S., Kimura, N., Kaneko, Y., Tomaras, S., Proft, F.N., Holzer, M-T, Gromova, M.A., Aharonov, O., Nagy-Vincze, M., Griger, Z., Hmamouchi, I., El bouchti, P.I., Baba, Z., Ima-Edomwonyi, U., Dedeke, I., Airenakho, E., Madu, N.H., Yerima, A., Olaosebikan, H., Chibuzo, O.C., Becky, A., Koussougbo, O.D., Palalane, E., Langguth, D., Limaye, V., Needham, M., Srivastava, N., Hudson, M., Landon-Cardinal, O., Zuleta, W.G.R., Arbeláez, Á., Cajas, J., Silva, J.A.P., Fonseca, J.E., Zimba, O., Bohdana, D., So, H., Ugarte-Gil, M.F., Chinchay, L., Bernaola, J.P., Pimentel, V., Tanveer Hasan, A.T.M., Saha, S., Vaidya, B., Fathi, H.M., Mohammed, R.H.A., Chen, Y-M, Harifi, G., El Kibbi, L., Halabi, H.M., Akawatcharangura, P., Katchamart, W., Fuentes-Silva, Y., Cabriza, K., Losanto, J., Colaman, N., Cachafeiro-Vilar, A., Bautista, G.G., Ho, E.J.G., González, R.A., Nunez, L.S., Vergara, C.M., Báez, J.T., Alonzo, H., Pastelin, C.B.S., Salinas, R.G., Obiols, A.Q., Chávez, N., Ordóñez, A.B., Argueta, S., Quijivix, D., Llerena, G.A.R., Sierra-Zorita, R., Arrieta, D., Hidalgo, E.R., Saenz, R., M., I.E., Morales, R., Calapaqui, W., Quezada, I., and Arredondo, G.
Abstract: Vaccine hesitancy is considered a major barrier to achieving herd immunity against COVID-19. While multiple alternative and synergistic approaches including heterologous vaccination, booster doses, and antiviral drugs have been developed, equitable vaccine uptake remains the foremost strategy to manage pandemic. Although none of the currently approved vaccines are live-attenuated, several reports of disease flares, waning protection, and acute-onset syndromes have emerged as short-term adverse events after vaccination. Hence, scientific literature falls short when discussing potential long-term effects in vulnerable cohorts. The COVAD-2 survey follows on from the baseline COVAD-1 survey with the aim to collect patient-reported data on the long-term safety and tolerability of COVID-19 vaccines in immune modulation. The e-survey has been extensively pilot-tested and validated with translations into multiple languages. Anticipated results will help improve vaccination efforts and reduce the imminent risks of COVID-19 infection, especially in understudied vulnerable groups.
Published: 2022

32. Is it really infective endocarditis? Distinguishing systemic vasculitis from its mimics

Author: Biglia, A, Monti, S, Morandi, V, Delvino, P, Bellis, E, Rossi, S, Cavagna, L, Mugellini, A, Canino, C, Bogliolo, L, Montecucco, C, Biglia, A, Monti, S, Morandi, V, Delvino, P, Bellis, E, Rossi, S, Cavagna, L, Mugellini, A, Canino, C, Bogliolo, L, and Montecucco, C
Published: 2022

33. D-dimer and reduced-dose apixaban for extended treatment after unprovoked venous thromboembolism: the Apidulcis study

Author: Palareti, G., Poli, D., Pesavento, R., Legnani, C., Antonucci, E., Bucherini, E., Testa, S., Paoletti, O., Chistolini, A., Ceccato, D., Martinelli, I., Bucciarelli, P., Falanga, A., Tosetto, A., Sarti, L., Mastroiacovo, D., Cosmi, B., Visona, A., Santoro, R. C., Zanatta, N., Grandone, E., Bertu, L., Pengo, V., Caiano, L., Prandoni, P., Lotti, E., Crudele, F., Ageno, W., Abenante, A., Colombo, G., Guarascio, M., Cancellieri, E., Morandini, R., Zambelli, S., Martini, S., Vastola, M., Serrao, A., Abbattista, M., Artoni, A., Capecchi, M., Gianniello, F., Scimeca, B., Barcella, L., Gamba, S., Lerede, T., Maggioni, A., Schieppati, F., Russo, L., Zunino, F., Artuso, A., Bellesso, S., Cadau, J., Carli, G., Nichele, I., Perbellini, O., Caronna, A., Gabrielli, F., Lami, F., Nicolini, A., Scaglioni, F., Pinelli, M., Desideri, G., Borgese, L., Favaretto, E., Libra, A., Migliaccio, L., Sartori, M., Panzavolta, C., Scandiuzzi, T., Zalunardo, B. -M., Ierardi, A., Leotta, M., Strangio, A., Guzzon, S., Colaizzo, D., Favuzzi, G., Lombardi, M. R., Ferrini, P. M., Tassoni, M. I., Corradini, S., Iotti, M., Lambertini, I., Veropalumbo, M. R., Lessiani, G., Parisi, R., Bortoluzzi, C., Vo, H. N., Chiarugi, P., Casini, M., Violo, C., Nuti, M., Angeloni, L., Carrozzi, L., Pancani, R., Chimera, D., Conti, V., Meschi, C., Cattaneo, M., Podda, G., Birocchi, S., Cuppini, S., Marzolo, M., Milan, M., Martini, G., Merelli, S., Pontoglio, S., Portesi, N., Villalta, S., De Lucchi, L., Sponghiado, A., Becattini, C., Giustozzi, M., Vinci, A., Pignatelli, P., Bucci, T., Menichelli, D., Pastori, D., Pomero, F., Casalis, S., Galli, E., Ciammaichella, M., Maida, R., De Cristofaro, Raimondo, Alberelli, M. A., Basso, M. R., De Candia, Erica, Di Gennaro, Leonardo, Mumoli, N., Capra, R., Orlando, M., Porta, C., Rotiroti, G., Demarco, M., Petrillo, P., Rossi, E., Bartolomei, Francesca, Soldati, D., Russo, U., Burgo, I., Ziliotti, M., Pataccini, C., Terroni, L., Ugolotti, M. C., Di Giorgio, A., Cavagna, L., Mete, F., Gino, M., Santoro, A., De Carlo, A., Cappelli, R., Bicchi, M., Dyrmo, L., Grifoni, E., Masotti, L., Ria, L., Spagnolo, M., Rupoli, S., Federici, I., Morsia, E., Scortechini, A. R., Torre, E., Franchini, M., Montorsi, P., Galgano, G., De Luca, A., Muiesan, M. L., Paini, A., Stassaldi, D., Denas, G., De Cristofaro R. (ORCID:0000-0002-8066-8849), De Candia E. (ORCID:0000-0003-0942-2819), Di Gennaro L., Bartolomei F., Palareti, G., Poli, D., Pesavento, R., Legnani, C., Antonucci, E., Bucherini, E., Testa, S., Paoletti, O., Chistolini, A., Ceccato, D., Martinelli, I., Bucciarelli, P., Falanga, A., Tosetto, A., Sarti, L., Mastroiacovo, D., Cosmi, B., Visona, A., Santoro, R. C., Zanatta, N., Grandone, E., Bertu, L., Pengo, V., Caiano, L., Prandoni, P., Lotti, E., Crudele, F., Ageno, W., Abenante, A., Colombo, G., Guarascio, M., Cancellieri, E., Morandini, R., Zambelli, S., Martini, S., Vastola, M., Serrao, A., Abbattista, M., Artoni, A., Capecchi, M., Gianniello, F., Scimeca, B., Barcella, L., Gamba, S., Lerede, T., Maggioni, A., Schieppati, F., Russo, L., Zunino, F., Artuso, A., Bellesso, S., Cadau, J., Carli, G., Nichele, I., Perbellini, O., Caronna, A., Gabrielli, F., Lami, F., Nicolini, A., Scaglioni, F., Pinelli, M., Desideri, G., Borgese, L., Favaretto, E., Libra, A., Migliaccio, L., Sartori, M., Panzavolta, C., Scandiuzzi, T., Zalunardo, B. -M., Ierardi, A., Leotta, M., Strangio, A., Guzzon, S., Colaizzo, D., Favuzzi, G., Lombardi, M. R., Ferrini, P. M., Tassoni, M. I., Corradini, S., Iotti, M., Lambertini, I., Veropalumbo, M. R., Lessiani, G., Parisi, R., Bortoluzzi, C., Vo, H. N., Chiarugi, P., Casini, M., Violo, C., Nuti, M., Angeloni, L., Carrozzi, L., Pancani, R., Chimera, D., Conti, V., Meschi, C., Cattaneo, M., Podda, G., Birocchi, S., Cuppini, S., Marzolo, M., Milan, M., Martini, G., Merelli, S., Pontoglio, S., Portesi, N., Villalta, S., De Lucchi, L., Sponghiado, A., Becattini, C., Giustozzi, M., Vinci, A., Pignatelli, P., Bucci, T., Menichelli, D., Pastori, D., Pomero, F., Casalis, S., Galli, E., Ciammaichella, M., Maida, R., De Cristofaro, Raimondo, Alberelli, M. A., Basso, M. R., De Candia, Erica, Di Gennaro, Leonardo, Mumoli, N., Capra, R., Orlando, M., Porta, C., Rotiroti, G., Demarco, M., Petrillo, P., Rossi, E., Bartolomei, Francesca, Soldati, D., Russo, U., Burgo, I., Ziliotti, M., Pataccini, C., Terroni, L., Ugolotti, M. C., Di Giorgio, A., Cavagna, L., Mete, F., Gino, M., Santoro, A., De Carlo, A., Cappelli, R., Bicchi, M., Dyrmo, L., Grifoni, E., Masotti, L., Ria, L., Spagnolo, M., Rupoli, S., Federici, I., Morsia, E., Scortechini, A. R., Torre, E., Franchini, M., Montorsi, P., Galgano, G., De Luca, A., Muiesan, M. L., Paini, A., Stassaldi, D., Denas, G., De Cristofaro R. (ORCID:0000-0002-8066-8849), De Candia E. (ORCID:0000-0003-0942-2819), Di Gennaro L., and Bartolomei F.
Abstract: D-dimer assay is used to stratify patients with unprovoked venous thromboembolism (VTE) for the risk of recurrence. However, this approach was never evaluated since direct oral anticoagulants are available. With this multicenter, prospective cohort study, we aimed to assess the value of an algorithm incorporating serial D-dimer testing and administration of reduced-dose apixaban (2.5 mg twice daily) only to patients with a positive test. A total of 732 outpatients aged 18 to 74 years, anticoagulated for ≥12 months after a first unprovoked VTE, were included. Patients underwent D-dimer testing with commercial assays and preestablished cutoffs. If the baseline D-dimer during anticoagulation was negative, anticoagulation was stopped and testing repeated after 15, 30, and 60 days. Patients with serially negative results (286 [39.1%]) were left without anticoagulation. At the first positive result, the remaining 446 patients (60.9%) were given apixaban for 18 months. All patients underwent follow-up planned for 18 months. The study was interrupted after a planned interim analysis for the high rate of primary outcomes (7.3%; 95% confidence interval [CI], 4.5-11.2), including symptomatic proximal deep vein thrombosis (DVT) or pulmonary embolism (PE) recurrence, death for VTE, and major bleeding occurring in patients off anticoagulation vs that in those receiving apixaban (1.1%; 95% CI, 0.4-2.6; adjusted hazard ratio [HR], 8.2; 95% CI, 3.2-25.3). In conclusion, in patients anticoagulated for ≥1 year after a first unprovoked VTE, the decision to further extend anticoagulation should not be based on D-dimer testing. The results confirmed the high efficacy and safety of reduced-dose apixaban against recurrences. This trial was registered at www.clinicaltrials.gov as #NCT03678506.
Published: 2022

34. HIGH FATIGUE SCORES IN PATIENTS WITH IDIOPATHIC INFLAMMATORY MYOPATHIES : A MULTIGROUP COMPARATIVE STUDY FROM THE COVAD E-SURVEY

Author: Grignaschi, S., Cavagna, L., Kim, M., R, N., Lilleker, J. B., Sen, P., Agarwal, V., Kardes, S., Day, J., Makol, A., Milchert, M., Gheita, T. A., Salim, B., Velikova, T., Gracia-Ramos, A. E., Parodis, Ioannis, Selva-O'callaghan, A., Nikiphorou, E., Chatterjee, T., Tan, A. L., Saavedra, M. A., Shinjo, S. Katsuyuki, Ziade, N., Knitza, J., Kuwana, M., Nune, A., Distler, O., Chinoy, H., Aggarwal, R., Gupta, L., Grignaschi, S., Cavagna, L., Kim, M., R, N., Lilleker, J. B., Sen, P., Agarwal, V., Kardes, S., Day, J., Makol, A., Milchert, M., Gheita, T. A., Salim, B., Velikova, T., Gracia-Ramos, A. E., Parodis, Ioannis, Selva-O'callaghan, A., Nikiphorou, E., Chatterjee, T., Tan, A. L., Saavedra, M. A., Shinjo, S. Katsuyuki, Ziade, N., Knitza, J., Kuwana, M., Nune, A., Distler, O., Chinoy, H., Aggarwal, R., and Gupta, L.
Published: 2022
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35. TREATMENT PATTERNS OF IDIOPATHIC INFLAMMATORY MYOPATHIES : RESULTS FROM AN INTERNATIONAL COHORT OF OVER 1,400 PATIENTS

Author: Aoude, M., Gupta, L., Hmamouchi, I., Grignaschi, S., Cavagna, L., Kim, M., R, N., Lilleker, J. B., Sen, P., Agarwal, V., Kardes, S., Day, J., Makol, A., Milchert, M., Gheita, T. A., Salim, B., Velikova, T., Gracia-Ramos, A. E., Parodis, Ioannis, Selva-O'callaghan, A., Nikiphorou, E., Chatterjee, T., Tan, A. L., Saavedra, M. A., Shinjo, S. Katsuyuki, Knitza, J., Kuwana, M., Nune, A., Distler, O., Chinoy, H., Aggarwal, R., Ziade, N., Aoude, M., Gupta, L., Hmamouchi, I., Grignaschi, S., Cavagna, L., Kim, M., R, N., Lilleker, J. B., Sen, P., Agarwal, V., Kardes, S., Day, J., Makol, A., Milchert, M., Gheita, T. A., Salim, B., Velikova, T., Gracia-Ramos, A. E., Parodis, Ioannis, Selva-O'callaghan, A., Nikiphorou, E., Chatterjee, T., Tan, A. L., Saavedra, M. A., Shinjo, S. Katsuyuki, Knitza, J., Kuwana, M., Nune, A., Distler, O., Chinoy, H., Aggarwal, R., and Ziade, N.
Published: 2022
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36. COVID-19 SEVERITY AND VACCINE BREAKTHROUGH INFECTIONS IN IDIOPATHIC INFLAMMATORY MYOPATHIES, OTHER SYSTEMIC AUTOIMMUNE AND INFLAMMATORY DISEASES, AND HEALTHY INDIVIDUALS : RESULTS FROM THE COVID-19 VACCINATION IN AUTOIMMUNE DISEASES (COVAD) STUDY.

Author: Gupta, L., Hoff, L. S., R, N., Sen, P., Katsuyuki Shinjo, S., Day, J., Lilleker, J. B., Agarwal, V., Kardes, S., Kim, M., Makol, A., Milchert, M., Gheita, T. A., Salim, B., Velikova, T., Gracia-Ramos, A. E., Parodis, Ioannis, Selva-O'callaghan, A., Nikiphorou, E., Chatterjee, T., Tan, A. L., Nune, A., Cavagna, L., Saavedra, M. A., Ziade, N., Knitza, J., Kuwana, M., Distler, O., Chinoy, H., Aggarwal, R., Gupta, L., Hoff, L. S., R, N., Sen, P., Katsuyuki Shinjo, S., Day, J., Lilleker, J. B., Agarwal, V., Kardes, S., Kim, M., Makol, A., Milchert, M., Gheita, T. A., Salim, B., Velikova, T., Gracia-Ramos, A. E., Parodis, Ioannis, Selva-O'callaghan, A., Nikiphorou, E., Chatterjee, T., Tan, A. L., Nune, A., Cavagna, L., Saavedra, M. A., Ziade, N., Knitza, J., Kuwana, M., Distler, O., Chinoy, H., and Aggarwal, R.
Published: 2022
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37. IMPAIRED PROMIS PHYSICAL FUNCTION IN IDIOPATHIC INFLAMMATORY MYOPATHY PATIENTS : RESULTS FROM THE MULTICENTER COVAD PATIENT REPORTED E-SURVEY

Author: Yoshida, A., Kim, M., Kuwana, M., R, N., Lilleker, J. B., Sen, P., Agarwal, V., Kardes, S., Day, J., Makol, A., Milchert, M., Gheita, T. A., Salim, B., Velikova, T., Gracia-Ramos, A. E., Parodis, Ioannis, Selva-O'callaghan, A., Nikiphorou, E., Chatterjee, T., Tan, A. L., Nune, A., Cavagna, L., Saavedra, M. A., Katsuyuki Shinjo, S., Ziade, N., Knitza, J., Distler, O., Chinoy, H., Aggarwal, R., Gupta, L., Yoshida, A., Kim, M., Kuwana, M., R, N., Lilleker, J. B., Sen, P., Agarwal, V., Kardes, S., Day, J., Makol, A., Milchert, M., Gheita, T. A., Salim, B., Velikova, T., Gracia-Ramos, A. E., Parodis, Ioannis, Selva-O'callaghan, A., Nikiphorou, E., Chatterjee, T., Tan, A. L., Nune, A., Cavagna, L., Saavedra, M. A., Katsuyuki Shinjo, S., Ziade, N., Knitza, J., Distler, O., Chinoy, H., Aggarwal, R., and Gupta, L.
Published: 2022
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38. COVID-19 VACCINATION-RELATED ADVERSE EVENTS AMONG AUTOIMMUNE DISEASE PATIENTS : RESULTS FROM THE COVID-19 VACCINATION IN AUTOIMMUNE DISEASES (COVAD) STUDY

Author: Sen, P., R, N., Nune, A., Lilleker, J. B., Agarwal, V., Kardes, S., Kim, M., Day, J., Milchert, M., Gheita, T. A., Salim, B., Velikova, T., Gracia-Ramos, A. E., Parodis, Ioannis, Selva-O'callaghan, A., Nikiphorou, E., Chatterjee, T., Tan, A. L., Cavagna, L., Saavedra, M. A., Shinjo, S. Katsuyuki, Ziade, N., Knitza, J., Kuwana, M., Distler, O., Chinoy, H., Aggarwal, R., Gupta, L., Sen, P., R, N., Nune, A., Lilleker, J. B., Agarwal, V., Kardes, S., Kim, M., Day, J., Milchert, M., Gheita, T. A., Salim, B., Velikova, T., Gracia-Ramos, A. E., Parodis, Ioannis, Selva-O'callaghan, A., Nikiphorou, E., Chatterjee, T., Tan, A. L., Cavagna, L., Saavedra, M. A., Shinjo, S. Katsuyuki, Ziade, N., Knitza, J., Kuwana, M., Distler, O., Chinoy, H., Aggarwal, R., and Gupta, L.
Published: 2022
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39. The added value of a European Reference Network on rare and complex connective tissue and musculoskeletal diseases:insights after the first 5 years of the ERN ReCONNET

Author: Talarico, R., Aguilera, S., Alexander, T., Amoura, Z., Andersen, J., Arnaud, L., Avcin, T., Marsal Barril, S., Beretta, L., Bombardieri, S., Bortoluzzi, A., Bouillot, C., Bulina, I., Burmester, G. R., Cannizzo, S., Cavagna, L., Chaigne, B., Cornet, A., Corti, P., Costedoat-Chalumeau, N., Dāvidsone, Z., Doria, A., Fenech, C., Ferraris, A., Fischer-Betz, R., Fonseca, J. E., Frank, C., Gaglioti, A., Galetti, I., Guimarães, V., Hachulla, E., Holmner, M., Houssiau, F., Iaccarino, L., Jacobsen, S., Limper, M., Malfait, F., Mariette, X., Marinello, D., Martin, T., Matthews, L., Matucci-Cerinic, M., Meyer, A., Milas-Ahić, J., Moinzadeh, P., Montecucco, C., Mouthon, L., Müller-Ladner, U., Nagy, G., Patarata, E., Pileckyte, M., Pruunsild, C., Rednic, S., Romão, V. C., Schneider, M., Scirè, C. A., Smith, V., Sulli, A., Tamirou, F., Tani, C., Taruscio, D., Taulaigo, A. V., Tincani, A., Ticciati, S., Turchetti, G., van Hagen, P. M., van Laar, J. M., Vieira, A., de Vries-Bouwstra, J. K., Zschocke, J., Cutolo, M., Mosca, Marta, Talarico, R., Aguilera, S., Alexander, T., Amoura, Z., Andersen, J., Arnaud, L., Avcin, T., Marsal Barril, S., Beretta, L., Bombardieri, S., Bortoluzzi, A., Bouillot, C., Bulina, I., Burmester, G. R., Cannizzo, S., Cavagna, L., Chaigne, B., Cornet, A., Corti, P., Costedoat-Chalumeau, N., Dāvidsone, Z., Doria, A., Fenech, C., Ferraris, A., Fischer-Betz, R., Fonseca, J. E., Frank, C., Gaglioti, A., Galetti, I., Guimarães, V., Hachulla, E., Holmner, M., Houssiau, F., Iaccarino, L., Jacobsen, S., Limper, M., Malfait, F., Mariette, X., Marinello, D., Martin, T., Matthews, L., Matucci-Cerinic, M., Meyer, A., Milas-Ahić, J., Moinzadeh, P., Montecucco, C., Mouthon, L., Müller-Ladner, U., Nagy, G., Patarata, E., Pileckyte, M., Pruunsild, C., Rednic, S., Romão, V. C., Schneider, M., Scirè, C. A., Smith, V., Sulli, A., Tamirou, F., Tani, C., Taruscio, D., Taulaigo, A. V., Tincani, A., Ticciati, S., Turchetti, G., van Hagen, P. M., van Laar, J. M., Vieira, A., de Vries-Bouwstra, J. K., Zschocke, J., Cutolo, M., and Mosca, Marta
Abstract: In order to address the main challenges related to the rare diseases (RDs) the European Commission launched the European Reference Networks (ERNs), virtual networks involving healthcare providers (HCPs) across Europe. The mission of the ERNs is to tackle low prevalence and RDs that require highly specialised treatment and a concentration of knowledge and resources. In fact, ERNs offer the potential to give patients and healthcare professionals across the EU access to the best expertise and timely exchange of lifesaving knowledge, trying to make the knowledge travelling more than patients. For this reason, ERNs were established as concrete European infrastructures, and this is particularly crucial in the framework of rare and complex diseases in which no country alone has the whole knowledge and capacity to treat all types of patients. It has been five years since their kick-off launch in Vilnius in 2017. The 24 ERNs have been intensively working on different transversal areas, including patient management, education, clinical practice guidelines, patients' care pathways and many other fundamental topics. The present work is therefore aimed not only at reporting a summary of the main activities and milestones reached so far, but also at celebrating the first 5 years of the ERN on Rare and Complex Connective Tissue and Musculo-skeletal Diseases (ReCONNET), in which the members of the network built together one of the 24 infrastructures that are hopefully going to change the scenario of rare diseases across the EU.
Published: 2022

40. Evidence for charge-based mimicry in anti dsDNA antibody generation

Author: Bruschi, M, Angeletti, A, Kajana, X, Moroni, G, Sinico, R, Fredi, M, Vaglio, A, Cavagna, L, Pratesi, F, Migliorini, P, Locatelli, F, Pazzola, G, Pesce, G, Bagnasco, M, Manfredi, A, Ramirez, G, Esposito, P, Negrini, S, Bui, F, Trezzi, B, Emmi, G, Cavazzana, I, Binda, V, Fenaroli, P, Pisani, I, Montecucco, C, Santoro, D, Scolari, F, Volpi, S, Mosca, M, Tincani, A, Candiano, G, Verrina, E, Franceschini, F, Ravelli, A, Prunotto, M, Meroni, P, Ghiggeri, G, Bruschi, Maurizio, Angeletti, Andrea, Kajana, Xhuliana, Moroni, Gabriella, Sinico, Renato Alberto, Fredi, Micaela, Vaglio, Augusto, Cavagna, Lorenzo, Pratesi, Federico, Migliorini, Paola, Locatelli, Francesco, Pazzola, Giulia, Pesce, Giampaola, Bagnasco, Marcello, Manfredi, Angelo, Ramirez, Giuseppe Alvise, Esposito, Pasquale, Negrini, Simone, Bui, Federica, Trezzi, Barbara, Emmi, Giacomo, Cavazzana, Ilaria, Binda, Valentina, Fenaroli, Paride, Pisani, Isabella, Montecucco, Carlomaurizio, Santoro, Domenico, Scolari, Francesco, Volpi, Stefano, Mosca, Marta, Tincani, Angela, Candiano, Giovanni, Verrina, Enrico, Franceschini, Franco, Ravelli, Angelo, Prunotto, Marco, Meroni, Pier Luigi, Ghiggeri, Gian Marco, Bruschi, M, Angeletti, A, Kajana, X, Moroni, G, Sinico, R, Fredi, M, Vaglio, A, Cavagna, L, Pratesi, F, Migliorini, P, Locatelli, F, Pazzola, G, Pesce, G, Bagnasco, M, Manfredi, A, Ramirez, G, Esposito, P, Negrini, S, Bui, F, Trezzi, B, Emmi, G, Cavazzana, I, Binda, V, Fenaroli, P, Pisani, I, Montecucco, C, Santoro, D, Scolari, F, Volpi, S, Mosca, M, Tincani, A, Candiano, G, Verrina, E, Franceschini, F, Ravelli, A, Prunotto, M, Meroni, P, Ghiggeri, G, Bruschi, Maurizio, Angeletti, Andrea, Kajana, Xhuliana, Moroni, Gabriella, Sinico, Renato Alberto, Fredi, Micaela, Vaglio, Augusto, Cavagna, Lorenzo, Pratesi, Federico, Migliorini, Paola, Locatelli, Francesco, Pazzola, Giulia, Pesce, Giampaola, Bagnasco, Marcello, Manfredi, Angelo, Ramirez, Giuseppe Alvise, Esposito, Pasquale, Negrini, Simone, Bui, Federica, Trezzi, Barbara, Emmi, Giacomo, Cavazzana, Ilaria, Binda, Valentina, Fenaroli, Paride, Pisani, Isabella, Montecucco, Carlomaurizio, Santoro, Domenico, Scolari, Francesco, Volpi, Stefano, Mosca, Marta, Tincani, Angela, Candiano, Giovanni, Verrina, Enrico, Franceschini, Franco, Ravelli, Angelo, Prunotto, Marco, Meroni, Pier Luigi, and Ghiggeri, Gian Marco
Abstract: Mechanisms for the generation of anti-dsDNA autoantibodies are still not completely elucidated. One theory states that dsDNA interacts for mimicry with antibodies raised versus other antigens but molecular features for mimicry are unknown. Here we show that, at physiological acid-base balance, anti-Annexin A1 binds IgG2 dsDNA in a competitive and dose-dependent way with Annexin A1 and that the competition between the two molecules is null at pH 9. On the other hand, these findings also show that dsDNA and Annexin A1 interact with their respective antibodies on a strictly pH-dependent basis: in both cases, the binding was minimal at pH 4 and maximal at pH9-10. The anionic charge of dsDNA is mainly conferred by the numerous phosphatidic residues. The epitope binding site of Annexin A1 for anti-Annexin A1 IgG2 was here characterized as a string of 34 amino acids at the NH2 terminus, 10 of which are anionic. Circulating levels of anti-dsDNA and anti-Annexin A1 IgG2 antibodies were strongly correlated in patients with systemic lupus erythematosus (n 496) and lupus nephritis (n 425) stratified for age, sex, etc. These results show that dsDNA competes with Annexin A1 for the binding with anti-Annexin A1 IgG2 on a dose and charged mediated base, being able to display an inhibition up to 75%. This study provides the first demonstration that dsDNA may interact with antibodies raised versus other anionic molecules (anti-Annexin A1 IgG2) because of charge mimicry and this interaction may contribute to anti-dsDNA antibodies generation.
Published: 2022

41. Development and implementation of the AIDA International Registry for patients with Behçet's disease

Author: Vitale, A, Della Casa, F, Ragab, G, Almaghlouth, Ia, Lopalco, G, Pereira, Rm, Guerriero, S, Govoni, M, Sfikakis, Pp, Giacomelli, R, Ciccia, F, Monti, S, Ruscitti, P, Piga, M, Lomater, C, Tufan, A, Opris-Belinski, D, Emmi, G, Hernández-Rodríguez, J, Şahin, A, Sebastiani, Gd, Bartoloni, E, Akkoç, N, Gündüz, Ö, Cattalini, M, Conti, Giorgio, Hatemi, G, Maier, A, Parronchi, P, Del Giudice, E, Erten, S, Insalaco, A, Li Gobbi, F, Maggio, Mc, Shahram, F, Caggiano, V, Hegazy, Mt, Asfina, Kn, Morrone, M, Prado, Ll, Dammacco, R, Ruffilli, F, Arida, A, Navarini, L, Pantano, I, Cavagna, L, Conforti, A, Cauli, A, Marucco, Em, Kucuk, H, Ionescu, R, Mattioli, I, Espinosa, G, Araújo, O, Karkaş, B, Canofari, C, Sota, J, Laymouna, Ah, Bedaiwi, Aa, Colella, S, Giardini, Ham, Albano, V, Lo Monaco, A, Fragoulis, Ge, Kardas, Rc, Berlengiero, V, Hussein, Ma, Ricci, F, La Torre, F, Rigante, Donato, Więsik-Szewczyk, E, Frassi, M, Gentileschi, S, Tosi, Gm, Dagostin, Ma, Mahmoud, Aaa, Tarsia, M, Alessio, G, Cimaz, R, Giani, T, Gaggiano, C, Iannone, F, Cipriani, P, Mourabi, M, Spedicato, V, Barneschi, S, Aragona, E, Balistreri, A, Frediani, B, Fabiani, C, Cantarini, L &amp, Autoinflammatory Diseases Alliance (AIDA), Network, Conti G (ORCID:0000-0002-8566-9365), Rigante D (ORCID:0000-0001-7032-7779), Vitale, A, Della Casa, F, Ragab, G, Almaghlouth, Ia, Lopalco, G, Pereira, Rm, Guerriero, S, Govoni, M, Sfikakis, Pp, Giacomelli, R, Ciccia, F, Monti, S, Ruscitti, P, Piga, M, Lomater, C, Tufan, A, Opris-Belinski, D, Emmi, G, Hernández-Rodríguez, J, Şahin, A, Sebastiani, Gd, Bartoloni, E, Akkoç, N, Gündüz, Ö, Cattalini, M, Conti, Giorgio, Hatemi, G, Maier, A, Parronchi, P, Del Giudice, E, Erten, S, Insalaco, A, Li Gobbi, F, Maggio, Mc, Shahram, F, Caggiano, V, Hegazy, Mt, Asfina, Kn, Morrone, M, Prado, Ll, Dammacco, R, Ruffilli, F, Arida, A, Navarini, L, Pantano, I, Cavagna, L, Conforti, A, Cauli, A, Marucco, Em, Kucuk, H, Ionescu, R, Mattioli, I, Espinosa, G, Araújo, O, Karkaş, B, Canofari, C, Sota, J, Laymouna, Ah, Bedaiwi, Aa, Colella, S, Giardini, Ham, Albano, V, Lo Monaco, A, Fragoulis, Ge, Kardas, Rc, Berlengiero, V, Hussein, Ma, Ricci, F, La Torre, F, Rigante, Donato, Więsik-Szewczyk, E, Frassi, M, Gentileschi, S, Tosi, Gm, Dagostin, Ma, Mahmoud, Aaa, Tarsia, M, Alessio, G, Cimaz, R, Giani, T, Gaggiano, C, Iannone, F, Cipriani, P, Mourabi, M, Spedicato, V, Barneschi, S, Aragona, E, Balistreri, A, Frediani, B, Fabiani, C, Cantarini, L &amp, Autoinflammatory Diseases Alliance (AIDA), Network, Conti G (ORCID:0000-0002-8566-9365), and Rigante D (ORCID:0000-0001-7032-7779)
Abstract: Objective: Purpose of the present paper is to point out the design, development and deployment of the AutoInflammatory Disease Alliance (AIDA) International Registry dedicated to pediatric and adult patients with Behçet’s disease (BD). Methods: The Registry is a clinical physician-driven population- and electronic-based instrument implemented for the retrospective and prospective collection of real-life data about demographics, clinical, therapeutic, laboratory, instrumental and socioeconomic information from BD patients; the Registry is based on the Research Electronic Data Capture (REDCap) tool, which is thought to collect standardised information for clinical real-life research, and has been realised to change over time according to future scientific acquisitions and potentially communicate with other existing and future Registries dedicated to BD. Results: Starting from January 31st to November 23rd, 2021, 99 centres from 20 countries in 4 continents have been involved. Forty-eight of these have already obtained the approval from their local Ethics Committees. Currently, the platform counts 265 users (99 Principal Investigators, 162 Site Investigators, 2 Lead Investigators, and 2 data managers). The Registry collects baseline and follow-up data using 5474 fields organised into 15 instruments, including patient’s demographics, history, clinical manifestations and symptoms, trigger/risk factors, therapies and healthcare access. Conclusions: The development of the AIDA International Registry for BD patients will facilitate the collection of standardised data leading to real-world evidence, enabling international multicentre collaborative research through data sharing, international consultation, dissemination of knowledge, inclusion of patients and families, and ultimately optimisation of scientific efforts and implementation of standardised care.
Published: 2022

42. AB1287 DESIGN OF A GLOBAL PHASE 2/3 RANDOMIZED, PLACEBO-CONTROLLED TRIAL EVALUATING THE EFFICACY AND SAFETY OF RAVULIZUMAB IN ADULTS WITH DERMATOMYOSITIS

Author: Cavagna, L., primary, Aggarwal, R., additional, Iikuni, N., additional, and Rakhade, S., additional
Published: 2022
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43. POS0924 THE ROLE OF RAYNAUD’S PHENOMENON ON MATERNAL AND FETAL OBSTETRICAL OUTCOMES

Author: Bellis, E., primary, Codullo, V., additional, Palermo, B. L., additional, Bottazzi, F., additional, Biglia, A., additional, Bellingeri, C., additional, Beneventi, F., additional, Spinillo, A., additional, Montecucco, C., additional, Zanframundo, G., additional, and Cavagna, L., additional
Published: 2022
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44. OP0161 TREATMENT PATTERNS OF IDIOPATHIC INFLAMMATORY MYOPATHIES: RESULTS FROM AN INTERNATIONAL COHORT OF OVER 1,400 PATIENTS

Author: Aoude, M., primary, Gupta, L., additional, Hmamouchi, I., additional, Grignaschi, S., additional, Cavagna, L., additional, Kim, M., additional, R, N., additional, Lilleker, J. B., additional, Sen, P., additional, Agarwal, V., additional, Kardes, S., additional, Day, J., additional, Makol, A., additional, Milchert, M., additional, Gheita, T. A., additional, Salim, B., additional, Velikova, T., additional, Gracia-Ramos, A. E., additional, Parodis, I., additional, Selva-O’callaghan, A., additional, Nikiphorou, E., additional, Chatterjee, T., additional, Tan, A. L., additional, Saavedra, M. A., additional, Katsuyuki Shinjo, S., additional, Knitza, J., additional, Kuwana, M., additional, Nune, A., additional, Distler, O., additional, Chinoy, H., additional, Aggarwal, R., additional, and Ziade, N., additional
Published: 2022
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45. POS0899 HIGH FATIGUE SCORES IN PATIENTS WITH IDIOPATHIC INFLAMMATORY MYOPATHIES: A MULTIGROUP COMPARATIVE STUDY FROM THE COVAD E-SURVEY.

Author: Grignaschi, S., primary, Cavagna, L., additional, Kim, M., additional, R, N., additional, Lilleker, J. B., additional, Sen, P., additional, Agarwal, V., additional, Kardes, S., additional, Day, J., additional, Makol, A., additional, Milchert, M., additional, Gheita, T. A., additional, Salim, B., additional, Velikova, T., additional, Gracia-Ramos, A. E., additional, Parodis, I., additional, Selva-O’callaghan, A., additional, Nikiphorou, E., additional, Chatterjee, T., additional, Tan, A. L., additional, Saavedra, M. A., additional, Katsuyuki Shinjo, S., additional, Ziade, N., additional, Knitza, J., additional, Kuwana, M., additional, Nune, A., additional, Distler, O., additional, Chinoy, H., additional, Aggarwal, R., additional, and Gupta, L., additional
Published: 2022
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46. POS1232 LONG-TERM OUTCOMES OF COVID-19 VACCINATION IN PATIENTS WITH RARE AND COMPLEX CONNECTIVE TISSUE DISEASES: AN AD-INTERIM ANALYSIS OF ERN-ReCONNET VACCINATE STUDY

Author: Di Cianni, F., primary, Cardelli, C., additional, Italiano, N., additional, Laurino, E., additional, Moretti, M., additional, Depascale, R., additional, Gamba, A., additional, Iaccarino, L., additional, Doria, A., additional, Sousa Bandeira, M. J., additional, Dinis, S. P., additional, C Romão, V., additional, Alessandri, E., additional, Gotelli, E., additional, Paolino, S., additional, DI Giosaffatte, N., additional, Grammatico, P., additional, Ferraris, A., additional, Cavagna, L., additional, Montecucco, C., additional, Longo, V., additional, Beretta, L., additional, Cavazzana, I., additional, Fredi, M., additional, Tincani, A., additional, D’urzo, R., additional, Bombardieri, S., additional, Burmester, G. R., additional, Cutolo, M., additional, Fonseca, J. E., additional, Frank, C. H., additional, Galetti, I., additional, Hachulla, E., additional, Houssiau, F., additional, Marinello, D., additional, Müller-Ladner, U., additional, Schneider, M., additional, Smith, V., additional, Talarico, R., additional, Van Laar, J. M., additional, Vieira, A., additional, Tani, C., additional, and Mosca, M., additional
Published: 2022
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47. POS0863 ANTI-NOR90 ANTIBODIES IN THE SETTING OF CONNECTIVE TISSUE DISEASE: CLINICAL SIGNIFICANCE AND COMPARISON WITH A COHORT OF PATIENTS WITH SYSTEMIC SCLEROSIS

Author: Biglia, A., primary, Dourado, E., additional, Palterer, B., additional, Parronchi, P., additional, Pellico, M. R., additional, Zanframundo, G., additional, Rivera Matias, P. A., additional, Martins, P., additional, Miranda, A., additional, Cabral Da Fonseca, J. E., additional, DI Agosta, E., additional, Cammelli, D., additional, Emmi, G., additional, Rosi, E., additional, Bixio, R., additional, Conticini, E., additional, Bellis, E., additional, Bruni, C., additional, Montecucco, C., additional, Matucci-Cerinic, M., additional, Rojas-Serrano, J., additional, and Cavagna, L., additional
Published: 2022
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48. POS0201 COVID-19 SEVERITY AND VACCINE BREAKTHROUGH INFECTIONS IN IDIOPATHIC INFLAMMATORY MYOPATHIES, OTHER SYSTEMIC AUTOIMMUNE AND INFLAMMATORY DISEASES, AND HEALTHY INDIVIDUALS: RESULTS FROM THE COVID-19 VACCINATION IN AUTOIMMUNE DISEASES (COVAD) STUDY.

Author: Gupta, L., primary, Hoff, L. S., additional, R, N., additional, Sen, P., additional, Katsuyuki Shinjo, S., additional, Day, J., additional, Lilleker, J. B., additional, Agarwal, V., additional, Kardes, S., additional, Kim, M., additional, Makol, A., additional, Milchert, M., additional, Gheita, T. A., additional, Salim, B., additional, Velikova, T., additional, Gracia-Ramos, A. E., additional, Parodis, I., additional, Selva-O’callaghan, A., additional, Nikiphorou, E., additional, Chatterjee, T., additional, Tan, A. L., additional, Nune, A., additional, Cavagna, L., additional, Saavedra, M. A., additional, Ziade, N., additional, Knitza, J., additional, Kuwana, M., additional, Distler, O., additional, Chinoy, H., additional, and Aggarwal, R., additional
Published: 2022
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49. AB0140 ROLE OF ENDOGLIN IN THE PATHOGENESIS OF SYSTEMIC SCLEROSIS: A PRISMA-DRIVEN SYSTEMATIC REVIEW

Author: Grignaschi, S., primary, Palermo, B. L., additional, Spinozzi, G., additional, Sbalchiero, A., additional, Cantarini, C., additional, Nardiello, C., additional, Olivieri, C., additional, and Cavagna, L., additional
Published: 2022
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50. POS0855 IMPAIRED PROMIS PHYSICAL FUNCTION IN IDIOPATHIC INFLAMMATORY MYOPATHY PATIENTS: RESULTS FROM THE MULTICENTER COVAD PATIENT REPORTED E-SURVEY

Author: Yoshida, A., primary, Kim, M., additional, Kuwana, M., additional, R, N., additional, Lilleker, J. B., additional, Sen, P., additional, Agarwal, V., additional, Kardes, S., additional, Day, J., additional, Makol, A., additional, Milchert, M., additional, Gheita, T. A., additional, Salim, B., additional, Velikova, T., additional, Gracia-Ramos, A. E., additional, Parodis, I., additional, Selva-O’callaghan, A., additional, Nikiphorou, E., additional, Chatterjee, T., additional, Tan, A. L., additional, Nune, A., additional, Cavagna, L., additional, Saavedra, M. A., additional, Katsuyuki Shinjo, S., additional, Ziade, N., additional, Knitza, J., additional, Distler, O., additional, Chinoy, H., additional, Aggarwal, R., additional, and Gupta, L., additional
Published: 2022
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