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1. Mortality risk factors in primary Sjögren syndrome: a real-world, retrospective, cohort study

Author

Arends, S., Treppo, E., Longhino, S., Manfrè, V., Rizzo, M., Baldini, C., Bombardieri, S., Bandeira, M., Silvéiro-António, M., Seror, R., Mariette, X., Nordmark, G., Danda, D., Wiland, P., Gerli, R., Kwok, S.K., Park, S.H., Kvarnstrom, M., Wahren-Herlenius, M., Downie-Doyle, S., Sene, D., Isenberg, D., Valim, V., Devauchelle-Pensec, V., Saraux, A., Morel, J., Morcillo, C., Díaz Cuiza, P.E., Herrera, B.E., González-de-Paz, L., Sisó-Almirall, A., Brito-Zerón, Pilar, Flores-Chávez, Alejandra, Horváth, Ildiko Fanny, Rasmussen, Astrid, Li, Xiaomei, Olsson, Peter, Vissink, Arjan, Priori, Roberta, Armagan, Berkan, Hernandez-Molina, Gabriela, Praprotnik, Sonja, Quartuccio, Luca, Inanç, Nevsun, Özkızıltaş, Burcugül, Bartoloni, Elena, Sebastian, Agata, Romão, Vasco C., Solans, Roser, Pasoto, Sandra G., Rischmueller, Maureen, Galisteo, Carlos, Suzuki, Yasunori, Trevisani, Virginia Fernandes Moça, Fugmann, Cecilia, González-García, Andrés, Carubbi, Francesco, Jurcut, Ciprian, Shimizu, Toshimasa, Retamozo, Soledad, Atzeni, Fabiola, Hofauer, Benedikt, Melchor-Díaz, Sheila, Gheita, Tamer, López-Dupla, Miguel, Fonseca-Aizpuru, Eva, Giacomelli, Roberto, Vázquez, Marcos, Consani, Sandra, Akasbi, Miriam, Nakamura, Hideki, Szántó, Antónia, Farris, A. Darise, Wang, Li, Mandl, Thomas, Gattamelata, Angelica, Kilic, Levent, Pirkmajer, Katja Perdan, Abacar, Kerem, Tufan, Abdurrahman, de Vita, Salvatore, Bootsma, Hendrika, and Ramos-Casals, Manuel

Published
2023
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2. Dynamic Contrast-Enhanced MRI Confirms Rapid And Sustained Improvement Of Rheumatoid Arthritis Induced By Tocilizumab Treatment: An Italian Multicentre Study

Author

Cimmino MA, Parodi M, Barbieri F, Bombardieri S, Zampogna G, Iagnocco A, Batticciotto A, Sconfienza LM, Sinigaglia L, De Benedetti F, Atzeni F, and Sarzi-Puttini P

Subjects
tocilizumab, rheumatoid arthritis, wrist, synovitis, low-field mri, dedicated mri, Medicine (General), R5-920
Abstract

Marco A Cimmino,1 Massimiliano Parodi,1 Francesca Barbieri,1 Stefano Bombardieri,2 Giuseppe Zampogna,1 Annamaria Iagnocco,3 Alberto Batticciotto,4 Luca Maria Sconfienza,5,6 Luigi Sinigaglia,7 Fabrizio De Benedetti,8 Fabiola Atzeni,9 Piercarlo Sarzi-Puttini10 1Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, Genoa, Italy; 2Rheumatology Unit, Santa Chiara Hospital, University of Pisa, Pisa, Italy; 3Academic Rheumatology Centre, Università degli Studi di Torino, Turin, Italy; 4Rheumatology Unit ASST-Settelaghi, Ospedale di Circolo – Fondazione Macchi, Varese, Varese, Italy; 5IRCCS Istituto Ortopedico Galeazzi, Milan, Italy; 6Dipartimento di Scienze Biomediche per la Salute, Università degli Studi di Milano, Milan, Italy; 7Rheumatology Unit, G. Pini Hospital, Milan, Italy; 8Division of Rheumatology, IRCCS, Bambin Gesù Paediatric Hospital, Rome, Italy; 9Rheumatology Unit, University of Messina, Messina, Italy; 10Rheumatology Unit, L. Sacco University Hospital, Milan, ItalyCorrespondence: Piercarlo Sarzi-PuttiniReumatology Department, L. Sacco University Hospital, ASST Fatebenefratelli – Sacco, Via G.B. Grassi, 74, Milan 20157, ItalyTel +39 02 39042208Email piercarlo.sarziputtini@gmail.comObjective: This open-label study evaluated the effects of combined tocilizumab (TCZ) and disease-modifying antirheumatic drugs (DMARDs) on magnetic resonance imaging (MRI) changes in synovial membrane enhancement, bone marrow edema (BME), and erosions in the wrist and hand joints of rheumatoid arthritis (RA) patients inadequately responding to DMARDs alone.Methods: The efficacy of intravenous TCZ 8 mg/kg administered every four weeks for 48 weeks was evaluated on six occasions. The primary endpoints were the changes in the extent and degree of wrist synovitis as measured using the RA MRI Score (RAMRIS) and dynamic, gadolinium-enhanced 0.2T MRI (DCE-MRI). A number of different parameters of DCE-MRI were evaluated.Results: Fifty-eight patients were treated, eight of whom (13.8%) discontinued the study prematurely. The mean RAMRIS significantly decreased after two weeks and the decrease was maintained for up to 48 weeks. By week 4, the mean RAMRIS synovitis score had significantly decreased from baseline (− 0.804± 1.575; p=0.018), but not the mean early enhancement (REE) or relative enhancement (RE). However, there were significant decreases in RE at week 24, in REE and Ntotal (total number of enhancing voxels)*IRE (initial rate of enhancement) at weeks 12, 24 and 48, and in Ntotal*ME (maximal enhancement) at weeks 24 and 48. Mean BME decreased from baseline to week 48, and bone erosions did not progress. The patients’ clinical parameters significantly improved from baseline until week 48.Conclusion: TCZ in combination with DMARDs improved wrist synovitis, BME and clinical parameters, without any progression in bone erosions. The RAMRIS for synovitis rapidly improved from as early as two weeks after the first TCZ infusion. (Funded by F. Hoffmann–La Roche; ACTRACE EudraCT No. 2009 012185-32).Keywords: tocilizumab, rheumatoid arthritis, wrist, synovitis, low-field MRI, dedicated MRI

Published
2020

3. Mortality risk factors in primary Sjögren syndrome: a real-world, retrospective, cohort study

Author

Brito-Zerón, Pilar, primary, Flores-Chávez, Alejandra, additional, Horváth, Ildiko Fanny, additional, Rasmussen, Astrid, additional, Li, Xiaomei, additional, Olsson, Peter, additional, Vissink, Arjan, additional, Priori, Roberta, additional, Armagan, Berkan, additional, Hernandez-Molina, Gabriela, additional, Praprotnik, Sonja, additional, Quartuccio, Luca, additional, Inanç, Nevsun, additional, Özkızıltaş, Burcugül, additional, Bartoloni, Elena, additional, Sebastian, Agata, additional, Romão, Vasco C., additional, Solans, Roser, additional, Pasoto, Sandra G., additional, Rischmueller, Maureen, additional, Galisteo, Carlos, additional, Suzuki, Yasunori, additional, Trevisani, Virginia Fernandes Moça, additional, Fugmann, Cecilia, additional, González-García, Andrés, additional, Carubbi, Francesco, additional, Jurcut, Ciprian, additional, Shimizu, Toshimasa, additional, Retamozo, Soledad, additional, Atzeni, Fabiola, additional, Hofauer, Benedikt, additional, Melchor-Díaz, Sheila, additional, Gheita, Tamer, additional, López-Dupla, Miguel, additional, Fonseca-Aizpuru, Eva, additional, Giacomelli, Roberto, additional, Vázquez, Marcos, additional, Consani, Sandra, additional, Akasbi, Miriam, additional, Nakamura, Hideki, additional, Szántó, Antónia, additional, Farris, A. Darise, additional, Wang, Li, additional, Mandl, Thomas, additional, Gattamelata, Angelica, additional, Kilic, Levent, additional, Pirkmajer, Katja Perdan, additional, Abacar, Kerem, additional, Tufan, Abdurrahman, additional, de Vita, Salvatore, additional, Bootsma, Hendrika, additional, Ramos-Casals, Manuel, additional, Arends, S., additional, Treppo, E., additional, Longhino, S., additional, Manfrè, V., additional, Rizzo, M., additional, Baldini, C., additional, Bombardieri, S., additional, Bandeira, M., additional, Silvéiro-António, M., additional, Seror, R., additional, Mariette, X., additional, Nordmark, G., additional, Danda, D., additional, Wiland, P., additional, Gerli, R., additional, Kwok, S.K., additional, Park, S.H., additional, Kvarnstrom, M., additional, Wahren-Herlenius, M., additional, Downie-Doyle, S., additional, Sene, D., additional, Isenberg, D., additional, Valim, V., additional, Devauchelle-Pensec, V., additional, Saraux, A., additional, Morel, J., additional, Morcillo, C., additional, Díaz Cuiza, P.E., additional, Herrera, B.E., additional, González-de-Paz, L., additional, and Sisó-Almirall, A., additional

Published
2023
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4. 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

5. Exposure to air pollution as an environmental determinant of how Sjögren's disease is expressed at diagnosis

Author

Brito-Zeron, P., Flores-Chavez, A., Ng, W. -F, Horvath, I. Fanny, Rasmussen, A., Priori, R., Baldini, C., Armagan, B., Ozkiziltas, B., Praprotnik, S., Suzuki, Y., Quartuccio, L., Hernandez-Molina, G., Abacar, K., Bartoloni, E., Rischmueller, M., Reis-de Oliveira, F., Trevisani, V. Fernandes Moca, Jurcut, C., Fugmann, Cecilia, Carubbi, F., Hofauer, B., Valim, V., Pasoto, S. G., Retamozo, S., Atzeni, F., Fonseca-Aizpuru, E., Lopez-Dupla, M., Giacomelli, R., Nakamura, H., Akasbi, M., Thompson, K., Szanto, A., Farris, A. D., Villa, M., Bombardieri, S., Kilic, L., Tufan, A., Pirkmajer, K. Perdan, Fujisawa, Y., De Vita, S., Inanc, N., Ramos-Casals, M., Brito-Zeron, P., Flores-Chavez, A., Ng, W. -F, Horvath, I. Fanny, Rasmussen, A., Priori, R., Baldini, C., Armagan, B., Ozkiziltas, B., Praprotnik, S., Suzuki, Y., Quartuccio, L., Hernandez-Molina, G., Abacar, K., Bartoloni, E., Rischmueller, M., Reis-de Oliveira, F., Trevisani, V. Fernandes Moca, Jurcut, C., Fugmann, Cecilia, Carubbi, F., Hofauer, B., Valim, V., Pasoto, S. G., Retamozo, S., Atzeni, F., Fonseca-Aizpuru, E., Lopez-Dupla, M., Giacomelli, R., Nakamura, H., Akasbi, M., Thompson, K., Szanto, A., Farris, A. D., Villa, M., Bombardieri, S., Kilic, L., Tufan, A., Pirkmajer, K. Perdan, Fujisawa, Y., De Vita, S., Inanc, N., and Ramos-Casals, M.

Abstract

ObjectiveTo analyse how the potential exposure to air pollutants can influence the key components at the time of diagnosis of Sjogren's phenotype (epidemiological profile, sicca symptoms, and systemic disease). MethodsFor the present study, the following variables were selected for harmonisation and refinement: age, sex, country, fulfilment of 2002/2016 criteria items, dry eyes, dry mouth, and overall ESSDAI score. Air pollution indexes per country were defined according to the OECD (1990-2021), including emission data of nitrogen and sulphur oxides (NO/SO), particulate matter (PM2.5 and 1.0), carbon monoxide (CO) and volatile organic compounds (VOC) calculated per unit of GDP, Kg per 1000 USD.ResultsThe results of the chi-square tests of independence for each air pollutant with the frequency of dry eyes at diagnosis showed that, except for one, all variables exhibited p-values <0.0001. The most pronounced disparities emerged in the dry eye prevalence among individuals inhabiting countries with the highest NO/SO exposure, a surge of 4.61 percentage points compared to other countries, followed by CO (3.59 points), non-methane (3.32 points), PM2.5 (3.30 points), and PM1.0 (1.60 points) exposures. Concerning dry mouth, individuals residing in countries with worse NO/SO exposures exhibited a heightened frequency of dry mouth by 2.05 percentage points (p<0.0001), followed by non-methane exposure (1.21 percentage points increase, p=0.007). Individuals inhabiting countries with the worst NO/SO, CO, and PM2.5 pollution levels had a higher mean global ESSDAI score than those in lower-risk nations (all p-values <0.0001). When systemic disease was stratified according to DAS into low, moderate, and high systemic activity levels, a heightened proportion of individuals manifesting moderate/severe systemic activity was observed in countries with worse exposures to NO/SO, CO, and PM2.5 pollutant levels. ConclusionFor the first time, we suggest that pollution levels could

Published
2023
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6. Influence of exposure to climate-related hazards in the phenotypic expression of primary Sjögren's syndrome

Author

Flores-Chavez, A., Brito-Zeron, P., Ng, W. -f., Szanto, A., Rasmussen, A., Priori, R., Baldini, C., Armagan, B., Oezkiziltas, B., Praprotnik, S., Suzuki, Y., Quartuccio, L., Hernandez-Molina, G., Inanc, N., Bartoloni, E., Rischmueller, M., Oliveira, F. Reis-de, Trevisani, V. Fernandes Moca, Jurcut, C., Nordmark, Gunnel, Carubbi, F., Hofauer, B., Valim, V., Pasoto, S. G., Retamozo, S., Atzeni, F., Fonseca-Aizpuru, E., Lopez-Dupla, M., Giacomelli, R., Nakamura, H., Akasbi, M., Thompson, K., Horvath, I. Fanny, Farris, A. D., Simoncelli, E., Bombardieri, S., Kilic, L., Tufan, A., Pirkmajer, K. Perdan, Fujisawa, Y., De Vita, S., Abacar, K., Ramos-Casals, M., Flores-Chavez, A., Brito-Zeron, P., Ng, W. -f., Szanto, A., Rasmussen, A., Priori, R., Baldini, C., Armagan, B., Oezkiziltas, B., Praprotnik, S., Suzuki, Y., Quartuccio, L., Hernandez-Molina, G., Inanc, N., Bartoloni, E., Rischmueller, M., Oliveira, F. Reis-de, Trevisani, V. Fernandes Moca, Jurcut, C., Nordmark, Gunnel, Carubbi, F., Hofauer, B., Valim, V., Pasoto, S. G., Retamozo, S., Atzeni, F., Fonseca-Aizpuru, E., Lopez-Dupla, M., Giacomelli, R., Nakamura, H., Akasbi, M., Thompson, K., Horvath, I. Fanny, Farris, A. D., Simoncelli, E., Bombardieri, S., Kilic, L., Tufan, A., Pirkmajer, K. Perdan, Fujisawa, Y., De Vita, S., Abacar, K., and Ramos-Casals, M.

Abstract

Objective To analyse how the key components at the time of diagnosis of the Sjogren's phenotype (epidemiological profile, sicca symptoms, and systemic disease) can be influenced by the potential exposure to climate-related natural hazards. Methods For the present study, the following variables were selected for harmonisation and refinement: age, sex, country, fulfilment of 2002/2016 criteria items, dry eyes, dry mouth, and overall ESSDAI score. Climate-related hazards per country were defined according to the OECD and included seven climate-related hazard types: extreme temperature, extreme precipitation, drought, wildfire, wind threats, river flooding, and coastal flooding. Climatic variables were defined as dichotomous variables according to whether each country is ranked among the ten countries with the most significant exposure. Results After applying data-cleaning techniques and excluding people from countries not included in the OECD climate rankings, the database study analysed 16,042 patients from 23 countries. The disease was diagnosed between 1 and 3 years earlier in people living in countries included among the top 10 worst exposed to extreme precipitation, wildfire, wind threats, river flooding, and coastal flooding. A lower frequency of dry eyes was observed in people living in countries exposed to wind threats, river flooding, and coastal flooding, with a level of statistical association being classified as strong (p<0.0001 for the three variables). The frequency of dry mouth was significantly lower in people living in countries exposed to river flooding (p<0.0001) and coastal flooding (p<0.0001). People living in countries included in the worse climate scenarios for extreme temperature (p<0.0001) and river flooding (p<0.0001) showed a higher mean ESSDAI score in comparison with people living in no-risk countries. In contrast, those living in countries exposed to worse climate scenarios for wind threats (p<0.0001) and coastal flooding

Published
2023
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7. COVID-19: The new challenge for rheumatologists. One year later

Author

Puxeddu, I, Ferro, F, Bartoloni, E, Elefante, E, Baldini, C, Scire, C, Talarico, R, Migliorini, P, Mosca, M, Bombardieri, S, Puxeddu I., Ferro F., Bartoloni E., Elefante E., Baldini C., Scire C. A., Talarico R., Migliorini P., Mosca M., Bombardieri S., Puxeddu, I, Ferro, F, Bartoloni, E, Elefante, E, Baldini, C, Scire, C, Talarico, R, Migliorini, P, Mosca, M, Bombardieri, S, Puxeddu I., Ferro F., Bartoloni E., Elefante E., Baldini C., Scire C. A., Talarico R., Migliorini P., Mosca M., and Bombardieri S.

Abstract

At the beginning of COVID-19, we underlined that this pandemic was a new challenge for rheumatologists. On the one hand, it was necessary to clarify the impact of this new viral disease on the natural history of many rheumatic diseases and, on the other hand, to define the beneficial or harmful effects of the synthetic or targeted therapies used for their treatment. In addition, we have postulated that in view of the common pathogenetic mechanisms involved, the therapeutic armamentarium currently employed in the management of viral or idiopathic systemic autoimmune rheumatic diseases could be useful to control the “cytokine storm” induced by SARS-COV-2. One year later, in the present review we have analysed the progress of the knowledge on both these aspects and updated the algorithms initially proposed for a rational use of the synthetic and targeted anti-inflammatory and immunomodulatory agents in the management of COVID-19.

Published
2021

9. Childhood-onset of primary Sjögren’s syndrome: phenotypic characterization at diagnosis of 158 children

Author

Ramos-Casals, M., Acar-Denizli, N., Vissink, A., Brito-Zeron, P., Li, X., Carubbi, F., Priori, R., Toplak, N., Baldini, C., Faugier-Fuentes, E., Kruize, A. A., Mandl, T., Tomiita, M., Gandolfo, S., Hashimoto, K., Hernandez-Molina, G., Hofauer, B., Mendieta-Zeron, S., Rasmussen, A., Sandhya, P., Sene, D., Trevisani, V. F. M., Isenberg, D., Sundberg, E., Pasoto, S. G., Sebastian, A., Suzuki, Y., Retamozo, S., Xu, B., Giacomelli, R., Gattamelata, A., Bizjak, M., Bombardieri, S., Loor-Chavez, R. -E., Hinrichs, A., Olsson, P., Bootsma, H., Lieberman, S. M., Kostov, B., Horvath, I. -F., Szanto, A., Seror, R., Mariette, X., Kvarnstrom, M., Wahren-Herlenius, M., Praprotnik, S., Solans, R., Nordmark, G., Hammenfors, D., Brun, J. G., Gheita, T. A., Atzeni, F., Armagan, B., Kilic, L., Kalyoncu, U., Nakamura, T., Takagi, Y., Consani, S., Solorzano, F. O., Translational Immunology Groningen (TRIGR), Personalized Healthcare Technology (PHT), Universitat Politècnica de Catalunya. Departament d'Estadística i Investigació Operativa, and Universitat Politècnica de Catalunya. ADBD - Anàlisi de Dades Complexes per a les Decisions Empresarials

Subjects
Male, Systemic disease, Anti-nuclear antibody, Epidemiology, Autoimmune diseases, Matemàtiques i estadística::Matemàtica aplicada a les ciències [Àrees temàtiques de la UPC], Disease, Severity of Illness Index, Parotid Gland, Medicine, CLASSIFICATION CRITERIA, Pharmacology (medical), Registries, Age of Onset, biology, 92 Biology and other natural sciences::92B Mathematical biology in general [Classificació AMS], Dry eyes, Phenotype, Sjogren's syndrome, Female, epidemiology, Antibody, medicine.symptom, PAROTITIS, medicine.medical_specialty, Biomatemàtica, Adolescent, 62 Statistics::62D05 Sampling theory, sample surveys [Classificació AMS], Childhood, Paediatrics, Humans, Sjogren's Syndrome, paediatrics, AGE, Rheumatology, Peripheral nerve, Rheumatoid factor, autoimmune diseases, Sampling (Statistics), Primary Sjögren Syndrome, childhood, Biomathematics, Matemàtiques i estadística::Estadística aplicada::Estadística biosanitària [Àrees temàtiques de la UPC], business.industry, CLINICAL-FEATURES, medicine.disease, Dry mouth, Dermatology, stomatognathic diseases, biology.protein, Sjogren’s syndrome, CONSENSUS, business, Mostreig (Estadística), Parotitis
Abstract

Objectives To characterize the phenotypic presentation at diagnosis of childhood-onset primary SS. Methods The Big Data Sjögren Project Consortium is an international, multicentre registry using worldwide data-sharing cooperative merging of pre-existing clinical SS databases from the five continents. For this study, we selected those patients in whom the disease was diagnosed below the age of 19 years according to the fulfilment of the 2002/2016 classification criteria. Results Among the 12 083 patients included in the Sjögren Big Data Registry, 158 (1.3%) patients had a childhood-onset diagnosis (136 girls, mean age of 14.2 years): 126 (80%) reported dry mouth, 111 (70%) dry eyes, 52 (33%) parotid enlargement, 118/122 (97%) positive minor salivary gland biopsy and 60/64 (94%) abnormal salivary US study, 140/155 (90%) positive ANA, 138/156 (89%) anti-Ro/La antibodies and 86/142 (68%) positive RF. The systemic EULAR Sjögren’s syndrome disease activity index (ESSDAI) domains containing the highest frequencies of active patients included the glandular (47%), articular (26%) and lymphadenopathy (25%) domains. Patients with childhood-onset primary SS showed the highest mean ESSDAI score and the highest frequencies of systemic disease in 5 (constitutional, lymphadenopathy, glandular, cutaneous and haematological) of the 12 ESSDAI domains, and the lowest frequencies in 4 (articular, pulmonary, peripheral nerve and CNS) in comparison with patients with adult-onset disease. Conclusions Childhood-onset primary SS involves around 1% of patients with primary SS, with a clinical phenotype dominated by sicca features, parotid enlargement and systemic disease. Age at diagnosis plays a key role in modulating the phenotypic expression of the disease.

Published
2021
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10. 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

11. Efficacy and Safety of Epratuzumab in Moderately to Severely Active Systemic Lupus Erythematosus: Results From Two Phase III Randomized, Double‐Blind, Placebo‐Controlled Trials

Author

Clowse, Megan E. B., Wallace, Daniel J., Furie, Richard A., Petri, Michelle A., Pike, Marilyn C., Leszczyński, Piotr, Neuwelt, C. Michael, Hobbs, Kathryn, Keiserman, Mauro, Duca, Liliana, Kalunian, Kenneth C., Galateanu, Catrinel, Bongardt, Sabine, Stach, Christian, Beaudot, Carolyn, Kilgallen, Brian, Gordon, Caroline, Batalov, A., Bojinca, M., Djerassi, R., Duca, L., Horak, P., Kolarov, Z., Milasiene, R., Monova, D., Otsa, K., Pileckyte, M., Popova, T., Radulescu, F., Rashkov, R., Rednic, S., Repin, M., Stoilov, R., Tegzova, D., Vezikova, N., Vitek, P., Zainea, C., East, Far, Baek, H., Chen, Y., Chiu, Y., Cho, C., Chou, C., Choe, J., Huang, C., Kang, Y., Kang, S., Lai, N., Lee, S., Park, W., Shim, S., Suh, C., Yoo, W., Armengol, H. Avila, Zapata, F. Avila, Santiago, M. Barreto, Cavalcanti, F., Chahade, W., Costallat, L., Keiserman, M., Alcala, J. Orozco, Remus, C. Ramos, Roimicher, L., AbuShakra, M., Agarwal, V., AgmonLevin, N., Kadel, J., Levy, Y., Mevorach, D., Paran, D., Reitblat, T., Rosner, I., Shobha, V., Sthoeger, Z., Zisman, D., Ayesu, K., Berney, S., Box, J., Busch, H., Buyon, J., Carter, J., Chi, J., Clowse, M., Collins, R., Dao, K., Diab, I., Dikranian, A., ElShahawy, M., Gaylis, N., Grossman, J., Halpert, E., Huff, J., Jarjour, W., Kao, A., Katz, R., Kennedy, A., Khan, M., Kivitz, A., Kohen, M., LawrenceFord, T., Lawson, J., Levesque, M., Lowenstein, M., Majjhoo, A., Mcarthur, R., McLain, D., Merrill, J., Murillo, A., Neucks, S., Niemer, G., Noaiseh, G., Parker, C., Pantojas, C., Pattanaik, D., Petri, M., Pickrell, P., Reveille, J., RomanMiranda, A., Rothfield, N., Sankoorikal, A., Sayers, M., Singhal, A., Snyder, A., Striebich, C., Vo, Q., von Feldt, J., Wallace, D., Wasko, M., Young, C., Adelstein, S., Hall, S., Littlejohn, G., Nicholls, D., Suranyi, M., Amoura, Z., Bannert, B., Behrens, F., Perez, L.Carreno, Chakravarty, K., Gonzales, F. Diaz, Davies, K., Doria, A., Emery, P., FernándezNebro, A., Govoni, M., Hachulla, E., Hellmich, B., Houssiau, F., Malaise, M., Margaux, J., Maugars, Y., MuñozFernández, S., Navarro, F., OrdiRos, J., Pellerito, R., PenaSagredo, J., Roussou, E., Schmidt, R. E., UcarAngulo, E., Viallard, JF., Westhovens, R., Worm, M., Yee, C. S., Nayiager, S., Reuter, H., Spargo, C., Bazela, B., Brzosko, M., Chudzik, D., Gasztonyi, B., Geher, P., Ionescu, R., Jeka, S., Kemeny, L., Kiss, E., Kotyla, P., Kovacs, L., Kovalenko, V., Kucharz, E., Kwiatkowska, B., Leszczynski, P., Levchenko, E., Lysenko, G., Majdan, M., Mihailov, C., Nalotov, S., Nedelciu, M., Pavel, M., Raskina, T., Rebrov, B., Rezus, E., Semen, T., Smakotina, S., Stanislavchuk, M., Stanislav, M., Szombati, I., Szucs, G., Udrea, G., Zajdel, J., ZonGiebel, A., Bonfiglioli, R., Bustamante, R., Klumb, E., Ramirez, G. Medrano, Neiva, C., Olguin, M., Gonzaga, J.Reyes, Scotton, A., Ayala, S. Sicsik, Ximenes, A., Sharma, R., Srikantiah, C., Aelion, J., Aranow, C., Baker, M., Chadha, A., Chao, J., Chatham, W., Chow, A., Clay, C., CohenGadol, S., Conaway, D., Denburg, J., Escalante, A., Espinoza, L., Fiechtner, J., Fortin, I., Fraser, A., Furie, R., Gladman, D., Goddard, D., Goldberg, M., GonzalezRivera, R., Gorman, J., Griffin, R., Haaland, D., Halter, D., Hemaiden, A., Hobbs, K., Joshi, V., Lim, S., Kalunian, K., Karpouzas, G., Khraishi, M., Lafyatis, R., Lee, S., Lidman, R., Lue, C., Mohan, M., Mease, P., Mehta, C., Mizutani, W., Nami, A., Nascimento, J., Neuwelt, C., Pappas, J., Pope, J., Porges, A., Roane, G., Rosenberg, D., Ross, S., Saadeh, C., Scoville, C., Sherrer, Y., Solomon, M., Surbeck, W., Valenzuela, G., Waller, P., Alten, R., Baerwald, C., Bienvenu, B., Bombardieri, S., Braun, J., Dival, L., Espinosa, G., Fernandez, I. Figueroa, GomezReino, J., Gordon, C., Hiepe, F., Hopkinson, N., Isenberg, D., Jacobi, A., Jorgensen, C., Guern, V. Le, Paul, C., PegoReigosa, J. M., Heredia, J. Rodriguez, RubbertRoth, A., Sabbadini, M., Schroeder, J., Schwarting, A., Spieler, W., Valesini, G., Wollenhaupt, J., Mendoza, A. Zea, and Zouboulis, C.

Published
2017
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12. 2016 American College of Rheumatology/European League Against Rheumatism classification criteria for primary Sjögrenʼs syndrome: A consensus and data-driven methodology involving three international patient cohorts

Author

Shiboski, Caroline H, Shiboski, Stephen C, Seror, Raphaèle, Criswell, Lindsey A, Labetoulle, Marc, Lietman, Thomas M, Rasmussen, Astrid, Scofield, Hal, Vitali, Claudio, Bowman, Simon J, Mariette, Xavier, Heidenreich, AM, Lanfranchi, H, Vollenweider, C, Schiødt, M., Devauchelle, V., Gottenberg, JE, Saraux, A, Pincemin, Maggy, Dörner, T, Tzoufias, A, Baldini, C, Bombardieri, S, De Vita, S, Kitagawa, K, Sumida, T, Umehara, H, Bootsma, H, Kruize, AA, Radstake, TR, Vissink, A, Jonsson, R, Ramos-Casals, M, Theander, E, Challacombe, S, Fisher, B, Kirkham, B, Larkin, G, Ng, F, Rauz, S, Akpek, E, Atkinson, J, Baer, AN, Carsons, S, Carteron, N, Daniels, T, Fox, B, Greenspan, J, Illei, G, Nelson, D, Parke, A, Pillemer, S, Segal, B, Sivils, K, Clair, EW St, Stone, D, Vivino, F, Wu, A, and Hammitt, Kathy

Published
2017
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13. 2016 American College of Rheumatology/European League Against Rheumatism Classification Criteria for Primary Sjögrenʼs Syndrome: A Consensus and Data‐Driven Methodology Involving Three International Patient Cohorts

Author

Shiboski, Caroline H., Shiboski, Stephen C., Seror, Raphaèle, Criswell, Lindsey A., Labetoulle, Marc, Lietman, Thomas M., Rasmussen, Astrid, Scofield, Hal, Vitali, Claudio, Bowman, Simon J., Mariette, Xavier, Heidenreich, A. M., Lanfranchi, H., Vollenweider, C, Schiødt, M., Devauchelle, V, Gottenberg, J. E, Saraux, A, Pincemin, Maggy, Dörner, T., Tzoufias, A, Baldini, C, Bombardieri, S, De Vita, S., Kitagawa, K, Sumida, T, Umehara, H, Bootsma, H, Kruize, A. A., Radstake, T. R., Vissink, A, Jonsson, R, Ramos‐Casals, M., Theander, E, Challacombe, S, Fisher, B, Kirkham, B, Larkin, G, Ng, F, Rauz, S, Akpek, E, Atkinson, J, Baer, A. N., Carsons, S, Carteron, N, Daniels, T., Fox, B, Greenspan, J, Illei, G, Nelson, D, Parke, A, Pillemer, S, Segal, B, Sivils, K, St.Clair, E. W., Stone, D, Vivino, F, and Wu, A.

Published
2017
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14. 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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16. List of Contributors

Author

Alunno, A., primary, Appel, S., additional, Astorri, E., additional, Baldini, C., additional, Barone, F., additional, Bartoloni, E., additional, Bombardieri, M., additional, Bombardieri, S., additional, Bowman, S.J., additional, Campos, J., additional, Carubbi, F., additional, Cipriani, P., additional, Colafrancesco, S., additional, De Vita, S., additional, Del Papa, N., additional, Devauchelle-Pensec, V., additional, Felten, R., additional, Fisher, B.A., additional, Fox, C.M., additional, Fox, R.I., additional, Gandolfo, S., additional, Gerli, R., additional, Giacomelli, R., additional, Gottenberg, J.-E., additional, Jonsson, R., additional, Kapsogeorgou, E.K., additional, Kyttaris, V.C., additional, Lucchesi, D., additional, Lunardi, C., additional, Manoussakis, M.N., additional, Mavragani, C.P., additional, Patuzzo, G., additional, Perricone, C., additional, Pers, J.-O., additional, Pitzalis, C., additional, Priori, R., additional, Quartuccio, L., additional, Shoenfeld, Y., additional, Sibilia, J., additional, Tinazzi, E., additional, Tsokos, G.C., additional, Tzioufas, A.G., additional, Valesini, G., additional, Vitali, C., additional, and Youinou, P., additional

Published
2016
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18. Influence of the age at diagnosis in the disease expression of primary Sjögren's syndrome: Analysis of 12,753 patients from the Sjögren Big Data Consortium

Author

Retamozo, S., Acar-Denizli, N., Horváth, I. F., Ng, W. -F, Rasmussen, A., Dong, X., Li, X., Baldini, C., Olsson, P., Priori, R., Seror, R., Gottenberg, J. -E, Kruize, A. A., Hernandez-Molina, G., Vissink, A., Sandhya, P., Armagan, B., Quartuccio, L., Sebastian, A., Praprotnik, S., Bartoloni, E., Kwok, S. -K, Kvarnstrom, M., Rischmueller, M., Soláns-Laqué, R., Sene, D., Pasoto, S. G., Suzuki, Y., Isenberg, D. A., Valim, V., Nordmark, G., Nakamura, H., Virginia Trevisani, Hofauer, B., Sisó-Almirall, A., Giacomelli, R., Devauchelle-Pensec, V., Bombardieri, M., Atzeni, F., Hammenfors, D., Maure, B., Carsons, S. E., Gheita, T., Sánchez-Berná, I., López-Dupla, M., Morel, J., Inanç, N., Fonseca-Aizpuru, E., Morcillo, C., Vollenweider, C., Melchor, S., Vázquez, M., Díaz-Cuiza, E., Consani-Fernández, S., De-Miguel-Campo, B., Szántó, A., Bombardieri, S., Gattamelata, A., Hinrichs, A., Sánchez-Guerrero, J., Danda, D., Kilic, L., Vita, S., Wiland, P., Gerli, R., Park, S. -H, Wahren-Herlenius, M., Bootsma, H., Mariette, X., Ramos-Casals, M., Brito-Zerón, P., Translational Immunology Groningen (TRIGR), and Personalized Healthcare Technology (PHT)

Subjects
immunological markers, MANIFESTATIONS, age, Sjogren's syndrome, ONSET, YOUNG, MANAGEMENT, LYMPHOMA, disease phenotype, CLASSIFICATION CRITERIA, CONSENSUS, PROJECT, SALIVARY FLOW
Abstract

Objective. To analyse how the main components of the disease phenotype (sicca symptoms, diagnostic tests, immunological markers and systemic disease) can be driven by the age at diagnosis of primary Sjogren's syndrome (pSS). Methods. By January 2021, the participant centres had included 12,753 patients from 25 countries that fulfilled the 2002/2016 classification criteria for pSS. The age at diagnosis was defined as the time when the attending physician confirmed fulfilment of the criteria. Patients were clustered according to age at diagnosis. 50 clusters with more than 100 observations (from 27 to 76 years) were used to study the influence of the age at diagnosis in the disease expression. Results. There was a consistent increase in the frequency of oral dryness according to the age at diagnosis, with a frequency of 95% in those diagnosed at the oldest ages. The smooth curves that best fitted a linear model were the frequency of dry mouth (adjusted R-2 0.87) and the frequency of abnormal oral tests (adjusted R-2 0.72). Therefore, for each 1-year increase in the age at diagnosis, the frequency of dry mouth increased by 0.13%, and the frequency of abnormal oral diagnostic tests by 0.11%. There was a consistent year-by-year decrease in the frequency of all autoantibodies and immunological markers except for cryoglobulins. According to the linear models, for each 1-year increase in the age at diagnosis, the frequency of a positive result decreased by 0.57% (for anti-Ro antibodies), 0.47% (for RF) and 0.42% (for anti-La antibodies). The ESSDAI domains which showed a more consistent decrease were glandular and lymph node involvement (for each 1-year increase in the age at diagnosis, the frequency of activity decreased by 0.18%), and constitutional, cutaneous, and haematological involvements (the frequency decreased by 0.09% for each 1-year increase). In contrast, other domains showed an ascending pattern, especially pulmonary involvement (for each 1-year increase in the age at diagnosis, the frequency of activity increased by 0.22%), and peripheral nerve involvement (the frequency increased by 0.09% for each 1-year increase). Conclusion. The influence of the age at diagnosis on the key phenotypic features of pSS is strong, and should be considered critical not only for designing a personalised diagnostic approach, but also to be carefully considered when analysing the results of diagnostic tests and immunological parameters, and when internal organ involvement is suspected at diagnosis.

Published
2021

20. Contributors

Author

Abdurakhmanov, Alexander, primary, Abu-Shakra, Mahmoud, additional, Afanasyeva, Marina, additional, Agmon-Levin, Nancy, additional, Albert, Paul J., additional, Almeida, Isabel, additional, Alves, Rute, additional, Amital, Howard, additional, Andrade, Paulo, additional, Antonelli, Alessandro, additional, Puccetti, Antonio, additional, Arango, María-Teresa, additional, Atzeni, Fabiola, additional, Baird, Alison E., additional, Balbir-Gurman, Alexandra, additional, Bashi, Tomer, additional, Batticciotto, Alberto, additional, Benucci, Maurizio, additional, Blank, Miri, additional, Bogdanos, Dimitrios P., additional, Bombardieri, S., additional, Borella, E., additional, Bosis, Samantha, additional, Bournia, Vasiliki Kalliopi, additional, Brandão, Mariana, additional, Braun-Moscovici, Yolanda, additional, Brender-Gotlieb, Neta, additional, Cainelli, Francesca, additional, Caldas, Cezar Augusto Muniz, additional, Campar, A., additional, Carvalheiras, Graziela, additional, Cervera, R., additional, Chapman, Joab, additional, Chastain, Emily M.L., additional, Claudio, Lunardi, additional, Cohen, Eytan, additional, Conti, Fabrizio, additional, Correia, J., additional, Carvalho, Jozélio Freire de, additional, Rossa, A. Della, additional, Detrick, Barbara, additional, Deutsch, Melanie, additional, Di Domenicantonio, Andrea, additional, Domeneghetti, M., additional, Domingues, Vital, additional, Doria, A., additional, Dow, Coad Thomas, additional, Dreyfus, David H., additional, Drouin, Elise E., additional, Dubaniewicz, Anna, additional, Durai, Malarvizhi, additional, Ebringer, Alan, additional, Ehrenfeld, Michael, additional, Elisa, Tinazzi, additional, Esposito, Susanna, additional, Fallahi, Poupak, additional, Faria, Raquel, additional, Farinha, Fátima, additional, Ferrannini, Ele, additional, Ferrari, Silvia Martina, additional, Ferreira, Alvaro, additional, Ferrão, C., additional, Garg, Ravindra Kumar, additional, Gatto, M., additional, Ghirardello, A., additional, Giovanna, Zanoni, additional, Giuseppe, Patuzzo, additional, Givaty, Gili, additional, Guilherme, Luiza, additional, Hammerstad, Sara Salehi, additional, Hodak, Emillia, additional, Hooks, John J., additional, Iaccarino, L., additional, Invernizzi, Pietro, additional, Israeli, Eitan, additional, Jamin, Christophe, additional, Janket, Sok-Ja, additional, Jarčuška, Peter, additional, Jones, Rodney P., additional, Kalil, Jorge, additional, Kanasi, Eleni, additional, Kivity, Shaye, additional, Konikoff, Tom, additional, Kozáková, D., additional, Krause, Ilan, additional, Lerner, Aaron, additional, Lidar, Merav, additional, Ling, Eduard, additional, Mahajna, Hussein, additional, Mahroum, Naim, additional, Maoz-Segal, Ramit, additional, Marinho, António, additional, Marshall, Trevor G., additional, Martinelli, Maria, additional, Marzia, Dolcino, additional, Mavragani, Clio P., additional, Mendonça, Teresa, additional, Miller, Stephen D., additional, Mimouni, Daniel, additional, Monteiro, Marta, additional, Moudgil, Kamal D., additional, Moulton, Vaishali R., additional, Moutsopoulos, Haralampos M., additional, Nagpal, Kamalpreet, additional, Neves, Esmeralda, additional, Nussenblatt, Robert, additional, Ollech, Ayelet, additional, Palma, L., additional, Pasoto, Sandra Gofinet, additional, Pella, Daniel, additional, Pereira, Cláudia, additional, Perricone, Carlo, additional, Petríková, Jana, additional, Proal, Amy D., additional, Rashid, Taha, additional, Reif, Shimon, additional, Renaudineau, Yves, additional, Ribeiro, Francinne Machado, additional, Rigante, Donato, additional, Rigopoulou, Eirini I., additional, Rose, Noel R., additional, Rosário, Cristina, additional, Rovenský, J., additional, Sakkas, Lazaros I., additional, Sarzi-Puttini, Piercarlo, additional, Seguro, Luciana Parente Costa, additional, Semino, Margherita, additional, Shoenfeld, Yehuda, additional, Silva, S., additional, Simonin, Laurent, additional, Smyk, Daniel S., additional, Sousa, Rita Catarina Medeiros, additional, Stagnaro, C., additional, Steere, Allen C., additional, Strle, Klemen, additional, Tektonidou, Maria G., additional, Tishler, Moshe, additional, Tomer, Yaron, additional, Toubi, Elias, additional, Tsokos, George C., additional, Vadasz, Zahava, additional, Valesini, Guido, additional, Vasconcelos, Carlos, additional, Vasconcelos, Júlia, additional, Vassilopoulos, Dimitrios, additional, Venkatesha, Shivaprasad H., additional, Vento, Sandro, additional, Viale, Christophe, additional, Villanueva, Ronald, additional, Vita, Pedro, additional, Wilson, Clyde, additional, Youinou, Pierre, additional, Záňová, E., additional, Zandman-Goddard, Gisele, additional, and Zen, M., additional

Published
2015
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21. Undifferentiated connective tissue disease: State of the art on clinical practice guidelines

Author

Antunes, M, Scire, C, Talarico, R, Alexander, T, Avcin, T, Belocchi, C, Doria, A, Franceschini, F, Galetti, I, Govoni, M, Hachulla, E, Launay, D, Lepri, G, Macieira, C, Matucci-Cerinic, M, Montecucco, C, Moraes-Fontes, M, Mouthon, L, Paolino, S, Ramoni, V, Tani, C, Tas, S, Tincani, A, Van Vollenhoven, R, Zen, M, Fonseca, J, Bombardieri, S, Schneider, M, Smith, V, Cutolo, M, Mosca, M, Beretta, L, Antunes M., Scire C. A., Talarico R., Alexander T., Avcin T., Belocchi C., Doria A., Franceschini F., Galetti I., Govoni M., Hachulla E., Launay D., Lepri G., Macieira C., Matucci-Cerinic M., Montecucco C. M., Moraes-Fontes M. F., Mouthon L., Paolino S., Ramoni V., Tani C., Tas S. W., Tincani A., Van Vollenhoven R., Zen M., Fonseca J., Bombardieri S., Fonseca J. E., Schneider M., Smith V., Cutolo M., Mosca M., Beretta L., Antunes, M, Scire, C, Talarico, R, Alexander, T, Avcin, T, Belocchi, C, Doria, A, Franceschini, F, Galetti, I, Govoni, M, Hachulla, E, Launay, D, Lepri, G, Macieira, C, Matucci-Cerinic, M, Montecucco, C, Moraes-Fontes, M, Mouthon, L, Paolino, S, Ramoni, V, Tani, C, Tas, S, Tincani, A, Van Vollenhoven, R, Zen, M, Fonseca, J, Bombardieri, S, Schneider, M, Smith, V, Cutolo, M, Mosca, M, Beretta, L, Antunes M., Scire C. A., Talarico R., Alexander T., Avcin T., Belocchi C., Doria A., Franceschini F., Galetti I., Govoni M., Hachulla E., Launay D., Lepri G., Macieira C., Matucci-Cerinic M., Montecucco C. M., Moraes-Fontes M. F., Mouthon L., Paolino S., Ramoni V., Tani C., Tas S. W., Tincani A., Van Vollenhoven R., Zen M., Fonseca J., Bombardieri S., Fonseca J. E., Schneider M., Smith V., Cutolo M., Mosca M., and Beretta L.

Abstract

The term a € undifferentiated connective tissue disease' (UCTD) is generally used to describe clinical entities characterised by clinical and serological manifestations of systemic autoimmune diseases but not fulfilling the criteria for defined connective tissue diseases (CTDs). In this narrative review, we summarise the results of a systematic literature research, which was performed as part of the ERN ReCONNET project, aimed at evaluating existing clinical practice guidelines (CPGs) or recommendations. No specific CPG on UCTD were found, potential areas of intervention are absence of a consensus definition of UCTD, need for specific monitoring and therapeutic protocols, stratification of UCTD based on the risk of developing a defined CTD and preventive measure for the future development of a more severe condition. Patients feel uncertainty regarding the name of the disease and feel the need of a better education and understanding of these conditions and its possible changes over time.

Published
2019

22. Idiopathic inflammatory myopathies: Narrative review of unmet needs in clinical practice guidelines

Author

Meyer, A, Scire, C, Talarico, R, Alexander, T, Amoura, Z, Avcin, T, Barsotti, S, Beretta, L, Blagojevic, J, Burmester, G, Cavazzana, I, Cherrin, P, Damian, L, Doria, A, Fonseca, J, Furini, F, Galetti, I, Houssiau, F, Krieg, T, Maddalena, L, Launay, D, Campanilho-Marques, R, Martin, T, Matucci-Cerinic, M, Moinzadeh, P, Montecucco, C, Moraes-Fontes, M, Mouthon, L, Neri, R, Paolino, S, Piette, Y, Rednic, S, Tamirou, F, Tincani, A, Toplak, N, Bombardieri, S, Hachulla, E, Mueller-Ladner, U, Schneider, M, Smith, V, Vieira, A, Cutolo, M, Mosca, M, Cavagna, L, Meyer A., Scire C. A., Talarico R., Alexander T., Amoura Z., Avcin T., Barsotti S., Beretta L., Blagojevic J., Burmester G., Cavazzana I., Cherrin P., Damian L., Doria A., Fonseca J. E., Furini F., Galetti I., Houssiau F., Krieg T., Maddalena L., Launay D., Campanilho-Marques R., Martin T., Matucci-Cerinic M., Moinzadeh P., Montecucco C., Moraes-Fontes M. F., Mouthon L., Neri R., Paolino S., Piette Y., Rednic S., Tamirou F., Tincani A., Toplak N., Bombardieri S., Hachulla E., Mueller-Ladner U., Schneider M., Smith V., Vieira A., Cutolo M., Mosca M., Cavagna L., Meyer, A, Scire, C, Talarico, R, Alexander, T, Amoura, Z, Avcin, T, Barsotti, S, Beretta, L, Blagojevic, J, Burmester, G, Cavazzana, I, Cherrin, P, Damian, L, Doria, A, Fonseca, J, Furini, F, Galetti, I, Houssiau, F, Krieg, T, Maddalena, L, Launay, D, Campanilho-Marques, R, Martin, T, Matucci-Cerinic, M, Moinzadeh, P, Montecucco, C, Moraes-Fontes, M, Mouthon, L, Neri, R, Paolino, S, Piette, Y, Rednic, S, Tamirou, F, Tincani, A, Toplak, N, Bombardieri, S, Hachulla, E, Mueller-Ladner, U, Schneider, M, Smith, V, Vieira, A, Cutolo, M, Mosca, M, Cavagna, L, Meyer A., Scire C. A., Talarico R., Alexander T., Amoura Z., Avcin T., Barsotti S., Beretta L., Blagojevic J., Burmester G., Cavazzana I., Cherrin P., Damian L., Doria A., Fonseca J. E., Furini F., Galetti I., Houssiau F., Krieg T., Maddalena L., Launay D., Campanilho-Marques R., Martin T., Matucci-Cerinic M., Moinzadeh P., Montecucco C., Moraes-Fontes M. F., Mouthon L., Neri R., Paolino S., Piette Y., Rednic S., Tamirou F., Tincani A., Toplak N., Bombardieri S., Hachulla E., Mueller-Ladner U., Schneider M., Smith V., Vieira A., Cutolo M., Mosca M., and Cavagna L.

Abstract

Idiopathic inflammatory myopathies (IIMs) encompass a heterogeneous group of rare autoimmune diseases characterised by muscle weakness and inflammation, but in antisynthetase syndrome arthritis and interstitial lung disease are more frequent and often inaugurate the disease. Clinical practice guidelines (CPGs) have been proposed for IIMs, but they are sparse and heterogeneous. This work aimed at identifying: i) current available CPGs for IIMs, ii) patients ' and clinicians' unmet needs not covered by CPGs. It has been performed in the framework of the European Reference Network on rare and complex connective tissue and musculoskeletal diseases (ReCONNET), a network of centre of expertise and patients funded by the European Union's Health Programme. Fourteen original CPGs were identified, notably recommending that: i) extra-muscular involvements should be assessed; ii) corticosteroids and methotrexate or azathioprine are first-line therapies of IIMs. ii) IVIG is a treatment of resistant-DM that may be also used in other resistant-IIMs; iii) physical therapy and sun protection (in DM patients) are part of the treatment; v) tumour screening for patients with DM include imaging of chest, abdomen, pelvis and breast (in woman) along with colonoscopy (in patients over 50 years); vi) disease activity and damages should be monitor using standardised and validated tools. Yet, only half of these CPGs were evidence-based. Crucial unmet needs were identified both by patients and clinicians. In particular, there was a lack of large multidisciplinary working group and of patients ' preferences. The following fields were not or inappropriately targeted: diagnosis; management of extra-muscular involvements other than skin; co-morbidities and severe manifestations.

Published
2019

23. IgG4-related diseases: State of the art on clinical practice guidelines

Author

Iaccarino, L, Talarico, R, Scire, C, Amoura, Z, Burmester, G, Doria, A, Faiz, K, Frank, C, Hachulla, E, Hie, M, Launay, D, Montecucco, C, Monti, S, Mouthon, L, Tincani, A, Toniati, P, Van Hagen, P, Van Vollenhoven, R, Bombardieri, S, Mueller-Ladner, U, Schneider, M, Smith, V, Cutolo, M, Mosca, M, Alexander, T, Iaccarino L., Talarico R., Scire C. A., Amoura Z., Burmester G., Doria A., Faiz K., Frank C., Hachulla E., Hie M., Launay D., Montecucco C., Monti S., Mouthon L., Tincani A., Toniati P., Van Hagen P. M., Van Vollenhoven R. F., Bombardieri S., Mueller-Ladner U., Schneider M., Smith V., Cutolo M., Mosca M., Alexander T., Iaccarino, L, Talarico, R, Scire, C, Amoura, Z, Burmester, G, Doria, A, Faiz, K, Frank, C, Hachulla, E, Hie, M, Launay, D, Montecucco, C, Monti, S, Mouthon, L, Tincani, A, Toniati, P, Van Hagen, P, Van Vollenhoven, R, Bombardieri, S, Mueller-Ladner, U, Schneider, M, Smith, V, Cutolo, M, Mosca, M, Alexander, T, Iaccarino L., Talarico R., Scire C. A., Amoura Z., Burmester G., Doria A., Faiz K., Frank C., Hachulla E., Hie M., Launay D., Montecucco C., Monti S., Mouthon L., Tincani A., Toniati P., Van Hagen P. M., Van Vollenhoven R. F., Bombardieri S., Mueller-Ladner U., Schneider M., Smith V., Cutolo M., Mosca M., and Alexander T.

Abstract

Immunoglobulin G4-related diseases (IgG4-RD) are a group of chronic relapsing-remitting inflammatory conditions, characterised by tissue infiltration with lymphocytes and IgG4-secreting plasma cells, fibrosis and a usually favourable response to steroids. In this narrative review, we summarise the results of a systematic literature research, which was performed as part of the European Reference Network ReCONNET, aimed at evaluating existing clinical practice guidelines (CPGs) and recommendations in IgG4-RD. From 167 publications initially obtained from a systematic literature search, only one was identified as a systematic multispecialist, evidence-based, consensus guidance statement on diagnosis and treatment of IgG4-RD, which may be recommended for use as CPG in IgG4-RD. With the recognition of a limited evidence based in this increasingly recognised disease, the group discussion has identified the following unmet needs: lack of shared classification criteria, absence of formal guidelines on diagnosis, no evidence-based therapeutic recommendations and lack of activity and damage indices. Areas of unmet needs include the difficulties in diagnosis, management and monitoring and the scarcity of expert centres.

Published
2019

25. Morbidity and mortality in the antiphospholipid syndrome during a 10-year period: a multicentre prospective study of 1000 patients

Author

Cervera, R, Serrano, R, Pons-Estel, G J, Ceberio-Hualde, L, Shoenfeld, Y, de Ramón, E, Buonaiuto, V, Jacobsen, S, Zeher, M M, Tarr, T, Tincani, A, Taglietti, M, Theodossiades, G, Nomikou, E, Galeazzi, M, Bellisai, F, Meroni, P L, Derksen, R H W M, de Groot, P G D, Baleva, M, Mosca, M, Bombardieri, S, Houssiau, F, Gris, J-C, Quéré, I, Hachulla, E, Vasconcelos, C, Fernández-Nebro, A, Haro, M, Amoura, Z, Miyara, M, Tektonidou, M, Espinosa, G, Bertolaccini, M L, and Khamashta, M A

Published
2015
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26. What are the topics you care about making trials in lupus more effective? Results of an Open Space meeting of international lupus experts.

Author

Mucke J., Alarcon-Riquelme M., Andersen J., Aringer M., Bombardieri S., Brinks R., Cervera R., Chehab G., Cornet A., Costedoat-Chalumeau N., Czirjak L., Doria A., Fischer-Betz R., Furie R.A., Gatto M., Houssiau F.A., Ines L., Liang M.H., Morand E., Mosca M., Pego-Reigosa J.M., Rua-Figueroa I., Ruiz-Irastorza G., Terrier B., Voss A., Schneider M., Mucke J., Alarcon-Riquelme M., Andersen J., Aringer M., Bombardieri S., Brinks R., Cervera R., Chehab G., Cornet A., Costedoat-Chalumeau N., Czirjak L., Doria A., Fischer-Betz R., Furie R.A., Gatto M., Houssiau F.A., Ines L., Liang M.H., Morand E., Mosca M., Pego-Reigosa J.M., Rua-Figueroa I., Ruiz-Irastorza G., Terrier B., Voss A., and Schneider M.

Abstract

Despite promising candidates for new therapeutic options in the treatment of systemic lupus erythematosus (SLE), many clinical trials have failed in the past few years. The disappointing results have been at least partly be attributed to trial designs. With the aim of stimulating new developments in SLE trial design, an international open space meeting was held on occasion of the European Lupus Meeting 2018 in Duesseldorf, Germany about 'What are the topics you care about for making trials in lupus more effective?'. The Open Space is a participant-driven technology, where the discussion topics and schedule are selected during the meeting by all participants and discussion rounds are led by the people attending encouraging active contributions. Eleven topics were selected for further discussion, of which 6 were voted to be more intensively discussed in two consecutive rounds. Major topics were the optimal handling of glucocorticoids in clinical trials, the improvement of outcome measures, reducing or controlling the placebo response and the identification of biomarkers and stratification parameters. Further, the importance of local and international networks was emphasised. By networking, collaborations are facilitated, patient recruitment is more efficient and treatment can be harmonised thus lead to more successful SLE trials. Further discussions are needed to substantiate the results and develop new trial designs. Copyright © 2020 American Society of Mechanical Engineers (ASME). All rights reserved.

Published
2021

27. The impact of COVID-19 on rare and complex connective tissue diseases: the experience of ERN ReCONNET

Author

Talarico, R, Aguilera, S, Alexander, T, Amoura, Z, Antunes, AMC, Arnaud, L, Avcin, T, Beretta, L, Bombardieri, S, Burmester, GR, Cannizzo, S, Cavagna, L, Chaigne, B, Cornet, A, Costedoat-Chalumeau, N, Doria, A, Ferraris, A, Fischer-Betz, R, Fonseca, JE, Frank, C, Gaglioti, A, Galetti, I, Grunert, J, Guimaraes, V, Hachulla, E, Houssiau, F, Iaccarino, L, Krieg, T, Limper, M, Malfait, F, Mariette, X, Marinello, D, Martin, T, Matthews, L, Matucci-Cerinic, M, Meyer, A, Montecucco, C, Mouthon, L, Muller-Ladner, U, Rednic, S, Romao, VC, Schneider, M, Smith, V, Sulli, A, Tamirou, F, Taruscio, D, Taulaigo, AV, Terol, E, Tincani, A, Ticciati, S, Turchetti, G, van Hagen, P.M., van Laar, JM, Vieira, A, de Vries-Bouwstra, JK, Cutolo, M, Mosca, M, Talarico, R, Aguilera, S, Alexander, T, Amoura, Z, Antunes, AMC, Arnaud, L, Avcin, T, Beretta, L, Bombardieri, S, Burmester, GR, Cannizzo, S, Cavagna, L, Chaigne, B, Cornet, A, Costedoat-Chalumeau, N, Doria, A, Ferraris, A, Fischer-Betz, R, Fonseca, JE, Frank, C, Gaglioti, A, Galetti, I, Grunert, J, Guimaraes, V, Hachulla, E, Houssiau, F, Iaccarino, L, Krieg, T, Limper, M, Malfait, F, Mariette, X, Marinello, D, Martin, T, Matthews, L, Matucci-Cerinic, M, Meyer, A, Montecucco, C, Mouthon, L, Muller-Ladner, U, Rednic, S, Romao, VC, Schneider, M, Smith, V, Sulli, A, Tamirou, F, Taruscio, D, Taulaigo, AV, Terol, E, Tincani, A, Ticciati, S, Turchetti, G, van Hagen, P.M., van Laar, JM, Vieira, A, de Vries-Bouwstra, JK, Cutolo, M, and Mosca, M

Abstract

During the COVID-19 pandemic, the need to provide high-level care for a large number of patients with COVID-19 has affected resourcing for, and limited the routine care of, all other conditions. The impact of this health emergency is particularly relevant in the rare connective tissue diseases (rCTDs) communities, as discussed in this Perspective article by the multi-stakeholder European Reference Network on Rare and Complex Connective Tissue and Musculoskeletal Diseases (ERN ReCONNET). The clinical, organizational and health economic challenges faced by health-care providers, institutions, patients and their families during the SARS-CoV-2 outbreak have demonstrated the importance of ensuring continuity of care in the management of rCTDs, including adequate diagnostics and monitoring protocols, and highlighted the need for a structured emergency strategy. The vulnerability of patients with rCTDs needs to be taken into account when planning future health policies, in preparation for not only the post-COVID era, but also any possible new health emergencies.

Published
2021

28. SARS-CoV-2 infection in patients with primary Sjögren syndrome: Characterization and outcomes of 51 patients

Author

Medicina i Cirurgia, Universitat Rovira i Virgili, Brito-Zerón P; Brito-Zerón P; Melchor S; Seror R; Seror R; Priori R; Priori R; Solans R; Solans R; Kostov B; Kostov B; Baldini C; Carubbi F; Carubbi F; Callejas JL; Guisado-Vasco P; Hernández-Molina G; Hernández-Molina G; Pasoto SG; Pasoto SG; Valim V; Valim V; Sisó-Almirall A; Sisó-Almirall A; Mariette X; Carreira P; Ramos-Casals M; Brito-Zerón P; Morcillo C; Flores-Chávez A; Acar-Denizli N; Horvath IF; Szanto A; Tarr T; Mandl T; Olsson P; Li X; Xu B; Baldini C; Bombardieri S; Gottenberg JE; Gandolfo S; De Vita S; Giardina F; Sánchez-Guerrero J; Kruize AA; Hinrichs A; Isenberg D; Rischmueller M; Downie-Doyle S; Kwok SK; Park SH; Nordmark G; Suzuki Y; Kawano M; Giacomelli R; Devauchelle-Pensec V; Saraux A; Hofauer B; Knopf A; Bootsma H; Vissink A; Morel J; Vollenveider C; Atzeni F; Retamozo S; Moça Trevisano V; Armagan B; Kilic L; Kalyoncu U; Consani-Fernández S; Callejas JL; López-Dupla M; Pérez-Alvarez R; Akasbi M; Sánchez I, Medicina i Cirurgia, Universitat Rovira i Virgili, and Brito-Zerón P; Brito-Zerón P; Melchor S; Seror R; Seror R; Priori R; Priori R; Solans R; Solans R; Kostov B; Kostov B; Baldini C; Carubbi F; Carubbi F; Callejas JL; Guisado-Vasco P; Hernández-Molina G; Hernández-Molina G; Pasoto SG; Pasoto SG; Valim V; Valim V; Sisó-Almirall A; Sisó-Almirall A; Mariette X; Carreira P; Ramos-Casals M; Brito-Zerón P; Morcillo C; Flores-Chávez A; Acar-Denizli N; Horvath IF; Szanto A; Tarr T; Mandl T; Olsson P; Li X; Xu B; Baldini C; Bombardieri S; Gottenberg JE; Gandolfo S; De Vita S; Giardina F; Sánchez-Guerrero J; Kruize AA; Hinrichs A; Isenberg D; Rischmueller M; Downie-Doyle S; Kwok SK; Park SH; Nordmark G; Suzuki Y; Kawano M; Giacomelli R; Devauchelle-Pensec V; Saraux A; Hofauer B; Knopf A; Bootsma H; Vissink A; Morel J; Vollenveider C; Atzeni F; Retamozo S; Moça Trevisano V; Armagan B; Kilic L; Kalyoncu U; Consani-Fernández S; Callejas JL; López-Dupla M; Pérez-Alvarez R; Akasbi M; Sánchez I

Abstract

Objective: To analyse the prognosis and outcomes of SARS-CoV-2 infection in patients with primary SS. Methods: We searched for patients with primary SS presenting with SARS-CoV-2 infection (defined following and according to the European Centre for Disease Prevention and Control guidelines) among those included in the Big Data Sjögren Registry, an international, multicentre registry of patients diagnosed according to the 2002/2016 classification criteria. Results: A total of 51 patients were included in the study (46 women, mean age at diagnosis of infection of 60 years). According to the number of patients with primary SS evaluated in the Registry (n = 8211), the estimated frequency of SARS-CoV-2 infection was 0.62% (95% CI 0.44, 0.80). All but two presented with symptoms suggestive of COVID-19, including fever (82%), cough (57%), dyspnoea (39%), fatigue/myalgias (27%) and diarrhoea (24%), and the most frequent abnormalities included raised lactate dehydrogenase (LDH) (88%), CRP (81%) and D-dimer (82%) values, and lymphopenia (70%). Infection was managed at home in 26 (51%) cases and 25 (49%) required hospitalization (five required admission to ICU, four died). Compared with patients managed at home, those requiring hospitalization had higher odds of having lymphopenia as laboratory abnormality (adjusted OR 21.22, 95% CI 2.39, 524.09). Patients with comorbidities had an older age (adjusted OR 1.05, 95% CI 1.00, 1.11) and showed a risk for hospital admission six times higher than those without (adjusted OR 6.01, 95% CI 1.72, 23.51) in the multivariate analysis. Conclusion: Baseline comorbidities were a key risk factor for a more complicated COVID-19 in patients with primary SS, with higher rates of hospitalization and poor outcomes in comparison with patients without co

Published
2021

29. Influence of the age at diagnosis in the disease expression of primary Sjögren's syndrome. Analysis of 12,753 patients from the Sjögren Big Data Consortium

Author

Universitat Rovira i Virgili, Retamozo S; Acar-Denizli N; Horváth IF; Ng WF; Rasmussen A; Dong X; Li X; Baldini C; Olsson P; Priori R; Seror R; Gottenberg JE; Kruize AA; Hernandez-Molina G; Vissink A; Sandhya P; Armagan B; Quartuccio L; Sebastian A; Praprotnik S; Bartoloni E; Kwok SK; Kvarnstrom M; Rischmueller M; Soláns-Laqué R; Sene D; Pasoto SG; Suzuki Y; Isenberg DA; Valim V; Nordmark G; Nakamura H; Trevisani VFM; Hofauer B; Sisó-Almirall A; Giacomelli R; Devauchelle-Pensec V; Bombardieri M; Atzeni F; Hammenfors D; Maure B; Carsons SE; Gheita T; Sánchez-Berná I; López-Dupla M; Morel J; Inanç N; Fonseca-Aizpuru E; Morcillo C; Vollenweider C; Melchor S; Vázquez M; Díaz-Cuiza E; Consani-Fernández S; De-Miguel-Campo B; Szántó A; Bombardieri S; Gattamelata A; Hinrichs A; Sánchez-Guerrero J; Danda D; Kilic L; De Vita S; Wiland P; Gerli R; Park SH; Wahren-Herlenius M; Bootsma H; Mariette X; Ramos-Casals M; Brito-Zerón P, Universitat Rovira i Virgili, and Retamozo S; Acar-Denizli N; Horváth IF; Ng WF; Rasmussen A; Dong X; Li X; Baldini C; Olsson P; Priori R; Seror R; Gottenberg JE; Kruize AA; Hernandez-Molina G; Vissink A; Sandhya P; Armagan B; Quartuccio L; Sebastian A; Praprotnik S; Bartoloni E; Kwok SK; Kvarnstrom M; Rischmueller M; Soláns-Laqué R; Sene D; Pasoto SG; Suzuki Y; Isenberg DA; Valim V; Nordmark G; Nakamura H; Trevisani VFM; Hofauer B; Sisó-Almirall A; Giacomelli R; Devauchelle-Pensec V; Bombardieri M; Atzeni F; Hammenfors D; Maure B; Carsons SE; Gheita T; Sánchez-Berná I; López-Dupla M; Morel J; Inanç N; Fonseca-Aizpuru E; Morcillo C; Vollenweider C; Melchor S; Vázquez M; Díaz-Cuiza E; Consani-Fernández S; De-Miguel-Campo B; Szántó A; Bombardieri S; Gattamelata A; Hinrichs A; Sánchez-Guerrero J; Danda D; Kilic L; De Vita S; Wiland P; Gerli R; Park SH; Wahren-Herlenius M; Bootsma H; Mariette X; Ramos-Casals M; Brito-Zerón P

Abstract

Objective. To analyse how the main components of the disease phenotype (sicca symptoms, diagnostic tests, immunological markers and systemic disease) can be driven by the age at diagnosis of primary Sjögren's syndrome (pSS). Methods. By January 2021, the participant centres had included 12,753 patients from 25 countries that fulfilled the 2002/2016 classification criteria for pSS. The age at diagnosis was defined as the time when the attending physician confirmed fulfilment of the criteria. Patients were clustered according to age at diagnosis. 50 clusters with more than 100 observations (from 27 to 76 years) were used to study the influence of the age at diagnosis in the disease expression. Results. There was a consistent increase in the frequency of oral dryness according to the age at diagnosis, with a frequency of <90% in patients diagnosed at the youngest ages and >95% in those diagnosed at the oldest ages. The smooth curves that best fitted a linear model were the frequency of dry mouth (adjusted R2 0.87) and the frequency of abnormal oral tests (adjusted R2 0.72). Therefore, for each 1-year increase in the age at diagnosis, the frequency of dry mouth increased by 0.13%, and the frequency of abnormal oral diagnostic tests by 0.11%. There was a consistent year-by-year decrease in the frequency of all autoantibodies and immunological markers except for cryoglobulins. According to the linear models, for each 1-year increase in the age at diagnosis, the frequency of a positive result decreased by 0.57% (for anti-Ro antibodies), 0.47% (for RF) and 0.42% (for anti-La antibodies). The ESSDAI domains which showed a more consistent decrease were glandular and lymph node involvement (for each 1-year increase in the age at diagnosis, the frequency of activity decreased by 0.

Published
2021

33. Antiphospholipid syndrome: State of the art on clinical practice guidelines

Author

Limper, M, Scire, C, Talarico, R, Amoura, Z, Avcin, T, Basile, M, Burmester, G, Carli, L, Cervera, R, Costedoat-Chalumeau, N, Doria, A, Dorner, T, Fonseca, J, Galetti, I, Hachulla, E, Launay, D, Lourenco, F, Macieira, C, Meroni, P, Montecucco, C, Moraes-Fontes, M, Mouthon, L, Nalli, C, Ramoni, V, Tektonidou, M, Van Laar, J, Bombardieri, S, Schneider, M, Smith, V, Vieira, A, Cutolo, M, Mosca, M, Tincani, A, Limper M., Scire C. A., Talarico R., Amoura Z., Avcin T., Basile M., Burmester G., Carli L., Cervera R., Costedoat-Chalumeau N., Doria A., Dorner T., Fonseca J. E., Galetti I., Hachulla E., Launay D., Lourenco F., Macieira C., Meroni P., Montecucco C. M., Moraes-Fontes M. F., Mouthon L., Nalli C., Ramoni V., Tektonidou M., Van Laar J. M., Bombardieri S., Schneider M., Smith V., Vieira A., Cutolo M., Mosca M., Tincani A., Limper, M, Scire, C, Talarico, R, Amoura, Z, Avcin, T, Basile, M, Burmester, G, Carli, L, Cervera, R, Costedoat-Chalumeau, N, Doria, A, Dorner, T, Fonseca, J, Galetti, I, Hachulla, E, Launay, D, Lourenco, F, Macieira, C, Meroni, P, Montecucco, C, Moraes-Fontes, M, Mouthon, L, Nalli, C, Ramoni, V, Tektonidou, M, Van Laar, J, Bombardieri, S, Schneider, M, Smith, V, Vieira, A, Cutolo, M, Mosca, M, Tincani, A, Limper M., Scire C. A., Talarico R., Amoura Z., Avcin T., Basile M., Burmester G., Carli L., Cervera R., Costedoat-Chalumeau N., Doria A., Dorner T., Fonseca J. E., Galetti I., Hachulla E., Launay D., Lourenco F., Macieira C., Meroni P., Montecucco C. M., Moraes-Fontes M. F., Mouthon L., Nalli C., Ramoni V., Tektonidou M., Van Laar J. M., Bombardieri S., Schneider M., Smith V., Vieira A., Cutolo M., Mosca M., and Tincani A.

Abstract

Antiphospholipid syndrome (APS) is a rare disease characterised by venous and/or arterial thrombosis, pregnancy complications and the presence of specific autoantibodies called antiphospholipid antibodies. This review aims to identify existing clinical practice guidelines (CPG) as part of the ERN ReCONNET project, aimed at evaluating existing CPGs or recommendations in rare and complex diseases. Seventeen papers providing important data were identified; however, the literature search highlighted the scarceness of reliable clinical data to develop CPGs. With no formal clinical guidelines in place, diagnosis and treatment of APS is largely based on consensus and expert opinion. Patients' unmet need refers to the understanding of the disease and its clinical picture and implications, the need of education for patients, family members and healthcare providers, as well as to the development of monitoring pathways involving multiple healthcare providers.

Published
2018

34. Systemic sclerosis: State of the art on clinical practice guidelines

Author

Smith, V, Scire, C, Talarico, R, Airo, P, Alexander, T, Allanore, Y, Bruni, C, Codullo, V, Dalm, V, De Vries-Bouwstra, J, Della Rossa, A, Distler, O, Galetti, I, Launay, D, Lepri, G, Mathian, A, Mouthon, L, Ruaro, B, Sulli, A, Tincani, A, Vandecasteele, E, Vanhaecke, A, Vanthuyne, M, Van Den Hoogen, F, Van Vollenhoven, R, Voskuyl, A, Zanatta, E, Bombardieri, S, Burmester, G, Eurico, F, Frank, C, Hachulla, E, Houssiau, F, Mueller-Ladner, U, Schneider, M, Van Laar, J, Vieira, A, Cutolo, M, Mosca, M, Matucci-Cerinic, M, Smith V., Scire C. A., Talarico R., Airo P., Alexander T., Allanore Y., Bruni C., Codullo V., Dalm V., De Vries-Bouwstra J., Della Rossa A., Distler O., Galetti I., Launay D., Lepri G., Mathian A., Mouthon L., Ruaro B., Sulli A., Tincani A., Vandecasteele E., Vanhaecke A., Vanthuyne M., Van Den Hoogen F., Van Vollenhoven R., Voskuyl A. E., Zanatta E., Bombardieri S., Burmester G., Eurico F. J., Frank C., Hachulla E., Houssiau F., Mueller-Ladner U., Schneider M., Van Laar J. M., Vieira A., Cutolo M., Mosca M., Matucci-Cerinic M., Smith, V, Scire, C, Talarico, R, Airo, P, Alexander, T, Allanore, Y, Bruni, C, Codullo, V, Dalm, V, De Vries-Bouwstra, J, Della Rossa, A, Distler, O, Galetti, I, Launay, D, Lepri, G, Mathian, A, Mouthon, L, Ruaro, B, Sulli, A, Tincani, A, Vandecasteele, E, Vanhaecke, A, Vanthuyne, M, Van Den Hoogen, F, Van Vollenhoven, R, Voskuyl, A, Zanatta, E, Bombardieri, S, Burmester, G, Eurico, F, Frank, C, Hachulla, E, Houssiau, F, Mueller-Ladner, U, Schneider, M, Van Laar, J, Vieira, A, Cutolo, M, Mosca, M, Matucci-Cerinic, M, Smith V., Scire C. A., Talarico R., Airo P., Alexander T., Allanore Y., Bruni C., Codullo V., Dalm V., De Vries-Bouwstra J., Della Rossa A., Distler O., Galetti I., Launay D., Lepri G., Mathian A., Mouthon L., Ruaro B., Sulli A., Tincani A., Vandecasteele E., Vanhaecke A., Vanthuyne M., Van Den Hoogen F., Van Vollenhoven R., Voskuyl A. E., Zanatta E., Bombardieri S., Burmester G., Eurico F. J., Frank C., Hachulla E., Houssiau F., Mueller-Ladner U., Schneider M., Van Laar J. M., Vieira A., Cutolo M., Mosca M., and Matucci-Cerinic M.

Abstract

Systemic sclerosis (SSc) is an orphan disease characterised by autoimmunity, fibrosis of the skin and internal organs, and vasculopathy. SSc may be associated with high morbidity and mortality. In this narrative review we summarise the results of a systematic literature research, which was performed as part of the European Reference Network on Rare and Complex Connective Tissue and Musculoskeletal Diseases project, aimed at evaluating existing clinical practice guidelines or recommendations. Only in the domains 'Vascular & Ulcers' (ie, non-pharmacological approach to digital ulcer), 'PAH' (ie, screening and treatment), 'Treatment' and 'Juveniles' (ie, evaluation of juveniles with Raynaud's phenomenon) evidence-based and consensus-based guidelines could be included. Hence there is a preponderance of unmet needs in SSc referring to the diagnosis and (non-)pharmacological treatment of several SSc-specific complications. Patients with SSc experience significant uncertainty concerning SSc-related taxonomy, management (both pharmacological and nonpharmacological) and education. Day-to-day impact of the disease (loss of self-esteem, fatigue, sexual dysfunction, and occupational, nutritional and relational problems) is underestimated and needs evaluation.

Published
2018

35. Relapsing polychondritis: State of the art on clinical practice guidelines

Author

Rednic, S, Damian, L, Talarico, R, Scire, C, Tobias, A, Costedoat-Chalumeau, N, Launay, D, Mathian, A, Mattews, L, Ponte, C, Toniati, P, Bombardieri, S, Frank, C, Schneider, M, Smith, V, Cutolo, M, Mosca, M, Arnaud, L, Rednic S., Damian L., Talarico R., Scire C. A., Tobias A., Costedoat-Chalumeau N., Launay D., Mathian A., Mattews L., Ponte C., Toniati P., Bombardieri S., Frank C., Schneider M., Smith V., Cutolo M., Mosca M., Arnaud L., Rednic, S, Damian, L, Talarico, R, Scire, C, Tobias, A, Costedoat-Chalumeau, N, Launay, D, Mathian, A, Mattews, L, Ponte, C, Toniati, P, Bombardieri, S, Frank, C, Schneider, M, Smith, V, Cutolo, M, Mosca, M, Arnaud, L, Rednic S., Damian L., Talarico R., Scire C. A., Tobias A., Costedoat-Chalumeau N., Launay D., Mathian A., Mattews L., Ponte C., Toniati P., Bombardieri S., Frank C., Schneider M., Smith V., Cutolo M., Mosca M., and Arnaud L.

Abstract

Due to the rarity of relapsing polychondritis (RP), many unmet needs remain in the management of RP. Here, we present a systematic review of clinical practice guidelines (CPGs) published for RP, as well as a list of the most striking unmet needs for this rare disease. We carried out a systematic search in PubMed and Embase based on controlled terms (medical subject headings and Emtree) and keywords of the disease and publication type (CPGs). The systematic literature review identified 20 citations, among which no CPGs could be identified. We identified 11 main areas with unmet needs in the field of RP: the diagnosis strategy for RP; the therapeutic management of RP; the management of pregnancy in RP; the management of the disease in specific age groups (for instance in paediatric-onset RP); the evaluation of adherence to treatment; the follow-up of patients with RP, including the frequency of screening for the potential complications and the optimal imaging tools for each involved region; perioperative and anaesthetic management (due to tracheal involvement); risk of neoplasms in RP, including haematological malignancies; the prevention and management of infections; tools for assessment of disease activity and damage; and patient-reported outcomes and quality of life indicators. Patients and physicians should work together within the frame of the ReCONNET network to derive valuable evidence for obtaining literature-informed CPGs.

Published
2018

36. Sjögren's syndrome: State of the art on clinical practice guidelines

Author

Romao, V, Talarico, R, Scire, C, Vieira, A, Alexander, T, Baldini, C, Gottenberg, J, Gruner, H, Hachulla, E, Mouthon, L, Orlandi, M, Pamfil, C, Pineton De Chambrun, M, Taglietti, M, Toplak, N, Van Daele, P, Van Laar, J, Bombardieri, S, Schneider, M, Smith, V, Cutolo, M, Mosca, M, Mariette, X, Romao V. C., Talarico R., Scire C. A., Vieira A., Alexander T., Baldini C., Gottenberg J. -E., Gruner H., Hachulla E., Mouthon L., Orlandi M., Pamfil C., Pineton De Chambrun M., Taglietti M., Toplak N., Van Daele P., Van Laar J. M., Bombardieri S., Schneider M., Smith V., Cutolo M., Mosca M., Mariette X., Romao, V, Talarico, R, Scire, C, Vieira, A, Alexander, T, Baldini, C, Gottenberg, J, Gruner, H, Hachulla, E, Mouthon, L, Orlandi, M, Pamfil, C, Pineton De Chambrun, M, Taglietti, M, Toplak, N, Van Daele, P, Van Laar, J, Bombardieri, S, Schneider, M, Smith, V, Cutolo, M, Mosca, M, Mariette, X, Romao V. C., Talarico R., Scire C. A., Vieira A., Alexander T., Baldini C., Gottenberg J. -E., Gruner H., Hachulla E., Mouthon L., Orlandi M., Pamfil C., Pineton De Chambrun M., Taglietti M., Toplak N., Van Daele P., Van Laar J. M., Bombardieri S., Schneider M., Smith V., Cutolo M., Mosca M., and Mariette X.

Abstract

Sjögren's syndrome (SS) is a complex autoimmune rheumatic disease that specifically targets salivary and lachrymal glands. As such, patients typically had ocular and oral dryness and salivary gland swelling. Moreover, skin, nasal and vaginal dryness are frequently present. In addition to dryness, musculoskeletal pain and fatigue are the hallmarks of this disease and constitute the classic symptom triad presented by the vast majority of patients. Up to 30% to 50 % of patients with SS may present systemic disease; moreover, there is an increased risk for the development of non-Hodgkin's lymphoma that occurs in a minority of patients. The present work was developed in the framework of the European Reference Network (ERN) dedicated to Rare and Complex Connective Tissue and Musculoskeletal Diseases (ReCONNET). In line with its goals of aiming to improve early diagnosis, treatment and care of rare connective and musculoskeletal diseases, ERN-ReCONNET set to review the current state of clinical practice guidelines (CPGs) in the rare and complex connective tissue diseases of interest of the network. Therefore, the present work was aimed at providing a state of the art of CPGs for SS.

Published
2018

37. Systemic lupus erythematosus: State of the art on clinical practice guidelines

Author

Tamirou, F, Arnaud, L, Talarico, R, Scire, C, Alexander, T, Amoura, Z, Avcin, T, Bortoluzzi, A, Cervera, R, Conti, F, Cornet, A, Devilliers, H, Doria, A, Frassi, M, Fredi, M, Govoni, M, Houssiau, F, Llado, A, Macieira, C, Martin, T, Massaro, L, Moraes-Fontes, M, Pamfil, C, Paolino, S, Tani, C, Tas, S, Tektonidou, M, Tincani, A, Van Vollenhoven, R, Bombardieri, S, Burmester, G, Eurico, F, Galetti, I, Hachulla, E, Mueller-Ladner, U, Schneider, M, Smith, V, Cutolo, M, Mosca, M, Costedoat-Chalumeau, N, Tamirou F., Arnaud L., Talarico R., Scire C. A., Alexander T., Amoura Z., Avcin T., Bortoluzzi A., Cervera R., Conti F., Cornet A., Devilliers H., Doria A., Frassi M., Fredi M., Govoni M., Houssiau F., Llado A., Macieira C., Martin T., Massaro L., Moraes-Fontes M. F., Pamfil C., Paolino S., Tani C., Tas S. W., Tektonidou M., Tincani A., Van Vollenhoven R. F., Bombardieri S., Burmester G., Eurico F. J., Galetti I., Hachulla E., Mueller-Ladner U., Schneider M., Smith V., Cutolo M., Mosca M., Costedoat-Chalumeau N., Tamirou, F, Arnaud, L, Talarico, R, Scire, C, Alexander, T, Amoura, Z, Avcin, T, Bortoluzzi, A, Cervera, R, Conti, F, Cornet, A, Devilliers, H, Doria, A, Frassi, M, Fredi, M, Govoni, M, Houssiau, F, Llado, A, Macieira, C, Martin, T, Massaro, L, Moraes-Fontes, M, Pamfil, C, Paolino, S, Tani, C, Tas, S, Tektonidou, M, Tincani, A, Van Vollenhoven, R, Bombardieri, S, Burmester, G, Eurico, F, Galetti, I, Hachulla, E, Mueller-Ladner, U, Schneider, M, Smith, V, Cutolo, M, Mosca, M, Costedoat-Chalumeau, N, Tamirou F., Arnaud L., Talarico R., Scire C. A., Alexander T., Amoura Z., Avcin T., Bortoluzzi A., Cervera R., Conti F., Cornet A., Devilliers H., Doria A., Frassi M., Fredi M., Govoni M., Houssiau F., Llado A., Macieira C., Martin T., Massaro L., Moraes-Fontes M. F., Pamfil C., Paolino S., Tani C., Tas S. W., Tektonidou M., Tincani A., Van Vollenhoven R. F., Bombardieri S., Burmester G., Eurico F. J., Galetti I., Hachulla E., Mueller-Ladner U., Schneider M., Smith V., Cutolo M., Mosca M., and Costedoat-Chalumeau N.

Abstract

Systemic lupus erythematosus (SLE) is the paradigm of systemic autoimmune diseases characterised by a wide spectrum of clinical manifestations with an unpredictable relapsing-remitting course. The aim of the present work was to identify current available clinical practice guidelines (CPGs) for SLE, to provide their review and to identify physicians' and patients' unmet needs. Twenty-Three original guidelines published between 2004 and 2017 were identified. Many aspects of disease management are covered, including global disease management, lupus nephritis and neuropsychiatric involvement, management of pregnancies, vaccinations and comorbidities monitoring. Unmet needs relate with disease management of some clinical manifestations and adherence to treatment. Many patient's unmet needs have been identified starting with faster diagnosis, need for more therapeutic options, guidelines on lifestyle issues, attention to quality of life and adequate education.

Published
2018

38. Ehlers-Danlos syndromes: State of the art on clinical practice guidelines

Author

Sulli, A, Talarico, R, Scire, C, Avcin, T, Castori, M, Ferraris, A, Frank, C, Grunert, J, Paolino, S, Bombardieri, S, Schneider, M, Smith, V, Cutolo, M, Mosca, M, Malfait, F, Sulli A., Talarico R., Scire C. A., Avcin T., Castori M., Ferraris A., Frank C., Grunert J., Paolino S., Bombardieri S., Schneider M., Smith V., Cutolo M., Mosca M., Malfait F., Sulli, A, Talarico, R, Scire, C, Avcin, T, Castori, M, Ferraris, A, Frank, C, Grunert, J, Paolino, S, Bombardieri, S, Schneider, M, Smith, V, Cutolo, M, Mosca, M, Malfait, F, Sulli A., Talarico R., Scire C. A., Avcin T., Castori M., Ferraris A., Frank C., Grunert J., Paolino S., Bombardieri S., Schneider M., Smith V., Cutolo M., Mosca M., and Malfait F.

Abstract

Objective To report the effort of the European Reference Network for Rare and Complex CONnective tissue and musculoskeletal diseases NETwork working group on Ehlers-Danlos syndromes (EDS) and related disorders to assess current available clinical practice guidelines (CPGs) specifically addressed to EDS, in order to identify potential clinician and patient unmet needs. Methods Systematic literature search in PUBMED and EMBASE based on controlled terms (MeSH and Emtree) and keywords of the disease and publication type (CPGs). All the published articles were revised in order to identify existing CPGs on diagnosis, monitoring and treatment of EDS. Results Literature revision detected the absence of papers reporting good quality CPGs to optimise EDS patient care. The current evidence-based literature regarding clinical guidelines for the EDS was limited in size and quality, and there is insufficient research exploring the clinical features and interventions, and clinical decision-making are currently based on theoretical and limited research evidences. Conclusions Many clinician and patient unmet needs have been identified.

Published
2018

39. SARS-CoV-2 infection in patients with primary Sjögren syndrome: characterization and outcomes of 51 patients

Author

Brito-Zerón, Pilar, Melchor, Sheila, Seror, Raphaèle, Priori, Roberta, Solans, Roser, Kostov, Belchin, Baldini, Chiara, Carubbi, Francesco, Callejas, Jose Luis, Guisado-Vasco, Pablo, Hernández-Molina, Gabriela, Pasoto, Sandra G, Valim, Valeria, Sisó-Almirall, Antoni, Mariette, Xavier, Carreira, Patricia, Ramos-Casals, Manuel, Brito-Zerón, P, Morcillo, C, Flores-Chávez, A, Ramos-Casals, M, Acar-Denizli, N, Horvath, I F, Szanto, A, Tarr, T, Seror, R, Mariette, X, Mandl, T, Olsson, P, Li, X, Xu, B, Baldini, C, Bombardieri, S, Gottenberg, J E, Gandolfo, S, De Vita, S, Priori, R, Giardina, F, Hernandez-Molina, G, Sánchez-Guerrero, J, Kruize, A A, Hinrichs, A, Valim, V, Isenberg, D, Solans, R, Rischmueller, M, Downie-Doyle, S, Kwok, S-K, Park, S-H, Nordmark, G, Suzuki, Y, Kawano, M, Giacomelli, R, Devauchelle-Pensec, V, Saraux, A, Hofauer, B, Knopf, A, Bootsma, H, Vissink, A, Morel, J, Vollenveider, C, Atzeni, F, Retamozo, S, Moça Trevisano, V, Armagan, B, Kilic, L, Kalyoncu, U, Pasoto, S G, Kostov, B, Sisó-Almirall, A, Consani-Fernández, S, Carubbi, F, Callejas, J L, López-Dupla, M, Pérez-Alvarez, R, Akasbi, M, Guisado-Vasco, P, Sánchez, I, Hospital Universitario 12 de Octubre [Madrid], EULAR standing committee of People with Arthritis/Rheumatism in Europe (PARE), H. CIMA-Sanitas, Barcelona, CELLEX-IDIBAPS Department of Autoimmune Diseases, Barcelona, Instituto Mexicano del Seguro Social [Mexico City, Mexico] (IMSS), Universidad de Colima [Mexico], Hospital Clinic [Barcelona, Spain], İstanbul Üniversitesi, İstanbul, Medical and Health Science Center, University of Debrecen, Debrecen, Hungary, MTA-Debreceni Egyetem, Immunologie des maladies virales, auto-immunes, hématologiques et bactériennes (IMVA-HB), Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris-Saclay, Center for Immunology of Viral Infections and Autoimmune Diseases, Le Kremlin Bicêtre, Skåne University Hospital [Malmö, Suède], Stockholm University, Wuhan University [China], Pisa University Hospital, Nuclear Medicine Unit, Humanitas Gavazzeni, Bergamo, Italy, University Hospitals, Strasbourg, Università degli Studi di Roma 'La Sapienza' = Sapienza University [Rome] (UNIROMA), Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán - National Institute of Medical Science and Nutrition Salvador Zubiran [Mexico], Federal University of Espírito Santo, University Hospital Vall d’Hebròn [Barcelona, Spain], Lymphocytes B, Autoimmunité et Immunothérapies (LBAI), Université de Brest (UBO)-Institut National de la Santé et de la Recherche Médicale (INSERM)-LabEX IGO Immunothérapie Grand Ouest, Nantes Université (Nantes Univ)-Nantes Université (Nantes Univ)-Institut Brestois Santé Agro Matière (IBSAM), Université de Brest (UBO), Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Institut de Génétique Moléculaire de Montpellier (IGMM), Université de Montpellier (UM)-Centre National de la Recherche Scientifique (CNRS), Institut d'Investigaciones Biomèdiques August Pi i Sunye (IDIBAPS), Università degli studi di Palermo - University of Palermo, Hospitales Universitarios de Granada/Universidad de Granada, Hospital Universitario Quironsalud, Università degli Studi di Roma 'La Sapienza' = Sapienza University [Rome], Lymphocyte B et Auto-immunité (LBAI), Université de Brest (UBO)-Institut Brestois Santé Agro Matière (IBSAM), Université de Brest (UBO)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre National de la Recherche Scientifique (CNRS)-Université de Montpellier (UM), Rheumatology Unit, Department of Internal Medicine, University of Palermo, Palermo, Italy, and Michel, Geneviève

Subjects
Male, medicine.medical_specialty, Multivariate analysis, [SDV.IMM] Life Sciences [q-bio]/Immunology, Coronavirus disease 2019 (COVID-19), Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), Comorbidity, Laboratory abnormality, comorbidities, outcomes, 03 medical and health sciences, 0302 clinical medicine, Rheumatology, Risk Factors, Internal medicine, medicine, Humans, In patient, Pharmacology (medical), 030212 general & internal medicine, Registries, Risk factor, Primary Sjögren Syndrome, AcademicSubjects/MED00360, ComputingMilieux_MISCELLANEOUS, 030203 arthritis & rheumatology, business.industry, SARS-CoV-2, COVID-19, Primary SS, SARS-Cov-2, Middle Aged, medicine.disease, 3. Good health, Hospitalization, Sjogren's Syndrome, [SDV.IMM]Life Sciences [q-bio]/Immunology, Original Article, Female, business
Abstract

Objective To analyse the prognosis and outcomes of SARS-CoV-2 infection in patients with primary SS. Methods We searched for patients with primary SS presenting with SARS-CoV-2 infection (defined following and according to the European Centre for Disease Prevention and Control guidelines) among those included in the Big Data Sjögren Registry, an international, multicentre registry of patients diagnosed according to the 2002/2016 classification criteria. Results A total of 51 patients were included in the study (46 women, mean age at diagnosis of infection of 60 years). According to the number of patients with primary SS evaluated in the Registry (n = 8211), the estimated frequency of SARS-CoV-2 infection was 0.62% (95% CI 0.44, 0.80). All but two presented with symptoms suggestive of COVID-19, including fever (82%), cough (57%), dyspnoea (39%), fatigue/myalgias (27%) and diarrhoea (24%), and the most frequent abnormalities included raised lactate dehydrogenase (LDH) (88%), CRP (81%) and D-dimer (82%) values, and lymphopenia (70%). Infection was managed at home in 26 (51%) cases and 25 (49%) required hospitalization (five required admission to ICU, four died). Compared with patients managed at home, those requiring hospitalization had higher odds of having lymphopenia as laboratory abnormality (adjusted OR 21.22, 95% CI 2.39, 524.09). Patients with comorbidities had an older age (adjusted OR 1.05, 95% CI 1.00, 1.11) and showed a risk for hospital admission six times higher than those without (adjusted OR 6.01, 95% CI 1.72, 23.51) in the multivariate analysis. Conclusion Baseline comorbidities were a key risk factor for a more complicated COVID-19 in patients with primary SS, with higher rates of hospitalization and poor outcomes in comparison with patients without comorbidities.

Published
2020
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40. EULAR recommendations for the management of Sjogren's syndrome with topical and systemic therapies

Author

Medicina i Cirurgia, Ramos-Casals, M Brito-Zeron, P; Bombardieri, S; Bootsma, H; De Vita, S; Dörner, T; Fisher, BA; Gottenberg, JE; Hernandez-Molina, G; Kocher, A; Kostov, B; Kruize, AA; Mandl, T; Ng, WF; Retamozo, S; Seror, R; Shoenfeld, Y; Siso-Almirall, A; Tzioufas, AG; Vitali, C; Bowman, S; Mariette, X, Medicina i Cirurgia, and Ramos-Casals, M Brito-Zeron, P; Bombardieri, S; Bootsma, H; De Vita, S; Dörner, T; Fisher, BA; Gottenberg, JE; Hernandez-Molina, G; Kocher, A; Kostov, B; Kruize, AA; Mandl, T; Ng, WF; Retamozo, S; Seror, R; Shoenfeld, Y; Siso-Almirall, A; Tzioufas, AG; Vitali, C; Bowman, S; Mariette, X

Abstract

The therapeutic management of Sjögren syndrome (SjS) has not changed substantially in recent decades: treatment decisions remain challenging in clinical practice, without a specific therapeutic target beyond the relief of symptoms as the most important goal. In view of this scenario, the European League Against Rheumatism (EULAR) promoted and supported an international collaborative study (EULAR SS Task Force) aimed at developing the first EULAR evidence and consensus-based recommendations for the management of patients with SjS with topical and systemic medications. The aim was to develop a rational therapeutic approach to SjS patients useful for healthcare professionals, physicians undergoing specialist training, medical students, the pharmaceutical industry and drug regulatory organisations following the 2014 EULAR standardised operating procedures. The Task Force (TF) included specialists in rheumatology, internal medicine, oral health, ophthalmology, gynaecology, dermatology and epidemiology, statisticians, general practitioners, nurses and patient representatives from 30 countries of the 5 continents. Evidence was collected from studies including primary SjS patients fulfilling the 2002/2016 criteria; when no evidence was available, evidence from studies including associated SjS or patients fulfilling previous sets of criteria was considered and extrapolated. The TF endorsed the presentation of general principles for the management of patients with SjS as three overarching, general consensus-based recommendations and 12 specific recommendations that form a logical sequence, starting with the management of the central triplet of symptoms (dryness, fatigue and pain) followed by the management of systemic disease. The recommendations address the use of topical oral (

Published
2020

43. A randomized controlled trial of rituximab for the treatment of severe cryoglobulinemic vasculitis

Author

De Vita, S., Quartuccio, L., Isola, M., Mazzaro, C., Scaini, P., Lenzi, M., Campanini, M., Naclerio, C., Tavoni, A., Pietrogrande, M., Ferri, C., Mascia, M. T., Masolini, P., Zabotti, A., Maset, M., Roccatello, D., Zignego, A. L., Pioltelli, P., Gabrielli, A., Filippini, D., Perrella, O., Migliaresi, S., Galli, M., Bombardieri, S., and Monti, G.

Published
2012
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49. Preliminary classification criteria for the cryoglobulinaemic vasculitis

Author

De Vita, S, Soldano, F, Isola, M, Monti, G, Gabrielli, A, Tzioufas, A, Ferri, C, Ferraccioli, G F, Quartuccio, L, Corazza, L, De Marchi, G, Casals, M Ramos, Voulgarelis, M, Lenzi, M, Saccardo, F, Fraticelli, P, Mascia, M T, Sansonno, D, Cacoub, P, Tomsic, M, Tavoni, A, Pietrogrande, M, Zignego, A L, Scarpato, S, Mazzaro, C, Pioltelli, P, Steinfeld, S, Lamprecht, P, Bombardieri, S, and Galli, M

Published
2011
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50. EULAR recommendations for the management of systemic lupus erythematosus with neuropsychiatric manifestations: report of a task force of the EULAR standing committee for clinical affairs

Author

Bertsias, G K, Ioannidis, J P A, Aringer, M, Bollen, E, Bombardieri, S, Bruce, I N, Cervera, R, Dalakas, M, Doria, A, Hanly, J G, Huizinga, T W J, Isenberg, D, Kallenberg, C, Piette, J C, Schneider, M, Scolding, N, Smolen, J, Stara, A, Tassiulas, I, Tektonidou, M, Tincani, A, van Buchem, M A, van Vollenhoven, R, Ward, M, Gordon, C, and Boumpas, D T

Published
2010
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