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1. A replication study confirms the association of TNFSF4 (OX40L) polymorphisms with Systemic Sclerosis in a large European cohort

Author

Airó P, Lie B A, Palm Ø, Nordin A, Padykov L, Claes K, Denton C, Fonseca C, Madhok R, Shiels P, Chee M M, Schuerwegh A J, Voskuyl A E, De Langhe E, Westhovens R, Smith V, De Keyser F, Houssiau F, Koeleman B P, Coenen MJH, Witte T, Riemekasten G, Hesselstrand R, González-Gay M A, Pros A, García de la Peña P, Follonosa V, Egurbide M V, Fernández-Nebro A, Gallego M, Castellví I, García-Hernández F J, Gómez-Gracia I, Román-Ivorra J A, Tolosa C, Rodríguez L, Vicente-Rabaneda E, González-Escribano M F, Navarrete N, Camps M T, Carreira P, Espinosa G, Ortego-Centeno N, Vonk M C, Beretta L, Simeon C P, Broen JCA, Bossini-Castillo L, Scorza R, van Laar J M, Hunzelmann N, Kreuter A, Herrick A, Worthington J, Radstake TRDJ, Martín J, and Rueda B

Subjects
Medicine
Published
2010
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3. Flemish network on rare connective tissue diseases (CTD):patient pathways in systemic sclerosis. First steps taken

Author

Piette, Y., Van den Bossche, F., Aerts, J., Aerts, N., Ajeganova, S., Badot, V., Berghen, N., Blockmans, D., Brusselle, G., Caeyers, N., De Decker, M., De Haes, P., De Cock, C., De Keyser, F., De Langhe, E., Delcroix, M., De Nutte, H., De Pauw, M., Depicker, A., De Sutter, A., De Sutter, J., Du Four, T., Frank, C., Goubau, J., Guiot, J., Gutermuth, J., Heeman, L., Houssiau, F., Hennes, I., Lenaerts, J., Lintermans, A., Loeys, B., Luyten, H., Maeyaert, B., Malfait, F., Moeyersoons, A., Mostmans, Y., Nijs, J., Poppe, B., Polfliet, K., Ruttens, D., Sabato, V., Schoeters, E., Slabbynck, H., Stuer, A., Tamirou, F., Thevissen, Kristof, Van Kersschaever, G., Vanneuville, B., Van Offel, J., Vanthuyne, M., Van Wabeke, J., Verbist, C., Vos, I., Westhovens, R., Wuyts, W., Yserbyt, J., Smith, V., Piette, Y., Van den Bossche, F., Aerts, J., Aerts, N., Ajeganova, S., Badot, V., Berghen, N., Blockmans, D., Brusselle, G., Caeyers, N., De Decker, M., De Haes, P., De Cock, C., De Keyser, F., De Langhe, E., Delcroix, M., De Nutte, H., De Pauw, M., Depicker, A., De Sutter, A., De Sutter, J., Du Four, T., Frank, C., Goubau, J., Guiot, J., Gutermuth, J., Heeman, L., Houssiau, F., Hennes, I., Lenaerts, J., Lintermans, A., Loeys, B., Luyten, H., Maeyaert, B., Malfait, F., Moeyersoons, A., Mostmans, Y., Nijs, J., Poppe, B., Polfliet, K., Ruttens, D., Sabato, V., Schoeters, E., Slabbynck, H., Stuer, A., Tamirou, F., Thevissen, Kristof, Van Kersschaever, G., Vanneuville, B., Van Offel, J., Vanthuyne, M., Van Wabeke, J., Verbist, C., Vos, I., Westhovens, R., Wuyts, W., Yserbyt, J., and Smith, V.

Abstract

Despite the low prevalence of each rare disease, the total burden is high. Patients with rare diseases encounter numerous barriers, including delayed diagnosis and limited access to high-quality treatments. In order to tackle these challenges, the European Commission launched the European Reference Networks (ERNs), cross-border networks of healthcare providers and patients representatives. In parallel, the aims and structure of these ERNs were translated at the federal and regional levels, resulting in the creation of the Flemish Network of Rare Diseases. In line with the mission of the ERNs and to ensure equal access to care, we describe as first patient pathways for systemic sclerosis (SSc), as a pilot model for other rare connective and musculoskeletal diseases. Consensus was reached on following key messages: 1. Patients with SSc should have multidisciplinary clinical and investigational evaluations in a tertiary reference expert centre at baseline, and subsequently every three to 5 years. Intermediately, a yearly clinical evaluation should be provided in the reference centre, whilst SSc technical evaluations are permissionably executed in a centre that follows SSc-specific clinical practice guidelines. In between, monitoring can take place in secondary care units, under the condition that qualitative examinations and care including interactive multidisciplinary consultations can be provided. 2. Patients with early diffuse cutaneous SSc, (progressive) interstitial lung disease and/or pulmonary arterial hypertension should undergo regular evaluations in specialised tertiary care reference institutions. 3. Monitoring of patients with progressive interstitial lung disease and/or pulmonary (arterial) hypertension will be done in agreement with experts of ERN LUNG.

Published
2024

4. Flemish network on rare connective tissue diseases (CTD): patient pathways in systemic sclerosis. First steps taken

Author

Piette, Y., primary, Van den Bossche, F., additional, Aerts, J., additional, Aerts, N., additional, Ajeganova, S., additional, Badot, V., additional, Berghen, N., additional, Blockmans, D., additional, Brusselle, G., additional, Caeyers, N., additional, De Decker, M., additional, De Haes, P., additional, De Cock, C., additional, De Keyser, F., additional, De Langhe, E., additional, Delcroix, M., additional, De Nutte, H., additional, De Pauw, M., additional, Depicker, A., additional, De Sutter, A., additional, De Sutter, J., additional, Du Four, T., additional, Frank, C., additional, Goubau, J., additional, Guiot, J., additional, Gutermuth, J., additional, Heeman, L., additional, Houssiau, F., additional, Hennes, I., additional, Lenaerts, J., additional, Lintermans, A., additional, Loeys, B., additional, Luyten, H., additional, Maeyaert, B., additional, Malfait, F., additional, Moeyersoons, A., additional, Mostmans, Y., additional, Nijs, J., additional, Poppe, B., additional, Polfliet, K., additional, Ruttens, D., additional, Sabato, V., additional, Schoeters, E., additional, Slabbynck, H., additional, Stuer, A., additional, Tamirou, F., additional, Thevissen, Kristof, additional, Van Kersschaever, G., additional, Vanneuville, B., additional, Van Offel, J., additional, Vanthuyne, M., additional, Van Wabeke, J., additional, Verbist, C., additional, Vos, I., additional, Westhovens, R., additional, Wuyts, W., additional, Yserbyt, J., additional, and Smith, V., additional

Published
2023
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5. 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

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

Author

Cavagna, L, Meloni, F, Meyer, A, Sambataro, G, Belliato, M, De Langhe, E, Cavazzana, I, Pipitone, N, Triantafyllias, K, Mosca, M, Barsotti, S, Zampogna, G, Biglia, A, Emmi, G, De Visser, M, Van Der Kooi, A, Parronchi, P, Hirschi, S, da Silva, J, Scire, C, Furini, F, Giannini, M, Martinez Gonzalez, O, Damian, L, Piette, Y, Smith, V, Mera-Valera, A, Bachiller-Corral, J, Cabezas Rodriguez, I, Brandy-Garcia, A, Maurier, F, Perrin, J, Gonzalez-Moreno, J, Drott, U, Delbruck, C, Schwarting, A, Arrigoni, E, Sebastiani, G, Iuliano, A, Nannini, C, Quartuccio, L, Rodriguez Cambron, A, Blazquez Canamero, M, Villa Blanco, I, Cagnotto, G, Pesci, A, Luppi, F, Dei, G, Romero Bueno, F, Franceschini, F, Chiapparoli, I, Zanframundo, G, Lettieri, S, De Stefano, L, Cutolo, M, Mathieu, A, Piga, M, Prieto-Gonzalez, S, Moraes-Fontes, M, Fonseca, J, Jovani, V, Riccieri, V, Santaniello, A, Montfort, S, Bilocca, D, Erre, G, Bartoloni, E, Gerli, R, Monti, M, Lorenz, H, Sambataro, D, Bellando Randone, S, Schneider, U, Valenzuela, C, Lopez-Mejias, R, Cifrian, J, Mejia, M, Gonzalez Perez, M, Wendel, S, Fornaro, M, De Luca, G, Orsolini, G, Rossini, M, Dieude, P, Knitza, J, Castaneda, S, Voll, R, Rojas-Serrano, J, Valentini, A, Vancheri, C, Matucci-Cerinic, M, Feist, E, Codullo, V, Iannone, F, Distler, J, Montecucco, C, Gonzalez-Gay, M, Cavagna L., Meloni F., Meyer A., Sambataro G., Belliato M., De Langhe E., Cavazzana I., Pipitone N., Triantafyllias K., Mosca M., Barsotti S., Zampogna G., Biglia A., Emmi G., De Visser M., Van Der Kooi A., Parronchi P., Hirschi S., da Silva J. A. P., Scire C. A., Furini F., Giannini M., Martinez Gonzalez O., Damian L., Piette Y., Smith V., Mera-Valera A., Bachiller-Corral J., Cabezas Rodriguez I., Brandy-Garcia A. M., Maurier F., Perrin J., Gonzalez-Moreno J., Drott U., Delbruck C., Schwarting A., Arrigoni E., Sebastiani G. D., Iuliano A., Nannini C., Quartuccio L., Rodriguez Cambron A. B., Blazquez Canamero M. A., Villa Blanco I., Cagnotto G., Pesci A., Luppi F., Dei G., Romero Bueno F. I., Franceschini F., Chiapparoli I., Zanframundo G., Lettieri S., De Stefano L., Cutolo M., Mathieu A., Piga M., Prieto-Gonzalez S., Moraes-Fontes M. F., Fonseca J. E., Jovani V., Riccieri V., Santaniello A., Montfort S., Bilocca D., Erre G. L., Bartoloni E., Gerli R., Monti M. C., Lorenz H. M., Sambataro D., Bellando Randone S., Schneider U., Valenzuela C., Lopez-Mejias R., Cifrian J., Mejia M., Gonzalez Perez M. -I., Wendel S., Fornaro M., De Luca G., Orsolini G., Rossini M., Dieude P., Knitza J., Castaneda S., Voll R. E., Rojas-Serrano J., Valentini A., Vancheri C., Matucci-Cerinic M., Feist E., Codullo V., Iannone F., Distler J. H., Montecucco C., Gonzalez-Gay M. A., Cavagna, L, Meloni, F, Meyer, A, Sambataro, G, Belliato, M, De Langhe, E, Cavazzana, I, Pipitone, N, Triantafyllias, K, Mosca, M, Barsotti, S, Zampogna, G, Biglia, A, Emmi, G, De Visser, M, Van Der Kooi, A, Parronchi, P, Hirschi, S, da Silva, J, Scire, C, Furini, F, Giannini, M, Martinez Gonzalez, O, Damian, L, Piette, Y, Smith, V, Mera-Valera, A, Bachiller-Corral, J, Cabezas Rodriguez, I, Brandy-Garcia, A, Maurier, F, Perrin, J, Gonzalez-Moreno, J, Drott, U, Delbruck, C, Schwarting, A, Arrigoni, E, Sebastiani, G, Iuliano, A, Nannini, C, Quartuccio, L, Rodriguez Cambron, A, Blazquez Canamero, M, Villa Blanco, I, Cagnotto, G, Pesci, A, Luppi, F, Dei, G, Romero Bueno, F, Franceschini, F, Chiapparoli, I, Zanframundo, G, Lettieri, S, De Stefano, L, Cutolo, M, Mathieu, A, Piga, M, Prieto-Gonzalez, S, Moraes-Fontes, M, Fonseca, J, Jovani, V, Riccieri, V, Santaniello, A, Montfort, S, Bilocca, D, Erre, G, Bartoloni, E, Gerli, R, Monti, M, Lorenz, H, Sambataro, D, Bellando Randone, S, Schneider, U, Valenzuela, C, Lopez-Mejias, R, Cifrian, J, Mejia, M, Gonzalez Perez, M, Wendel, S, Fornaro, M, De Luca, G, Orsolini, G, Rossini, M, Dieude, P, Knitza, J, Castaneda, S, Voll, R, Rojas-Serrano, J, Valentini, A, Vancheri, C, Matucci-Cerinic, M, Feist, E, Codullo, V, Iannone, F, Distler, J, Montecucco, C, Gonzalez-Gay, M, Cavagna L., Meloni F., Meyer A., Sambataro G., Belliato M., De Langhe E., Cavazzana I., Pipitone N., Triantafyllias K., Mosca M., Barsotti S., Zampogna G., Biglia A., Emmi G., De Visser M., Van Der Kooi A., Parronchi P., Hirschi S., da Silva J. A. P., Scire C. A., Furini F., Giannini M., Martinez Gonzalez O., Damian L., Piette Y., Smith V., Mera-Valera A., Bachiller-Corral J., Cabezas Rodriguez I., Brandy-Garcia A. M., Maurier F., Perrin J., Gonzalez-Moreno J., Drott U., Delbruck C., Schwarting A., Arrigoni E., Sebastiani G. D., Iuliano A., Nannini C., Quartuccio L., Rodriguez Cambron A. B., Blazquez Canamero M. A., Villa Blanco I., Cagnotto G., Pesci A., Luppi F., Dei G., Romero Bueno F. I., Franceschini F., Chiapparoli I., Zanframundo G., Lettieri S., De Stefano L., Cutolo M., Mathieu A., Piga M., Prieto-Gonzalez S., Moraes-Fontes M. F., Fonseca J. E., Jovani V., Riccieri V., Santaniello A., Montfort S., Bilocca D., Erre G. L., Bartoloni E., Gerli R., Monti M. C., Lorenz H. M., Sambataro D., Bellando Randone S., Schneider U., Valenzuela C., Lopez-Mejias R., Cifrian J., Mejia M., Gonzalez Perez M. -I., Wendel S., Fornaro M., De Luca G., Orsolini G., Rossini M., Dieude P., Knitza J., Castaneda S., Voll R. E., Rojas-Serrano J., Valentini A., Vancheri C., Matucci-Cerinic M., Feist E., Codullo V., Iannone F., Distler J. H., Montecucco C., and Gonzalez-Gay M. A.

Abstract

Objective To define the clinical spectrum time-course and prognosis of non-Asian patients positive for anti-MDA5 antibodies. Methods We conducted a multicentre, international, retrospective cohort study. Results 149 anti-MDA5 positive patients (median onset age 53 years, median disease duration 18 months), mainly females (100, 67%), were included. Dermatomyositis (64, 43%) and amyopathic dermatomyositis (47, 31%), were the main diagnosis; 15 patients (10%) were classified as interstitial pneumonia with autoimmune features (IPAF) and 7 (5%) as rheumatoid arthritis. The main clinical findings observed were myositis (84, 56%), interstitial lung disease (ILD) (108, 78%), skin lesions (111, 74%), and arthritis (76, 51%). The onset of these manifestations was not concomitant in 74 cases (50%). Of note, 32 (21.5%) patients were admitted to the intensive care unit for rapidly progressive-ILD, which occurred in median 2 months from lung involvement detection, in the majority of cases (28, 19%) despite previous immunosuppressive treatment. One-third of patients (47, 32% each) was ANA and anti-ENA antibodies negative and a similar percentage was anti-Ro52 kDa antibodies positive. Non-specific interstitial pneumonia (65, 60%), organising pneumonia (23, 21%), and usual interstitial pneumonia-like pattern (14, 13%) were the main ILD patterns observed. Twenty-six patients died (17%), 19 (13%) had a rapidly progressive-ILD. Conclusion The clinical spectrum of the anti-MDA5 antibodies-related disease is heterogeneous. Rapidly-progressive ILD deeply impacts the prognosis also in non-Asian patients, occurring early during the disease course. Anti-MDA5 antibody positivity should be considered even when baseline autoimmune screening is negative, anti-Ro52 kDa antibodies are positive, and radiology findings show a NSIP pattern.

Published
2022

10. 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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11. Correspondence Between Audible Crackles and Lung Function Test Results

Author

Gogaert, S., primary, Goos, T., additional, Vermant, M., additional, Carlon, M.S., additional, De Crem, N., additional, De Sadeleer, L., additional, De Langhe, E., additional, Dubbeldam, A., additional, Vanaudenaerde, B., additional, Verschakelen, J., additional, Weynand, B., additional, Yserbyt, J., additional, Bertrand, A., additional, De Vos, M., additional, and Wuyts, W.A., additional

Published
2023
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12. Current Definitions of Progressive Pulmonary Fibrosis Might Be Inaccurate: Data From a Cohort of Patients With Rheumatoid Arthritis Associated Interstitial Lung Disease

Author

Vermant, M., primary, Goos, T., additional, Gogaert, S., additional, Carlon, M.S., additional, De Crem, N., additional, De Sadeleer, L., additional, De Langhe, E., additional, Dubbeldam, A., additional, Vanaudenaerde, B., additional, Verbeken, E., additional, Verschakelen, J., additional, Weynand, B., additional, Yserbyt, J., additional, Westhovens, R., additional, Verschueren, P., additional, and Wuyts, W.A., additional

Published
2023
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13. Intrafamilial Correlation and Variability in the Clinical Evolution of Pulmonary Fibrosis

Author

Goos, T., primary, Dubbeldam, A., additional, Vermant, M., additional, Gogaert, S., additional, De Sadeleer, L.J., additional, De Crem, N., additional, De Langhe, E., additional, Yserbyt, J., additional, Weynand, B., additional, Carlon, M.S., additional, Verschakelen, J., additional, Vermeer, S., additional, Verleden, S.E., additional, and Wuyts, W.A., additional

Published
2023
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14. E-book: Reumatologie – Uitgave 2023

Author

Carron, P., primary, De Craemer, A.-S., additional, Van den Bosch, F., additional, Vulsteke, J.-B., additional, Bossuyt, X., additional, De Langhe, E., additional, Willers, N., additional, Berteloot, P., additional, Wittevronghel, I., additional, Jacomen, G., additional, Schelfhout, V., additional, Vanwambeke, K., additional, Desmedt, S., additional, Desmedt, V., additional, Billiet, T., additional, George, C., additional, Meersseman, W., additional, D'Heygere, F., additional, De Bondt, E., additional, Betrains, A., additional, Vanderschueren, S., additional, Hanssens, J., additional, Werbrouck, B., additional, Terryn, W., additional, Deconinck, B., additional, Nollet, A., additional, Cokelaere, K., additional, Timmermans, K., additional, Nachtergaele, M., additional, Vanfraechem, C., additional, Bogaert, A.-M., additional, Hublou, W., additional, Gijsen, M., additional, Declercq, P., additional, Spriet, I., additional, Van der Linden, L., additional, and Quintens, C., additional

Published
2023
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15. Family history of ILD predicts pulmonary function decline in IPF patients

Author

Goos, T, primary, Vermant, M, additional, De Sadeleer, L J, additional, Verstraete, K, additional, De Crem, N, additional, De Langhe, E, additional, Dubbeldam, A, additional, Verbeken, E K, additional, Verschakelen, J, additional, Weynand, B, additional, Yserbyt, J, additional, Vermeer, S, additional, Verleden, S E, additional, and Wuyts, W A, additional

Published
2022
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16. Evolution of diffusion capacity in interstitial lung disease associated with rheumatoid arthritis

Author

Vermant, M, primary, Goos, T, additional, Bode, H, additional, De Sadeleer, L J, additional, Verstraete, K, additional, De Crem, N, additional, De Langhe, E, additional, Dubbeldam, A, additional, Verbeken, E, additional, Verschakelen, J, additional, Weynand, B, additional, Yserbyt, J, additional, Westhovens, R, additional, Verschueren, P, additional, and Wuyts, W A, additional

Published
2022
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20. Myositis-specific and myositis-associated autoantibodies predict trajectories of damage over time in idiopathic inflammatory myopathies

Author

Espinosa-Ortega, F., Holmqvist, M., Lodin, K., Dastmalchi, M., Danieli, M. G., Venkovsky, J., Knitza, J., Oliveira, S., Diederichsen, L. P., Wedderburn, L., Andersson, H., del Mercado, M. Vazquez, Gomez-Martin, D., Torres, J., Callaghan, A. Selva O., Benveniste, O., De Visser, M., De Bleecker, J., McCann, L., De Langhe, E., Rietveld, A., Chinoy, H., Krogh, N. S., Alexanderson, H., Lundberg, I. E., and Grp, Euro-myositis

Published
2021

24. A new molecular classification to drive precision treatment strategies in primary Sjögren's syndrome

Author

Agence Nationale de la Recherche (France), European Commission, Soret, P., Le Dantec, C., Desvaux, E., Foulquier, N., Chassagnol, B., Hubert, S., Jamin, C., Barturen, Guillermo, Desachy, G., Devauchelle-Pensec, V., Boudjeniba, C., Cornec, D., Saraux, A., Jousse-Joulin, S., Barbarroja, N., Rodríguez-Pintó, I., De Langhe, E., Beretta, L., Chizzolini, C., Kovács, L., Witte, T., PRECISESADS Clinical Consortium, PRECISESADS Flow Cytometry Consortium, Bettacchioli, E., Buttgereit, A., Makowska, Z., Lesche, R., Borghi, M. O., Martín, J., Courtade-Gaiani, S, Xuereb, L., Guedj, M, Moingeon, P., Alarcón-Riquelme, M. E., Laigle, L., Pers, Jacques-Olivier, Agence Nationale de la Recherche (France), European Commission, Soret, P., Le Dantec, C., Desvaux, E., Foulquier, N., Chassagnol, B., Hubert, S., Jamin, C., Barturen, Guillermo, Desachy, G., Devauchelle-Pensec, V., Boudjeniba, C., Cornec, D., Saraux, A., Jousse-Joulin, S., Barbarroja, N., Rodríguez-Pintó, I., De Langhe, E., Beretta, L., Chizzolini, C., Kovács, L., Witte, T., PRECISESADS Clinical Consortium, PRECISESADS Flow Cytometry Consortium, Bettacchioli, E., Buttgereit, A., Makowska, Z., Lesche, R., Borghi, M. O., Martín, J., Courtade-Gaiani, S, Xuereb, L., Guedj, M, Moingeon, P., Alarcón-Riquelme, M. E., Laigle, L., and Pers, Jacques-Olivier

Abstract

There is currently no approved treatment for primary Sjögren’s syndrome, a disease thatprimarily affects adult women. The difficulty in developing effective therapies is -in part-because of the heterogeneity in the clinical manifestation and pathophysiology of the disease.Finding common molecular signatures among patient subgroups could improve our under-standing of disease etiology, and facilitate the development of targeted therapeutics. Here,we report, in a cross-sectional cohort, a molecular classification scheme for Sjögren’s syn-drome patients based on the multi-omic profiling of whole blood samples from a Europeancohort of over 300 patients, and a similar number of age and gender-matched healthyvolunteers. Using transcriptomic, genomic, epigenetic, cytokine expression andflow cyto-metry data, combined with clinical parameters, we identify four groups of patients withdistinct patterns of immune dysregulation. The biomarkers we identify can be used bymachine learning classifiers to sort future patients into subgroups, allowing the re-evaluationof response to treatments in clinical trials.

Published
2021

25. The autoimmune disease-associated IL2RA locus is involved in the clinical manifestations of systemic sclerosis

Author

Martin, J-E, Carmona, F D, Broen, J C A, Simeón, C P, Vonk, M C, Carreira, P, Ríos-Fernández, R, Espinosa, G, Vicente-Rabaneda, E, Tolosa, C, García-Hernández, F J, Castellví, I, Fonollosa, V, González-Gay, M A, Sáez-Comet, L, Portales, R García, de la Peña, P García, Fernández-Castro, M, Díaz, B, Martínez-Estupiñán, L, Coenen, M, Voskuyl, A E, Schuerwegh, A J, Vanthuyne, M, Houssiau, F, Smith, V, de Keyser, F, De Langhe, E, Riemekasten, G, Witte, T, Hunzelmann, N, Kreuter, A, Palm, Ø, Chee, M M, van Laar, J M, Denton, C, Herrick, A, Worthington, J, Koeleman, B P C, Radstake, T R D J, Fonseca, C, and Martín, J

Published
2012
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28. Riociguat in patients with early diffuse cutaneous systemic sclerosis (RISE-SSc): randomised, double-blind, placebo-controlled multicentre trial.

Author

Khanna, D, Allanore, Y, Denton, CP, Kuwana, M, Matucci-Cerinic, M, Pope, JE, Atsumi, T, Bečvář, R, Czirják, L, Hachulla, E, Ishii, T, Ishikawa, O, Johnson, SR, De Langhe, E, Stagnaro, C, Riccieri, V, Schiopu, E, Silver, RM, Smith, V, Steen, V, Stevens, W, Szücs, G, Truchetet, M-E, Wosnitza, M, Laapas, K, de Oliveira Pena, J, Yao, Z, Kramer, F, Distler, O, Khanna, D, Allanore, Y, Denton, CP, Kuwana, M, Matucci-Cerinic, M, Pope, JE, Atsumi, T, Bečvář, R, Czirják, L, Hachulla, E, Ishii, T, Ishikawa, O, Johnson, SR, De Langhe, E, Stagnaro, C, Riccieri, V, Schiopu, E, Silver, RM, Smith, V, Steen, V, Stevens, W, Szücs, G, Truchetet, M-E, Wosnitza, M, Laapas, K, de Oliveira Pena, J, Yao, Z, Kramer, F, and Distler, O

Abstract

OBJECTIVES: Riociguat is approved for pulmonary arterial hypertension and has antiproliferative, anti-inflammatory and antifibrotic effects in animal models of tissue fibrosis. We evaluated the efficacy and safety of riociguat in patients with early diffuse cutaneous systemic sclerosis (dcSSc) at high risk of skin fibrosis progression. METHODS: In this randomised, double-blind, placebo-controlled, phase IIb trial, adults with dcSSc of <18 months' duration and a modified Rodnan skin score (mRSS) 10-22 units received riociguat 0.5 mg to 2.5 mg orally three times daily (n=60) or placebo (n=61). The primary endpoint was change in mRSS from baseline to week 52. RESULTS: At week 52, change from baseline in mRSS units was -2.09±5.66 (n=57) with riociguat and -0.77±8.24 (n=52) with placebo (difference of least squares means -2.34 (95% CI -4.99 to 0.30; p=0.08)). In patients with interstitial lung disease, forced vital capacity declined by 2.7% with riociguat and 7.6% with placebo. At week 14, average Raynaud's condition score had improved ≥50% in 19 (41.3%)/46 patients with riociguat and 13 (26.0%)/50 patients with placebo. Safety assessments showed no new signals with riociguat and no treatment-related deaths. CONCLUSIONS: Riociguat did not significantly benefit mRSS versus placebo at the predefined p<0.05. Secondary and exploratory analyses showed potential efficacy signals that should be tested in further trials. Riociguat was well tolerated.

Published
2020

29. Genome-wide whole blood transcriptome profiling in a large European cohort of systemic sclerosis patients

Author

European Commission, Beretta, L., Barturen, Guillermo, Vigone, B, Bellocchi, C., Hunzelmann, Nicolas, De Langhe, E., Cervera, R., Gerosa, M., Kovács, L., Ortega Castro, R., Almeida, I., Cornec, D., Chizzolini, C., Pers, J.O., Makowska, Z., Lesche, R., Kerick, Martin, Alarcón-Riquelme, M. E., Martín, J., European Commission, Beretta, L., Barturen, Guillermo, Vigone, B, Bellocchi, C., Hunzelmann, Nicolas, De Langhe, E., Cervera, R., Gerosa, M., Kovács, L., Ortega Castro, R., Almeida, I., Cornec, D., Chizzolini, C., Pers, J.O., Makowska, Z., Lesche, R., Kerick, Martin, Alarcón-Riquelme, M. E., and Martín, J.

Abstract

Objectives: The analysis of annotated transcripts from genome-wide expression studies may help to understand the pathogenesis of complex diseases, such as systemic sclerosis (SSc). We performed a whole blood (WB) transcriptome analysis on RNA collected in the context of the European PRECISESADS project, aiming at characterising the pathways that differentiate SSc from controls and that are reproducible in geographically diverse populations. Methods: Samples from 162 patients and 252 controls were collected in RNA stabilisers. Cases and controls were divided into a discovery (n=79+163; Southern Europe) and validation cohort (n=83+89; Central-Western Europe). RNA sequencing was performed by an Illumina assay. Functional annotations of Reactome pathways were performed with the Functional Analysis of Individual Microarray Expression (FAIME) algorithm. In parallel, immunophenotyping of 28 circulating cell populations was performed. We tested the presence of differentially expressed genes/pathways and the correlation between absolute cell counts and RNA transcripts/FAIME scores in regression models. Results significant in both populations were considered as replicated. Results: Overall, 15 224 genes and 1277 functional pathways were available; of these, 99 and 225 were significant in both sets. Among replicated pathways, we found a deregulation in type-I interferon, Toll-like receptor cascade, tumour suppressor p53 protein function, platelet degranulation and activation. RNA transcripts or FAIME scores were jointly correlated with cell subtypes with strong geographical differences; neutrophils were the major determinant of gene expression in SSc-WB samples. Conclusions: We discovered a set of differentially expressed genes/pathways validated in two independent sets of patients with SSc, highlighting a number of deregulated processes that have relevance for the pathogenesis of autoimmunity and SSc.

Published
2020

35. OP0249 LONG-TERM EXTENSION RESULTS OF RISE-SSC, A RANDOMIZED TRIAL OF RIOCIGUAT IN PATIENTS WITH EARLY DIFFUSE CUTANEOUS SYSTEMIC SCLEROSIS (DCSSC)

Author

Khanna, D., primary, Pope, J., additional, Matucci-Cerinic, M., additional, Kuwana, M., additional, Denton, C., additional, Allanore, Y., additional, Wosnitza, M., additional, Truchetet, M. E., additional, Szücs, G., additional, Stevens, W., additional, Steen, V., additional, Stagnaro, C., additional, Smith, V., additional, Silver, R., additional, Schiopu, E., additional, Riccieri, V., additional, Kramer, F., additional, Johnson, S., additional, Ishikawa, O., additional, Ishii, T., additional, Hachlla, E., additional, De Langhe, E., additional, Czirják, L., additional, Bečvář, R., additional, Atsumi, T., additional, and Distler, O., additional

Published
2020
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36. FRI0575 BIOMARKER ANALYSIS FROM THE RISE-SSC STUDY OF RIOCIGUAT IN EARLY DIFFUSE CUTANEOUS SYSTEMIC SCLEROSIS (DCSSC)

Author

Distler, O., primary, Kramer, F., additional, Höfler, J., additional, Ghadessi, M., additional, Sandner, P., additional, Allanore, Y., additional, Denton, C., additional, Kuwana, M., additional, Matucci-Cerinic, M., additional, Pope, J., additional, Atsumi, T., additional, Bečvář, R., additional, Czirják, L., additional, De Langhe, E., additional, Hachlla, E., additional, Ishii, T., additional, Ishikawa, O., additional, Johnson, S., additional, Laapas, K., additional, Riccieri, V., additional, Schiopu, E., additional, Silver, R., additional, Smith, V., additional, Stagnaro, C., additional, Steen, V., additional, Stevens, W., additional, Szücs, G., additional, Truchetet, M. E., additional, Wosnitza, M., additional, and Khanna, D., additional

Published
2020
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37. THU0367 INCIDENCE AND PREVALENCE OF SYSTEMIC SCLEROSIS-ASSOCIATED INTERSTITIAL LUNG DISEASE IN FLANDERS: A 12-YEARS COLLABORATIVE MULTICENTER PROSPECTIVE COHORT STUDY.

Author

Vandecasteele, E., primary, Melsens, K., additional, Blockmans, D., additional, Carton, C., additional, De Keyser, F., additional, De Langhe, E., additional, Lauwerys, B., additional, Piette, Y., additional, Vanhaecke, A., additional, Verbeke, K., additional, Wuyts, W., additional, Brusselle, G., additional, and Smith, V., additional

Published
2020
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38. The effect of highly ionising particles on the CMS silicon strip tracker

Author

Adam, W., Bergauer, T., Friedl, M., Fruehwirth, R., Hrubec, J., Krammer, M., Pernicka, M., Waltenberger, W., Beaumont, W., de Langhe, E., de Wolf, E., Tasevsky, M., Bouhali, O., Clerbaux, B., de Lentdecker, G., Dewulf, J.-P., Neuckermans, L., Vander-Velde, C., Vanlaer, P., Wickens, J., D’Hondt, J., Goorens, R., Heyninck, J., Lowette, S., Tavernier, S., Van Lancker, L., Yu, C., Assouak, S., Bonnet, J.-L., Bruno, G., De-Callatay, B., De-Favereau-de-Jeneret, J., Delaere, C., De-Visscher, S., Favart, D., Gregoire, G., Keutgen, Th., Leibenguth, G., Lemaitre, V., Michotte, D., Militaru, O., Ninane, A., Ovyn, S., Piotrzkowski, K., Roberfroid, V., Rouby, X., Van-der-Aa, O., Vander-Donckt, M., Boulogne, I., Daubie, E., Defontaines, F., Herquet, P., Czellar, S., Härkönen, J., Karimäki, V., Katajisto, H., Linden, T., Luukka, P., Lampen, T., Mäenpää, T., Tuominen, E., Tuominiemi, J., Tuuva, T., Ageron, M., Chabanat, E., Contardo, D., Estre, N., Haroutunian, R., Lumb, N., Mirabito, L., Perries, S., Trocme, B., Blaes, R., Charles, F., Drouhin, F., Ernenwein, J.P., Fontaine, J.C., Berst, J.D., Brom, J.M., Didierjean, F., Goerlach, U., Gross, L., Juillot, P., Lounis, A., Maazouzi, C., Olivetto, C., Strub, R., Vanhove, P., Vintache, D., Adolphi, R., Brauer, R., Braunschweig, W., Esser, H., Feld, L., Heister, A., Karpinski, W., Klein, K., König, S., Kukulies, C., Olzem, J., Ostaptchouk, A., Pandoulas, D., Pierschel, G., Raupach, F., Schael, S., Schultz von Dratzig, A., Schwering, G., Siedling, R., Thomas, M., Wlochal, M., Beissel, F., Boffin, K.-D., Duda, M., Flossdorf, A., Flugge, G., Franke, T., Hangarter, K., Hegner, B., Hermanns, Th., Kasselmann, S., Kress, Th., Linn, A., Mnich, J., Nowack, A., Poettgens, M., Pooth, O., Reinhold, B., Bleyl, M., Holm, U., Klanner, R., Pein, U., Schleper, P., Schirm, N., Steinbrück, G., Stoye, M., Tesch, S., van Staa, R., Wick, K., Atz, B., Barvich, T., Blum, P., de Boer, W., Boegelspacher, F., Dirkes, G., Fahrer, M., Fernandez, J., Frey, M., Furgeri, A., Grigoriev, E., Hartmann, F., Heier, S., Muller, T., Ortega-Gomez, T., Simonis, H.-J., Steck, P., Theel, A., Weiler, T., Zhukov, V., Creanza, D., De Filippis, N., de Palma, M., De Robertis, G., Fiore, L., Giordano, D., Maggi, G., Mennea, M., My, S., Radicci, V., Selvaggi, G., Silvestris, L., Tempesta, P., Zito, G., Albergo, S., Bellini, V., Chiorboli, M., Costa, S., Potenza, R., Sutera, C., Tricomi, A., Tuvè, C., Bocci, A., Ciulli, V., Civinini, C., D’Alessandro, R., Focardi, E., Landi, G., Macchiolo, A., Magini, N., Mersi, S., Marchettini, C., Meschini, M., Paoletti, S., Parrini, G., Ranieri, R., Sani, M., Bacchetta, N., Bisello, D., Candelori, A., Dorigo, T., Giubilato, P., Kaminsky, A., Loreti, M., Nigro, M., Paccagnella, A., Rando, R., Angarano, M.M., Babucci, E., Benedetti, D., Biasini, M., Bilei, G.M., Brunetti, M.T., Checcucci, B., Dinu, N., Fanò, L., Giorgi, M., Lariccia, P., Mantovani, G., Postolache, V., Puscalau, M., Ricci, D., Santinelli, R., Santocchia, A., Servoli, L., Zucchetti, C., Azzurri, P., Bagliesi, G., Basti, A., Bernardini, J., Boccali, T., Borrello, L., Bosi, F., Castaldi, R., D’Alfonso, M., Dell’Orso, R., Dutta, S., Foa, L., Gennai, S., Giammanco, A., Giassi, A., Lomtadze, T., Mangano, B., Messineo, A., Moggi, A., Palla, F., Palmonari, F., Raffaelli, F., Rizzi, A., Rizzi, D., Segneri, G., Sentenac, D., Sguazzoni, G., Spagnolo, P., Tonelli, G., Verdini, P.G., Biino, C., Costa, M., Demaria, N., Favro, G., Trapani, P., Peroni, C., Romero, A., Migliore, E., Abbaneo, D., Ahmed, F., Bartalini, P., Bernardino-Rodriguez, N., Breuker, H., Buchmuller, O., Carrone, E., Cattai, A., Chierici, R., Cucciarelli, S., Dierlamm, A., Eppard, M., Frey, A., Gill, K., Grabit, R., Honma, A., Huhtinen, M., Magazzu, G., Mannelli, M., Marchioro, A., Onnela, A., Perea-Solano, B., Petagna, P., Postema, H., Risoldi, M., Rolandi, G., Siegrist, P., Troska, I., Tsirou, A., Vasey, F., Weber, M., Wittmer, B., Bertl, W., Gabathuler, K., Horisberger, R., Kästli, H.-Ch., Kotlinski, D., MacPherson, A., Rohe, T., Freudenreich, K., Lustermann, W., Pauss, F., Eichler, R., Erdmann, W., Grab, C., Schoning, A., Amsler, C., Chiochia, V., Dorokhov, A., Hörmann, C., Pruys, H., Prokofiev, K., Regenfus, C., Robmann, P., Speer, T., Bell, K.W., Coughlan, J., French, M., Halsall, R., Jones, L., Pearson, M., Rogers, G., Tomalin, I., Bainbridge, R., Barrillon, P., Colling, D., Dris, S., Foudas, C., Fulcher, J., Hall, G., Iles, G., Jones, J., Leaver, J., Macevoy, B.C., Noy, M., Raymond, D.M., Takahashi, M., Zorba, O., Barnett, B., Chien, C.-Y., Kim, D.W., Liang, G., Swartz, M., Atac, M., Demarteau, M., Joshi, U., Kwan, S., Spiegel, L., Tkaczyk, S., Gerber, C.E., Shabalina, E., Ten, T., Lander, R., Pellett, D., Gobbi, B., Kubantsev, M., Malik, S., Tilden, R., Baringer, P., Bean, A., Christofek, L., Coppage, D., Bolton, T.A., Demina, R., Kahl, W.E., Khanov, A., Korjenevski, S., Pukhaeva, N., Reay, N.W., Rizatdinova, F., Sidwell, R.A., Stanton, N.R., Cremaldi, L., Sanders, D., Bartz, E., Doroshenko, J., Koeth, T., Perera, L., Schnetzer, S., Stone, R., Worm, S., Gartung, P., Hanson, G.G., Jeng, G.Y., Páztor, G., Eusebi, R., Halkiadakis, E., Hocker, A., Tipton, P., Affolder, A., Campagnari, C., Hale, D., Incandela, J., Kyre, S., Lamb, J., Taylor, R., White, D., Bolla, G., Bortoletto, D., Garfinkel, A., Rott, C., and Roy, A.

Published
2005
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39. Incidence and risk factors for gangrene in patients with systemic sclerosis from the EUSTAR cohort

Author

Mihai, Carina, Distler, Oliver, Gheorghiu, Ana Maria, Constantin, Paul I, Dobrota, Rucsandra, Jordan, Suzana, Smith, Vanessa, Hachulla, Eric, Henes, Jörg, Siegert, Elise, Vettori, Serena, Müller-Ladner, Ulf, Matucci Cerinic, Marco, Allanore, Yannick, Lepri, G, Jaeger, Vk, Walker, Ua, Iannone, F, Cacciapaglia, F, Tomčík, M, Becvar, R, Rednic, S, Petcu, A, Szabo, I, Codullo, V, Caporali, R, Montecucco, C, Carreira, P, Ioven, B, Minier, T, Czirják, L, Chizzolini, C, Allali, D, Zanatta, E, Doria, A, Gabrielli, A, Airò, P, Lazzaroni, Mg, Radić, M, Martinovic, D, Braun-Moscovici, Y, Balbir-Gurman, A, Hunzelmann, N, Caramaschi, P, Morovic-Vergles, J, Denton, C, Santamaria, V, Heitmann, S, Krasowska, D, Michalska-Jakubus, M, Seidel, M, Foeldvari, I, Helmus, N, Salvador, M, Stamenkovic, B, Stankovic, A, Ananieva, L, Herrick, A, Engelhart, M, De La Puente, C, Hoffmann-Vold, Am, Midtvedt, Ø, Launay, D, Sobanski, V, Riccieri, V, Opris-Belinski, D, Groseanu, L, Ionescu, R, Bojinca, M, Sunderkötter, C, Distler, J, Ingegnoli, F, van der Haecke, A, Ullman, S, Pozzi, Mr, Eyerich, K, Vanthuyne, M, Erler, A, Aringer, M, De Langhe, E, Baresic, M, Mayer, M, Anic, B, Yavuz, S, Granel, B, Popa, S, Agachi, S, Zenone, T, Mathieu, A, Vacca, A, Solanki, K, Veale, D, Loyo, E, Tineo, C, Gigante, A, Rosato, E, Oksel, F, Yagurcu, F, Tănăseanu, Cm, Visalli, E, Benenati, A, Foti, R, Ancuta, C, Dan, D, Adler, S, Villiger, P, Fathi, N, de la Peña Lefebvre PG, González Martín, J, Chatelus, E, Sibilia, J, Litinsky, I, Del Galdo, F, Ann Sakettkoo, L, Kerzberg, E, Bianchi, Wa, Bianchi, Bv, Castellví, I, Limonta, M, Rimar, D, Couto, M, Ribi, C, Spertini, F, Kahl, S, Hsu, V, Poindron, V, Meghit, K, Martin, T, Kolstad, K, Chung, L, Thiele, A, Schmeiser, T, Zdrojewski, Z, Riemekasten, G, Levy, Y, Cardoneanu, A, Burlui, A, Rezus, E, Pamuk, On, Talotta, R, Bongiovanni, S, Puttini, Ps., Mihai, Carina, Distler, Oliver, Gheorghiu, Ana Maria, Constantin, Paul I, Dobrota, Rucsandra, Jordan, Suzana, Smith, Vanessa, Hachulla, Eric, Henes, Jörg, Siegert, Elise, Vettori, Serena, Müller-Ladner, Ulf, Matucci Cerinic, Marco, Allanore, Yannick, Giovanna, Cuomo, Chizzolini, Carlo, Allali, Danièle, and University of Zurich

Subjects
Adult, Male, medicine.medical_specialty, Databases, Factual, systemic sclerosis, digital ulcer, 610 Medicine & health, Disease, ddc:616.07, Logistic regression, Systemic scleroderma, Cohort Studies, Rheumatology, Risk Factors, Internal medicine, Cox proportional hazards regression, medicine, Humans, Pharmacology (medical), In patient, digital ulcers, gangrene, vasculopathy, Aged, Gangrene, Scleroderma, Systemic, business.industry, Incidence (epidemiology), Incidence, 10051 Rheumatology Clinic and Institute of Physical Medicine, food and beverages, Middle Aged, medicine.disease, Cross-Sectional Studies, Cohort, Female, business, systemic sclerosi
Abstract

Objective In patients with SSc, peripheral vasculopathy can promote critical ischaemia and gangrene. The aim of this study was to investigate the prevalence, incidence and risk factors for gangrene in the EUSTAR cohort. Methods We included patients from the EUSTAR database fulfilling the ACR 1980 or the ACR/EULAR 2013 classification criteria for SSc, with at least one visit recording data on gangrene. Centres were asked for supplementary data on traditional cardiovascular risk factors. We analysed the cross-sectional relationship between gangrene and its potential risk factors by univariable and multivariable logistic regression. Longitudinal data were analysed by Cox proportional hazards regression. Results 1757 patients were analysed (age 55.9 [14.5] years, disease duration 7.9 [10.3] years, male sex 16.7%, 24.6% diffuse cutaneous subset [dcSSc]). At inclusion, 8.9% of patients had current or previous digital gangrene, 16.1% had current digital ulcers (DUs) and 42.7% had ever had DUs (current or previous). Older age, DUs ever and dcSSc were statistically significant risk factors for gangrene in the cross-sectional multivariable model. During a median follow-up of 13.1 months, 16/771 (0.9%) patients developed gangrene. All 16 patients who developed gangrene had previously had DUs and gangrene. Further risk factors for incident gangrene were the dcSSc subset and longer disease duration. Conclusion In unselected SSc patients, gangrene occurs in about 9% of SSc patients. DUs ever and, to a lesser extent, the dcSSc subset are strongly and independently associated with gangrene, while traditional cardiovascular risk factors could not be identified as risk factors.

Published
2019

40. Predictors of disease worsening defined by progression of organ damage in diffuse systemic sclerosis: a European Scleroderma Trials and Research (EUSTAR) analysis

Author

Becker M, Graf N, Sauter R, Curram J, Denton C, Khanna D, Pena J, Pope J, Distler O, Matucci-Cerinic M, Guiducci S, Walker U, Jaeger V, Bannert B, Lapadula G, Becvarare R, Cutolo M, Valentini G, Siegert E, Rednic S, Allanore Y, Montecucco C, Carreira P, Novak S, Czirjak L, Varju C, Chizzolini C, Allai D, Kucharz E, Cozzi F, Rozman B, Mallia C, Gabrielli A, Bancel D, Airo P, Hesselstrand R, Martinovic D, Balbir-Gurman A, Braun-Moscovici Y, Hunzelmann N, Pellerito R, Caramaschi P, Black C, Damjanov N, Henes J, Santamaria V, Heitmann S, Seidel M, Da Silva J, Stamenkovic B, Selmi C, Tikly M, Denisov L, Muller-Ladner U, Engelhart M, Hachulla E, Riccieri V, Ionescu R, Mihai C, Sunderkotter C, Kuhn A, Schett G, Distler J, Meroni P, Ingegnoli F, Mouthon L, De Keyser F, Smith V, Cantatore F, Corrado A, Ullman S, Iversen L, Pozzi M, Eyerich K, Hein R, Knott E, Wiland P, Szmyrka-Kaczmarek M, Sokolik R, Morgiel E, Madej M, Alegre-Sancho J, Krummel-Lorenz B, Saar P, Aringer M, Gunther C, Anne E, Westhovens R, De Langhe E, Lenaerts J, Anic B, Baresic M, Mayer M, Uprus M, Otsa K, Yavuz S, Radominski S, Muller C, Azevedo V, Popa S, Zenone T, Stebbings S, Highton J, Mathieu A, Vacca A, Stamp L, Chapman P, O'Donnell J, Solanki K, Doube A, Veale D, O'Rourke M, Loyo E, Li M, Rosato E, Amoroso A, Gigante A, Oksel F, Yargucu F, Tanaseanu C, Popescu M, Dumitrascu A, Tiglea I, Foti R, Visalli E, Benenati A, Amato G, Ancuta C, Chirieac R, Villiger P, Adler S, Dan D, Lefebvre P, Rubio S, Exposito M, Sibilia J, Chatelus E, Gottenberg J, Chifflot H, Litinsky I, Del Galdo F, Venalis A, Saketkoo L, Lasky J, Kerzberg E, Montoya F, Cosentino V, Limonta M, Brucato A, Lupi E, Spertini F, Ribi C, Buss G, Martin T, Guffroy A, Poindron V, Chung L, Schmeiser T, Zebryk P, Riso N, Riemekasten G, Rezus E, Puttini P, and EUSTAR Collaborators

Abstract

Objectives Mortality and worsening of organ function are desirable endpoints for clinical trials in systemic sclerosis (SSc). The aim of this study was to identify factors that allow enrichment of patients with these endpoints, in a population of patients from the European Scleroderma Trials and Research group database. Methods Inclusion criteria were diagnosis of diffuse SSc and follow-up over 12 +/- 3 months. Disease worsening/organ progression was fulfilled if any of the following events occurred: new renal crisis; decrease of lung or heart function; new echocardiography-suspected pulmonary hypertension or death. In total, 42 clinical parameters were chosen as predictors for the analysis by using (1) imputation of missing data on the basis of multivariate imputation and (2) least absolute shrinkage and selection operator regression. Results Of 1451 patients meeting the inclusion criteria, 706 had complete data on outcome parameters and were included in the analysis. Of the 42 outcome predictors, eight remained in the final regression model. There was substantial evidence for a strong association between disease progression and age, active digital ulcer (DU), lung fibrosis, muscle weakness and elevated C-reactive protein (CRP) level. Active DU, CRP elevation, lung fibrosis and muscle weakness were also associated with a significantly shorter time to disease progression. A bootstrap validation step with 10 000 repetitions successfully validated the model. Conclusions The use of the predictive factors presented here could enable cohort enrichment with patients at risk for overall disease worsening in SSc clinical trials.

Published
2019

41. Influence of Antisynthetase Antibodies Specificities on Antisynthetase Syndrome Clinical Spectrum Time Course

Author

Cavagna, L, Trallero-Araguás, E, Meloni, F, Cavazzana, I, Rojas-Serrano, J, Feist, E, Zanframundo, G, Morandi, V, Meyer, A, Pereira da Silva, J, Matos Costa, C, Molberg, O, Andersson, H, Codullo, V, Mosca, M, Barsotti, S, Neri, R, Scirè, C, Govoni, M, Furini, F, Lopez-Longo, F, Martinez-Barrio, J, Schneider, U, Lorenz, H, Doria, A, Ghirardello, A, Ortego-Centeno, N, Confalonieri, M, Tomietto, P, Pipitone, N, Rodriguez Cambron, A, Blázquez Cañamero, M, Voll, R, Wendel, S, Scarpato, S, Maurier, F, Limonta, M, Colombelli, P, Giannini, M, Geny, B, Arrigoni, E, Bravi, E, Migliorini, P, Mathieu, A, Piga, M, Drott, U, Delbrueck, C, Bauhammer, J, Cagnotto, G, Vancheri, C, Sambataro, G, De Langhe, E, Sainaghi, P, Monti, C, Gigli Berzolari, F, Romano, M, Bonella, F, Specker, C, Schwarting, A, Villa Blanco, I, Selmi, C, Ceribelli, A, Nuno, L, Mera-Varela, A, Perez Gomez, N, Fusaro, E, Parisi, S, Sinigaglia, L, Del Papa, N, Benucci, M, Cimmino, M, Riccieri, V, Conti, F, Sebastiani, G, Iuliano, A, Emmi, G, Cammelli, D, Sebastiani, M, Manfredi, A, Bachiller-Corral, J, Sifuentes Giraldo, W, Paolazzi, G, Saketkoo, L, Giorgi, R, Salaffi, F, Cifrian, J, Caporali, R, Locatelli, F, Marchioni, E, Pesci, A, Dei, G, Pozzi, M, Claudia, L, Distler, J, Knitza, J, Schett, G, Iannone, F, Fornaro, M, Franceschini, F, Quartuccio, L, Gerli, R, Bartoloni, E, Bellando Randone, S, Zampogna, G, Gonzalez Perez, M, Mejia, M, Vicente, E, Triantafyllias, K, Lopez-Mejias, R, Matucci-Cerinic, M, Selva-O'Callaghan, A, Castañeda, S, Montecucco, C, Gonzalez-Gay, M, Cavagna, Lorenzo, Trallero-Araguás, Ernesto, Meloni, Federica, Cavazzana, Ilaria, Rojas-Serrano, Jorge, Feist, Eugen, Zanframundo, Giovanni, Morandi, Valentina, Meyer, Alain, Pereira da Silva, Jose Antonio, Matos Costa, Carlo Jorge, Molberg, Oyvind, Andersson, Helena, Codullo, Veronica, Mosca, Marta, Barsotti, Simone, Neri, Rossella, Scirè, Carlo, Govoni, Marcello, Furini, Federica, Lopez-Longo, Francisco Javier, Martinez-Barrio, Julia, Schneider, Udo, Lorenz, Hanns-Martin, Doria, Andrea, Ghirardello, Anna, Ortego-Centeno, Norberto, Confalonieri, Marco, Tomietto, Paola, Pipitone, Nicolò, Rodriguez Cambron, Ana Belen, Blázquez Cañamero, María Ángeles, Voll, Reinhard Edmund, Wendel, Sarah, Scarpato, Salvatore, Maurier, Francois, Limonta, Massimiliano, Colombelli, Paolo, Giannini, Margherita, Geny, Bernard, Arrigoni, Eugenio, Bravi, Elena, Migliorini, Paola, Mathieu, Alessandro, Piga, Matteo, Drott, Ulrich, Delbrueck, Christiane, Bauhammer, Jutta, Cagnotto, Giovanni, Vancheri, Carlo, Sambataro, Gianluca, De Langhe, Ellen, Sainaghi, Pier Paolo, Monti, Cristina, Gigli Berzolari, Francesca, Romano, Mariaeva, Bonella, Francesco, Specker, Christof, Schwarting, Andreas, Villa Blanco, Ignacio, Selmi, Carlo, Ceribelli, Angela, Nuno, Laura, Mera-Varela, Antonio, Perez Gomez, Nair, Fusaro, Enrico, Parisi, Simone, Sinigaglia, Luigi, Del Papa, Nicoletta, Benucci, Maurizio, Cimmino, Marco Amedeo, Riccieri, Valeria, Conti, Fabrizio, Sebastiani, Gian Domenico, Iuliano, Annamaria, Emmi, Giacomo, Cammelli, Daniele, Sebastiani, Marco, Manfredi, Andreina, Bachiller-Corral, Javier, Sifuentes Giraldo, Walter Alberto, Paolazzi, Giuseppe, Saketkoo, Lesley Ann, Giorgi, Roberto, Salaffi, Fausto, Cifrian, Jose, Caporali, Roberto, Locatelli, Francesco, Marchioni, Enrico, Pesci, Alberto, Dei, Giulia, Pozzi, Maria Rosa, Claudia, Lomater, Distler, Jorg, Knitza, Johannes, Schett, George, Iannone, Florenzo, Fornaro, Marco, Franceschini, Franco, Quartuccio, Luca, Gerli, Roberto, Bartoloni, Elena, Bellando Randone, Silvia, Zampogna, Giuseppe, Gonzalez Perez, Montserrat I, Mejia, Mayra, Vicente, Esther, Triantafyllias, Konstantinos, Lopez-Mejias, Raquel, Matucci-Cerinic, Marco, Selva-O'Callaghan, Albert, Castañeda, Santos, Montecucco, Carlomaurizio, Gonzalez-Gay, Miguel Angel, Cavagna, L, Trallero-Araguás, E, Meloni, F, Cavazzana, I, Rojas-Serrano, J, Feist, E, Zanframundo, G, Morandi, V, Meyer, A, Pereira da Silva, J, Matos Costa, C, Molberg, O, Andersson, H, Codullo, V, Mosca, M, Barsotti, S, Neri, R, Scirè, C, Govoni, M, Furini, F, Lopez-Longo, F, Martinez-Barrio, J, Schneider, U, Lorenz, H, Doria, A, Ghirardello, A, Ortego-Centeno, N, Confalonieri, M, Tomietto, P, Pipitone, N, Rodriguez Cambron, A, Blázquez Cañamero, M, Voll, R, Wendel, S, Scarpato, S, Maurier, F, Limonta, M, Colombelli, P, Giannini, M, Geny, B, Arrigoni, E, Bravi, E, Migliorini, P, Mathieu, A, Piga, M, Drott, U, Delbrueck, C, Bauhammer, J, Cagnotto, G, Vancheri, C, Sambataro, G, De Langhe, E, Sainaghi, P, Monti, C, Gigli Berzolari, F, Romano, M, Bonella, F, Specker, C, Schwarting, A, Villa Blanco, I, Selmi, C, Ceribelli, A, Nuno, L, Mera-Varela, A, Perez Gomez, N, Fusaro, E, Parisi, S, Sinigaglia, L, Del Papa, N, Benucci, M, Cimmino, M, Riccieri, V, Conti, F, Sebastiani, G, Iuliano, A, Emmi, G, Cammelli, D, Sebastiani, M, Manfredi, A, Bachiller-Corral, J, Sifuentes Giraldo, W, Paolazzi, G, Saketkoo, L, Giorgi, R, Salaffi, F, Cifrian, J, Caporali, R, Locatelli, F, Marchioni, E, Pesci, A, Dei, G, Pozzi, M, Claudia, L, Distler, J, Knitza, J, Schett, G, Iannone, F, Fornaro, M, Franceschini, F, Quartuccio, L, Gerli, R, Bartoloni, E, Bellando Randone, S, Zampogna, G, Gonzalez Perez, M, Mejia, M, Vicente, E, Triantafyllias, K, Lopez-Mejias, R, Matucci-Cerinic, M, Selva-O'Callaghan, A, Castañeda, S, Montecucco, C, Gonzalez-Gay, M, Cavagna, Lorenzo, Trallero-Araguás, Ernesto, Meloni, Federica, Cavazzana, Ilaria, Rojas-Serrano, Jorge, Feist, Eugen, Zanframundo, Giovanni, Morandi, Valentina, Meyer, Alain, Pereira da Silva, Jose Antonio, Matos Costa, Carlo Jorge, Molberg, Oyvind, Andersson, Helena, Codullo, Veronica, Mosca, Marta, Barsotti, Simone, Neri, Rossella, Scirè, Carlo, Govoni, Marcello, Furini, Federica, Lopez-Longo, Francisco Javier, Martinez-Barrio, Julia, Schneider, Udo, Lorenz, Hanns-Martin, Doria, Andrea, Ghirardello, Anna, Ortego-Centeno, Norberto, Confalonieri, Marco, Tomietto, Paola, Pipitone, Nicolò, Rodriguez Cambron, Ana Belen, Blázquez Cañamero, María Ángeles, Voll, Reinhard Edmund, Wendel, Sarah, Scarpato, Salvatore, Maurier, Francois, Limonta, Massimiliano, Colombelli, Paolo, Giannini, Margherita, Geny, Bernard, Arrigoni, Eugenio, Bravi, Elena, Migliorini, Paola, Mathieu, Alessandro, Piga, Matteo, Drott, Ulrich, Delbrueck, Christiane, Bauhammer, Jutta, Cagnotto, Giovanni, Vancheri, Carlo, Sambataro, Gianluca, De Langhe, Ellen, Sainaghi, Pier Paolo, Monti, Cristina, Gigli Berzolari, Francesca, Romano, Mariaeva, Bonella, Francesco, Specker, Christof, Schwarting, Andreas, Villa Blanco, Ignacio, Selmi, Carlo, Ceribelli, Angela, Nuno, Laura, Mera-Varela, Antonio, Perez Gomez, Nair, Fusaro, Enrico, Parisi, Simone, Sinigaglia, Luigi, Del Papa, Nicoletta, Benucci, Maurizio, Cimmino, Marco Amedeo, Riccieri, Valeria, Conti, Fabrizio, Sebastiani, Gian Domenico, Iuliano, Annamaria, Emmi, Giacomo, Cammelli, Daniele, Sebastiani, Marco, Manfredi, Andreina, Bachiller-Corral, Javier, Sifuentes Giraldo, Walter Alberto, Paolazzi, Giuseppe, Saketkoo, Lesley Ann, Giorgi, Roberto, Salaffi, Fausto, Cifrian, Jose, Caporali, Roberto, Locatelli, Francesco, Marchioni, Enrico, Pesci, Alberto, Dei, Giulia, Pozzi, Maria Rosa, Claudia, Lomater, Distler, Jorg, Knitza, Johannes, Schett, George, Iannone, Florenzo, Fornaro, Marco, Franceschini, Franco, Quartuccio, Luca, Gerli, Roberto, Bartoloni, Elena, Bellando Randone, Silvia, Zampogna, Giuseppe, Gonzalez Perez, Montserrat I, Mejia, Mayra, Vicente, Esther, Triantafyllias, Konstantinos, Lopez-Mejias, Raquel, Matucci-Cerinic, Marco, Selva-O'Callaghan, Albert, Castañeda, Santos, Montecucco, Carlomaurizio, and Gonzalez-Gay, Miguel Angel

Abstract

Antisynthetase syndrome (ASSD) is a rare clinical condition that is characterized by the occurrence of a classic clinical triad, encompassing myositis, arthritis, and interstitial lung disease (ILD), along with specific autoantibodies that are addressed to different aminoacyl tRNA synthetases (ARS). Until now, it has been unknown whether the presence of a different ARS might affect the clinical presentation, evolution, and outcome of ASSD. In this study, we retrospectively recorded the time of onset, characteristics, clustering of triad findings, and survival of 828 ASSD patients (593 anti-Jo1, 95 anti-PL7, 84 anti-PL12, 38 anti-EJ, and 18 anti-OJ), referring to AENEAS (American and European NEtwork of Antisynthetase Syndrome) collaborative group's cohort. Comparisons were performed first between all ARS cases and then, in the case of significance, while using anti-Jo1 positive patients as the reference group. The characteristics of triad findings were similar and the onset mainly began with a single triad finding in all groups despite some differences in overall prevalence. The "ex-novo" occurrence of triad findings was only reduced in the anti-PL12-positive cohort, however, it occurred in a clinically relevant percentage of patients (30%). Moreover, survival was not influenced by the underlying anti-aminoacyl tRNA synthetase antibodies' positivity, which confirmed that antisynthetase syndrome is a heterogeneous condition and that antibody specificity only partially influences the clinical presentation and evolution of this condition.

Published
2019

42. Generation of a Core Set of Items to Develop Classification Criteria for Scleroderma Renal Crisis Using Consensus Methodology

Author

Butler, E-A, Baron, M, Fogo, AB, Frech, T, Ghossein, C, Hachulla, E, Hoa, S, Johnson, SR, Khanna, D, Mouthon, L, Nikpour, M, Proudman, S, Steen, V, Stern, E, Varga, J, Denton, C, Hudson, M, Barnado, A, Bernstein, EJ, Boin, F, Braun-Moscovici, Y, Castelino, FV, Catoggio, LJ, Matucci-Cerinic, M, Chung, L, Clements, P, Csuka, ME, De langhe, E, Distler, J, Distler, O, Farge, DC, Fischer, A, Gabrielli, A, Hasegawa, M, Hayat, S, Herrick, A, Hesselstrand, R, Hsu, V, Hughes, M, Hunzelmann, N, Hummers, L, Iannone, F, Ingegnoli, F, Jacobsen, S, Kawaguchi, Y, Koenig, M, Kuwana, M, Lenaerts, J, Martin, T, Mayes, MD, McMahan, Z, Medsger, T, Merkel, P, Narain, S, Ong, V, Pauling, JD, Pope, J, de la Puente, C, Rischmueller, M, Sofia Rodriguez-Reyna, T, Sahhar, J, Saketkoo, LA, Senecal, J-L, Shah, A, Shah, AA, Alberto Sifuentes-Giraldo, W, Silver, R, Stevens, W, Sutton, E, Thakkar, V, Valentini, G, de Vries-Bouwstra, J, Vonk, M, Walker, UA, Butler, E-A, Baron, M, Fogo, AB, Frech, T, Ghossein, C, Hachulla, E, Hoa, S, Johnson, SR, Khanna, D, Mouthon, L, Nikpour, M, Proudman, S, Steen, V, Stern, E, Varga, J, Denton, C, Hudson, M, Barnado, A, Bernstein, EJ, Boin, F, Braun-Moscovici, Y, Castelino, FV, Catoggio, LJ, Matucci-Cerinic, M, Chung, L, Clements, P, Csuka, ME, De langhe, E, Distler, J, Distler, O, Farge, DC, Fischer, A, Gabrielli, A, Hasegawa, M, Hayat, S, Herrick, A, Hesselstrand, R, Hsu, V, Hughes, M, Hunzelmann, N, Hummers, L, Iannone, F, Ingegnoli, F, Jacobsen, S, Kawaguchi, Y, Koenig, M, Kuwana, M, Lenaerts, J, Martin, T, Mayes, MD, McMahan, Z, Medsger, T, Merkel, P, Narain, S, Ong, V, Pauling, JD, Pope, J, de la Puente, C, Rischmueller, M, Sofia Rodriguez-Reyna, T, Sahhar, J, Saketkoo, LA, Senecal, J-L, Shah, A, Shah, AA, Alberto Sifuentes-Giraldo, W, Silver, R, Stevens, W, Sutton, E, Thakkar, V, Valentini, G, de Vries-Bouwstra, J, Vonk, M, and Walker, UA

Abstract

OBJECTIVE: To generate a core set of items to develop classification criteria for scleroderma renal crisis (SRC) using consensus methodology. METHODS: An international, multidisciplinary panel of experts was invited to participate in a 3-round Delphi exercise developed using a survey based on items identified by a scoping review. In round 1, participants were asked to identify omissions and clarify ambiguities regarding the items in the survey. In round 2, participants were asked to rate the validity and feasibility of the items using Likert-type scales ranging from 1 to 9 (where 1 = very invalid/unfeasible, 5 = uncertain, and 9 = very valid/feasible). In round 3, participants reviewed the results and comments from round 2 and were asked to provide final ratings. Items rated as highly valid and feasible (median scores ≥7 for each) in round 3 were selected as the provisional core set of items. A consensus meeting using a nominal group technique was conducted to further reduce the core set of items. RESULTS: Ninety-nine experts from 16 countries participated in the Delphi exercise. Of the 31 items in the survey, consensus was achieved on 13, in the categories hypertension, renal insufficiency, proteinuria, and hemolysis. Eleven experts took part in the nominal group technique discussion, where consensus was achieved in 5 domains: blood pressure, acute kidney injury, microangiopathic hemolytic anemia, target organ dysfunction, and renal histopathology. CONCLUSION: A core set of items that characterize SRC was identified using consensus methodology. This core set will be used in future data-driven phases of this project to develop classification criteria for SRC.

Published
2019

44. Update of EULAR recommendations for the treatment of systemic sclerosis

Author

Kowal-Bielecka O., Fransen J., Avouac J., Becker M., Kulak A., Allanore Y., Distler O., Clements P., Cutolo M., Czirjak L., Damjanov N., Del Galdo F., Denton C. P., Distler J. H. W., Foeldvari I., Figelstone K., Frerix M., Furst D. E., Guiducci S., Hunzelmann N., Khanna D., Matucci-Cerinic M., Herrick A. L., Van Den Hoogen F., Van Laar J. M., Riemekasten G., Silver R., Smith V., Sulli A., Tarner I., Tyndall A., Welling J., Wigley F., Valentini G., Walker U. A., Zulian F., Muller-Ladner U., Daikeler T., Lanciano E., Becvar R., Tomcik M., Gindzienska-Sieskiewicz E., Cuomo G., Iudici M., Rednic S., Vlachoyiannopoulos P. G., Caporali R., Carreira P. E., Novak S., Minier T., Kucharz E. J., Gabrielli A., Moroncini G., Airo' P., Hesselstrand R., Martinovic D., Radic M., Marasovic-Krstulovic D., Braun-Moscovici Y., Balbir-Gurman A., Lo Monaco A., Caramaschi P., Morovic-Vergles J., Henes J., Ortiz Santamaria V., Heitmann S., Krasowska D., Seidel M. F., Hasler P., Pereira Da Silva J. A., Salvador M. J., Stamenkovic B., Stankovic A., Tikly M., Ananieva L. P., Beretta L., Szucs G., Szamosi S., de la Puente Bujidos C., Midtvedt O., Hoffmann-Vold A. -M., Launay D., Hachulla E., Riccieri V., Ionescu R., Opris D., Mihai C., Herrgott I., Beyer C., Ingegnoli F., von Muhlen C. A., Alegre-Sancho J. J., Beltran-Catalan E., Aringer M., Fantana J., Leuchten N., Tausche A. -K., De Langhe E., Vanthuyne M., Anic B., Baresic M., Mayer M., Uprus M., Otsa K., Yavuz S., Granel B., Azevedo V. F., Muller C., Jimenez S. A., Popa S., Agachi S., Zenone T., Stebbings S., Dockerty J., Vacca A., Schollum J., Veale D. J., Toloza S., Xu D., Olas J., Rosato E., Foti R., Adler S., Dan D., Wiesik-Szewczyk E., Olesinska M., Kayser C., Fathi N., de la Pena Lefebvre P. G., Imbert B., Kowal-Bielecka, O., Fransen, J., Avouac, J., Becker, M., Kulak, A., Allanore, Y., Distler, O., Clements, P., Cutolo, M., Czirjak, L., Damjanov, N., Del Galdo, F., Denton, C. P., Distler, J. H. W., Foeldvari, I., Figelstone, K., Frerix, M., Furst, D. E., Guiducci, S., Hunzelmann, N., Khanna, D., Matucci-Cerinic, M., Herrick, A. L., Van Den Hoogen, F., Van Laar, J. M., Riemekasten, G., Silver, R., Smith, V., Sulli, A., Tarner, I., Tyndall, A., Welling, J., Wigley, F., Valentini, G., Walker, U. A., Zulian, F., Muller-Ladner, U., Daikeler, T., Lanciano, E., Becvar, R., Tomcik, M., Gindzienska-Sieskiewicz, E., Cuomo, G., Iudici, M., Rednic, S., Vlachoyiannopoulos, P. G., Caporali, R., Carreira, P. E., Novak, S., Minier, T., Kucharz, E. J., Gabrielli, A., Moroncini, G., Airo', P., Hesselstrand, R., Martinovic, D., Radic, M., Marasovic-Krstulovic, D., Braun-Moscovici, Y., Balbir-Gurman, A., Lo Monaco, A., Caramaschi, P., Morovic-Vergles, J., Henes, J., Ortiz Santamaria, V., Heitmann, S., Krasowska, D., Seidel, M. F., Hasler, P., Pereira Da Silva, J. A., Salvador, M. J., Stamenkovic, B., Stankovic, A., Tikly, M., Ananieva, L. P., Beretta, L., Szucs, G., Szamosi, S., de la Puente Bujidos, C., Midtvedt, O., Hoffmann-Vold, A. -M., Launay, D., Hachulla, E., Riccieri, V., Ionescu, R., Opris, D., Mihai, C., Herrgott, I., Beyer, C., Ingegnoli, F., von Muhlen, C. A., Alegre-Sancho, J. J., Beltran-Catalan, E., Aringer, M., Fantana, J., Leuchten, N., Tausche, A. -K., De Langhe, E., Vanthuyne, M., Anic, B., Baresic, M., Mayer, M., Uprus, M., Otsa, K., Yavuz, S., Granel, B., Azevedo, V. F., Muller, C., Jimenez, S. A., Popa, S., Agachi, S., Zenone, T., Stebbings, S., Dockerty, J., Vacca, A., Schollum, J., Veale, D. J., Toloza, S., Xu, D., Olas, J., Rosato, E., Foti, R., Adler, S., Dan, D., Wiesik-Szewczyk, E., Olesinska, M., Kayser, C., Fathi, N., de la Pena Lefebvre, P. G., Imbert, B., UCL - SSS/IREC/SLUC - Pôle St.-Luc, UCL - (MGD) Service de rhumatologie, Kowal Bielecka, Otylia, Fransen, Jaap, Avouac, Jerome, Becker, Mike, Kulak, Agnieszka, Allanore, Yannick, Distler, Oliver, Clements, Philip, Cutolo, Maurizio, Czirjak, Laszlo, Damjanov, Nemanja, del Galdo, Francesco, Denton, Christopher P., Distler, Jörg H. W., Foeldvari, Ivan, Figelstone, Kim, Frerix, Marc, Furst, Daniel E., Guiducci, Serena, Hunzelmann, Nicola, Khanna, Dinesh, Matucci Cerinic, Marco, Herrick, Ariane L., van den Hoogen, Frank, van Laar, Jacob M., Riemekasten, Gabriela, Silver, Richard, Smith, Vanessa, Sulli, Alberto, Tarner, Ingo, Tyndall, Alan, Welling, Joep, Wigley, Frederic, Valentini, Gabriele, Walker, Ulrich A., Zulian, Francesco, Müller Ladner, Ulf, Daikeler, Thoma, Lanciano, Elisabetta, Becvã¡r, Radim, Tomcik, Michal, Gindzienska Sieskiewicz, Ewa, Iudici, Michele, Rednic, Simona, Vlachoyiannopoulos, Panayiotis G., Caporali, Roberto, Carreira, Patricia E., Novak, Srdan, Minier, Tã¼nde, Kucharz, Eugene J., Gabrielli, Armando, Moroncini, Gianluca, Airo, Paolo, Hesselstrand, Roger, Martinovic, Duska, Radic, Mislav, Marasovic Krstulovic, Daniela, Braun Moscovici, Yolanda, Monaco, Andrea Lo, Morovic Vergles, Jadranka, Culo, Melanie I., Henes, Jã¶rg, Santamaria, Vera Ortiz, Heitmann, Stefan, Krasowska, Dorota, Michalska Jakubus, Malgorzata, Seidel, Matthias F., Klinik III, Medizinische, Hasler, Paul, Da Silva, José A. Pereira, Salvador, Maria J., Stamenkovic, Bojana, Stankovic, Aleksandra, Tikly, Mohammed, Ananieva, Lidia P., Beretta, Lorenzo, Szucs, Gabriella, Szamosi, Szilvia, de la Puente Bujidos, Carlo, Midtvedt, Øyvind, Hoffmann Vold, Anna Maria, Launay, David, Hachulla, Eric, Riccieri, Valeria, Ionescu, Ruxandra, Opris, Daniela, Mihai, Carina, Herrgott, Ilka, Beyer, Christian, Ingegnoli, Francesca, von Mühlen, Carlos Alberto, Alegre Sancho, Juan José, Beltran Catalan, Emma, Aringer, Martin, Fantana, Julia, Leuchten, Nicolai, Tausche, Anne Kathrin, Langhe, Ellen De, Vanthuyne, Marie, Anic, Branimir, Bareå¡ic, Marko, Mayer, Miroslav, Ãœprus, Maria, Otsa, Kati, Yavuz, Sule, Granel, Brigitte, Jimenez, Sergio A., Popa, Serghei, Agachi, Svetlana, Zenone, Thierry, Stebbings, Simon, Dockerty, Joanne, Vacca, Alessandra, Schollum, Joanna, Veale, Douglas J., Toloza, Sergio, Xu, Dong, Olas, Jacek, Rosato, Edoardo, Foti, Rosario, Adler, Sabine, Dan, Diana, Wiesik Szewczyk, Ewa, Olesinska, Marzena, Kayser, Cristiane, Fathi, Nihal, de la Peña Lefebvre, Paloma García, Imbert, Bernard, and Cuomo, Giovanna

Subjects
Endothelin Receptor Antagonists, Lung Diseases, Kidney Disease, Delphi Technique, Gastrointestinal Diseases, systemic sclerosis, Scleroderma Renal Crisis, Placebo-controlled study, Angiotensin-Converting Enzyme Inhibitors, Lung Disease, Scleroderma, 0302 clinical medicine, Glucocorticoid, Phosphodiesterase 5 Inhibitor, Immunology and Allergy, skin and connective tissue diseases, BIOMEDICINA I ZDRAVSTVO. Kliničke medicinske znanosti. Interna medicina, integumentary system, treatment, genetics and molecular biology (all), Hematopoietic Stem Cell Transplantation, cyclophosphamide, methotrexate, Pulmonary, Orvostudományok, Serotonin Uptake Inhibitor, 3. Good health, Europe, Systematic review, Hypertension, Serotonin Uptake Inhibitors, Cyclophosphamide, Methotrexate, Systemic Sclerosis, Treatment, Fingers, Fluoxetine, Glucocorticoids, Humans, Hypertension, Pulmonary, Kidney Diseases, Phosphodiesterase 5 Inhibitors, Prostaglandins I, Pyrazoles, Pyrimidines, Raynaud Disease, Rheumatology, Scleroderma, Systemic, Ulcer, Immunology, Biochemistry, Genetics and Molecular Biology (all), 030211 gastroenterology & hepatology, Endothelin Receptor Antagonist, Selective Serotonin Reuptake Inhibitors, medicine.drug, Human, medicine.medical_specialty, Gastrointestinal Disease, Klinikai orvostudományok, Riociguat, General Biochemistry, Genetics and Molecular Biology, 03 medical and health sciences, medicine, Finger, biochemistry, Intensive care medicine, BIOMEDICINE AND HEALTHCARE. Clinical Medical Sciences. Internal Medicine, Systemic Sclerosi, 030203 arthritis & rheumatology, business.industry, Systemic, Angiotensin-Converting Enzyme Inhibitor, medicine.disease, Transplantation, Clinical research, Pyrimidine, immunology and allergy, rheumatology, immunology, Pyrazole, Physical therapy, business, Rheumatism
Abstract

The aim was to update the 2009 European League against Rheumatism (EULAR) recommendations for the treatment of systemic sclerosis (SSc), with attention to new therapeutic questions. Update of the previous treatment recommendations was performed according to EULAR standard operating procedures. The task force consisted of 32 SSc clinical experts from Europe and the USA, 2 patients nominated by the pan-European patient association for SSc (Federation of European Scleroderma Associations (FESCA)), a clinical epidemiologist and 2 research fellows. All centres from the EULAR Scleroderma Trials and Research group were invited to submit and select clinical questions concerning SSc treatment using a Delphi approach. Accordingly, 46 clinical questions addressing 26 different interventions were selected for systematic literature review. The new recommendations were based on the available evidence and developed in a consensus meeting with clinical experts and patients. The procedure resulted in 16 recommendations being developed (instead of 14 in 2009) that address treatment of several SSc-related organ complications: Raynaud's phenomenon (RP), digital ulcers (DUs), pulmonary arterial hypertension (PAH), skin and lung disease, scleroderma renal crisis and gastrointestinal involvement. Compared with the 2009 recommendations, the 2016 recommendations include phosphodiesterase type 5 (PDE-5) inhibitors for the treatment of SSc-related RP and DUs, riociguat, new aspects for endothelin receptor antagonists, prostacyclin analogues and PDE-5 inhibitors for SSc-related PAH. New recommendations regarding the use of fluoxetine for SSc-related RP and haematopoietic stem cell transplantation for selected patients with rapidly progressive SSc were also added. In addition, several comments regarding other treatments addressed in clinical questions and suggestions for the SSc research agenda were formulated. These updated data-derived and consensus-derived recommendations will help rheumatologists to manage patients with SSc in an evidence-based way. These recommendations also give directions for future clinical research in SSc.

Published
2017

45. Educational needs and preferences of young European clinicians and physician researchers working in the field of rheumatology

Author

Beyer, C, Ramiro, S, Sivera, F, Mandl, P, Machado, P, Ospelt, C, Molto, A, Radner, H, Iagnocco, A, Bijlsma, J, Lundberg, I, Grigoriou, A, Fanouriakis, A, Hueber, A, Maurer, B, Scire, C, Gaujoux-Viala, C, Karpec, D, Van Der Woude, D, Rimar, D, Deseatnicova, E, Nikiphorou, E, De Langhe, E, Haavardsholm, E, Starodubtseva, I, Nagy, G, Biliavska, I, Sznajd, J, Karlsson, J, Zepa, J, Cassar, K, Senolt, L, Ghukasyan, L, Baresic, M, Frank-Bertoncelj, M, Ziade, N, Gobejishvili, N, Ostojic, P, Ostgard, R, Dobrota, R, Shumnalieva, R, Talli, S, Panchal, S, Volpinari, S, Rannio, T, Romao, V, Beyer C., Ramiro S., Sivera F., Mandl P., MacHado P. M., Ospelt C., Molto A., Radner H., Iagnocco A., Bijlsma J. W., Lundberg I. E., Grigoriou A., Fanouriakis A., Hueber A., Maurer B., Scire C. A., Gaujoux-Viala C., Karpec D., Van Der Woude D., Rimar D., Deseatnicova E., Nikiphorou E., De Langhe E., Haavardsholm E. A., Starodubtseva I., Nagy G., Biliavska I. V., Sznajd J., Karlsson J. A., Zepa J., Cassar K., Senolt L., Ghukasyan L., Baresic M., Frank-Bertoncelj M., Ziade N., Gobejishvili N., Ostojic P., Ostgard R., Dobrota R., Shumnalieva R., Talli S., Panchal S., Volpinari S., Rannio T., Romao V. C., Beyer, C, Ramiro, S, Sivera, F, Mandl, P, Machado, P, Ospelt, C, Molto, A, Radner, H, Iagnocco, A, Bijlsma, J, Lundberg, I, Grigoriou, A, Fanouriakis, A, Hueber, A, Maurer, B, Scire, C, Gaujoux-Viala, C, Karpec, D, Van Der Woude, D, Rimar, D, Deseatnicova, E, Nikiphorou, E, De Langhe, E, Haavardsholm, E, Starodubtseva, I, Nagy, G, Biliavska, I, Sznajd, J, Karlsson, J, Zepa, J, Cassar, K, Senolt, L, Ghukasyan, L, Baresic, M, Frank-Bertoncelj, M, Ziade, N, Gobejishvili, N, Ostojic, P, Ostgard, R, Dobrota, R, Shumnalieva, R, Talli, S, Panchal, S, Volpinari, S, Rannio, T, Romao, V, Beyer C., Ramiro S., Sivera F., Mandl P., MacHado P. M., Ospelt C., Molto A., Radner H., Iagnocco A., Bijlsma J. W., Lundberg I. E., Grigoriou A., Fanouriakis A., Hueber A., Maurer B., Scire C. A., Gaujoux-Viala C., Karpec D., Van Der Woude D., Rimar D., Deseatnicova E., Nikiphorou E., De Langhe E., Haavardsholm E. A., Starodubtseva I., Nagy G., Biliavska I. V., Sznajd J., Karlsson J. A., Zepa J., Cassar K., Senolt L., Ghukasyan L., Baresic M., Frank-Bertoncelj M., Ziade N., Gobejishvili N., Ostojic P., Ostgard R., Dobrota R., Shumnalieva R., Talli S., Panchal S., Volpinari S., Rannio T., and Romao V. C.

Abstract

Objectives: To understand the educational needs and preferences of young clinicians and physician researchers in the field of rheumatology in Europe. Methods: An international online survey was performed as a joint venture of ESCET and EMEUNET. The survey assessed the acceptance of and the access to the current European League Against Rheumatism (EULAR) educational portfolio, as well as the unmet educational needs and learning preferences among individuals below the age of 40 years working in rheumatology in Europe. Results: Among 568 European clinicians and physician researchers, 65% indicated that the existing EULAR educational portfolio adequately covers their educational needs. Within the EULAR portfolio, the online course on rheumatic diseases and the postgraduate course were the most appreciated. Participants were very much in favour of new educational courses on imaging techniques, and 63% of participants indicated a particular interest in musculoskeletal ultrasound. A strong interest in refresher (60%) and general review (55%) courses was observed. Lack of funding was considered the major obstacle to participating in existing EULAR programmes. Finally, participants showed diverse preferences regarding learning modalities with common interests in live courses and conferences. Conclusions: EULAR's training opportunities are well appreciated among young clinicians and physician researchers in rheumatology. The results from this survey will help to develop EULAR's future educational portfolio.

Published
2016

47. Update of EULAR recommendations for the treatment of systemic sclerosis

Author

Kowal-Bielecka, O. Fransen, J. Avouac, J. Becker, M. Kulak, A. Allanore, Y. Distler, O. Clements, P. Cutolo, M. Czirjak, L. Damjanov, N. Del Galdo, F. Denton, C.P. Distler, J.H.W. Foeldvari, I. Figelstone, K. Frerix, M. Furst, D.E. Guiducci, S. Hunzelmann, N. Khanna, D. Matucci-Cerinic, M. Herrick, A.L. Van Den Hoogen, F. Van Laar, J.M. Riemekasten, G. Silver, R. Smith, V. Sulli, A. Tarner, I. Tyndall, A. Welling, J. Wigley, F. Valentini, G. Walker, U.A. Zulian, F. Müller-Ladner, U. EUSTAR Coauthors Daikeler, T. Lanciano, E. Becvár, R. Tomcik, M. Gińdzieńska-Sieskiewicz, E. Cuomo, G. Iudici, M. Rednic, S. Vlachoyiannopoulos, P.G. Caporali, R. Carreira, P.E. Novak, S. Minier, T. Kucharz, E.J. Gabrielli, A. Moroncini, G. Airo', P. Hesselstrand, R. Martinovic, D. Radic, M. Marasovic-Krstulovic, D. Braun-Moscovici, Y. Balbir-Gurman, A. Lo Monaco, A. Caramaschi, P. Morovic-Vergles, J. Henes, J. Ortiz Santamaria, V. Heitmann, S. Krasowska, D. Seidel, M.F. Hasler, P. Pereira Da Silva, J.A. Salvador, M.J. Stamenkovic, B. Stankovic, A. Tikly, M. Ananieva, L.P. Beretta, L. Szucs, G. Szamosi, S. de la Puente Bujidos, C. Midtvedt, Ø. Hoffmann-Vold, A.-M. Launay, D. Hachulla, E. Riccieri, V. Ionescu, R. Opris, D. Mihai, C. Herrgott, I. Beyer, C. Ingegnoli, F. von Mühlen, C.A. Alegre-Sancho, J.J. Beltrán-Catalán, E. Aringer, M. Fantana, J. Leuchten, N. Tausche, A.-K. De Langhe, E. Vanthuyne, M. Anic, B. Barešic, M. Mayer, M. Üprus, M. Otsa, K. Yavuz, S. Granel, B. Azevedo, V.F. Muller, C. Jimenez, S.A. Popa, S. Agachi, S. Zenone, T. Stebbings, S. Dockerty, J. Vacca, A. Schollum, J. Veale, D.J. Toloza, S. Xu, D. Olas, J. Rosato, E. Foti, R. Adler, S. Dan, D. Wiesik-Szewczyk, E. Olesińska, M. Kayser, C. Fathi, N. de la Peña Lefebvre, P.G. Imbert, B.

Subjects
integumentary system, skin and connective tissue diseases
Abstract

The aim was to update the 2009 European League against Rheumatism (EULAR) recommendations for the treatment of systemic sclerosis (SSc), with attention to new therapeutic questions. Update of the previous treatment recommendations was performed according to EULAR standard operating procedures. The task force consisted of 32 SSc clinical experts from Europe and the USA, 2 patients nominated by the pan-European patient association for SSc (Federation of European Scleroderma Associations (FESCA)), a clinical epidemiologist and 2 research fellows. All centres from the EULAR Scleroderma Trials and Research group were invited to submit and select clinical questions concerning SSc treatment using a Delphi approach. Accordingly, 46 clinical questions addressing 26 different interventions were selected for systematic literature review. The new recommendations were based on the available evidence and developed in a consensus meeting with clinical experts and patients. The procedure resulted in 16 recommendations being developed (instead of 14 in 2009) that address treatment of several SSc-related organ complications: Raynaud's phenomenon (RP), digital ulcers (DUs), pulmonary arterial hypertension (PAH), skin and lung disease, scleroderma renal crisis and gastrointestinal involvement. Compared with the 2009 recommendations, the 2016 recommendations include phosphodiesterase type 5 (PDE-5) inhibitors for the treatment of SSc-related RP and DUs, riociguat, new aspects for endothelin receptor antagonists, prostacyclin analogues and PDE-5 inhibitors for SSc-related PAH. New recommendations regarding the use of fluoxetine for SSc-related RP and haematopoietic stem cell transplantation for selected patients with rapidly progressive SSc were also added. In addition, several comments regarding other treatments addressed in clinical questions and suggestions for the SSc research agenda were formulated. These updated data-derived and consensus-derived recommendations will help rheumatologists to manage patients with SSc in an evidence-based way. These recommendations also give directions for future clinical research in SSc. © Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.

Published
2017

48. pneumonia in patients treated for systemic autoimmune disorders: a retrospective analysis of patient characteristics and outcome.

Author

Verhaert, M, Blockmans, D, De Langhe, E, and Henckaerts, L

Subjects
PNEUMOCYSTIS pneumonia, DISEASES, LUNG diseases, INTENSIVE care units, IMMUNOCOMPROMISED patients, THERAPEUTIC use of glucocorticoids, AUTOIMMUNE diseases, FUNGI, RETROSPECTIVE studies, IMMUNOSUPPRESSIVE agents, DISEASE complications
Abstract

Objectives: Pneumocystis jirovecii is an opportunistic fungus. Pneumocystis jirovecii pneumonia (PJP) is well known in the human immunodeficiency virus (HIV)-infected population, but in non-HIV-related immunosuppressed patients, risk factors are largely unknown. We studied the characteristics and outcome of patients treated for systemic autoimmune disorders infected with P. jirovecii, aiming to clarify risk stratification to guide prophylaxis. Method: Clinical charts collected between 2010 and 2016 at the University Hospital of Leuven (Belgium) were reviewed. Information on type of systemic disorder, organ involvement, immunosuppressant use, and comorbidity was collected, and laboratory results were consulted. Results: In total, 39 cases of non-HIV PJP were retrieved, 24 of whom had pre-existing pulmonary disease. All were on immunosuppressant medication at the time of infection, the majority (36/39) taking glucocorticoids, with a median dose of 16 mg methylprednisolone over the past 3 months. Of the 39 cases, 21 were admitted to the intensive care unit and mortality reached 35%. Age and pulmonary disease correlated positively and methotrexate use negatively with mortality. When applying current prophylactic strategies to our cohort, 50% of infections could theoretically have been prevented. Conclusion: PJP is a rare but relevant clinical problem when caring for immunosuppressed patients with autoimmune systemic disorders. Pulmonary disease and age are risk factors for acquiring the infection and carry a worse prognosis. More studies are needed to further define prophylactic criteria. [ABSTRACT FROM AUTHOR]

Published
2020
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