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218 results on '"Kucharz, E. J."'

1. 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

2. 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

3. A Decreased Serum Leptin Level in Patients with Systemic Sclerosis

Author: Kotulska, A., Kucharz, E. J., Brzezińska-Wcisło, L., and Wadas, U.
Published: 2001
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4. 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, JÃ¶rg 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, MÃ¼ller 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, JosÃ© 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, Ã˜yvind, Hoffmann Vold, Anna Maria, Launay, David, Hachulla, Eric, Riccieri, Valeria, Ionescu, Ruxandra, Opris, Daniela, Mihai, Carina, Herrgott, Ilka, Beyer, Christian, Ingegnoli, Francesca, von MÃ¼hlen, Carlos Alberto, Alegre Sancho, Juan JosÃ©, 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 PeÃ±a Lefebvre, Paloma GarcÃa, 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

5. What have multicentre registries across the world taught us about the disease features of systemic sclerosis?

Author: Proudman S. M., Huq M., Stevens W., Wilson M. E., Sahhar J., Baron M., Hudson M., Pope J., Allanore Y., Distler O., Kowal-Bielecka O., Matucci-Cerinic M., H. L. Low A., Teng G. G., Law W. G., Santosa A., Nikpour M., Hill C., Lester S., Nash P., Ngian G. -S., Proudman S., Rischmueller M., Roddy J., Strickland G., Thakkar V., Walker J., Zochling J., Markland J., Robinson D., Jones N., Khalidi N., Docherty P., Kaminska E., Masetto A., Sutton E., Mathieu J. -P., Ligier S., Grodzicky T., LeClercq S., Thorne C., Gyger G., Smith D., Fortin P. R., Larche M., Abu-Hakima M., Rodriguez-Reyna T. S., Cabral A. R., Fritzler M., Avouac J., Walker U. A., Guiducci S., Riemekasten G., Air P., Hachulla E., Valentini G., Carreira P. E., Cozzi F., Gurman A. B., Braun-Moscovici Y., Damjanov N., Ananieva L. P., Scorza R., Jimenez S., Busquets J., Li M., Muller-Ladner U., Maurer B., Tyndall A., Lapadula G., Iannone F., Becvar R., Sierakowsky S., Cutolo M., Sulli A., Cuomo G., Vettori S., Rednic S., Nicoara I., Vlachoyiannopoulos P., Montecucco C., Caporali R., Novak S., Czirjak L., Varju C., Chizzolini C., Kucharz E. J., 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., Lo Monaco A., Hunzelmann N., Pellerito R., Bambara L. M., Caramaschi P., Black C., Denton C., Henes J., Santamaria V. O., Heitmann S., Krasowska D., Seidel M., Oleszowsky M., Burkhardt H., Himsel A., Salvador M. J., Stamenkovic B., Stankovic A., Tikly M., Starovoytova M. N., Engelhart M., Strauss G., Nielsen H., Damgaard K., Szucs G., Mendoza A. Z., de la Puente Buijdos C., Giraldo W. A. S., Midtvedt O., Garen T., Launay D., Valesini G., Riccieri V., Ionescu R. M., Opris D., Groseanu L., Wigley F. M., Mihai C. M., Cornateanu R. S., Ionitescu R., Gherghe A. M., Gorga M., Dobrota R., Bojinca M., Schett G., Distler J. H., Meroni P., Zeni S., Mouthon L., De Keyser F., Smith V., Cantatore F. P., Corrado A., Ullman S., Iversen L., Pozzi M. R., Eyerich K., Hein R., Knott E., Szechinski J., Wiland P., Szmyrka-Kaczmarek M., Sokolik R., Morgiel E., Krummel-Lorenz B., Saar P., Aringer M., Gunther C., Anic B., Baresic M., Mayer M., Radominski S. C., de Souza Muller C., Azevedo V. F., Agachi S., Groppa L., Chiaburu L., Russu E., Zenone T., Stebbings S., Highton J., Stamp L., Chapman P., O'Donnell J., Solanki K., Doube A., Veale D., O'Rourke M., Loyo E., Rosato E., Pisarri S., Tanaseanu C. -M., Popescu M., Dumitrascu A., Tiglea I., Chirieac R., Ancuta C., Furst D. E., Kafaja S., Garcia de la Pena Lefebvre P., Rubio S. R., Exposito M. V., Sibilia J., Chatelus E., Gottenberg J. E., Chifflot H., Litinsky I., Venalis A., Butrimiene I., Venalis P., Rugiene R., Karpec D., Kerzberg E., Montoya F., Cosentino V., Low A. H. L., Teng G., Chan G., Lim A. Y. N., Ng S. C., Proudman, S. M., Huq, M., Stevens, W., Wilson, M. E., Sahhar, J., Baron, M., Hudson, M., Pope, J., Allanore, Y., Distler, O., Kowal-Bielecka, O., Matucci-Cerinic, M., H. L. Low, A., Teng, G. G., Law, W. G., Santosa, A., Nikpour, M., Hill, C., Lester, S., Nash, P., Ngian, G. -S., Proudman, S., Rischmueller, M., Roddy, J., Strickland, G., Thakkar, V., Walker, J., Zochling, J., Markland, J., Robinson, D., Jones, N., Khalidi, N., Docherty, P., Kaminska, E., Masetto, A., Sutton, E., Mathieu, J. -P., Ligier, S., Grodzicky, T., Leclercq, S., Thorne, C., Gyger, G., Smith, D., Fortin, P. R., Larche, M., Abu-Hakima, M., Rodriguez-Reyna, T. S., Cabral, A. R., Fritzler, M., Avouac, J., Walker, U. A., Guiducci, S., Riemekasten, G., Air, P., Hachulla, E., Valentini, G., Carreira, P. E., Cozzi, F., Gurman, A. B., Braun-Moscovici, Y., Damjanov, N., Ananieva, L. P., Scorza, R., Jimenez, S., Busquets, J., Li, M., Muller-Ladner, U., Maurer, B., Tyndall, A., Lapadula, G., Iannone, F., Becvar, R., Sierakowsky, S., Cutolo, M., Sulli, A., Cuomo, G., Vettori, S., Rednic, S., Nicoara, I., Vlachoyiannopoulos, P., Montecucco, C., Caporali, R., Novak, S., Czirjak, L., Varju, C., Chizzolini, C., Kucharz, E. J., 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., Lo Monaco, A., Hunzelmann, N., Pellerito, R., Bambara, L. M., Caramaschi, P., Black, C., Denton, C., Henes, J., Santamaria, V. O., Heitmann, S., Krasowska, D., Seidel, M., Oleszowsky, M., Burkhardt, H., Himsel, A., Salvador, M. J., Stamenkovic, B., Stankovic, A., Tikly, M., Starovoytova, M. N., Engelhart, M., Strauss, G., Nielsen, H., Damgaard, K., Szucs, G., Mendoza, A. Z., de la Puente Buijdos, C., Giraldo, W. A. S., Midtvedt, O., Garen, T., Launay, D., Valesini, G., Riccieri, V., Ionescu, R. M., Opris, D., Groseanu, L., Wigley, F. M., Mihai, C. M., Cornateanu, R. S., Ionitescu, R., Gherghe, A. M., Gorga, M., Dobrota, R., Bojinca, M., Schett, G., Distler, J. H., Meroni, P., Zeni, S., Mouthon, L., De Keyser, F., Smith, V., Cantatore, F. P., Corrado, A., Ullman, S., Iversen, L., Pozzi, M. R., Eyerich, K., Hein, R., Knott, E., Szechinski, J., Wiland, P., Szmyrka-Kaczmarek, M., Sokolik, R., Morgiel, E., Krummel-Lorenz, B., Saar, P., Aringer, M., Gunther, C., Anic, B., Baresic, M., Mayer, M., Radominski, S. C., de Souza Muller, C., Azevedo, V. F., Agachi, S., Groppa, L., Chiaburu, L., Russu, E., Zenone, T., Stebbings, S., Highton, J., Stamp, L., Chapman, P., O'Donnell, J., Solanki, K., Doube, A., Veale, D., O'Rourke, M., Loyo, E., Rosato, E., Pisarri, S., Tanaseanu, C. -M., Popescu, M., Dumitrascu, A., Tiglea, I., Chirieac, R., Ancuta, C., Furst, D. E., Kafaja, S., Garcia de la Pena Lefebvre, P., Rubio, S. R., Exposito, M. V., Sibilia, J., Chatelus, E., Gottenberg, J. E., Chifflot, H., Litinsky, I., Venalis, A., Butrimiene, I., Venalis, P., Rugiene, R., Karpec, D., Kerzberg, E., Montoya, F., Cosentino, V., Low, A. H. L., Teng, G., Chan, G., Lim, A. Y. N., and Ng, S. C.
Subjects: 0301 basic medicine, medicine.medical_specialty, Pediatrics, Survival, Immunology, Disease, Scleroderma, 03 medical and health sciences, 0302 clinical medicine, Rheumatology, Internal medicine, Immunology and Allergy, Medicine, Multicentre registrie, 030203 arthritis & rheumatology, Clinical features, Cohort study, Multicentre registries, Systemic sclerosis, business.industry, Interstitial lung disease, Autoantibody, Clinical features, medicine.disease, 030104 developmental biology, Clinical feature, Cohort, business, Cohort study, Rheumatism
Abstract: Introduction The aim of this study is to compare the clinical features, mortality and causes of death of systemic sclerosis (SSc) patients in four large multicentre registries. Methods Patients seen at least once in the Australian Scleroderma Cohort Study (ASCS) (n = 1714), the Canadian Scleroderma Research Group (CSRG) (n = 1628), the European League Against Rheumatism Scleroderma Trials and Research (EUSTAR) Network (n = 13,996) and the Systemic Sclerosis Cohort in Singapore (SCORE) (n = 500) before August 2016 were included. Clinical manifestations and survival in cohorts and disease subtypes were compared. Results Among 17,838 SSc patients, most were female (86.1%), Caucasian (84.6%) and had the limited cutaneous subtype (lcSSc) (65.0%). The anti-centromere autoantibody was the most prevalent (37.6%). More patients in SCORE had the diffuse subtype (dcSSc) (49.3%) and Scl-70 autoantibody (38.8%) (pConclusions This meta-cohort of SSc patients, the largest reported to date, provides insights into the impact of race and sex on disease manifestations and survival and confirms the early mortality in this disease.
Published: 2017

6. Premictional volume and contractility of the urinary bladder in patients with systemic sclerosis

Author: Kucharz, E. J., Jonderko, G., Rubisz-Brzezinska, J., Końca, A., and Jarczyk, R.
Published: 1996
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7. Thyroid disorders in patients with progressive systemic sclerosis: A review

Author: Kucharz, E. J.
Published: 1993
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8. Serum cystatin C level in patients with rheumatoid arthritis after single infusion of infliximab

Author: Kopec-Medrek, M., Widuchowska, M., Kotulska, A., Zycinska-Dębska, E., and Kucharz, E. J.
Published: 2011
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9. Serum lipid profile in patients with systemic sclerosis: relationship to the thyreometabolic state

Author: Kotyla, P. J., Gozdzik, J., Lewicki, M., Kotulska, A. T., and Kucharz, E. J.
Published: 2006
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10. Acute-Phase Proteins in Patients with Systemic Sclerosis

Author: Kucharz, E. J., Grucka-Mamczar, E., Mamczar, A., and Brzezinska-Wcislo, L.
Published: 2000
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11. Incidence and predictors of cutaneous manifestations during the early course of systemic sclerosis. a 10-year longitudinal study from the EUSTAR database

Author: Wirz, E. G., Jaeger, V. K., Allanore, Y., Riemekasten, G., Hachulla, E., Distler, O., Airo, P., Carreira, P. E., Tikly, M., Vettori, S., Gurman, A. B., Damjanov, N., Muller-Ladner, U., Distler, J., Li, M., Hausermann, P., Walker, U. A., Ananieva, L., Heitmann, S., Rednic, S., Jimenez, S., Riccieri, V., Szmyrka-Kaczmarek, M., Farge, D., Lapadula, G., Matucci-Cerinic, M., Guiducci, S., Hunzelmann, N., Rosa Pozzi, M., Mihai, C., Veale, D., Hesselstrand, R., Mariok, E., Smith, V., Kucharz, E. J., Czirjak, L., Martinovic, D., Solanki, K., Mihaela Ancuta, C., Sibilia, J., Paola, C., Hassanien, M., Kahl, S., Woods, A., Vanthuyne, M., Ruxandra, I., Radominski, S. C., Lo Monaco, A., Corrado, A., Koehm, M., Maurizio, M., Radim, B., Loyo, E., Uprus, M., Pellerito, R., Zenone, T., Gabrielli, A., Kowal-Bielecka, O., Rozman, B., Scorza, R., Ann Saketkoo, L., Midtvedt, O., von Muhlen, C. A., Henes, J., Branimir, A., Hasler, P., Yavuz, S., Villiger, P., Krummel-Lorenz, B., Posa, M., Engelhart, M., Denton, C., Krasowska, D., de la Pena Lefebvre, P. G., Cozzi, F., Mouthon, L., Rosato, E., Carlo, S., Alegre Sancho, J. J., Mallia, C., Limonta, M., Seidel, M., Foti, R., Stamp, L., Ullman, S., Stebbings, S., Ortiz Santamaria, V., Del Galdo, F., De Langhe, E., Mathieu, A., Sunderkotter, C., Eyerich, K., Stamenkovic, B., Novak, S., Sampaio-Barros, P. D., Kayser, C., Litinsky, I., Couto, M., University of Zurich, Walker, U A, Wirz, Eg, Jaeger, Vk, Allanore, Y, Riemekasten, G, Hachulla, E, Distler, O, Airò, P, Carreira, Pe, Tikly, M, Vettori, Serena, Balbir Gurman, A, Damjanov, N, Müller Ladner, U, Distler, J, Li, M, Häusermann, P, Walker, Ua, and Eustar, Coauthors
Subjects: Male, Genetics and Molecular Biology (all), Pathology, Longitudinal study, Time Factors, Databases, Factual, Epidemiology, systemic sclerosis, 2745 Rheumatology, Kaplan-Meier Estimate, Severity of Illness Index, Biochemistry, Scleroderma, Risk Factors, Medizinische Fakultät, Immunology and Allergy, Longitudinal Studies, Prospective Studies, 610 Medicine & health, integumentary system, Incidence (epidemiology), Incidence, 10051 Rheumatology Clinic and Institute of Physical Medicine, Middle Aged, Connective tissue disease, 3. Good health, Autoantibodies, Systemic Sclerosis, Cohort, 2723 Immunology and Allergy, Female, Adult, medicine.medical_specialty, Immunology, General Biochemistry, Genetics and Molecular Biology, Sex Factors, Rheumatology, 1300 General Biochemistry, Genetics and Molecular Biology, Internal medicine, Skin Ulcer, medicine, Humans, ddc:610, Proportional Hazards Models, 2403 Immunology, Scleroderma, Systemic, business.industry, medicine.disease, Clinical trial, Biochemistry, Genetics and Molecular Biology (all), Scleroderma, Diffuse, business
Abstract: Objectives To longitudinally map the onset and identify risk factors for skin sclerosis and digital ulcers (DUs) in patients with systemic sclerosis (SSc) from an early time point after the onset of Raynaud9s phenomenon (RP) in the European Scleroderma Trials and Research (EUSTAR) cohort. Methods 695 patients with SSc with a baseline visit within 1 year after RP onset were followed in the prospective multinational EUSTAR database. During the 10-year observation period, cumulative probabilities of cutaneous lesions were assessed with the Kaplan–Meier method. Cox proportional hazards regression analysis was used to evaluate risk factors. Results The median modified Rodnan skin score (mRSS) peaked 1 year after RP onset, and was 15 points. The 1-year probability to develop an mRSS ≥2 in at least one area of the arms and legs was 69% and 25%, respectively. Twenty-five per cent of patients developed diffuse cutaneous involvement in the first year after RP onset. This probability increased to 36% during the subsequent 2 years. Only 6% of patients developed diffuse cutaneous SSc thereafter. The probability to develop DUs increased to a maximum of 70% at the end of the 10-year observation. The main factors associated with diffuse cutaneous SSc were the presence of anti-RNA polymerase III autoantibodies, followed by antitopoisomerase autoantibodies and male sex. The main factor associated with incident DUs was the presence of antitopoisomerase autoantibodies. Conclusion Early after RP onset, cutaneous manifestations exhibit rapid kinetics in SSc. This should be accounted for in clinical trials aiming to prevent skin worsening.
Published: 2016

12. Elevated serum level of interleukin-10 in patients with systemic sclerosis

Author: Kucharz, E. J., Brzezinska-Wcislo, L., Kotulska, A., Gasiorowska-Kielkowska, W., and Gozdzik, J.
Published: 1997
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13. Characteristics of joint involvement and relationships with systemic inflammation in systemic sclerosis: results from the EULAR Scleroderma Trial and Research Group (EUSTAR) database

Author: Avouac, Jerome, Walker, Ulrich, Tyndall, Alan, Kahan, André, Matucci Cerinic, Marco, Allanore, Yannick, Miniati, I., Müller, A., Iannone, F., Giacomelli, R., Distler, O., Becvar, R., Sierakowsky, S., Kowal Bielecka, O., Coelho, P., Cabane, J., Cutolo, M., Shoenfeld, Y., Rovensky, J., Riemekasten, G., Nicoara, I., Vlachoyiannopoulos, P., Caporali, R., Jiri, S., Inanc, M., Gorska, I. Zimmermann, Carreira, P., Novak, S., Czirjak, L., Ramos, F. Oliveira, Jendro, M., Chizzolini, C., Kucharz, E. J., Richter, J., Cozzi, F., Rozman, B., Mallia, C. M., Gabrielli, A., Farge, D., Kiener, H. P., Schöffel, D., Sticherling, M., Airo, P., Wollheim, F., Martinovic, D., Trotta, F., Hunzelmann, N., Jablonska, S., Reich, K., Bombardieri, S., Siakka, P., Pellerito, R., Bambara, L. M., Morovic Vergles, J., Denton, C., Hinrichs, R., Van Den Hoogen, F., Damjanov, N., Kötter, I., Ortiz, V., Heitmann, S., Krasowska, D., Seidel, M., Hasler, P., Van Laar, J. M., Kaltwasser, J. P., Foeldvari, I., Juan Mas, A., Bajocchi, G., Wislowska, M., Pereira Da Silva, J. A., Jacobsen, S., Worm, M., Graniger, W., Kuhn, A., Stankovic, A., Cossutta, R., Majdan, M., Rajcevska, L. Damjanovska, Tikly, M., Nasonov, E. L., Steinbrink, K., Herrick, A., Müller Ladner, U., Dinc, A., Scorza, R., Sondergaard, K., Indiveri, F., Nielsen, H., Szekanecz, Z., Silver, R. M., Antivalle, M., Espinosa, I. B., García De La Pena Lefebvre, P., Midtvedt, O., Launay, D., Valesini, F., Tuvik, P., Ionescu, R. M., Del Papa, N., Pinto, S., Wigley, F., Mihai, C., Capranu, M. Sinziana, Sunderkötter, C., Jun, J. B., Derk, C., Alhasani, S., Distler, J. H., Ton, E., Soukup, T., Seibold, J., Zeni, S., Nash, P., Mouthon, L., De Keyser, F., Duruöz, M. T., Cantatore, F. P., Strauss, G., Von Mülhen, C. A., Pozzi, M. R., Eyerich, K., Szechinski, J., Keiserman, M., Houssiau, F. A., Rom Ivorra, J. A., Krummel Lorenz, B., Aringer, M., Westhovens, R., Bellisai, F., Mayer, M., Stoeckl, F., Üprus, M., Volpe, A., Buslau, M., Yavuz, S., Granel, B., Feijó, A. Valderílio, Del Galdo, F., Popa, S., Zenone, T., Machado, X. Ricardo, Pileckyte, M., Stebbings, S., Mathieu, A., Tulli, A., Tourinho, T., Souza, R., Acayaba De Toledo, R., Stamp, L., Solanki, K., Veale, D., Neto, J. Francisco Marques, Bagnato, G. F., Loyo, E., Toloza, S., Li, M., Mohamed, W. Ahmed Abdel Atty, Cobankara, V., Olas, J., Salsano, F., Oksel, F., Tanaseanu, C. M., Foti, R., Ancuta, C., Vonk, M., Caramashi, P., Beretta, L., Balbir, A., Shine, B., Chiàla, A., Simic, K. Pasalic, Ghio, M., Stamenkovic, B., Rednic, S., Host, N., Hachulla, E., Furst, D. E., VALENTINI, Gabriele, Avouac, Jerome, Walker, Ulrich, Tyndall, Alan, Kahan, André, Matucci Cerinic, Marco, Allanore, Yannick, Miniati, I., Müller, A., Iannone, F., Giacomelli, R., Distler, O., Becvar, R., Sierakowsky, S., Kowal Bielecka, O., Coelho, P., Cabane, J., Cutolo, M., Shoenfeld, Y., Valentini, Gabriele, Rovensky, J., Riemekasten, G., Nicoara, I., Vlachoyiannopoulos, P., Caporali, R., Jiri, S., Inanc, M., Gorska, I. Zimmermann, Carreira, P., Novak, S., Czirjak, L., Ramos, F. Oliveira, Jendro, M., Chizzolini, C., Kucharz, E. J., Richter, J., Cozzi, F., Rozman, B., Mallia, C. M., Gabrielli, A., Farge, D., Kiener, H. P., Schöffel, D., Sticherling, M., Airo, P., Wollheim, F., Martinovic, D., Trotta, F., Hunzelmann, N., Jablonska, S., Reich, K., Bombardieri, S., Siakka, P., Pellerito, R., Bambara, L. M., Morovic Vergles, J., Denton, C., Hinrichs, R., Van Den Hoogen, F., Damjanov, N., Kötter, I., Ortiz, V., Heitmann, S., Krasowska, D., Seidel, M., Hasler, P., Van Laar, J. M., Kaltwasser, J. P., Foeldvari, I., Juan Mas, A., Bajocchi, G., Wislowska, M., Pereira Da Silva, J. A., Jacobsen, S., Worm, M., Graniger, W., Kuhn, A., Stankovic, A., Cossutta, R., Majdan, M., Rajcevska, L. Damjanovska, Tikly, M., Nasonov, E. L., Steinbrink, K., Herrick, A., Müller Ladner, U., Dinc, A., Scorza, R., Sondergaard, K., Indiveri, F., Nielsen, H., Szekanecz, Z., Silver, R. M., Antivalle, M., Espinosa, I. B., García De La Pena Lefebvre, P., Midtvedt, O., Launay, D., Valesini, F., Tuvik, P., Ionescu, R. M., Del Papa, N., Pinto, S., Wigley, F., Mihai, C., Capranu, M. Sinziana, Sunderkötter, C., Jun, J. B., Derk, C., Alhasani, S., Distler, J. H., Ton, E., Soukup, T., Seibold, J., Zeni, S., Nash, P., Mouthon, L., De Keyser, F., Duruöz, M. T., Cantatore, F. P., Strauss, G., Von Mülhen, C. A., Pozzi, M. R., Eyerich, K., Szechinski, J., Keiserman, M., Houssiau, F. A., Rom Ivorra, J. A., Krummel Lorenz, B., Aringer, M., Westhovens, R., Bellisai, F., Mayer, M., Stoeckl, F., Üprus, M., Volpe, A., Buslau, M., Yavuz, S., Granel, B., Feijó, A. Valderílio, Del Galdo, F., Popa, S., Zenone, T., Machado, X. Ricardo, Pileckyte, M., Stebbings, S., Mathieu, A., Tulli, A., Tourinho, T., Souza, R., Acayaba De Toledo, R., Stamp, L., Solanki, K., Veale, D., Neto, J. Francisco Marque, Bagnato, G. F., Loyo, E., Toloza, S., Li, M., Mohamed, W. Ahmed Abdel Atty, Cobankara, V., Olas, J., Salsano, F., Oksel, F., Tanaseanu, C. M., Foti, R., Ancuta, C., Vonk, M., Caramashi, P., Beretta, L., Balbir, A., Shine, B., Chiàla, A., Simic, K. Pasalic, Ghio, M., Stamenkovic, B., Rednic, S., Host, N., Hachulla, E., Furst, D. E., Chizzolini, Carlo, and Westhovens, Rene
Subjects: Male, Systemic disease, Databases, Factual, Cross-sectional study, Joint Diseases/etiology/pathology/physiopathology, Systemic inflammation, Joint involvement, Scleroderma, systemic sclrosis, systemic inflemmetion, joint involvement, Tendons, Systemic sclerosi, Scleroderma, Localized, 0302 clinical medicine, data base, Immunopathology, joint radiography, Immunology and Allergy, Joints/pathology, scleroderma, 030212 general & internal medicine, nuclear magnetic resonance imaging, Range of Motion, Articular, skin and connective tissue diseases, rheumatic disease, ddc:616, interstitial lung disease, Joint contracture, Clinical Trials as Topic, Synovitis, Inflammation/etiology/pathology/physiopathology, integumentary system, article, Tendons/pathology, Middle Aged, musculoskeletal system, cohort analysis, Connective tissue disease, priority journal, Joint, Synoviti, Systemic sclerosis, Female, medicine.symptom, Joint Diseases, Human, musculoskeletal diseases, Adult, medicine.medical_specialty, hand radiography, Immunology, Scleroderma, Localized/etiology/*pathology, Auto-immunity, transplantation and immunotherapy [N4i 4], 03 medical and health sciences, SYSTEMIC SCLEROSIS, JOINT INVOLVEMENT, SYNOVITIS, JOINT CONTRACTURE, TENDON FRICTION RUB, Tendon friction rub, Rheumatology, Internal medicine, medicine, Humans, Health care ethics [NCEBP 5], Tendon, Aged, 030203 arthritis & rheumatology, Cross-Sectional Studie, Inflammation, skin disease, Scleroderma, Systemic, business.industry, echography, medicine.disease, major clinical study, tenosynovitis, Synovitis/etiology/pathology, clinical feature, body regions, Cross-Sectional Studies, Evaluation of complex medical interventions [NCEBP 2], Scleroderma, Systemic/complications/pathology/physiopathology, Joints, disease duration, business, Joint Disease, disease activity
Abstract: Objective.To determine the prevalence of and independent factors associated with joint involvement in a large population of patients with systemic sclerosis (SSc).Methods.This study was cross-sectional, based on data collected on patients included in the European League Against Rheumatism (EULAR) Scleroderma Trials and Research (EUSTAR) registry. We queried this database to extract data regarding global evaluation of patients with SSc and the presence of any clinical articular involvement: synovitis (tender and swollen joints), tendon friction rubs (rubbing sensation detected as the tendon was moved), and joint contracture (stiffness of the joints that decreased their range of motion). Overall joint involvement was defined by the occurrence of synovitis and/or joint contracture and/or tendon friction rubs.Results.We recruited 7286 patients with SSc; their mean age was 56 ± 14 years, disease duration 10 ± 9 years, and 4210 (58%) had a limited cutaneous disease subset. Frequencies of synovitis, tendon friction rubs, and joint contractures were 16%, 11%, and 31%, respectively. Synovitis, tendon friction rubs, and joint contracture were more prevalent in patients with the diffuse cutaneous subset and were associated together and with severe vascular, muscular, renal, and interstitial lung involvement. Moreover, synovitis had the highest strength of association with elevated acute-phase reactants taken as the dependent variable.Conclusion.Our results highlight the striking level of articular involvement in SSc, as evaluated by systematic examination in a large cohort of patients with SSc. Our data also show that synovitis, joint contracture, and tendon friction rubs are associated with a more severe disease and with systemic inflammation.
Published: 2010

14. Prediction of worsening of skin fibrosis in patients with diffuse cutaneous systemic sclerosis using the EUSTAR database

Author: Maurer, B., Graf, N., Michel, B. A., Muller Ladner, U., Czirjak, L., Denton, C. P., Tyndall, A., Metzig, C., Lanius, V., Khanna, D., Distler, O., Arner, I. H., Cerinic, M. M., Guiducci, S., Walker, U., Lapadula, G., Iannone, F., Becvar, R., Sierakowsky, S., Bielecka, O. K., Cutolo, M., Sulli, A., Valentini, G., Cuomo, G., Vettori, S., Riemekasten, G., Rednic, S., Nicoara, I., Kahan, A., Allanore, Y., Vlachoyiannopoulos, P., Montecucco, C., Caporali, R., Carreira, P. E., Novak, S., Varju, C., Chizzolini, C., Kucharz, E. J., Kotulska, A., Kopec Medrek, M., Widuchowska, M., Cozzi, F., Rozman, B., Mallia, C., Coleiro, B., Gabrielli, A., Farge, D., Hij, A., Airo, P., Hesselstrand, R., Scheja, A., Wollheim, F., Martinovic, D., Gurman, A. B., Braun Moscovici, Y., Govoni, Marcello, LO MONACO, Andrea, Hunzelmann, N., Pellerito, R., Bambara, L. M., Caramaschi, P., Black, C., Damjanov, N., Santamaria, V. O., Heitmann, S., Krasowska, D., Seidel, M., Oleszowsky, M., Burkhardt, H., Himsel, A., Salvador, M. J., Stamenkovic, B., Stankovic, A., Tikly, M., Starovoytova, M. N., Ananieva, L. P., Scorza, R., Engelhart, M., Strauss, G., Nielsen, H., Damgaard, K., Szucs, G., Mendoza, A. Z., Buijdos, C. d. l. P., Sifuentes Giraldo, W. A., Midtvedt, O., Garen, T., Hachulla, E., Launay, D., Valesini, G., Riccieri, V., Ionescu, R. M., Opris, D., Groseanu, L., Wigley, F. M., Mihai, C. M., Cornateanu, R. S., Ionitescu, R., Gherghe, A. M., Gorga, M., Dobrota, R., Bojinca, M., Schett, G., Distler, J. H., Meroni, P., Zeni, P., Mouthon, L., Keyser, F. D., Cantatore, F. P., Corrado, A., Ullman, S., Iversen, L., Pozzi, M. R., Eyerich, K., Hein, R., Knott, E., Szechinski, J., Wiland, P., Szmyrka Kaczmarek, M., Sokolik, R., Morgiel, E., Krummel Lorenz, B., Saar, P., Aringer, M., Gunther, C., Anic, B., Baresic, M., Mayer, M., Radominski, S. C., Muller, C. d. S., Azevedo, V. F., Agachi, S., Groppa, L., Chiaburu, L., Russu, E., Zenone, T., Highton, J., Stamp, L., Chapman, P., O'Donnell, J., Solanki, K., Doube, A., Veale, D., O'Rourke, M., Loyo, E., Li, M., Rosato, E., Pisarri, S., Tanaseanu, C. M., Popescu, M., Dumitrascu, A., Tiglea, I., Chirieac, R., Ancuta, C., Furst, D. E., Kafaja, S., Lefebvre, P. G. d. l. P., Rubio, S. R., Exposito, M. V., Sibilia, J., Chatelus, E., Gottenberg, J. E., Chifflot, H., Litinsky, I., Venalis, A., Butrimiene, I., Venalis, P., Rugiene, R., Karpec, D., Kerzberg, E., Montoya, F., Cosentino, V., Chizzolini, Carlo, Maurer, Britta, Graf, Nicole, Michel, Beat A, Müller Ladner, Ulf, Czirják, László, Denton, Christopher P, Tyndall, Alan, Metzig, Carola, Lanius, Vivian, Khanna, Dinesh, Distler, Oliver, Tarner, Ingo H, Cerinic, Marco Matucci, Guiducci, Serena, Walker, Ulrich, Lapadula, Giovanni, Iannone, Florenzo, Becvar, Radim, Sierakowsky, Stanislaw, Bielecka, Otylia Kowal, Cutolo, Maurizio, Sulli, Alberto, Valentini, Gabriele, Cuomo, Giovanna, Vettori, Serena, Riemekasten, Gabriele, Rednic, Simona, Nicoara, Ileana, Kahan, André, Allanore, Yannick, Vlachoyiannopoulos, P, Montecucco, Carlomaurizio, Caporali, Roberto, Carreira, Patricia E, Novak, Srdan, Varju, Cecilia, Kucharz, Eugene J, Kotulska, Anna, Kopec Medrek, Magdalena, Widuchowska, Malgorzata, Cozzi, Franco, Rozman, Blaz, Mallia, Carmel, Coleiro, Bernard, Gabrielli, Armando, Farge, Dominique, Hij, Adrian, Airò, Paolo, Hesselstrand, Roger, Scheja, Agneta, Wollheim, Frank, Martinovic, Duska, Gurman, Alexandra Balbir, Braun Moscovici, Yolanda, Govoni, M, Monaco, Andrea Lo, Hunzelmann, Nicola, Pellerito, Raffaele, Bambara, Lisa Maria, Caramaschi, Paola, Black, Carol, Damjanov, Nemanja, Santamaria, Vera Ortiz, Heitmann, Stefan, Krasowska, Dorota, Seidel, Matthia, Oleszowsky, Mara, Burkhardt, Harald, Himsel, Andrea, Salvador, Maria J, Stamenkovic, Bojana, Stankovic, Aleksandra, Tikly, Mohammed, Starovoytova, Maya N, Ananieva, Lidia P, Scorza, Raffaella, Engelhart, Merete, Strauss, Gitte, Nielsen, Henrik, Damgaard, Kirsten, Szücs, Gabriella, Mendoza, Antonio Zea, Buijdos, Carlos de la Puente, Giraldo, Walter A. Sifuente, Midtvedt, Øyvind, Garen, Torhild, Hachulla, Eric, Launay, David, Valesini, Guido, Riccieri, Valeria, Ionescu, Ruxandra Maria, Opris, Daniela, Groseanu, Laura, Wigley, Fredrick M, Mihai, Carmen M, Cornateanu, Roxana Sfrent, Ionitescu, Razvan, Gherghe, Ana Maria, Gorga, Marilena, Dobrota, Rucsandra, Bojinca, Mihai, Schett, Georg, Distler, Jörg HW, Meroni, Pierluigi, Zeni, Silvana, Mouthon, Luc, Keyser, Filip De, Cantatore, Francesco P, Corrado, Ada, Ullman, Susanne, Iversen, Line, Pozzi, Maria R, Eyerich, Kilian, Hein, Rüdiger, Knott, Elisabeth, Szechinski, Jacek, Wiland, Piotr, Szmyrka Kaczmarek, Magdalena, Sokolik, Renata, Morgiel, Ewa, Krummel Lorenz, Brigitte, Saar, Petra, Aringer, Martin, Günther, Claudia, Anic, Branimir, Baresic, Marko, Mayer, Miroslav, Radominski, Sebastião C, Müller, Carolina de Souza, Azevedo, Valderílio F, Agachi, Svetlana, Groppa, Liliana, Chiaburu, Lealea, Russu, Eugen, Zenone, Thierry, Highton, John, Stamp, Lisa, Chapman, Peter, O'Donnell, John, Solanki, Kamal, Doube, Alan, Veale, Dougla, O’Rourke, Marie, Loyo, Esthela, Li, Mengtao, Rosato, Edoardo, Pisarri, Simonetta, Tanaseanu, Cristina Mihaela, Popescu, Monica, and University of Zurich
Subjects: Genetics and Molecular Biology (all), Male, Time Factors, Databases, Factual, systemic sclerosis, 2745 Rheumatology, computer.software_genre, Biochemistry, Severity of Illness Index, Outcomes Research, Qualitative Research, Systemic Sclerosis, Adult, Cohort Studies, Creatine Kinase, Decision Support Techniques, Deglutition Disorders, Dyspnea, Female, Fibrosis, Humans, Logistic Models, Middle Aged, Multivariate Analysis, Scleroderma, Diffuse, Sex Factors, Skin, Synovitis, Disease Progression, Rheumatology, Immunology, Biochemistry, Genetics and Molecular Biology (all), Immunology and Allergy, Medicine (all), Scleroderma, skin fibrosis, skin and connective tissue diseases, ddc:616, EUSTAR, Univariate analysis, Database, integumentary system, 10051 Rheumatology Clinic and Institute of Physical Medicine, Orvostudományok, Diffuse, Connective tissue disease, Cohort, 2723 Immunology and Allergy, Cohort study, medicine.medical_specialty, 610 Medicine & health, Klinikai orvostudományok, General Biochemistry, Genetics and Molecular Biology, NO, outcomes research, qualitative research, Databases, 1300 General Biochemistry, Genetics and Molecular Biology, Internal medicine, Severity of illness, medicine, Factual, 2403 Immunology, business.industry, medicine.disease, business, computer
Abstract: ObjectivesTo identify predictive parameters for the progression of skin fibrosis within 1 year in patients with diffuse cutaneous SSc (dcSSc).MethodsAn observational study using the EUSTAR database was performed. Inclusion criteria were dcSSc, American College of Rheumatology (ACR) criteria fulfilled, modified Rodnan skin score (MRSS) ≥7 at baseline visit, valid data for MRSS at 2nd visit, and available follow-up of 12±2 months. Worsening of skin fibrosis was defined as increase in MRSS >5 points and ≥25% from baseline to 2nd visit. In the univariate analysis, patients with progressive fibrosis were compared with non-progressors, and predictive markers with pResultsA total of 637 dcSSc patients were eligible. Univariate analyses identified joint synovitis, short disease duration (≤15 months), short disease duration in females/patients without creatine kinase (CK) elevation, low baseline MRSS (≤22/51), and absence of oesophageal symptoms as potential predictors for progressive skin fibrosis. In the multivariate analysis, by employing combinations of the predictors, 17 models with varying prediction success were generated, allowing cohort enrichment from 9.7% progressive patients in the whole cohort to 44.4% in the optimised enrichment cohort. Using a second validation cohort of 188 dcSSc patients, short disease duration, low baseline MRSS and joint synovitis were confirmed as independent predictors of progressive skin fibrosis within 1 year resulting in a 4.5-fold increased prediction success rate.ConclusionsOur study provides novel, evidence-based criteria for the enrichment of dcSSc cohorts with patients who experience worsening of skin fibrosis which allows improved clinical trial design.
Published: 2014

15. Depression and anxiety in patients with systemic sclerosis

Author: Straszecka, J., Kucharz, E. J., Jonderko, G., Kotulska, A., Bednarczyk-Kaluzny, M., Brzezińska-Wcislo, L., and Rubisz-Brzezinska, J.
Published: 1996
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16. High serum level of prolactin in patients with systemic sclerosis

Author: Kucharz, E. J., Jarczyk, R., Jonderko, G., Rubisz-Brzezińska, J., and Brzezińska-Wcislo, L.
Published: 1996
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17. Interleukin-1-production by monocytes from patients with systemic lupus erythematosus

Author: Sierakowski, S., Kucharz, E. J., Lightfoot, Jr., R. W., and Goodwin, J. S.
Published: 1987
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18. Decreased activity of interleukin-2 inhibitor in plasma of patients with systemic lupus erythematosus

Author: Kucharz, E. J., Sierakowski, S., and Goodwin, J. S.
Published: 1988
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19. Serum cystatin C level in patients with rheumatoid arthritis after single infusion of infliximab

Author: Kopec-Medrek, M., primary, Widuchowska, M., additional, Kotulska, A., additional, Zycinska-Dębska, E., additional, and Kucharz, E. J., additional
Published: 2010
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20. Kolageni elastin u jetri štakora otrovanih živinim kloridom

Author: Krystyna Olczyk, Kucharz, E. J., Wieczorek, M., Szczebara, M., and Sonecki, P.
Abstract: Intoxication of rats with mercuric chloride (0.5 mg Hg/kg of body weight, daily for 10 weeks) increased the hepatic contents of soluble and insoluble collagen and elastin. The increase was associated with elevated serum aminotransferase and alkaline phosphatase activities, and decreased total protein level in serum. Inflammatory changes were found in the liver. An increase in the fibrous protein content suggests that inflammatory reaction to mercuric chloride can result in hepatic fibrosis., Trovanje štakora živinim kloridom (0,5 mg Hg/kg tjelesne težine na dan tijekom deset tjedana) imalo je za rezultat povećan sadržaj topljivog i netopljivog kolagena i elastina u jetri. Povećanje je dovedeno u vezu s povišenim aktivnostima aminotransferaze i alkalne fosfataze u serumu, i sa smanjenim nivoom ukupnog proteina u serumu. U jetri su zamijećene upalne promjene. Povišen sadržaj vlaknastog proteina upućuje na to da upalna reakcija na živin klorid može dovesti do fibroze jetre.
Published: 1990

21. Serum lipid profile in patients with systemic sclerosis: relationship to the thyreometabolic state

Author: Kotyla, P. J., primary, Gozdzik, J., additional, Lewicki, M., additional, Kotulska, A. T., additional, and Kucharz, E. J., additional
Published: 2005
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22. Glycosaminoglycan-collagen interactions in vitro in the hydralazine-induced collagen disease-like syndrome.

Author: Głowacki, A, primary, Kucharz, E J, additional, and Olczyk, K, additional
Published: 1993
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23. Serum glycoproteins in patients with hypo- and hyperthyroidism.

Author: Kucharz, E J, primary, Olczyk, K, additional, Głowacki, A, additional, and Duraj-Kassem, M, additional
Published: 1993
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24. Dynamics of serum interleukin-6 level in patients with acute myocardial infarction

Author: Kucharz, E. J. and Wilk, T.
Published: 2000
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25. Free radical activity and antioxidant defense mechanisms in patients with hyperthyroidism due to Graves` disease during therapy

Author: Komosinska-Vassev, K., Olczyk, K., Kucharz, E. J., Marcisz, C., Winsz-Szczotka, K., and Kotulska, A.
Published: 2000
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26. Is there a role for TNF-alpha antagonists in the treatment of SSc? EUSTAR expert consensus development using the Delphi technique

Author: Distler, J. H. W., Jordan, S., Airo, P., Alegre-Sancho, J. J., Allanore, Y., Gurman, A. B., Caporali, R., Caramaschi, P., Carreira, P. E., Chizzolini, C., Cutolo, M., Duruoz, M. T., Farge-Bancel, D., Hesselstrand, R., Iannone, F., Keyser, F., Kucharz, E. J., David Launay, Lefebvre, P. G. D., Lukacova, O., Marasini, B., Martinovic, D., Neto, J. F. M., Radic, M., Rednic, S., Riemekasten, G., Rovensky, J., Seidel, M. F., Senel, S., Smith, V., Sunderkotter, C., Ton, E., Laar, J. M., Matucci-Cerinic, M., Muller-Ladner, U., Distler, O., [Jordan, S. -- Distler, O.] Univ Zurich Hosp, Dept Rheumatol, CH-8091 Zurich, Switzerland -- [Distler, J. H. W.] Univ Erlangen Nurnberg, Dept Rheumatol, Erlangen, Germany -- [Airo, P.] Spedali Civil Brescia, Unit Rheumatol & Clin Immunol, I-25125 Brescia, Italy -- [Alegre-Sancho, J. J.] Hosp Univ Dr Peset Valencia, Valencia, Spain -- [Allanore, Y.] Univ Paris 05, Paris, France -- [Allanore, Y.] Hop Cochin, Serv Rhumatol A, F-75674 Paris, France -- [Gurman, A. Balbir] Rambam Hlth Care Campus, B Shine Rheumatol Unit, Haifa, Israel -- [Caporali, R.] Univ Pavia, Div Rheumatol, IRCCS S, Matteo Fdn, I-27100 Pavia, Italy -- [Caramaschi, P.] Rheumatol Unit, Verona, Italy -- [Carreira, P. E.] Hosp Univ 12 Octubre, Serv Reumatol, Madrid, Spain -- [Chizzolini, C.] Univ Hosp Geneva, Geneva, Switzerland -- [Cutolo, M.] Univ Genoa, Dept Internal Med, Res Lab, I-16126 Genoa, Italy -- [Cutolo, M.] Univ Genoa, Dept Internal Med, Acad Unit Clin Rheumatol, I-16126 Genoa, Italy -- [Duruoz, M. Tuncay] Celal Bayar Univ, Sch Med, PM&R Dept, Div Rheumatol, Manisa, Turkey -- [Farge-Bancel, D.] Hop St Louis, INSERM, U976, Serv Med Interne & Pathol Vasc, Paris, France -- [Hesselstrand, R.] Univ Lund Hosp, Dept Rheumatol, S-22185 Lund, Sweden -- [Iannone, F.] Univ Bari, Rheumatol Unit, DiMIMP, I-70121 Bari, Italy -- [De Keyser, F. -- Smith, V.] Ghent Univ Hosp, Dept Rheumatol, Ghent, Belgium -- [Kucharz, E. J.] Med Univ Silesia, Dept Internal Med & Rheumatol, Katowice, Poland -- [Launay, D.] Univ Lille 2, Dept Internal Med, Lille, France -- [de la Pena Lefebvre, P. Garcia] Hosp Univ Madrid Norte Sanchinarro, Madrid, Spain -- [Lukacova, O. -- Rovensky, J.] Natl Inst Rheumat Dis, Piestany, Slovakia -- [Marasini, B.] Univ Milan, Ist Clin Humanitas, I-20122 Milan, Italy -- [Martinovic, D. -- Radic, M.] Univ Hosp Split, Dept Rheumatol, Split, Croatia -- [Marques Neto, J. F.] Univ Estadual Campinas, FCM, Campinas, Brazil -- [Rednic, S.] Univ Med & Pharm Iuliu Hatieganu Cluj, Clin Reumatol, Cluj Napoca, Romania -- [Riemekasten, G.] Charite, Med Klin Schwerpunkt Rheumatol & Klin Immunol, D-13353 Berlin, Germany -- [Seidel, M. F.] Med Klin & Poliklin 1, Bonn, Germany -- [Senel, S.] Cumhuriyet Univ, Sch Med, Div Rheumatol, Sivas, Turkey -- [Sunderkoetter, C.] Univ Hosp Munster, Dept Dermatol, Munster, Germany -- [Ton, E.] Univ Med Ctr Utrecht, Dept Rheumatol & Clin Immunol, Utrecht, Netherlands -- [van Laar, J. M.] Med Sch Newcastle Upon Tyne, Inst Cellular Med, Musculoskeletal Res Grp, Newcastle Upon Tyne NE2 4HH, Tyne & Wear, England -- [Matucci-Cerinic, M.] Univ Florence, Dept Biomed, Div Rheumatol AOUC, Florence, Italy -- [Mueller-Ladner, U.] Univ Giessen, Dept Rheumatol & Clin Immunol, Kerckhoff Clin Bad Nauheim, Bad Nauheim, Germany, Radic, Mislav -- 0000-0003-0350-6800, Launay, David -- 0000-0003-1840-1817, Duruoz, Mehmet Tuncay -- 0000-0003-3584-2788, Carreira, Patricia -- 0000-0001-8279-3806, and Iannone, Florenzo -- 0000-0003-0474-5344
Subjects: fibrosis, scleroderma, TNF-alpha
Abstract: WOS: 000291117200007, PubMed ID: 21586217, Objective: To obtain experiences and expert opinion on treatment of SSc patients with TNF-alpha antagonists. Methods: An investigation was carried out among the EUSTAR centres into their expertise on use of TNF-alpha antagonists. Assessment forms on the frequency of TNF-alpha inhibitor use were distributed to EULAR Scleroderma Trials and Research Group (EUSTAR) centres. Afterwards, a three round Delphi exercise was performed to obtain expert consensus on the use of TNF-alpha inhibitors in SSc. Results: Seventy-nine centres returned information on use of TNF-alpha antagonists in SSc patients. A total of 65 patients were treated with TNF-alpha inhibitors in 14 different centres. Forty-eight of the 65 patients treated with TNF-alpha inhibitors improved. Improvement was mainly seen in patients with arthritis, whereas the effects on fibrosis varied. In the first round of the subsequent Delphi approach, 71 out of 79 experts stated that they would use TNF-alpha antagonists in SSc. Arthritis was suggested as an indication for TNF alpha antagonists by 75% of the experts. However; after the third stage of the Delphi exercise, the acceptance for the off-label use of TNF-alpha antagonists decreased and 59% recommended that TNF-alpha antagonists should not be used or only used in clinical trials in SSc patients, while 38% of the experts suggested the use of TNF-alpha antagonists for arthritis associated with SSc. Conclusions: Most of the experts do not recommend the routine use of TNF-alpha antagonists in systemic sclerosis. Arthritis might be a potential indication in SSc, although controlled clinical trials with TNF-alpha antagonists are needed before general recommendations can be given., Novartis; Bayer Schering Pharma; Cell Gen Therapeutics; NicOx; Array BioPharma; Bristol-Myers Squibb; Ergonex Pharma GmbH; Actelion Pharmaceuticals; Pfizer; GlaxoSmithKline; Merck; Amgen; Abbott; Roche; EUSTAR/EULAR; Sanofi-Aventis; United BioSource Corporation; Medac; Biovitrium; Active Biotech; Ergonex, Dr Jorg Distler has served as a consultant for Actelion Pharmaceuticals, Pfizer, and GlaxoSmithKline; has received grant support from Novartis, Bayer Schering Pharma, Cell Gen Therapeutics, NicOx, Array BioPharma, Bristol-Myers Squibb, and Ergonex Pharma GmbH; has received payment for development of educational presentations (including service on speakers' bureaus) from Actelion Pharmaceuticals, Pfizer, GlaxoSmithKline, and Bayer Schering Pharma.; Dr F. lannone has received speaker's and consultancy fees from Merck.; Dr Riemekasten has been invited for a meeting by Humira-People (Amgen) and has received speaker's fees and support for travel costs riembursed by Amgen.; Dr M.F. Seidel has received research grants from Pfizer and Abbott.; Dr J. van Laar has received speaker's and consultancy fees and research grants from Roche.; Dr U. Muller-Ladner has received research grants from EUSTAR/EULAR.; Dr Oliver Distler has a consultancy relationship and/or has received research funding from Actelion Pharmaceuticals, Pfizer, Ergonex Pharma GmbH, Bristol-Myers Squibb, Sanofi-Aventis, United BioSource Corporation, Medac, Biovitrium, Novartis and Active Biotech in the area of potential treatments of scleroderma and its complications. He has received lecture honoraria from Actelion Pharmaceuticals, Pfizer and Ergonex.

27. Indices of collagen metabolism in pregnancy and postpartum period.

Author: Kucharz, Eugeniusz J. and Kucharz, E J
Published: 1986
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28. Inhibitors of collagenolytic enzymes in the serum of patients with chronic liver disorders.

Author: Kucharz, E J
Abstract: Inhibitory effect of serum on collagenolysis was studied in rat hepatic slices. Sera of patients with cirrhosis contained low concentrations of inhibitors. Higher concentrations were found in sera from patients with chronic active hepatitis and chronic persistent hepatitis, than in healthy individuals. It is suggested that changes of the serum inhibitory activity are responsible for the control of collagen degradation in the injured liver. [ABSTRACT FROM PUBLISHER]
Published: 1985

29. Serum amyloid A - An acute-phase protein involved in systemic sclerosis pathogenesis?,Surowiczy amyloid A - Białko ostrej fazy zwia̧zane z patogenez̧ twardziny układowej?

Author: Anna Lis-Święty, Widuchowska, M., Wcisoł-Dziadecka, D., Brzezinśka-Wcisło, L., and Kucharz, E. J.

30. The consensus on rituximab administration in the treatment of patients with rheumatoid arthritis,Konsensus dotycza̧cy stosowania rituksymabu w leczeniu chorych na reumatoidalne zapalenie stawów

Author: Szechiński, J., Piotr Głuszko, Kucharz, E. J., Maśliński, W., Samborski, W., Tłustochowicz, W., and Wiland, P.

31. Glycosaminoglycans--structure and function,Glikozoaminoglikany--struktura i funkcja

Author: Głowacki, A., Koźma, E. M., Krystyna Olczyk, and Kucharz, E. J.

32. Position of the Expert Panel of the National Consultant for Rheumatology concerning diagnosis and treatment of rheumatoid arthritis,Stanowisko Zespołu Ekspertów Konsultanta Krajowego ds. Reumatologii w sprawie diagnostyki i terapii reumatoidalnego zapalenia stawów

Author: Tłustochowicz, W., Brzosko, M., Filipowicz-Sosnowska, A., Głuszko, P., Kucharz, E. J., Włodzimierz Maśliński, Samborski, W., Szechiński, J., and Wiland, P.

33. The Expert Panel of the National Consultant for Rheumatology recommendations for the management of ankylosing spondylitis,Rekomendacje w postȩpowaniu diagnostycznym i terapeutycznym u chorych na zesztywniaja̧ce zapalenie stawów krȩgosł upa Opracowane przez zespół konsultanta krajowego z dziedziny reumatologii

Author: Wiland, P., Filipowicz-Sosnowska, A., Piotr Głuszko, Kucharz, E. J., Maśliński, W., Samborski, W., Szechiński, J., Tłustochowicz, W., and Brzosko, M.

34. Serum matrix-degrading enzymes in rats intoxicated with selenium

Author: Olczyk, K., Kucharz, E. J., Komosińska-Vassev, K., and Ewa Maria Koźma

35. A decreased serum interleukin-7 level in patients with myocardial infarction

Author: Kucharz, E. J. and Wilk, T.
Published: 2001
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36. Influence of chronic intoxication with selenium on collagen and elastin content in tissues of rat

Author: Kucharz, E. J. and Olczyk, K.
Published: 1993
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37. Stratification in systemic sclerosis according to autoantibody status versus skin involvement: a study of the prospective EUSTAR cohort

Author: Muriel Elhai, Nanthara Sritharan, Marouane Boubaya, Alexandra Balbir-Gurman, Elise Siegert, Eric Hachulla, Jeska de Vries-Bouwstra, Gabriela Riemekasten, Jörg H W Distler, Edoardo Rosato, Francesco Del Galdo, Fabian A Mendoza, Daniel E Furst, Carlos de la Puente, Anna-Maria Hoffmann-Vold, Armando Gabrielli, Oliver Distler, Coralie Bloch-Queyrat, Yannick Allanore, Marco Matucci Cerinic, Ulrich Walker, Florenzo Iannone, Suzana Jordan, Radim Becvar, Otylia Kowal Bielecka, Maurizio Cutolo, Giovanna Cuomo, Claudia Kedor, Simona Rednic, Jérome Avouac, P. Vlachoyiannopoulos, C. Montecucco, Jiri Stork, Murat Inanc, Patricia E. Carreira, Srdan Novak, László Czirják, Michele Iudici, Eugene J. Kucharz, Elisabetta Zanatta, Katja Perdan-Pirkmajer, Bernard Coleiro, Gianluca Moroncini, Dominique Farge Bancel, Paolo Airò, Roger Hesselstrand, Mislav Radic, Yolanda Braun-Moscovici, Andrea Lo Monaco, Nicolas Hunzelmann, Raffaele Pellerito, Alessandro Giollo, Jadranka Morovic-Vergles, Christopher Denton, Madelon Vonk, Nemanja Damjanov, Jörg Henes, Vera Ortiz Santamaria, Stefan Heitmann, Dorota Krasowska, Paul Hasler, Michaela Kohm, Ivan Foeldvari, Gianluigi Bajocchi, Maria João Salvador, Bojana Stamenkovic, Carlo Francesco Selmi, Mohammed Tikly, Lidia P. Ananieva, Ariane Herrick, Ulf Müller-Ladner, Raffaele De Palma, Merete Engelhart, Gabriela Szücs, Cristina Sobrino Grande, Øyvind Midtvedt, David Launay, Valeria Riccieri, Ruxandra Maria Ionescu, Ami Sha, Ana Maria Gheorghiu, Cord Sunderkötter, Francesca Ingegnoli, Luc Mouthon, Vanessa Smith, Francesco Paolo Cantatore, Susanne Ullman, Carlos Alberto von Mühlen, Maria Rosa Pozzi, Kilian Eyerich, Piotr Wiland, Marie Vanthuyne, Juan Jose Alegre-Sancho, Kristine Herrmann, Ellen De Langhe, Branimir Anic, Maria Üprus, Sule Yavuz, Brigitte Granel, Carolina de Souza Müller, Joanna Busquets, Svetlana Agachi, Simon Stebbings, D'Alessandro Mathieu, Percival D. Sampaio-Barros, Lisa Stamp, Kamal Solanki, Douglas Veale, Esthela Loyo, Mengtao Li, Walid Ahmed Abdel Atty Mohamed, Antonietta Gigante, Fahrettin Oksel, Cristina-Mihaela Tanaseanu, Rosario Foti, Codrina Ancuta, Britta Maurer, Jacob van Laar, Cristiane Kayser, Nihal Fathi, Paloma García de la Peña Lefebvre, Jean Sibilia, Ira Litinsky, Giuseppina Abignano, Goda Seskute, Lesley Ann Saketkoo, Eduardo Kerzberg, Washington Bianchi, Ivan Castellví, Massimiliano Limonta, Doron Rimar, Maura Couto, François Spertini, Antonella Marcoccia, Sarah Kahl, Ivien M. Hsu, Thierry Martin, Sergey Moiseev, Lorinda S. Chung, Tim Schmeiser, Dominik Majewski, Zbigniew Zdrojewski, Julia Martínez-Barrio, Vera Bernardino, Sabine Sommerlatte, Yair Levy, Elena Rezus, Omer Nuri Pamuk, Piercarlo Sarzi Puttini, Hadi Poormoghim, Ina Kötter, Francis Gaches, Laura Belloli, Petros Sfikakis, Juliana Markus, Gary R Feldman, Ana-Maria Ramazan, H.U. Scherer, Marie-Elise Truchetet, Alain Lescoat, Lorenzo Dagna, J.M. van Laar, Lidia Rudnicka, Susana Oliveira, Fabiola Atzeni, Masataka Kuwana, Arsene Mekinian, Mickaël Martin, Yoshiya Tanaka, Elhai, M., Sritharan, N., Boubaya, M., Balbir-Gurman, A., Siegert, E., Hachulla, E., de Vries-Bouwstra, J., Riemekasten, G., Distler, J. H. W., Rosato, E., Del Galdo, F., Mendoza, F. A., Furst, D. E., de la Puente, C., Hoffmann-Vold, A. -M., Gabrielli, A., Distler, O., Bloch-Queyrat, C., Allanore, Y., Matucci Cerinic, M., Walker, U., Iannone, F., Jordan, S., Becvar, R., Kowal Bielecka, O., Cutolo, M., Cuomo, G., Kedor, C., Rednic, S., Avouac, J., Vlachoyiannopoulos, P., Montecucco, C., Stork, J., Inanc, M., Carreira, P. E., Novak, S., Czirjak, L., Iudici, M., Kucharz, E. J., Zanatta, E., Perdan-Pirkmajer, K., Coleiro, B., Moroncini, G., Farge Bancel, D., Airo, P., Hesselstrand, R., Radic, M., Braun-Moscovici, Y., Lo Monaco, A., Hunzelmann, N., Pellerito, R., Giollo, A., Morovic-Vergles, J., Denton, C., Vonk, M., Damjanov, N., Henes, J., Ortiz Santamaria, V., Heitmann, S., Krasowska, D., Hasler, P., Kohm, M., Foeldvari, I., Bajocchi, G., Salvador, M. J., Stamenkovic, B., Selmi, C. F., Tikly, M., Ananieva, L. P., Herrick, A., Muller-Ladner, U., De Palma, R., Engelhart, M., Szucs, G., Sobrino Grande, C., Midtvedt, O., Launay, D., Riccieri, V., Ionescu, R. M., Sha, A., Gheorghiu, A. M., Sunderkotter, C., Ingegnoli, F., Mouthon, L., Smith, V., Cantatore, F. P., Ullman, S., Alberto von Muhlen, C., Pozzi, M. R., Eyerich, K., Wiland, P., Vanthuyne, M., Alegre-Sancho, J. J., Herrmann, K., De Langhe, E., Anic, B., Uprus, M., Yavuz, S., Granel, B., de Souza Muller, C., Busquets, J., Agachi, S., Stebbings, S., Mathieu, D. A., Sampaio-Barros, P. D., Stamp, L., Solanki, K., Veale, D., Loyo, E., Li, M., Abdel Atty Mohamed, W. A., Gigante, A., Oksel, F., Tanaseanu, C. -M., Foti, R., Ancuta, C., Maurer, B., van Laar, J., Kayser, C., Fathi, N., Garcia de la Pena Lefebvre, P., Sibilia, J., Litinsky, I., Abignano, G., Seskute, G., Saketkoo, L. A., Kerzberg, E., Bianchi, W., Castellvi, I., Limonta, M., Rimar, D., Couto, M., Spertini, F., Marcoccia, A., Kahl, S., Hsu, I. M., Martin, T., Moiseev, S., Chung, L. S., Schmeiser, T., Majewski, D., Zdrojewski, Z., Martinez-Barrio, J., Bernardino, V., Sommerlatte, S., Levy, Y., Rezus, E., Nuri Pamuk, O., Sarzi Puttini, P., Poormoghim, H., Kotter, I., Gaches, F., Belloli, L., Sfikakis, P., Markus, J., Feldman, G. R., Ramazan, A. -M., Scherer, H. U., Truchetet, M. -E., Lescoat, A., Dagna, L., van Laar, J. M., Rudnicka, L., Oliveira, S., Atzeni, F., Kuwana, M., Mekinian, A., Martin, M., and Tanaka, Y.
Subjects: Rheumatology, Immunology, Immunology and Allergy
Abstract: Background: The current subclassification of systemic sclerosis into cutaneous subtypes does not fully capture the heterogeneity of the disease. We aimed to compare the performances of stratification into LeRoy's cutaneous subtypes versus stratification by autoantibody status in systemic sclerosis. Methods: For this cohort study, we assessed people with systemic sclerosis in the multicentre international European Scleroderma Trials and Research (EUSTAR) database. Individuals positive for systemic-sclerosis autoantibodies of two specificities were excluded, and remaining individuals were classified by cutaneous subtype, according to their systemic sclerosis-specific autoantibodies, or both. We assessed the performance of each model to predict overall survival, progression-free survival, disease progression, and different organ involvement. The three models were compared by use of the area under the curve (AUC) of the receiver operating characteristic and the net reclassification improvement (NRI). Missing data were imputed. Findings: We assessed the database on July 26, 2019. Of 16 939 patients assessed for eligibility, 10 711 patients were included: 1647 (15·4%) of 10 709 were male, 9062 (84·6%) were female, mean age was 54·4 (SD 13·8) years, and mean disease duration was 7·9 (SD 8·2) years. Information regarding cutaneous subtype was available for 10 176 participants and antibody data were available for 9643 participants. In the prognostic analysis, there was no difference in AUC for overall survival (0·82, 95% CI 0·81–0·84 for cutaneous only vs 0·84, 0·82–0·85 for antibody only vs 0·84, 0·83–0·86 for combined) or for progression-free survival (0·70, 0·69–0·71 vs 0·71, 0·70–0·72 vs 0·71, 0·70–0·72). However, at 4 years the NRI showed substantial improvement for the antibody-only model compared with the cutaneous-only model in prediction of overall survival (0·57, 0·46–0·71 for antibody only vs 0·29, 0·19–0·39 for cutaneous only) and disease progression (0·36, 0·29–0·46 vs 0·21, 0·14–0·28). The antibody-only model did better than the cutaneous-only model in predicting renal crisis (AUC 0·72, 0·70–0·74 for antibody only vs 0·66, 0·64–0·69 for cutaneous only) and lung fibrosis leading to restrictive lung function (AUC 0·76, 0·75–0·77 vs 0·71, 0·70–0·72). The combined model improved the prediction of digital ulcers and elevated systolic pulmonary artery pressure, but did poorly for cardiac involvement. Interpretation: The autoantibody-only model outperforms cutaneous-only subsetting for risk stratifying people with systemic sclerosis in the EUSTAR cohort. Physicians should be aware of these findings at the time of decision making for patient management. Funding: World Scleroderma Foundation.
Published: 2022

38. Progressive skin fibrosis is associated with a decline in lung function and worse survival in patients with diffuse cutaneous systemic sclerosis in the european scleroderma trials and research (eustar) cohort

Author: Wu, Wanlong, Jordan, Suzana, Graf, Nicole, Pena, Janethe de Oliveira, Curram, John, Allanore, Yannick, Matucci-Cerinic, Marco, Pope, Janet E., Denton, Christopher P., Khanna, Dinesh, Distler, Oliver, Guiducci, Serena, Walker, Ulrich, Jaeger, Veronika, Bannert, Bettina, Lapadula, Giovanni, Becvarare, Radim, Cutolo, Maurizio, Valentini, Gabriele, Siegert, Elise, Rednic, Simona, Montecucco, C., Carreira, Patricia E., Novak, Srdan, Czirjak, Laszlo, Varju, Cecilia, Chizzolini, Carlo, Allai, Daniela, Kucharz, Eugene J., Cozzi, Franco, Rozman, Blaz, Mallia, Carmel, Gabrielli, Armando, Bancel, Dominique Farge, Airo, Paolo, Hesselstrand, Roger, Martinovic, Duska, Balbir-Gurman, Alexandra, Braun-Moscovici, Yolanda, Hunzelmann, Nicolas, Pellerito, Raffaele, Caramaschi, Paola, Black, Carol, Damjanov, Nemanja, Henes, Joerg, Ortiz Santamaria, Vera, Heitmann, Stefan, Seidel, Matthias, Pereira Da Silva, Jose Antonio, Stamenkovic, Bojana, Selmi, Carlo Francesco, Tikly, Mohammed, Denisov, Lev N., Mueller-Ladner, Ulf, Engelhart, Merete, Hachulla, Eric, Riccieri, Valeria, Ionescu, Ruxandra Maria, Mihai, Carina, Sunderkoetter, Cord, Kuhn, Annegret, Schett, Georg, Distler, Joerg, Meroni, Pierluigi, Ingegnoli, Francesca, Mouthon, Luc, De Keyser, Filip, Smith, Vanessa, Cantatore, Francesco Paolo, Corrado, Ada, Ullman, Susanne, Iversen, Line, Pozzi, Maria Rosa, Eyerich, Kilian, Hein, Ruediger, Knott, Elisabeth, Wiland, Piotr, Szmyrka-Kaczmarek, Magdalena, Sokolik, Renata, Morgiel, Ewa, Madej, Marta, Jose Alegre-Sancho, Juan, Krummel-Lorenz, Brigitte, Saar, Petra, Aringer, Martin, Guenther, Claudia, Anne, Erler, Westhovens, Rene, De Langhe, Ellen, Lenaerts, Jan, Anic, Branimir, Baresic, Marko, Mayer, Miroslav, Uprus, Maria, Otsa, Kati, Yavuz, Sule, Radominski, Sebastiao Cezar, Mueller, Carolina de Souza, Azevedo, Valderilio Feijo, Popa, Sergei, Zenone, Thierry, Stebbings, Simon, Highton, John, Mathieu, Alessandro, Vacca, Alessandra, Stamp, Lisa, Chapman, Peter, O'Donnell, John, Solanki, Kamal, Doube, Alan, Veale, Douglas, O'Rourke, Marie, Loyo, Esthela, Li, Mengtao, Rosato, Edoardo, Amoroso, Antonio, Gigante, Antonietta, Oksel, Fahrettin, Yargucu, Figen, Tanaseanu, Cristina-Mihaela, Popescu, Monica, Dumitrascu, Alina, Tiglea, Isabela, Foti, Rosario, Visalli, Elisa, Benenati, Alessia, Amato, Giorgio, Ancuta, Codrina, Chirieac, Rodica, Villiger, Peter, Adler, Sabine, Dan, Diana, de la Pena Lefebvre, Paloma Garcia, Rodriguez Rubio, Silvia, Valero Exposito, Marta, Sibilia, Jean, Chatelus, Emmanuel, Gottenberg, Jacques Eric, Chifflot, Helene, Litinsky, Ira, Del Galdo, Francesco, Venalis, Algirdas, Saketkoo, Lesley Ann, Lasky, Joseph A., Kerzberg, Eduardo, Montoya, Fabiana, Cosentino, Vanesa, Limonta, Massimiliano, Brucato, Antonio Luca, Lupi, Elide, Spertini, Francois, Ribi, Camillo, Buss, Guillaume, Martin, Thierry, Guffroy, Aurelien, Poindron, Vincent, Chung, Lori, Schmeiser, Tim, Zebryk, Pawel, Riso, Nuno, Riemekasten, Gabriela, Rezus, Elena, Puttini, Piercarlo Sarzi, Wu, W., Jordan, S., Graf, N., de Oliveira Pena, J., Curram, J., Allanore, Y., Matucci-Cerinic, M., Pope, J. E., Denton, C. P., Khanna, D., Distler, O., Guiducci, S., Walker, U., Jaeger, V., Bannert, B., Lapadula, G., Becvarare, R., Cutolo, M., Valentini, G., Siegert, E., Rednic, S., Montecucco, C., Carreira, P. E., Novak, S., Czirjak, L., Varju, C., Chizzolini, C., Allai, D., Kucharz, E. J., Cozzi, F., Rozman, B., Mallia, C., Gabrielli, A., Bancel, D. F., 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. O., Heitmann, S., Seidel, M., Pereira Da Silva, J. A., Stamenkovic, B., Selmi, C. F., Tikly, M., Denisov, L. N., Muller-Ladner, U., Engelhart, M., Hachulla, E., Riccieri, V., Ionescu, R. M., Mihai, C., Sunderkotter, C., Kuhn, A., Schett, G., Distler, J., Meroni, P., Ingegnoli, F., Mouthon, L., De Keyser, F., Smith, V., Cantatore, F. P., Corrado, A., Ullman, S., Iversen, L., Pozzi, M. R., Eyerich, K., Hein, R., Knott, E., Wiland, P., Szmyrka-Kaczmarek, M., Sokolik, R., Morgiel, E., Madej, M., Alegre-Sancho, J. 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. C., de Souza Muller, C., Azevedo, V. F., 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. -M., Popescu, M., Dumitrascu, A., Tiglea, I., Foti, R., Visalli, E., Benenati, A., Amato, G., Ancuta, C., Chirieac, R., Villiger, P., Adler, S., Dan, D., de la Pena Lefebvre, P. G., Rubio, S. R., Exposito, M. V., Sibilia, J., Chatelus, E., Gottenberg, J. E., Chifflot, H., Litinsky, I., Del Galdo, F., Venalis, A., Saketkoo, L. A., Lasky, J. A., Kerzberg, E., Montoya, F., Cosentino, V., Limonta, M., Brucato, A. L., 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., Sarzi Puttini, P., Ege Üniversitesi, Chizzolini, Carlo, Allali, Danièle, University of Zurich, and Distler, Oliver
Subjects: INVOLVEMENT, Male, BIOMEDICINE AND HEALTHCARE. Clinical Medical Sciences, diffuse, Time Factors, Databases, Factual, Skin Diseases/etiology/mortality/physiopathology, PREDICTION, Fibrosi, 2745 Rheumatology, Diffuse/complications/mortality/pathology, Immunology, General Biochemistry, Genetics and Molecular Biology, Immunology and Allergy, Rheumatology, Kaplan-Meier Estimate, ddc:616.07, Severity of Illness Index, Scleroderma, Cohort Studies, PROGNOSTIC-FACTORS, Fibrosis, Medicine and Health Sciences, scleroderma, Lung, Skin, integumentary system, progressive skin fibrosis, Lung function decline, BIOMEDICINA I ZDRAVSTVO. Kliničke medicinske znanosti, 10051 Rheumatology Clinic and Institute of Physical Medicine, DEATH, Middle Aged, ddc, Europe, VARIABILITY, factual, Cohort, Visceral organ progression, 2723 Immunology and Allergy, Disease Progression, Female, Survival Analysi, Life Sciences & Biomedicine, Cohort study, Human, Adult, Skin/pathology, medicine.medical_specialty, databases, All-cause death, risk analysis, diffuse cutaneous systemic sclerosis, 610 Medicine & health, IMPROVEMENT, Systemic Sclerosis, Skin Diseases, THICKNESS SCORE, VALIDATION, Databases, FEV1/FVC ratio, 1300 General Biochemistry, Genetics and Molecular Biology, Internal medicine, medicine, Humans, Factual, Survival analysis, 2403 Immunology, Science & Technology, Proportional hazards model, business.industry, Surrogate endpoint, MORTALITY, Skin Disease, fibrosis, Progressive skin fibrosi, Lung/physiopathology, biomarkers, Diffuse cutaneous systemic sclerosi, medicine.disease, Survival Analysis, all-cause death, lung function decline, visceral organ progression, adult, cohort studies, databases, factual, disease progression, female, humans, Scleroderma, Diffuse, Cohort Studie, business
Abstract: PubMed: 30852552, Objectives To determine whether progressive skin fibrosis is associated with visceral organ progression and mortality during follow-up in patients with diffuse cutaneous systemic sclerosis (dcSSc). Methods We evaluated patients from the European Scleroderma Trials and Research database with dcSSc, baseline modified Rodnan skin score (mRSS) ?7, valid mRSS at 12±3 months after baseline and ?1 annual follow-up visit. Progressive skin fibrosis was defined as an increase in mRSS >5 and ?25% from baseline to 12±3 months. Outcomes were pulmonary, cardiovascular and renal progression, and all-cause death. Associations between skin progression and outcomes were evaluated by Kaplan-Meier survival analysis and multivariable Cox regression. Results Of 1021 included patients, 78 (7.6%) had progressive skin fibrosis (skin progressors). Median follow-up was 3.4 years. Survival analyses indicated that skin progressors had a significantly higher probability of FVC decline ?10% (53.6% vs 34.4%; p, Bayer Bayer, 1Department of Rheumatology, University Hospital Zurich, Zurich, switzerland 2Graf Biostatistics, Winterthur, switzerland 3Clinical Development Pulmonology, Bayer Us llC, Whippany, new Jersey, Usa 4Data science and analytics, Bayer plc, Reading, UK 5Rheumatology a Department, Paris Descartes University, inseRM U1016, sorbonne, Paris Cité, Cochin Hospital, Paris, France 6Division of Rheumatology, University of Florence, Florence, italy 7Department of Medicine, Division of Rheumatology, University of Western Ontario, st. Joseph’s Health Care, london, Western Ontario, Canada 8Department of Rheumatology, Royal Free Hospital, University College london, london, UK 9scleroderma Program, Department of internal Medicine, Division of Rheumatology, University of Michigan, ann arbor, Michigan, Usa Acknowledgements The authors thank nicole schneider for excellent administration and data entry into the eUsTaR cohort. Medical writing assistance was provided by adelphi Communications ltd (Bollington, UK), funded by Bayer aG (Berlin, Germany)., This study was supported by a grant from Bayer aG.
Published: 2019

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

Author: Becker, Mike, Graf, Nicole, Sauter, Rafael, Allanore, Yannick, Curram, John, Denton, Christopher P., Khanna, Dinesh, Matucci-Cerinic, Marco, Pena, Janethe de Oliveira, Pope, Janet E., Distler, Oliver, Guiducci, Serena, Walker, Ulrich, Jaeger, Veronika, Bannert, Bettina, Lapadula, Giovanni, Becvarare, Radim, Cutolo, Maurizio, Valentini, Gabriele, Siegert, Elise, Rednic, Simona, Montecucco, C., Carreira, Patricia E., Novak, Srdan, Czirjak, Laszlo, Varju, Cecilia, Chizzolini, Carlo, Allai, Daniela, Kucharz, Eugene J., Cozzi, Franco, Rozman, Blaz, Mallia, Carmel, Gabrielli, Armando, Bancel, Dominique Farge, Airo, Paolo, Hesselstrand, Roger, Martinovic, Duska, Balbir-Gurman, Alexandra, Braun-Moscovici, Yolanda, Hunzelmann, Nicolas, Pellerito, Raffaele, Caramaschi, Paola, Black, Carol, Damjanov, Nemanja, Henes, Joerg, Ortiz Santamaria, Vera, Heitmann, Stefan, Seidel, Matthias, Pereira Da Silva, Jose Antonio, Stamenkovic, Bojana, Selmi, Carlo Francesco, Tikly, Mohammed, Denisov, Lev N., Mueller-Ladner, Ulf, Engelhart, Merete, Hachulla, Eric, Riccieri, Valeria, Ionescu, Ruxandra Maria, Mihai, Carina, Sunderkoetter, Cord, Kuhn, Annegret, Schett, Georg, Distler, Joerg, Meroni, Pierluigi, Ingegnoli, Francesca, Mouthon, Luc, De Keyser, Filip, Smith, Vanessa, Cantatore, Francesco Paolo, Corrado, Ada, Ullman, Susanne, Iversen, Line, Pozzi, Maria Rosa, Eyerich, Kilian, Hein, Ruediger, Knott, Elisabeth, Wiland, Piotr, Szmyrka-Kaczmarek, Magdalena, Sokolik, Renata, Morgiel, Ewa, Madej, Marta, Jose Alegre-Sancho, Juan, Krummel-Lorenz, Brigitte, Saar, Petra, Aringer, Martin, Guenther, Claudia, Anne, Erler, Westhovens, Rene, De langhe, Ellen, Lenaerts, Jan, Anic, Branimir, Baresic, Marko, Mayer, Miroslav, Uprus, Maria, Otsa, Kati, Yavuz, Sule, Radominski, Sebastiao Cezar, Mueller, Carolina de Souza, Azevedo, Valderilio Feijo, Popa, Sergei, Zenone, Thierry, Stebbings, Simon, Highton, John, Mathieu, Alessandro, Vacca, Alessandra, Stamp, Lisa, Chapman, Peter, O'Donnell, John, Solanki, Kamal, Doube, Alan, Veale, Douglas, O'Rourke, Marie, Loyo, Esthela, Li, Mengtao, Rosato, Edoardo, Amoroso, Antonio, Gigante, Antonietta, Oksel, Fahrettin, Yargucu, Figen, Tanaseanu, Cristina-Mihaela, Popescu, Monica, Dumitrascu, Alina, Tiglea, Isabela, Foti, Rosario, Visalli, Elisa, Benenati, Alessia, Amato, Giorgio, Ancuta, Codrina, Chirieac, Rodica, Villiger, Peter, Adler, Sabine, Dan, Diana, de la Pena Lefebvre, Paloma Garcia, Rodriguez Rubio, Silvia, Valero Exposito, Marta, Sibilia, Jean, Chatelus, Emmanuel, Gottenberg, Jacques Eric, Chifflot, Helene, Litinsky, Ira, Del Galdo, Francesco, Venalis, Algirdas, Saketkoo, Lesley Ann, Lasky, Joseph A., Kerzberg, Eduardo, Montoya, Fabiana, Cosentino, Vanesa, Limonta, Massimiliano, Brucato, Antonio Luca, Lupi, Elide, Spertini, Francois, Ribi, Camillo, Buss, Guillaume, Martin, Thierry, Guffroy, Aurelien, Poindron, Vincent, Chung, Lori, Schmeiser, Tim, Zebryk, Pawel, Riso, Nuno, Riemekasten, Gabriela, Rezus, Elena, Puttini, Piercarlo Sarzi, Ege Üniversitesi, University of Zurich, Distler, Oliver, Chizzolini, Carlo, Allai, Daniela, Becker, M., Graf, N., Sauter, R., Allanore, Y., Curram, J., Denton, C. P., Khanna, D., Matucci-Cerinic, M., de Oliveira Pena, J., Pope, J. E., Distler, O., Guiducci, S., Walker, U., Jaeger, V., Bannert, B., Lapadula, G., Becvarare, R., Cutolo, M., Valentini, G., Siegert, E., Rednic, S., Montecucco, C., Carreira, P. E., Novak, S., Czirjak, L., Varju, C., Chizzolini, C., Allai, D., Kucharz, E. J., Cozzi, F., Rozman, B., Mallia, C., Gabrielli, A., Bancel, D. F., 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. O., Heitmann, S., Seidel, M., Pereira Da Silva, J. A., Stamenkovic, B., Selmi, C. F., Tikly, M., Denisov, L. N., Muller-Ladner, U., Engelhart, M., Hachulla, E., Riccieri, V., Ionescu, R. M., Mihai, C., Sunderkotter, C., Kuhn, A., Schett, G., Distler, J., Meroni, P., Ingegnoli, F., Mouthon, L., De Keyser, F., Smith, V., Cantatore, F. P., Corrado, A., Ullman, S., Iversen, L., Pozzi, M. R., Eyerich, K., Hein, R., Knott, E., Wiland, P., Szmyrka-Kaczmarek, M., Sokolik, R., Morgiel, E., Madej, M., Alegre-Sancho, J. 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. C., de Souza Muller, C., Azevedo, V. F., 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. -M., Popescu, M., Dumitrascu, A., Tiglea, I., Foti, R., Visalli, E., Benenati, A., Amato, G., Ancuta, C., Chirieac, R., Villiger, P., Adler, S., Dan, D., de la Pena Lefebvre, P. G., Rubio, S. R., Exposito, M. V., Sibilia, J., Chatelus, E., Gottenberg, J. E., Chifflot, H., Litinsky, I., Del Galdo, F., Venalis, A., Saketkoo, L. A., Lasky, J. A., Kerzberg, E., Montoya, F., Cosentino, V., Limonta, M., Brucato, A. L., 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., and Sarzi Puttini, P.
Subjects: INVOLVEMENT, SELECTION, BIOMEDICINE AND HEALTHCARE. Clinical Medical Sciences, diffuse, predictive factors, systemic sclerosis, 2745 Rheumatology, epidemiologic methods, morbidity, Disease, Immunology, General Biochemistry, Genetics and Molecular Biology, Immunology and Allergy, Rheumatology, INTERSTITIAL LUNG-DISEASE, disease worsening, mortality, predictive factors, systemic sclerosis, predictive factor, disease worsening, DESIGN, middle aged, Medicine and Health Sciences, FIBROSIS, scleroderma, SKIN THICKNESS SCORE, mortality, skin and connective tissue diseases, Prospective cohort study, humans, lung diseases, ddc:616, education.field_of_study, heart diseases, integumentary system, clinical trials as topic, BIOMEDICINA I ZDRAVSTVO. Kliničke medicinske znanosti, 10051 Rheumatology Clinic and Institute of Physical Medicine, Interstitial lung disease, follow-up studies, ddc, female, Cohort, 2723 Immunology and Allergy, europe, Life Sciences & Biomedicine, CLINICAL-TRIALS, survival rate, medicine.medical_specialty, Population, 610 Medicine & health, disease progression, male, 1300 General Biochemistry, Genetics and Molecular Biology, Internal medicine, Severity of illness, REGRESSION, medicine, severity of illness index, education, Survival rate, METAANALYSIS, 2403 Immunology, Science & Technology, Scleroderma, Systemic, business.industry, MORTALITY, systemic, medicine.disease, prospective studies, Clinical trial, prognosis, scleroderma, diffuse, scleroderma, systemic, Scleroderma, Diffuse, business
Abstract: PubMed: 31227488, 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. © Author(s) (or their employer(s)) 2019. Re-use permitted under CC BY. Published by BMJ., Bayer Bayer, United Kingdom Université Paris Descartes Li Ka Shing Foundation, LKSF University of Michigan, U-M, 1Department of Rheumatology and the Centre of experimental Rheumatology, University Hospital Zurich, Zurich, switzerland 2Graf Biostatistics, Winterthur, switzerland 3Big Data institute, li Ka shing Centre for Health information and Discovery, nuffield Department of Medicine, University of Oxford, Oxford, UK 4Rheumatology a Department, Paris Descartes University, sorbonne Paris Cité, Cochin Hospital, Paris, France 5Data science and analytics, Bayer plc, Reading, UK 6UCl Division of Medicine, Royal Free Campus, london, UK 7Division of Rheumatology, Department of internal Medicine, University of Michigan scleroderma Program, University of Michigan, ann arbor, Michigan, Usa 8Department of experimental and Clinical Medicine, University of Florence, Florence, italy 9Bayer Us llC, Whippany, new Jersey, Usa 10Department of Medicine, Division of Rheumatology, University of Western Ontario, st. Joseph’s Health Care, london, Ontario, Canada Acknowledgements The R-code for the linear Mi-lassO was received from Qixuan Chen.21 Medical writing assistance was provided by adelphi Communications ltd (Bollington, UK), funded by Bayer aG (Berlin, Germany)., Contributors study conception and design, acquisition of data, analysis and interpretation of data and drafting and revising the article: OD and MB; analysis and interpretation of data: OD, MB, Rs and nG. all authors have critically reviewed and approved the final submitted version to be published. Funding This study was supported by a grant from Bayer aG. Bayer employees are coauthors of this paper and supported the study design and interpretation of the data, but otherwise Bayer had no influence on the study., Competing interests MOB declares no conflict of interest. OD has had consultancy relationships with actelion, Bayer, Biogen idec, Boehringer ingelheim, Chemomab, espeRare foundation, Genentech/Roche, GsK, inventiva, italfarmaco, lilly, medac, Medimmune, Mitsubishi Tanabe Pharma, Pharmacyclics, novartis, Pfizer, sanofi, sinoxa and UCB in the area of potential treatments of scleroderma and its complications. OD has received research funding from actelion, Bayer, Boehringer ingelheim, Mitsubishi Tanabe Pharma and Roche in the area of potential treatments of scleroderma and its complications. OD has a patent for mir-29 licensed for the treatment of systemic sclerosis. DK has consultancy relationships and/or has received grant/research support from Bayer, Bristol-Myers squibb, Boehringer ingelheim, Genentech/Roche, niH, Pfizer, sanofi-aventis Pharmaceuticals, actelion Pharmaceuticals Us, Chemomab, Corbus, Covis, Cytori, eicos, eMD serono, Gilead, GlaxosmithKline, and UCB Pharma. He is a shareholder of eicos. CPD has consultancy relationships with and/or has received speakers’ bureau fees from actelion Pharmaceuticals Us, Bayer aG, GlaxosmithKline, Csl Behring, Merck serono, Roche Pharmaceuticals, Genentech and Biogen iDeC inc., inventiva, sanofi-aventis Pharmaceuticals and Boehringer ingelheim. JeP has consultancy relationships with and/or has received grant/research support from actelion, Bayer aG, Bristol-Myers squibb, Merck, Pfizer inc. and Roche. MM-C has consultancy relationships and/ or has received grant/research support from Pfizer, Bristol-Myers squibb, actelion, UCB Pharma, Bayer, Chemomab, Genentech/Roche, inventiva and lilly. Ya has consultancy relationships with and/or has received grant/research support from actelion, Pharmaceuticals Us, Bayer aG, Bristol-Myers squibb, inventiva, Medac, Pfizer inc., Roche Pharmaceuticals, Genentech and Biogen iDeC inc., sanofi-aventis Pharmaceuticals and servier. JdOP and JC are employees of Bayer. nTG has nothing to disclose.
Published: 2019

40. Who might be predisposed to the development of serious side effects when treated with TNF-alpha antagonist?

Author: Kotyla PJ and Kucharz EJ
Subjects: Contraindications, Female, Heart Failure physiopathology, Humans, Infliximab, Risk Factors, Tachycardia, Ventricular physiopathology, Ventricular Dysfunction, Left etiology, Ventricular Dysfunction, Left physiopathology, Antibodies, Monoclonal adverse effects, Arthritis, Rheumatoid complications, Arthritis, Rheumatoid drug therapy, Arthritis, Rheumatoid physiopathology, Heart Failure etiology, Tachycardia, Ventricular etiology, Tumor Necrosis Factor-alpha antagonists & inhibitors
Published: 2006

41. Circadian profile of serum melatonin in patients with systemic sclerosis.

Author: Kotulska A, Brzezinska-Wcislo L, and Kucharz EJ
Subjects: Adult, Circadian Rhythm physiology, Female, Humans, Melatonin blood, Scleroderma, Systemic blood, Melatonin biosynthesis, Scleroderma, Systemic metabolism
Published: 2005

42. A single infusion of infliximab increases the serum endostatin level in patients with rheumatoid arthritis.

Author: Kucharz EJ, Gozdzik J, Kopec M, Kotulska A, Lewicki M, Pieczyrak R, Widuchowska M, Zakliczynska H, Szarzynska-Ruda M, and Zycinska-Debska E
Subjects: Adult, Arthritis, Rheumatoid blood, Endostatins, Female, Humans, Infliximab, Infusions, Parenteral, Middle Aged, Neovascularization, Pathologic, Angiogenesis Inhibitors blood, Antibodies, Monoclonal administration & dosage, Antirheumatic Agents administration & dosage, Arthritis, Rheumatoid drug therapy, Collagen blood, Peptide Fragments blood
Published: 2003

43. [The effect of raloxifene on bone tissue in postmenopausal women].

Author: Kopeć M and Kucharz EJ
Subjects: Female, Humans, Middle Aged, Bone Density drug effects, Osteoporosis, Postmenopausal prevention & control, Raloxifene Hydrochloride pharmacology, Receptors, Estrogen drug effects
Published: 2001

44. [The Third Congress of the European Federation of Internal Medicine, Edinburgh, May 9-12, 2001].

Author: Kucharz EJ
Subjects: Europe, Humans, International Cooperation, Societies, Medical, Internal Medicine
Published: 2001

45. Influence of short-time application of a low sodium diet on blood pressure in patients with hyperthyroidism or hypothyroidism during therapy.

Author: Marcisz C, Jonderko G, and Kucharz EJ
Subjects: Adolescent, Adult, Female, Humans, Male, Middle Aged, Renin blood, Time Factors, Blood Pressure, Diet, Sodium-Restricted, Hyperthyroidism physiopathology, Hypothyroidism physiopathology
Abstract: Hyperthyroidism or hypothyroidism are commonly associated with altered blood pressure (BP). Restriction of sodium in the diet produces a decrease in BP in some individuals. It is also well known that hormones other than thyroid affect BP. The present study was designed to evaluate the influence of a low sodium diet on BP in patients with hyperthyroidism or hypothyroidism during therapy. The occurrence of salt-sensitive or salt-nonsensitive BP was compared with hormonal levels (plasma renin activity, aldosterone, atrial natriuretic peptide, and arginine vasopressin). Patients with hyperthyroidism (75 subjects) were investigated before the initiation of treatment, 2 weeks after the treatment, and after the attainment of euthyroid state. Patients with hypothyroidism (31 subjects) were studied before the treatment and in the euthyroid state. Control values were obtained from 37 healthy individuals. Blood pressure, changes of plasma volume, serum aldosterone, atrial natriuretic peptide, vasopressin levels, and plasma renin activity were measured in all investigated subjects after application of a normal sodium diet and after 3 days on a low sodium diet. Elevated systolic BP was found in patients with hyperthyroidism and hypothyroidism. Mean arterial BP was higher only in the untreated hypothyroid patients. The high incidence of salt-sensitive BP was found only in untreated hypothyroid patients. Also in hypothyroid patients the application of a low sodium diet led to a lower increase in plasma renin activity in subjects with salt-sensitive BP than in individuals with salt-resistant BP. Therefore, different mechanisms are responsible for BP elevation in patients with hyperthyroidism or hypothyroidism.
Published: 2001
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46. [Cyclooxygenases, structure, biological functions and the role in inflammation processes].

Author: Kotyla PJ, Sliwińska-Kotyla B, and Kucharz EJ
Subjects: Anti-Inflammatory Agents, Non-Steroidal pharmacology, Cyclooxygenase 1, Cyclooxygenase 2, Cyclooxygenase 2 Inhibitors, Cyclooxygenase Inhibitors pharmacology, Humans, Isoenzymes drug effects, Membrane Proteins, Prostaglandin-Endoperoxide Synthases drug effects, Structure-Activity Relationship, Anti-Inflammatory Agents, Non-Steroidal therapeutic use, Cyclooxygenase Inhibitors therapeutic use, Inflammation metabolism, Isoenzymes metabolism, Prostaglandin-Endoperoxide Synthases metabolism
Published: 2001

47. [Mixed cryoglobulinemia].

Author: Kopeć M and Kucharz EJ
Subjects: Cryoglobulins metabolism, Hepacivirus, Humans, Cryoglobulinemia diagnosis, Cryoglobulinemia etiology, Cryoglobulinemia physiopathology, Cryoglobulinemia therapy, Hepatitis C complications, Hepatitis C therapy
Published: 2001

48. [Professor Akihiro Igata awarded with the Medal of the Polish Society of Internal Medicine].

Author: Kucharz EJ
Subjects: History, 20th Century, Japan, Poland, Societies, Medical, Awards and Prizes, Internal Medicine history
Published: 2001

49. [Psoriatic arthritis].

Author: Widuchowska M and Kucharz EJ
Subjects: Antibodies, Monoclonal therapeutic use, Antirheumatic Agents therapeutic use, Humans, Receptors, Tumor Necrosis Factor therapeutic use, Arthritis, Psoriatic classification, Arthritis, Psoriatic diagnosis, Arthritis, Psoriatic drug therapy
Published: 2001

50. [Kawasaki disease].

Author: Kotulska A, Pajewska-Łaba A, and Kucharz EJ
Subjects: Central Nervous System Diseases etiology, Cerebrovascular Disorders etiology, Child, Humans, Peripheral Nervous System Diseases etiology, Prognosis, Mucocutaneous Lymph Node Syndrome complications, Mucocutaneous Lymph Node Syndrome diagnosis, Mucocutaneous Lymph Node Syndrome etiology, Mucocutaneous Lymph Node Syndrome physiopathology
Published: 2001

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