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5. 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 EB, 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, Abu‐Shakra, M, Agarwal, V, Agmon‐Levin, 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, El‐Shahawy, M, Gaylis, N, Grossman, J, Halpert, E, Huff, J, Jarjour, W, Kao, A, Katz, R, Kennedy, A, Khan, M, Kivitz, A, Kohen, M, and Lawrence‐Ford, T

Subjects
Autoimmune Disease, Clinical Trials and Supportive Activities, Clinical Research, Lupus, 6.1 Pharmaceuticals, Evaluation of treatments and therapeutic interventions, Inflammatory and immune system, Adult, Antibodies, Monoclonal, Humanized, Double-Blind Method, Female, Humans, Lupus Erythematosus, Systemic, Male, Severity of Illness Index, Treatment Outcome, EMBODY Investigator Group, Clinical Sciences, Immunology, Public Health and Health Services, Arthritis & Rheumatology
Abstract

ObjectiveEpratuzumab, a monoclonal antibody that targets CD22, modulates B cell signaling without substantial reductions in the number of B cells. The aim of this study was to report the results of 2 phase III multicenter randomized, double-blind, placebo-controlled trials, the EMBODY 1 and EMBODY 2 trials, assessing the efficacy and safety of epratuzumab in patients with moderately to severely active systemic lupus erythematosus (SLE).MethodsPatients met ≥4 of the American College of Rheumatology revised classification criteria for SLE, were positive for antinuclear antibodies and/or anti-double-stranded DNA antibodies, had an SLE Disease Activity Index 2000 (SLEDAI-2K) score of ≥6 (increased disease activity), had British Isles Lupus Assessment Group 2004 index (BILAG-2004) scores of grade A (severe disease activity) in ≥1 body system or grade B (moderate disease activity) in ≥2 body systems (in the mucocutaneous, musculoskeletal, or cardiorespiratory domains), and were receiving standard therapy, including mandatory treatment with corticosteroids (5-60 mg/day). BILAG-2004 grade A scores in the renal and central nervous system domains were excluded. Patients were randomized 1:1:1 to receive either placebo, epratuzumab 600 mg every week, or epratuzumab 1,200 mg every other week, with infusions delivered for the first 4 weeks of each 12-week dosing cycle, for 4 cycles. Patients across all 3 treatment groups also continued with their standard therapy. The primary end point was the response rate at week 48 according to the BILAG-based Combined Lupus Assessment (BICLA) definition, requiring improvement in the BILAG-2004 score, no worsening in the BILAG-2004 score, SLEDAI-2K score, or physician's global assessment of disease activity, and no disallowed changes in concomitant medications. Patients who discontinued the study medication were classified as nonresponders.ResultsIn the EMBODY 1 and EMBODY 2 trials of epratuzumab, 793 patients and 791 patients, respectively, were randomized, 786 (99.1%) and 788 (99.6%), respectively, received study medication, and 528 (66.6%) and 533 (67.4%), respectively, completed the study. There was no statistically significant difference in the primary end point between the groups, with the week 48 BICLA response rates being similar between the epratuzumab groups and the placebo group (response rates ranging from 33.5% to 39.8%). No new safety signals were identified.ConclusionIn patients with moderate or severely active SLE, treatment with epratuzumab + standard therapy did not result in improvements in response rates over that observed in the placebo + standard therapy group.

Published
2017

6. A Computerized Assessment of Verbal and Visuospatial Memory (Dys)functions in Patients with Rheumatoid Arthritis

Author

Petra CV, Visu-Petra L, Buta M, Tămaș MM, Benga O, and Rednic S

Subjects
rheumatoid arthritis, verbal working memory, visuospatial working memory, cognitive dysfunction, neuropsychological assessment., Psychology, BF1-990, Industrial psychology, HF5548.7-5548.85
Abstract

Cristian Vasile Petra,1 Laura Visu-Petra,2 Monica Buta,2 Maria Magdalena Tămaș,1 Oana Benga,2 Simona Rednic1 1Department of Rheumatology, “Iuliu Hatieganu” University of Medicine and Pharmacy, Cluj-Napoca, Romania; 2Developmental Psychology Lab, Department of Psychology, Babeş-Bolyai University, Cluj-Napoca, RomaniaCorrespondence: Laura Visu-PetraDepartment of Psychology, Babeș-Bolyai University, Republicii Str. No. 37, Cluj-Napoca 400015, RomaniaTel +40264 590 559Email laurapetra@psychology.roPurpose: Rheumatoid arthritis (RA) is a chronic inflammatory systemic disease associated with various degrees of impairment across different cognitive domains. We aimed to provide a detailed computerized investigation of verbal and visuospatial short-term and working memory (dys)functions in RA patients, assessing both accuracy and response speed, while relating them to age, disease-related activity, affective problems, psychomotor speed and other clinical parameters.Patients and Methods: The study included 29 RA patients (mean age 50.6 ± 12.3 years, 79% female) and 30 controls (matched according to age, gender and education), assessed with short-term and working memory tasks from the Cambridge Neuropsychological Test Automated Battery (CANTAB) and the Automated Working Memory Assessment (AWMA).Results: RA patients were significantly slower on the basic processing speed test (Motor Screening Test, p =0.003). Their short-term information storage (verbal and visuospatial) was comparable to controls, yet this similar accuracy came at the expense of a longer response time to retain information correctly (on spatial span, p = 0.04). On tasks with higher executive demands, both visuospatial and verbal working memory were compromised, as RA patients took longer (p = 0.004) and had a higher number of total errors (p = 0.02) when conducting a strategic memory-guided search (Spatial Working Memory), and had a significantly lower verbal working memory span on the backwards digit recall test (p = 0.02).Conclusion: The findings of this study emphasize the usefulness of performing computerized tests to detect subtle signs of cognitive impairment and of intact performance, which can inform memory training protocols for this vulnerable population.Keywords: rheumatoid arthritis, verbal working memory, visuospatial working memory, cognitive dysfunction, neuropsychological assessment

Published
2020

7. Cohort Enrichment Strategies for Progressive Interstitial Lung Disease in Systemic Sclerosis From European Scleroderma Trials and Research

Author

Hoffmann-Vold A. -M., Brunborg C., Airo P., Ananyeva L. P., Czirjak L., Guiducci S., Hachulla E., Li M., Mihai C., Riemekasten G., Sfikakis P. P., Valentini G., Kowal-Bielecka O., Allanore Y., Distler O., Vacca A., Giollo A., Balbir-Gurman A., Gheorghiu A. M., Marcoccia A., Herrick A., Radic M., Stamenkovic B., Anic B., Granel B., Ribi C., Selmi C. F., Carlos de la Puente M., de Souza Muller C., Denton C., Kayser C., Tanaseanu C. -M., Majewski D., Rimar D., Krasowska D., Veale D., Walker U., Kerzberg E., Rezus E., Zanatta E., Siegert E., De Langhe E., Oksel F., Ingegnoli F., Cantatore F. P., Szucs G., Cuomo G., Seskute G., Litinsky V., Castellvi I., Morovic-Vergles J., Sibilia J., Henes J., Solanki K., Perdan-Pirkmajer K., Herrmann K., Saketkoo L. A., Stamp L., Mouthon L., Salvador M. J., Pozzi M. R., Uprus M., Vanthuyne M., Engelhart M., Kohm M., Iudici M., Inanc M., Fathi N., Pamuk N., Garcia de la Pena Lefebv P., Carreira P. E., Bancel D. F., Moroncini L., Montecucco C., Ancuta C., Sunderkotter C., Muller-Ladner U., Rosato E., Kucharz E. J., Iannone F., Del Galdo F., Poormoghim H., Kotter I., Distler J., Cutolo M., Tikly M., Damjanov N., Hunzelmann N., Vlachoyiannopoulos P., Hasler P., Sarzi Puttini P., Wiland P., Becvar R., Yavuz S., Zdrojewski Z., Pellerito R., Foti R., Ionescu R. M., Adler S., Kahl S., Moiseev S., Stebbings S., Rednic S., Negrini S., Heitmann S., Ullman S., Agachi S., Martin T., Schmeiser T., Riccieri V., Smith V., Bernardino V., Ortiz-Santamaria V., Hsu V. M., Abdel Atty Mohamed W. A., Hoffmann-Vold, A. -M., Brunborg, C., Airo, P., Ananyeva, L. P., Czirjak, L., Guiducci, S., Hachulla, E., Li, M., Mihai, C., Riemekasten, G., Sfikakis, P. P., Valentini, G., Kowal-Bielecka, O., Allanore, Y., Distler, O., Vacca, A., Giollo, A., Balbir-Gurman, A., Gheorghiu, A. M., Marcoccia, A., Herrick, A., Radic, M., Stamenkovic, B., Anic, B., Granel, B., Ribi, C., Selmi, C. F., Carlos de la Puente, M., de Souza Muller, C., Denton, C., Kayser, C., Tanaseanu, C. -M., Majewski, D., Rimar, D., Krasowska, D., Veale, D., Walker, U., Kerzberg, E., Rezus, E., Zanatta, E., Siegert, E., De Langhe, E., Oksel, F., Ingegnoli, F., Cantatore, F. P., Szucs, G., Cuomo, G., Seskute, G., Litinsky, V., Castellvi, I., Morovic-Vergles, J., Sibilia, J., Henes, J., Solanki, K., Perdan-Pirkmajer, K., Herrmann, K., Saketkoo, L. A., Stamp, L., Mouthon, L., Salvador, M. J., Pozzi, M. R., Uprus, M., Vanthuyne, M., Engelhart, M., Kohm, M., Iudici, M., Inanc, M., Fathi, N., Pamuk, N., Garcia de la Pena Lefebv, P., Carreira, P. E., Bancel, D. F., Moroncini, L., Montecucco, C., Ancuta, C., Sunderkotter, C., Muller-Ladner, U., Rosato, E., Kucharz, E. J., Iannone, F., Del Galdo, F., Poormoghim, H., Kotter, I., Distler, J., Cutolo, M., Tikly, M., Damjanov, N., Hunzelmann, N., Vlachoyiannopoulos, P., Hasler, P., Sarzi Puttini, P., Wiland, P., Becvar, R., Yavuz, S., Zdrojewski, Z., Pellerito, R., Foti, R., Ionescu, R. M., Adler, S., Kahl, S., Moiseev, S., Stebbings, S., Rednic, S., Negrini, S., Heitmann, S., Ullman, S., Agachi, S., Martin, T., Schmeiser, T., Riccieri, V., Smith, V., Bernardino, V., Ortiz-Santamaria, V., Hsu, V. M., and Abdel Atty Mohamed, W. A.

Subjects
interstitial lung disease, Pulmonary and Respiratory Medicine, enrichment, systemic sclerosis, clinical trial, Cardiology and Cardiovascular Medicine, Critical Care and Intensive Care Medicine
Abstract

BACKGROUND: Enrichment strategies from clinical trials for progressive systemic sclerosis-associated interstitial lung disease (SSc-ILD) have not been tested in a real-life cohort.RESEARCH QUESTION: Do enrichment strategies for progressive ILD impact efficacy, repre-sentativeness, and feasibility in patients with SSc-ILD from the European Scleroderma Trials and Research (EUSTAR) database?STUDY DESIGN AND METHODS: We applied the inclusion criteria of major recent SSc-ILD trials (Study of the Efficacy and Safety of Tocilizumab in Participants With Systemic Sclerosis [focuSSced], Scleroderma Lung Study II [SLS II], and Safety and Efficacy of Nintedanib in Systemic Sclerosis [SENSCIS]) and assessed progressive ILD, which was defined as absolute change in FVC and as significant progression (FVC decline $10%). Data were compared with all patients and with patients who did not fulfill any inclusion criteria. RESULTS: In total, 2,258 patients with SSc-ILD were included: 31.2% of the patients met SENSCIS criteria; 5.8% of the patients met SLS II criteria; 1.6% of the patients met focuSSced criteria, and 67.7% (1,529) of the patients did not meet any criteria. In the first 12 + 3 months, the absolute FVC decline in all patients and in patients who fulfilled criteria from SENSCIS was -0.1%, in patients who fulfilled criteria from focuSSced was -3.7%, and in patients who fulfilled criteria from SLS II was 2.3%, with accompanying more progressors in focuSSced. The patient populations that fulfilled the different study inclusion criteria significantly differed in various clinical parameters. In the second 12-month period, SENSCIS-enriched patients had a further absolute FVC% decline as described for the total cohort. In contrast, patients who fulfilled the focuSSced and SLS II criteria showed numeric improvement of lung function. There were no significant associations of enrichment criteria and ILD progression.INTERPRETATION: The application of enrichment criteria from previous clinical trials showed enrichment for progression with variable success, which led to selected patient populations reducing feasibility of recruitment. These findings are important for future clinical trial design and interpretation of the results of published trials.CHEST 2023; 163(3):586-598

Published
2023
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8. ERN ReCONNET points to consider for treating patients living with autoimmune rheumatic diseases with antiviral therapies and anti-SARS-CoV-2 antibody products

Author

Talarico, R, Ramirez, G, Barreira, S, Cardamone, C, Triggianese, P, Aguilera, S, Andersen, J, Avcin, T, Benistan, K, Bertsias, G, Bortoluzzi, A, Bouillot, C, Bulina, I, Burmester, G, Callens, S, Carreira, P, Cervera, R, Cutolo, M, Damian, L, Della-Torre, E, Faria, R, Fonseca, J, Galetti, I, Hachulla, E, Iaccarino, L, Jacobsen, S, Khmelinskii, N, Limper, M, Marinello, D, Meyer, A, Moroncini, G, Nagy, G, Olesinska, M, Pamfil, C, Pileckyte, M, Pistello, M, Rednic, S, Richez, C, Romao, V, Schneider, M, Sciascia, S, Scire, C, Simonini, G, Smith, V, Sulli, A, Tani, C, Tas, S, Tincani, A, Vonk, M, Tektonidou, M, Mosca, M, Talarico R., Ramirez G. A., Barreira S. C., Cardamone C., Triggianese P., Aguilera S., Andersen J., Avcin T., Benistan K., Bertsias G., Bortoluzzi A., Bouillot C., Bulina I., Burmester G. R., Callens S., Carreira P. E., Cervera R., Cutolo M., Damian L., Della-Torre E., Faria R., Fonseca J. E., Galetti I., Hachulla E., Iaccarino L., Jacobsen S., Khmelinskii N., Limper M., Marinello D., Meyer A., Moroncini G., Nagy G., Olesinska M., Pamfil C., Pileckyte M., Pistello M., Rednic S., Richez C., Romao V. C., Schneider M., Sciascia S., Scire C. A., Simonini G., Smith V., Sulli A., Tani C., Tas S. W., Tincani A., Vonk M. C., Tektonidou M., Mosca M., Talarico, R, Ramirez, G, Barreira, S, Cardamone, C, Triggianese, P, Aguilera, S, Andersen, J, Avcin, T, Benistan, K, Bertsias, G, Bortoluzzi, A, Bouillot, C, Bulina, I, Burmester, G, Callens, S, Carreira, P, Cervera, R, Cutolo, M, Damian, L, Della-Torre, E, Faria, R, Fonseca, J, Galetti, I, Hachulla, E, Iaccarino, L, Jacobsen, S, Khmelinskii, N, Limper, M, Marinello, D, Meyer, A, Moroncini, G, Nagy, G, Olesinska, M, Pamfil, C, Pileckyte, M, Pistello, M, Rednic, S, Richez, C, Romao, V, Schneider, M, Sciascia, S, Scire, C, Simonini, G, Smith, V, Sulli, A, Tani, C, Tas, S, Tincani, A, Vonk, M, Tektonidou, M, Mosca, M, Talarico R., Ramirez G. A., Barreira S. C., Cardamone C., Triggianese P., Aguilera S., Andersen J., Avcin T., Benistan K., Bertsias G., Bortoluzzi A., Bouillot C., Bulina I., Burmester G. R., Callens S., Carreira P. E., Cervera R., Cutolo M., Damian L., Della-Torre E., Faria R., Fonseca J. E., Galetti I., Hachulla E., Iaccarino L., Jacobsen S., Khmelinskii N., Limper M., Marinello D., Meyer A., Moroncini G., Nagy G., Olesinska M., Pamfil C., Pileckyte M., Pistello M., Rednic S., Richez C., Romao V. C., Schneider M., Sciascia S., Scire C. A., Simonini G., Smith V., Sulli A., Tani C., Tas S. W., Tincani A., Vonk M. C., Tektonidou M., and Mosca M.

Abstract

Recent studies have shown that people who are immunocompromised may inadvertently play a role in spurring the mutations of the virus that create new variants. This is because some immunocompromised individuals remain at risk of getting COVID-19 despite vaccination, experience more severe disease, are susceptible to being chronically infected and remain contagious for longer if they become infected and considering that immunocompromised individuals represent approximately 2% of the overall population, this aspect should be carefully considered. So far, some autoimmune rheumatic disease (ARD) patients with COVID-19 have been treated with antiviral therapies or anti-SARS-CoV-2 antibody products. However, there is no homogeneous approach to these treatment strategies. This issue was addressed within the European Reference Network (ERN) on Rare and Complex Connective Tissue and Musculoskeletal Diseases (ReCONNET) in a discussion among experts and patient's representatives in the context of the rare and complex connective tissue diseases (rCTDs) covered by the Network. ERN ReCONNET is one of the 24 ERNs launched by the European Commission in 2017 with the aim of tackling low prevalence and rare diseases that require highly specialised treatment and promoting concentration of knowledge and resources through virtual networks involving healthcare providers (HCPs) across the European Union (EU). Considering the urgent need to provide guidance not only to the rCTDs community, but also to the whole ARDs community, a multidisciplinary Task Force, including expert clinicians and European Patient Advocacy Group (ePAG) Advocates, was created in the framework of ERN ReCONNET with the aim of developing overarching principles (OP) and points-to-consider (PtC) on a homogenous approach to treat immunocompromised patients with ARDs (with a particular focus on CTDs) affected by COVID-19 using antiviral therapies and anti-SARS-CoV-2 antibody products. The present work reports the final OP

Published
2023

9. 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

10. AB1309 CLINICAL CHARACTERISTICS OF NEW-ONSET IDIOPATHIC INFLAMMATORY MYOPATHIES FOLLOWING SARS-CoV-2 INFECTION OR VACCINATION – ANALYSIS OF A CASE SERIES

Author

Marian, A., primary, Tamas, M. M., additional, Muntean, L., additional, Resteu, O. M., additional, Vaida-Voevod, D. A., additional, Felea, I., additional, Damian, L., additional, Filipescu, I., additional, Pamfil, C., additional, Szabo, I., additional, Simon, S. P., additional, and Rednic, S., additional

Published
2023
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12. OP0238 IMMUNOSUPPRESSION WITH TARGETED DMARDS REDUCES MORBIDITY AND MORTALITY IN PRE-CAPILLARY PULMONARY HYPERTENSION ASSOCIATED WITH SYSTEMIC SCLEROSIS: A EUSTAR ANALYSIS

Author

Bruni, C., primary, Tofani, L., additional, Fretheim, H., additional, Weber, Y., additional, Hachulla, E., additional, Carreira, P., additional, Giuggioli, D., additional, Airò, P., additional, Siegert, E., additional, Müller-Ladner, U., additional, Matucci-Cerinic, M., additional, Riemekasten, G., additional, Simeon Aznar, C. P., additional, De Vries-Bouwstra, J., additional, Saketkoo, L. A., additional, Distler, J., additional, Balbir-Gurman, A., additional, Castellví, I., additional, Zanatta, E., additional, Smith, V., additional, Denton, C. P., additional, Maurer, B., additional, Giollo, A., additional, Iannone, F., additional, Dagna, L., additional, Truchetet, M. E., additional, Kuwana, M., additional, Allanore, Y., additional, Tanaka, Y., additional, Martin, M., additional, Rosato, E., additional, Gheorghiu, A. M., additional, Del Galdo, F., additional, Solanki, K., additional, Vacca, A., additional, Resende, C., additional, Vieira, S., additional, Czirják, L., additional, Baresic, M., additional, Cantatore, F. P., additional, Riccieri, V., additional, Andréasson, K., additional, Chung, L., additional, Souza Muller, C., additional, Opris-Belinski, D., additional, Rednic, S., additional, Sfikakis, P., additional, Levy, Y., additional, Hsu, V., additional, Heitmann, S., additional, Henes, J., additional, Moroncini, G., additional, Iudici, M., additional, De Langhe, E., additional, Herrick, A., additional, Montecucco, C., additional, Hoffmann-Vold, A. M., additional, and Distler, O., additional

Published
2023
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13. POS0370 2023 EULAR RECOMMENDATIONS FOR THE MANAGEMENT OF FATIGUE IN PEOPLE WITH INFLAMMATORY RHEUMATIC AND MUSCULOSKELETAL DISEASES

Author

Dures, E., primary, Farisogullari, B., additional, Santos, E., additional, Moltó, A., additional, Feldthusen, C., additional, Harris, C., additional, Elling-Audersch, C., additional, Connolly, D., additional, Elefante, E., additional, Estevez-Lopez, F., additional, Bini, I., additional, Primdahl, J., additional, Hoeper, K., additional, Urban, M., additional, Laar, M. V. D., additional, Redondo, M., additional, Böhm, P., additional, Amarnani, R., additional, Hayward, R., additional, Geenen, R., additional, Rednic, S., additional, Pettersson, S., additional, Thomsen, T., additional, Uhlig, T., additional, Ritschl, V., additional, and Machado, P., additional

Published
2023
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15. OP0234 2023 UPDATE OF EULAR RECOMMENDATIONS FOR THE TREATMENT OF SYSTEMIC SCLEROSIS

Author

Del Galdo, F., primary, Lescoat, A., additional, Conaghan, P. G., additional, Ananyeva, L. P., additional, Balbir-Gurman, A., additional, Bertoldo, E., additional, Boyadzhieva, V., additional, Castellví, I., additional, Colic, J., additional, Denton, C. P., additional, Distler, O., additional, El Aoufy, K., additional, Emmel, J., additional, Farrington, S., additional, Gabrielli, A., additional, Galetti, I., additional, Hoffmann-Vold, A. M., additional, Kowal-Bielecka, O., additional, Matucci Cerinic, M., additional, Müller-Ladner, U., additional, Ostojic, P., additional, Rednic, S., additional, Santiago, T., additional, Suliman, Y. A., additional, Tarasova, A., additional, Vonk, M., additional, and Allanore, Y., additional

Published
2023
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18. Detection of Novel Biomarkers in Pediatric Autoimmune Hepatitis by Proteomic Profiling.

Author

Sîrbe, C., Badii, O.M., Crisan, T.O., Bența, G., Grama, A., Joosten, L.A.B., Rednic, S., Pop, T.L., Sîrbe, C., Badii, O.M., Crisan, T.O., Bența, G., Grama, A., Joosten, L.A.B., Rednic, S., and Pop, T.L.

Abstract

Item does not contain fulltext, Autoimmune hepatitis (AIH) is characterized by immune-mediated hepatocyte injury resulting in the destruction of liver cells, causing inflammation, liver failure, and fibrosis. Pediatric (AIH) is an autoimmune inflammatory disease that usually requires immunosuppression for an extended period. Frequent relapses after treatment discontinuation demonstrate that current therapies do not control intrahepatic immune processes. This study describes targeted proteomic profiling data in patients with AIH and controls. A total of 92 inflammatory and 92 cardiometabolic plasma markers were assessed for (i) pediatric AIH versus controls, (ii) AIH type 1 versus type 2, (iii) AIH and AIH-autoimmune sclerosing cholangitis overlapping syndrome and (iv) correlations with circulating vitamin D levels in AIH. A total of 16 proteins showed a nominally significant differential abundance in pediatric patients with AIH compared to controls. No clustering of AIH subphenotypes based on all protein data was observed, and no significant correlation of vitamin D levels was observed for the identified proteins. The proteins that showed variable expression include CA1, CA3, GAS6, FCGR2A, 4E-BP1 and CCL19, which may serve as potential biomarkers for patients with AIH. CX3CL1, CXCL10, CCL23, CSF1 and CCL19 showed homology to one another and may be coexpressed in AIH. CXCL10 seems to be the central intermediary link for the listed proteins. These proteins were involved in relevant mechanistic pathways for liver diseases and immune processes in AIH pathogenesis. This is the first report on the proteomic profile of pediatric AIH. The identified markers could potentially lead to new diagnostic and therapeutic tools. Nevertheless, considering the complex pathogenesis of AIH, more extensive studies are warranted to replicate and validate the present study's findings.

Published
2023

19. ERN ReCONNET points to consider for treating patients living with autoimmune rheumatic diseases with antiviral therapies and anti-SARS-CoV-2 antibody products.

Author

Talarico, R., Ramirez, G.A., Barreira, S.C., Cardamone, C., Triggianese, P., Aguilera, S., Andersen, J., Avcin, T., Benistan, K., Bertsias, G., Bortoluzzi, A., Bouillot, C., Bulina, I., Burmester, G.R., Callens, S., Carreira, P.E., Cervera, R., Cutolo, M., Damian, L., Della-Torre, E., Faria, R., Fonseca, J.E., Galetti, I., Hachulla, E., Iaccarino, L., Jacobsen, S., Khmelinskii, N., Limper, M., Marinello, D., Meyer, A, Moroncini, G., Nagy, G., Olesinska, M., Pamfil, C., Pileckyte, M., Pistello, M., Rednic, S., Richez, C., Romão, V.C., Schneider, M., Sciascia, S., Scirè, C.A., Simonini, G., Smith, V., Sulli, A., Tani, C., Tas, S.W., Tincani, A., Vonk, M.C., Tektonidou, M., Mosca, M., Talarico, R., Ramirez, G.A., Barreira, S.C., Cardamone, C., Triggianese, P., Aguilera, S., Andersen, J., Avcin, T., Benistan, K., Bertsias, G., Bortoluzzi, A., Bouillot, C., Bulina, I., Burmester, G.R., Callens, S., Carreira, P.E., Cervera, R., Cutolo, M., Damian, L., Della-Torre, E., Faria, R., Fonseca, J.E., Galetti, I., Hachulla, E., Iaccarino, L., Jacobsen, S., Khmelinskii, N., Limper, M., Marinello, D., Meyer, A, Moroncini, G., Nagy, G., Olesinska, M., Pamfil, C., Pileckyte, M., Pistello, M., Rednic, S., Richez, C., Romão, V.C., Schneider, M., Sciascia, S., Scirè, C.A., Simonini, G., Smith, V., Sulli, A., Tani, C., Tas, S.W., Tincani, A., Vonk, M.C., Tektonidou, M., and Mosca, M.

Abstract

Item does not contain fulltext, Recent studies have shown that people who are immunocompromised may inadvertently play a role in spurring the mutations of the virus that create new variants. This is because some immunocompromised individuals remain at risk of getting COVID-19 despite vaccination, experience more severe disease, are susceptible to being chronically infected and remain contagious for longer if they become infected and considering that immunocompromised individuals represent approximately 2% of the overall population, this aspect should be carefully considered. So far, some autoimmune rheumatic disease (ARD) patients with COVID-19 have been treated with antiviral therapies or anti-SARS-CoV-2 antibody products. However, there is no homogeneous approach to these treatment strategies. This issue was addressed within the European Reference Network (ERN) on Rare and Complex Connective Tissue and Musculoskeletal Diseases (ReCONNET) in a discussion among experts and patient's representatives in the context of the rare and complex connective tissue diseases (rCTDs) covered by the Network. ERN ReCONNET is one of the 24 ERNs launched by the European Commission in 2017 with the aim of tackling low prevalence and rare diseases that require highly specialised treatment and promoting concentration of knowledge and resources through virtual networks involving healthcare providers (HCPs) across the European Union (EU). Considering the urgent need to provide guidance not only to the rCTDs community, but also to the whole ARDs community, a multidisciplinary Task Force, including expert clinicians and European Patient Advocacy Group (ePAG) Advocates, was created in the framework of ERN ReCONNET with the aim of developing overarching principles (OP) and points-to-consider (PtC) on a homogenous approach to treat immunocompromised patients with ARDs (with a particular focus on CTDs) affected by COVID-19 using antiviral therapies and anti-SARS-CoV-2 antibody products. The present work reports the final OP

Published
2023

20. Hyperuricemia remodels the serum proteome toward a higher inflammatory state.

Author

Cabău, G., Gaal, O., Badii, O.M., Nica, V., Mirea, A.M., Hotea, I., Pamfil, C., Popp, R.A., Netea, M.G., Rednic, S., Crisan, T.O., Joosten, L.A.B., Cabău, G., Gaal, O., Badii, O.M., Nica, V., Mirea, A.M., Hotea, I., Pamfil, C., Popp, R.A., Netea, M.G., Rednic, S., Crisan, T.O., and Joosten, L.A.B.

Abstract

Item does not contain fulltext, Gout is an autoinflammatory disease triggered by a complex innate immune response to MSU crystals and inflammatory triggers. While hyperuricemia is an obligatory risk factor for the development of gout, the majority of individuals with hyperuricemia never develop gout but have an increased risk of developing cardiometabolic disorders. Current management of gout aims at MSU crystal dissolution by lowering serum urate. We apply a targeted proteomic analysis, using Olink inflammation panel, to a large group of individuals with gout, asymptomatic hyperuricemia, and normouricemic controls, and we show a urate-driven inflammatory signature. We add in vivo evidence of persistent immune activation linked to urate exposure and describe immune pathways involved in the pathogenesis of gout. Our results support a pro-inflammatory effect of asymptomatic hyperuricemia and pave the way for new research into targetable mechanisms in gout and cardiometabolic complications of asymptomatic hyperuricemia.

Published
2023

21. ERN ReCONNET points to consider for treating patients living with autoimmune rheumatic diseases with antiviral therapies and anti-SARS-CoV-2 antibody products

Author

Talarico, Rosaria R., Ramirez, G. A., Barreira, S. C., Cardamone, C., Triggianese, P., Aguilera, S., Andersen, J., Avcin, T., Benistan, K., Bertsias, G., Bortoluzzi, A., Bouillot, C., Bulina, I., Burmester, G. R., Callens, S., Carreira, P. E., Cervera, R., Cutolo, M., Damian, L., Torre, E. Della, Faria, R., Fonseca, J. E., Galetti, I., Hachulla, E., Iaccarino, L., Jacobsen, S., Khmelinskii, N., Limper, M., Marinello, D., Meyer, A., Moroncini, G., Nagy, G., Olesinska, M., Pamfil, C., Pileckyte, M., Pistello, M., Rednic, S., Richez, C., Romão, V. C., Schneider, M., Sciascia, S., Scirè, C. A., Simonini, G., Smith, V., Sulli, A., Tani, C., Tas, S. W., Tincani, A., Vonk, M. C., Tektonidou, M., Mosca, M., Talarico, Rosaria R., Ramirez, G. A., Barreira, S. C., Cardamone, C., Triggianese, P., Aguilera, S., Andersen, J., Avcin, T., Benistan, K., Bertsias, G., Bortoluzzi, A., Bouillot, C., Bulina, I., Burmester, G. R., Callens, S., Carreira, P. E., Cervera, R., Cutolo, M., Damian, L., Torre, E. Della, Faria, R., Fonseca, J. E., Galetti, I., Hachulla, E., Iaccarino, L., Jacobsen, S., Khmelinskii, N., Limper, M., Marinello, D., Meyer, A., Moroncini, G., Nagy, G., Olesinska, M., Pamfil, C., Pileckyte, M., Pistello, M., Rednic, S., Richez, C., Romão, V. C., Schneider, M., Sciascia, S., Scirè, C. A., Simonini, G., Smith, V., Sulli, A., Tani, C., Tas, S. W., Tincani, A., Vonk, M. C., Tektonidou, M., and Mosca, M.

Abstract

Recent studies have shown that people who are immunocompromised may inadvertently play a role in spurring the mutations of the virus that create new variants. This is because some immunocompromised individuals remain at risk of getting COVID-19 despite vaccination, experience more severe disease, are susceptible to being chronically infected and remain contagious for longer if they become infected and considering that immunocompromised individuals represent approximately 2% of the overall population, this aspect should be carefully considered. So far, some autoimmune rheumatic disease (ARD) patients with COVID-19 have been treated with antiviral therapies or anti-SARS-CoV-2 antibody products. However, there is no homogeneous approach to these treatment strategies. This issue was addressed within the European Reference Network (ERN) on Rare and Complex Connective Tissue and Musculoskeletal Diseases (ReCONNET) in a discussion among experts and patient’s representatives in the context of the rare and complex connective tissue diseases (rCTDs) covered by the Network. ERN ReCONNET is one of the 24 ERNs launched by the European Commission in 2017 with the aim of tackling low prevalence and rare diseases that require highly specialised treatment and promoting concentration of knowledge and resources through virtual networks involving healthcare providers (HCPs) across the European Union (EU). Considering the urgent need to provide guidance not only to the rCTDs community, but also to the whole ARDs community, a multidisciplinary Task Force, including expert clinicians and European Patient Advocacy Group (ePAG) Advocates, was created in the framework of ERN ReCONNET with the aim of developing overarching principles (OP) and points-to-consider (PtC) on a homogenous approach to treat immunocompromised patients with ARDs (with a particular focus on CTDs) affected by COVID-19 using antiviral therapies and anti-SARS-CoV-2 antibody products. The present work reports the final OP, Recent studies have shown that people who are immunocompromised may inadvertently play a role in spurring the mutations of the virus that create new variants. This is because some immunocompromised individuals remain at risk of getting COVID-19 despite vaccination, experience more severe disease, are susceptible to being chronically infected and remain contagious for longer if they become infected and considering that immunocompromised individuals represent approximately 2% of the overall population, this aspect should be carefully considered. So far, some autoimmune rheumatic disease (ARD) patients with COVID-19 have been treated with antiviral therapies or anti-SARS-CoV-2 antibody products. However, there is no homogeneous approach to these treatment strategies. This issue was addressed within the European Reference Network (ERN) on Rare and Complex Connective Tissue and Musculoskeletal Diseases (ReCONNET) in a discussion among experts and patient's representatives in the context of the rare and complex connective tissue diseases (rCTDs) covered by the Network. ERN ReCONNET is one of the 24 ERNs launched by the European Commission in 2017 with the aim of tackling low prevalence and rare diseases that require highly specialised treatment and promoting concentration of knowledge and resources through virtual networks involving healthcare providers (HCPs) across the European Union (EU). Considering the urgent need to provide guidance not only to the rCTDs community, but also to the whole ARDs community, a multidisciplinary Task Force, including expert clinicians and European Patient Advocacy Group (ePAG) Advocates, was created in the framework of ERN ReCONNET with the aim of developing overarching principles (OP) and points-to-consider (PtC) on a homogenous approach to treat immunocompromised patients with ARDs (with a particular focus on CTDs) affected by COVID-19 using antiviral therapies and anti-SARS-CoV-2 antibody products. The present work reports the final

Published
2023

22. Efficacy and safety of upadacitinib in patients with active psoriatic arthritis and axial involvement: results from two phase 3 studies

Author

Baraliakos, X, Ranza, R, Östör, A, Ciccia, F, Coates, LC, Rednic, S, Walsh, JA, Douglas, K, Gao, T, Kato, K, Song, I-H, Ganz, F, Deodhar, A, Baraliakos, Xenofon, Ranza, Roberto, Östör, Andrew, Ciccia, Francesco, Coates, Laura C, Rednic, Simona, Walsh, Jessica A, Douglas, Kevin, Gao, Tianming, Kato, Koji, Song, In-Ho, Ganz, Fabiana, and Deodhar, Atul

Subjects
Ankylosing Spondylitis Disease Activity Score (ASDAS), Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), Axial involvement, Psoriatic arthritis (PsA), Upadacitinib, Adalimumab, Safety, Janus kinase (JAK) inhibitor
Abstract

Background The objective of this post-hoc analysis was to assess the efficacy and safety of upadacitinib in psoriatic arthritis (PsA) patients with axial involvement. Methods Post-hoc analysis of SELECT-PsA 1 and SELECT-PsA 2 in patients randomized to upadacitinib 15 mg (UPA15), placebo (switched to UPA15 at week 24), or adalimumab 40 mg (ADA; SELECT-PsA 1 only). Axial involvement was determined by investigator judgement (yes or no; based on the totality of available clinical information, such as duration and characteristics of back pain, age of onset, and previous lab investigations and imaging, if available) alone, or investigator judgement and patient-reported outcome (PRO)-based criteria (Bath Ankylosing Spondylitis Disease Activity Index [BASDAI] ≥ 4 and BASDAI Q2 ≥ 4). Efficacy outcomes that describe axial disease activity, including BASDAI endpoints, such as change from baseline in the overall BASDAI score or proportion of patients achieving BASDAI50 (≥ 50% improvement from baseline), as well as Ankylosing Spondylitis Disease Activity Score (ASDAS) endpoints, such as mean change from baseline in overall ASDAS or proportion of patients achieving ASDAS inactive disease or low disease activity, were evaluated at weeks 12, 24, and 56, with nominal P-values shown. Treatment-emergent adverse events (TEAEs) are summarized through week 56. Results 30.9% of patients in SELECT-PsA 1 and 35.7% in SELECT-PsA 2 had axial involvement by investigator judgement alone; 22.6% (SELECT-PsA 1) and 28.6% (SELECT-PsA 2) had axial involvement by investigator judgement and PRO-based criteria. Greater proportions of patients achieved BASDAI50 with UPA15 versus placebo using either criterion, and versus ADA using investigator judgement alone, at week 24 in SELECT-PsA 1 (investigator alone: UPA15, 59.0%, placebo, 26.9%, P P = 0.015; investigator and PRO-based: UPA15, 60.4%, placebo, 29.3%, P P = 0.074), with comparable findings in SELECT-PsA 2. Similar results were observed with UPA15 for additional BASDAI and ASDAS endpoints at weeks 12 and 24, with improvements maintained at week 56. Rates of TEAEs were generally similar across sub-groups irrespective of axial involvement status. Conclusions PsA patients with axial involvement determined by predefined criteria showed greater BASDAI and ASDAS responses with UPA15 versus placebo, and numerically similar/greater responses versus ADA. Safety results were generally comparable between patients with or without axial involvement. Trial registration ClinicalTrials.gov: SELECT-PsA 1, NCT03104400; SELECT-PsA 2, NCT0310437.

Published
2023
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23. Persistent inflammation in systemic sclerosis is strongly associated with mortality: a EUSTAR database analysis

Author

Guler, S A, primary, Sarbu, A, additional, Stadler, O, additional, Allanore, Y, additional, Bernardino, V, additional, Distler, J H, additional, Gabrielli, A, additional, Hoffmann-Vold, A, additional, Matucci-Cerinic, M, additional, Müller-Ladner, U, additional, Ortiz-Santamaria, V, additional, Rednic, S, additional, Riccieri, V, additional, Smith, V, additional, Ullman, S, additional, Walker, U, additional, Geiser, T, additional, Distler, O, additional, Maurer, B, additional, and Kollert, F, additional

Published
2022
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24. ACE inhibitors in SSc patients display a risk factor for scleroderma renal crisis—a EUSTAR analysis

Author

Bütikofer L, Varisco PA, Distler O, Kowal-Bielecka O, Allanore Y, Riemekasten G, Villiger PM, Adler S, Avouac J, Walker UA, Guiducci S, Airò P, Hachulla E, Valentini G, Carreira PE, Cozzi F, Gurman AB, Braun-Moscovici Y, Damjanov N, Ananieva LP, Scorza R, Jimenez S, Busquets J, Li M, Müller-Ladner U, Maurer B, Tyndall A, Lapadula G, Iannone F, Becvar R, Sierakowsky S, Bielecka OK, Cutolo M, Sulli A, Cuomo G, Vettori S, Rednic S, Nicoara I, Vlachoyiannopoulos P, Montecucco C, Caporali R, Novak S, Czirják L, Varju C, Chizzolini C, Kucharz EJ, Kotulska A, Kopec-Medrek M, Widuchowska M, Rozman B, Mallia C, Coleiro B, Gabrielli A, Farge D, Hij A, Hesselstrand R, Scheja A, Wollheim F, Martinovic D, Govoni M, Monaco AL, Hunzelmann N, Pellerito R, Bambara LM, Caramaschi P, Black C, Denton C, Henes J, Santamaria VO, Heitmann S, Krasowska D, Seidel M, Oleszowsky M, Burkhardt H, Himsel A, Salvador MJ, Stamenkovic B, Stankovic A, Tikly M, Starovoytova MN, Engelhart M, Strauss G, Nielsen H, Damgaard K, Szücs G, Mendoza AZ, de la Puente Buijdos C, Sifuentes Giraldo WA, Midtvedt Ø, Garen T, Launay D, Valesini G, Riccieri V, Ionescu RM, Opris D, Groseanu L, Wigley FM, Mihai CM, Cornateanu RS, Ionitescu R, Gherghe AM, Gorga M, Dobrota R, Bojinca M, Schett G, Distler JHW, Meroni P, Zeni S, Mouthon L, De Keyser F, Smith V, Cantatore FP, Corrado A, Ullman S, Iversen L, Pozzi MR, Eyerich K, Hein R, Knott E, Szechinski J, Wiland P, Szmyrka-Kaczmarek M, Sokolik R, Morgiel E, Krummel-Lorenz B, Saar P, Aringer M, Günther C, Anic B, Baresic M, Mayer M, Radominski SC, de Souza Müller C, Azevedo VF, Agachi S, Groppa L, Chiaburu L, Russu E, Zenone T, Stebbings S, Highton J, Stamp L, Chapman P, Baron M, O'Donnell J, Solanki K, Doube A, Veale D, O'Rourke M, Loyo E, Rosato E, Pisarri S, Tanaseanu CM, Popescu M, Dumitrascu A, Tiglea I, Chirieac R, Ancuta C, Furst DE, Kafaja S, de la Peña Lefebvre PG, Rubio SR, Exposito MV, Sibilia J, Chatelus E, Gottenberg JE, Chifflot H, Litinsky I, Venalis A, Butrimiene I, Venalis P, Rugiene R, Karpec D, Kerzberg E, Montoya F, Cosentino V, Castellvi I., Publica, Bütikofer, L, Varisco, Pa, Distler, O, Kowal-Bielecka, O, Allanore, Y, Riemekasten, G, Villiger, Pm, Adler, S, Avouac, J, Walker, Ua, Guiducci, S, Airò, P, Hachulla, E, Valentini, G, Carreira, Pe, Cozzi, F, Gurman, Ab, Braun-Moscovici, Y, Damjanov, N, Ananieva, Lp, Scorza, R, Jimenez, S, Busquets, J, Li, M, Müller-Ladner, U, Maurer, B, Tyndall, A, Lapadula, G, Iannone, F, Becvar, R, Sierakowsky, S, Bielecka, Ok, Cutolo, M, Sulli, A, Cuomo, G, Vettori, S, Rednic, S, Nicoara, I, Vlachoyiannopoulos, P, Montecucco, C, Caporali, R, Novak, S, Czirják, L, Varju, C, Chizzolini, C, Kucharz, Ej, Kotulska, A, Kopec-Medrek, M, Widuchowska, M, Rozman, B, Mallia, C, Coleiro, B, Gabrielli, A, Farge, D, Hij, A, Hesselstrand, R, Scheja, A, Wollheim, F, Martinovic, D, Govoni, M, Monaco, Al, Hunzelmann, N, Pellerito, R, Bambara, Lm, Caramaschi, P, Black, C, Denton, C, Henes, J, Santamaria, Vo, Heitmann, S, Krasowska, D, Seidel, M, Oleszowsky, M, Burkhardt, H, Himsel, A, Salvador, Mj, Stamenkovic, B, Stankovic, A, Tikly, M, Starovoytova, Mn, Engelhart, M, Strauss, G, Nielsen, H, Damgaard, K, Szücs, G, Mendoza, Az, de la Puente Buijdos, C, Sifuentes Giraldo, Wa, Midtvedt, Ø, Garen, T, Launay, D, Valesini, G, Riccieri, V, Ionescu, Rm, Opris, D, Groseanu, L, Wigley, Fm, Mihai, Cm, Cornateanu, R, Ionitescu, R, Gherghe, Am, Gorga, M, Dobrota, R, Bojinca, M, Schett, G, Distler, Jhw, Meroni, P, Zeni, S, Mouthon, L, De Keyser, F, Smith, V, Cantatore, Fp, Corrado, A, Ullman, S, Iversen, L, Pozzi, Mr, Eyerich, K, Hein, R, Knott, E, Szechinski, J, Wiland, P, Szmyrka-Kaczmarek, M, Sokolik, R, Morgiel, E, Krummel-Lorenz, B, Saar, P, Aringer, M, Günther, C, Anic, B, Baresic, M, Mayer, M, Radominski, Sc, de Souza Müller, C, Azevedo, Vf, Agachi, S, Groppa, L, Chiaburu, L, Russu, E, Zenone, T, Stebbings, S, Highton, J, Stamp, L, Chapman, P, Baron, M, O'Donnell, J, Solanki, K, Doube, A, Veale, D, O'Rourke, M, Loyo, E, Rosato, E, Pisarri, S, Tanaseanu, Cm, Popescu, M, Dumitrascu, A, Tiglea, I, Chirieac, R, Ancuta, C, Furst, De, Kafaja, S, de la Peña Lefebvre, Pg, Rubio, Sr, Exposito, Mv, Sibilia, J, Chatelus, E, Gottenberg, Je, Chifflot, H, Litinsky, I, Venalis, A, Butrimiene, I, Venalis, P, Rugiene, R, Karpec, D, Kerzberg, E, Montoya, F, Cosentino, V, and Castellvi, I.

Subjects
INVOLVEMENT, Male, Hypertension, Renal, ACE inhibitors, lcsh:Diseases of the musculoskeletal system, Scleroderma Renal Crisis, MULTICENTER, Angiotensin-Converting Enzyme Inhibitors, Scleroderma, Scleroderma renal crisis, 0302 clinical medicine, Risk Factors, Medicine and Health Sciences, Medicine, 030212 general & internal medicine, Prospective Studies, 610 Medicine & health, Renal, Antihypertensive drugs, Outcome, antihypertensive drugs, arterial hypertension, outcome, scleroderma renal crisis, Incidence (epidemiology), Incidence, Hazard ratio, Acute Kidney Injury, Middle Aged, Europe, Treatment Outcome, Population Surveillance, Cohort, Hypertension, Female, 360 Social problems & social services, Proto-oncogene tyrosine-protein kinase Src, Research Article, Arterial hypertension, medicine.medical_specialty, 03 medical and health sciences, ENDOTHELIN-1, Internal medicine, Humans, Risk factor, Aged, 030203 arthritis & rheumatology, Scleroderma, Systemic, business.industry, SYSTEMIC-SCLEROSIS, Systemic, medicine.disease, Concomitant, lcsh:RC925-935, business
Abstract

Objectives To investigate the effect of ACE inhibitors (ACEi) on the incidence of scleroderma renal crisis (SRC) when given prior to SRC in the prospectively collected cohort from the European Scleroderma Trial and Research Group (EUSTAR). Methods SSc patients without prior SRC and at least one follow-up visit were included and analyzed regarding SRC, arterial hypertension, and medication focusing on antihypertensive medication and glucocorticoids (GC). Results Out of 14,524 patients in the database, we identified 7648 patients with at least one follow-up. In 27,450 person-years (py), 102 patients developed SRC representing an incidence of 3.72 (3.06–4.51) per 1000 py. In a multivariable time-to-event analysis adjusted for age, sex, disease severity, and onset, 88 of 6521 patients developed SRC. The use of ACEi displayed an increased risk for the development of SRC with a hazard ratio (HR) of 2.55 (95% confidence interval (CI) 1.65–3.95). Adjusting for arterial hypertension resulted in a HR of 2.04 (95%CI 1.29–3.24). There was no evidence for an interaction of ACEi and arterial hypertension (HR 0.83, 95%CI 0.32–2.13, p = 0.69). Calcium channel blockers (CCB), angiotensin receptor blockers (ARB), endothelin receptor antagonists, and GC—mostly in daily dosages below 15 mg of prednisolone—did not influence the hazard for SRC. Conclusions ACEi in SSc patients with concomitant arterial hypertension display an independent risk factor for the development of SRC but are still first choice in SRC treatment. ARBs might be a safe alternative, yet the overall safety of alternative antihypertensive drugs in SSc patients needs to be further studied.

Published
2020
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25. Late Skin Fibrosis in Systemic Sclerosis: A Study from the EUSTAR Cohort

Author

Michael Hughes 1 2, Suiyuan Huang 3 4, Juan Jose Alegre-Sancho 5, Patricia E Carreira 6, Merete Engelhart 7, Eric Hachulla 8, Joerg Henes 9, Eduardo Kerzberg 10, Maria Rosa Pozzi 11, Gabriela Riemekasten 12, Vanessa Smith 13, Gabriella Szücs 14, Marie Vanthuyne 15, Elisabetta Zanatta 16, Oliver Distler 17, Armando G Gabrielli 18, Anna-Maria Hoffmann-Vold 19, Virginia D Steen 20, Dinesh Khanna 3 21, EUSTAR Airò P, Allanore A, Ananieva Lp, Anic B, Balbir-Gurman A, Becvar R, Benvenuti F, Cantatore F P, Chung L S, Cuomo G, Cutolo M, Czirják L, Damjanov N, de Vries-Bouwstra J, Del Galdo F, Distler J, Eyerich K, Farge D, Foti R, Gheorghiu A M, Giollo A, Heitmann S, Herrick A, Hesselstrand R, Hsu I M, Hunzelmann N, Iannone F, Iudici M, Ionescuc M R, Ingegnoli F, Jose J, Joven B E, Kerzberg E, Kucharz E J, Kuwana M, Langhe E D, Launay D, Lefebvre P, Litinsky I, García de la Peña Lefebvre P, González-Martín J J, Li M, Loyo E, Martin T, Matucci-Cerinic M, Maurer B, Moroncini G, Mouthon L, Müller Cs, Müller-Ladner U, Novak S, Pastor P, Pecher A-C, Pellerito R, Pozzi M R, Oksel F, Rednic S, Rezus E, Riccieri V, Rosato E, Saketkoo L A, Salvador M J, Schmeiser T, Selmi C F, Sibilia J, Siegert E, Solanki K, Sommerlatte S, Spertini F, Stamenkovic B, Stamp L, Tanaseanu C-M, Tikly M, Tineo C, Ullman S, Üprus M, Vanthuyne M, Veale D, Walker U, Wiland P, Yargucu F, Yavuz S, University of Zurich, Michael Hughes, 1 2, Suiyuan Huang, 3 4, Juan Jose Alegre-Sancho, 5, Patricia, E Carreira 6, Merete Engelhart, 7, Eric Hachulla, 8, Joerg Henes, 9, Eduardo Kerzberg, 10, Maria Rosa Pozzi, 11, Gabriela Riemekasten, 12, Vanessa Smith, 13, Gabriella Szücs, 14, Marie Vanthuyne, 15, Elisabetta Zanatta, 16, Oliver Distler, 17, Armando, G Gabrielli 18, Anna-Maria Hoffmann-Vold, 19, Virginia, D Steen 20, Dinesh Khanna, 3 21, EUSTAR Airò, P, Allanore, A, Ananieva, Lp, Anic, B, Balbir-Gurman, A, Becvar, R, Benvenuti, F, Cantatore, F P, Chung, L S, Cuomo, G, Cutolo, M, Czirják, L, Damjanov, N, de Vries-Bouwstra, J, Del Galdo, F, Distler, J, Eyerich, K, Farge, D, Foti, R, Gheorghiu, A M, Giollo, A, Heitmann, S, Herrick, A, Hesselstrand, R, Hsu, I M, Hunzelmann, N, Iannone, F, Iudici, M, Ionescuc, M R, Ingegnoli, F, Jose, J, Joven, B E, Kerzberg, E, Kucharz, E J, Kuwana, M, Langhe, E D, Launay, D, Lefebvre, P, Litinsky, I, García de la Peña Lefebvre, P, González-Martín, J J, Li, M, Loyo, E, Martin, T, Matucci-Cerinic, M, Maurer, B, Moroncini, G, Mouthon, L, Müller, C, Müller-Ladner, U, Novak, S, Pastor, P, Pecher, A-C, Pellerito, R, Pozzi, M R, Oksel, F, Rednic, S, Rezus, E, Riccieri, V, Rosato, E, Saketkoo, L A, Salvador, M J, Schmeiser, T, Selmi, C F, Sibilia, J, Siegert, E, Solanki, K, Sommerlatte, S, Spertini, F, Stamenkovic, B, Stamp, L, Tanaseanu, C-M, Tikly, M, Tineo, C, Ullman, S, Üprus, M, Vanthuyne, M, Veale, D, Walker, U, Wiland, P, Yargucu, F, and Yavuz, S

Subjects
Rheumatology, Cohort enrichment, 10051 Rheumatology Clinic and Institute of Physical Medicine, Late disease, Systemic sclerosis, Pharmacology (medical), 610 Medicine & health, Fibrosis, Clinical trial design, Scleroderma, Skin
Abstract

Objectives The early trajectory of skin fibrosis provides insights into the disease course of systemic sclerosis (SSc) including mortality; however, little is known about late skin fibrosis. The aims of our study were to ascertain the prevalence and characteristics of late skin fibrosis in SSc. Methods We developed and tested three conceptual scenarios of late (>5 years after first non-RP feature) skin fibrosis including new worsening of skin disease, and failure to improve after worsening within 5-year window. We defined skin worsening as change in modified Rodnan skin score (mRSS) ≥5 units or ≥25%. Using strict inclusion criteria including complete mRSS, we identified 1,043 (out of 19 115) patients within the EUSTAR database for our analysis. We further restricted analysis within 887 (out of 1043) patients who had lcSSc or dcSSc at baseline. Results One-fifth of patients among the whole cohort (n = 208/1043, 19.9%) experienced mRSS worsening, including in patients with lcSSc or dcSSc at baseline (n = 193/887, 21.8%). This was largely due to new skin worsening after the 5-year window or failure to improve with worsening within the 5-year window. Patients with lower baseline mRSS and lcSSc were more likely to develop late skin fibrosis. Anti-Scl-70 was associated with progression from baseline lcSSc to dcSSc, and anticentromere was protective. Conclusions Late skin fibrosis is not uncommon in SSc. We have identified different patterns relevant to clinical practice and trial design. Late skin fibrosis is a neglected manifestation of SSc and warrants further investigation including to determine clinical outcomes and optimal therapeutic strategy.

Published
2022

27. 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

31. POS0383 CLINICAL CHARACTERISTICS AND PROGNOSIS OF PATIENTS WITH SYSTEMIC SCLEROSIS SINE SCLERODERMA: DATA FROM THE INTERNATIONAL EUSTAR DATABASE.

Author

Lescoat, A., primary, Huang, S., additional, Carreira, P., additional, Siegert, E., additional, De Vries-Bouwstra, J., additional, Distler, J. H. W., additional, Smith, V., additional, Del Galdo, F., additional, Anic, B., additional, Damjanov, N., additional, Rednic, S., additional, Ribi, C., additional, Farge, D., additional, Hoffmann-Vold, A. M., additional, Gabrielli, A., additional, Distler, O., additional, Khanna, D., additional, and Allanore, Y., additional

Published
2022
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32. POS0873 PERSISTENT INFLAMMATION IN SYSTEMIC SCLEROSIS IS STRONGLY ASSOCIATED WITH SEVERE DISEASE AND MORTALITY: AN ANALYSIS FROM THE EUSTAR DATABASE

Author

Sarbu, A. C., primary, Guler, S., additional, Stadler, O., additional, Allanore, Y., additional, Bernardino, V., additional, Distler, J. H. W., additional, Gabrielli, A., additional, Hoffmann-Vold, A. M., additional, Matucci-Cerinic, M., additional, Müller-Ladner, U., additional, Ortiz-Santamaria, V., additional, Rednic, S., additional, Riccieri, V., additional, Smith, V., additional, Ullman, S., additional, Walker, U., additional, Geiser, T., additional, Distler, O., additional, Maurer, B., additional, and Kollert, F., additional

Published
2022
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33. POS0934 EFFICACY OF UPADACITINIB ON PSORIATIC ARTHRITIS WITH AXIAL INVOLVEMENT DEFINED BY INVESTIGATOR ASSESSMENT AND PRO-BASED CRITERIA: RESULTS FROM TWO PHASE 3 STUDIES

Author

Baraliakos, X., primary, Ranza, R., additional, Ostor, A., additional, Ciccia, F., additional, Coates, L., additional, Rednic, S., additional, Walsh, J. A., additional, Gao, T., additional, Lertratanakul, A., additional, Song, I. H., additional, Ganz, F., additional, Douglas, K., additional, and Deodhar, A., additional

Published
2022
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34. CLINICAL CHARACTERISTICS AND PROGNOSIS OF PATIENTS WITH SYSTEMIC SCLEROSIS SINE SCLERODERMA: DATA FROM THE INTERNATIONAL EUSTAR DATABASE

Author

Lescoat, A, Huang, S., Carreira, P, Siegert, E., de Vries-Bouwstra, J., Distler, Jörg, Smith, V., del Galdo, F., Anic, B., Damjanov, N., Rednic, S., Ribi, C., Farge, D., Hoffmann-Vold, A. M., Gabrielli, A., Distler, O., Khanna, D., Allanore, Y, Institut de recherche en santé, environnement et travail (Irset), Université d'Angers (UA)-Université de Rennes (UR)-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), École des Hautes Études en Santé Publique [EHESP] (EHESP), University of Michigan [Ann Arbor], University of Michigan System, Hospital Universitario 12 de Octubre [Madrid], Charité - UniversitätsMedizin = Charité - University Hospital [Berlin], Universiteit Leiden, Friedrich-Alexander Universität Erlangen-Nürnberg (FAU), Ghent University Hospital, University of Leeds, University Hospital Centre Zagreb, Partenaires INRAE, University of Belgrade [Belgrade], Babes-Bolyai University [Cluj-Napoca] (UBB), Lausanne University Hospital, Hopital Saint-Louis [AP-HP] (AP-HP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Oslo University Hospital [Oslo], Università Politecnica delle Marche [Ancona] (UNIVPM), University hospital of Zurich [Zurich], Hôpital Cochin [AP-HP], Universiteit Leiden [Leiden], and Chard-Hutchinson, Xavier

Subjects
[SDV] Life Sciences [q-bio], [SDV]Life Sciences [q-bio]
Abstract

International audience

Published
2022
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35. PERSISTENT INFLAMMATION IN SYSTEMIC SCLEROSIS IS STRONGLY ASSOCIATED WITH SEVERE DISEASE AND MORTALITY: AN ANALYSIS FROM THE EUSTAR DATABASE

Author

Sarbu, A.C., Guler, S., Stadler, O., Allanore, Y., Bernardino, V., Distler, J. H. W., Gabrielli, A., Hoffmann-Vold, A. M., Matucci-Cerinic, M., Müller-Ladner, U., Ortiz-Santamaria, V., Rednic, S., Riccieri, V., Smith, V., Ullman, S., Walker, U., Geiser, T., Distler, O., Maurer, B., and Kollert, F.

Subjects
610 Medicine & health
Published
2022
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36. Rare clinical manifestations in systemic lupus erythematosus: A review on frequency and clinical presentation

Author

Tani, C. Elefante, E. Arnaud, L. Barreira, S.C. Bulina, I. Cavagna, L. Costedoat-Chalumeau, N. Doria, A. Fonseca, J.E. Franceschini, F. Fredi, M. Iaccarino, L. Limper, M. Majnik, J. Nagy, G. Pamfil, C. Rednic, S. Reynolds, J.A. Tektonidou, M.G. Troldborg, A. Zanframundo, G. Mosca, M.

Abstract

Objectives. The purpose of this study was to review the frequency and clinical presentation of the rarest clinical manifestations of systemic lupus erythematosus (SLE). Methods. A list of 6 rare SLE manifestations were defined: gastrointestinal, liver, pulmonary, cardiac, ocular and neurological manifestations. Each topic was assigned to a couple of authors to perform a literature search and article review. Results. In total, 149 articles were included in the literature review: 37 for gastrointestinal manifestations, 6 for liver manifestations, 27 for pulmonary manifestations, 50 for cardiac manifestations, 16 for ocular manifestations, 13 for neurological manifestations. Gastrointestinal disorders included several clinical presentations with variable frequency (from 0.5% to 10.7% of the cases); liver involvement included lupusrelated hepatitis (9.3%) and autoimmune hepatitis (2.3%). The rarest pulmonary manifestations identified were shrinking lung syndrome, described in 1.5% of patients, while interstitial lung disease and lupus pneumonia were reported in 4% and 3% of patients, respectively. Myocarditis and pulmonary hypertension were also rarely described in SLE patients although ranging from 0.4-16% and 1-14% respectively, depending on the methodology used for its identification. Ocular manifestations in SLE included some rare manifestations (reported in less than 5% of patients) and lupus retinopathy that is described in 1.2-28.8% of patients depending on methods of ascertainment. Aseptic meningitis and chorea were also confirmed as very rare manifestations being reported in less than 1% and in 0.3-2.4% of cases respectively. Conclusion. The results of this literature review provide the basis for a better understanding of some less-known manifestations of SLE and for stressing the need for a higher awareness in diagnostic and therapeutic protocols regarding these rare disease aspects. © Copyright Clinical and Experimental Rheumatology 2022.

Published
2022

37. 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

45. EFFECTIVENESS AND SAFETY OF TOCILIZUMAB IN PATIENTS WITH SYSTEMIC SCLEROSIS: A PROPENSITY SCORE CONTROL MATCHED OBSERVATIONAL STUDY OF THE EUSTAR COHORT

Author

Kuster S, Jordan S, Elhai M, Held U, Steigmiller K, Bruni C, Iannone F, Vettori S, Siegert E, Rednic S, Codullo V, Airo P, Braun-Moscovici Y, Hunzelmann N, Salvador M, Riccieri V, Gheorghiu A, Sancho J, Romanowska-Prochnicka K, Castellvi I, Koetter I, Truchetet M, Lopez-Longo F, Novikov P, Giollo A, Shirai Y, Belloli L, Zanatta E, Hachulla E, Smith V, Denton C, Ionescu R, Schmeiser T, Distler J, Gabrielli A, Hoffmann-Vold A, Kuwana M, Allanore Y, Distler O, and EUSTAR

Published
2021

46. EFFECTIVENESS AND SAFETY OF TOCILIZUMAB IN PATIENTS WITH SYSTEMIC SCLEROSIS: A PROPENSITY SCORE CONTROL MATCHED OBSERVATIONAL STUDY OF THE EUSTAR COHORT

Author

Kuster, S., Jordan, S., Elhai, M. D., Held, U., Steigmiller, K., Bruni, C., Iannone, F., Vettori, S., Siegert, E., Rednic, S., Codullo, V., Airo, P., Braun-Moscovici, Y., Hunzelmann, N., Salvador, M. J., Riccieri, V., Gheorghiu, A. M., Alegre Sancho, J. J., Romanowska-Prochnicka, K., Castellvi, I., Koetter, I., Truchetet, M. E., Lopez-Longo, F. J., Novikov, P., Giollo, A., Shirai, Y., Belloli, L., Zanatta, E., Hachulla, E., Smith, V., Denton, C., Ionescu, R., Schmeiser, T., Distler, J. H. W., Gabrielli, A., Hoffmann-Vold, A. M., Kuwana, M., Allanore, Y., Distler, O., Kuster, S., Jordan, S., Elhai, M. D., Held, U., Steigmiller, K., Bruni, C., Iannone, F., Vettori, S., Siegert, E., Rednic, S., Codullo, V., Airo, P., Braun-Moscovici, Y., Hunzelmann, N., Salvador, M. J., Riccieri, V., Gheorghiu, A. M., Alegre Sancho, J. J., Romanowska-Prochnicka, K., Castellvi, I., Koetter, I., Truchetet, M. E., Lopez-Longo, F. J., Novikov, P., Giollo, A., Shirai, Y., Belloli, L., Zanatta, E., Hachulla, E., Smith, V., Denton, C., Ionescu, R., Schmeiser, T., Distler, J. H. W., Gabrielli, A., Hoffmann-Vold, A. M., Kuwana, M., Allanore, Y., and Distler, O.

Published
2021

47. 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

49. POS0861 EFFECTIVENESS AND SAFETY OF TOCILIZUMAB IN PATIENTS WITH SYSTEMIC SCLEROSIS: A PROPENSITY SCORE CONTROL MATCHED OBSERVATIONAL STUDY OF THE EUSTAR COHORT

Author

Kuster, S., primary, Jordan, S., additional, Elhai, M. D., additional, Held, U., additional, Steigmiller, K., additional, Bruni, C., additional, Iannone, F., additional, Vettori, S., additional, Siegert, E., additional, Rednic, S., additional, Codullo, V., additional, Airò, P., additional, Braun-Moscovici, Y., additional, Hunzelmann, N., additional, Salvador, M. J., additional, Riccieri, V., additional, Gheorghiu, A. M., additional, Alegre Sancho, J. J., additional, Romanowska-Prochnicka, K., additional, Castellví, I., additional, Koetter, I., additional, Truchetet, M. E., additional, López-Longo, F. J., additional, Novikov, P., additional, Giollo, A., additional, Shirai, Y., additional, Belloli, L., additional, Zanatta, E., additional, Hachulla, E., additional, Smith, V., additional, Denton, C., additional, Ionescu, R., additional, Schmeiser, T., additional, Distler, J. H. W., additional, Gabrielli, A., additional, Hoffmann-Vold, A. M., additional, Kuwana, M., additional, Allanore, Y., additional, and Distler, O., additional

Published
2021
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