115 results on '"Carreira, P. E."'
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2. Does regression of skin thickening predict improvement of internal organ involvement and survival in patients with diffuse cutaneous systemic sclerosis? A EUSTAR analysis
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Wyss, Anja, Jordan, Suzana, Graf, Nicole, Carreira, Patricia E., Distler, Jörg, Cerinic, Marco Matucci, Siegert, Elise, Henes, Jörg, Zanatta, Elisabetta, Riccieri, Valeria, Truchetet, Marie-Elise, Oksel, Fahrettin, Li, Mengtao, Kucharz, Eugene J., Eyerich, Kilian, Del Galdo, Francesco, Vonk, Madelon C., Vold, Anna-Maria Hoffman, Gabrielli, Armando, and Distler, Oliver
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- 2024
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3. Retrotransposon instability dominates the acquired mutation landscape of mouse induced pluripotent stem cells
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Gerdes, Patricia, Lim, Sue Mei, Ewing, Adam D., Larcombe, Michael R., Chan, Dorothy, Sanchez-Luque, Francisco J., Walker, Lucinda, Carleton, Alexander L., James, Cini, Knaupp, Anja S., Carreira, Patricia E., Nefzger, Christian M., Lister, Ryan, Richardson, Sandra R., Polo, Jose M., and Faulkner, Geoffrey J.
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- 2022
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4. Diffusing Capacity of the Lungs for Carbon Monoxide and Echocardiographic Parameters in Identifying Mild Pulmonary Hypertension in the EUSTAR Cohort of Patients With Systemic Sclerosis
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Colalillo, Amalia, Hachulla, Eric, Pellicano, Chiara, Smith, Vanessa, Bergmann, Christina, Riemekasten, Gabriela, Zanatta, Elisabetta, Henes, Jörg, Launay, David, Marcoccia, Antonella, Gheorghiu, Ana Maria, Truchetet, Marie-Elise, Iannone, Florenzo, Simeón Aznar, Carmen Pilar, Oliveira, Susana, Vonk, Madelon, Del Galdo, Francesco, Rosato, Edoardo, Distler, Oliver, Becker, Mike, De Decker, Melissa, Fegatelli, Danilo Alunni, Siegert, Elise, Castellví, Ivan, Cauli, Alberto, Solanki, Kamal, Dagna, Lorenzo, Martin, Mickaël, Moroncini, Gianluca, Poormoghim, Hadi, Kuwana, Masataka, Carreira, Patricia E., Airò, Paolo, Bergmann, Christina, Spierings, Julia, Tanaka, Yoshiya, Selvi, Enrico, and Soukup, Tomas
- Abstract
The 2022 European Society of Cardiology/European Respiratory Society guidelines define pulmonary hypertension (PH) as a resting mean pulmonary artery pressure (mPAP) > 20 mm Hg at right heart catheterization (RHC). Previously, patients with an mPAP between 21 and 24 mm Hg were classified in a “gray zone” of unclear clinical significance.
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- 2024
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5. Cohort Enrichment Strategies for Progressive Interstitial Lung Disease in Systemic Sclerosis From European Scleroderma Trials and Research
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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.
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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
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- 2023
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6. TBP and SNAP50 transcription factors bind specifically to the Pr77 promoter sequence from trypanosomatid non-LTR retrotransposons
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Macías, Francisco, Afonso-Lehmann, Raquel, Carreira, Patricia E., and Thomas, M. Carmen
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- 2021
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7. ERN ReCONNET points to consider for treating patients living with autoimmune rheumatic diseases with antiviral therapies and anti-SARS-CoV-2 antibody products
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Talarico, R, Ramirez, G, Barreira, S, Cardamone, C, Triggianese, P, Aguilera, S, Andersen, J, Avcin, T, Benistan, K, Bertsias, G, Bortoluzzi, A, Bouillot, C, Bulina, I, Burmester, G, Callens, S, Carreira, P, Cervera, R, Cutolo, M, Damian, L, Della-Torre, E, Faria, R, Fonseca, J, Galetti, I, Hachulla, E, Iaccarino, L, Jacobsen, S, Khmelinskii, N, Limper, M, Marinello, D, Meyer, A, Moroncini, G, Nagy, G, Olesinska, M, Pamfil, C, Pileckyte, M, Pistello, M, Rednic, S, Richez, C, Romao, V, Schneider, M, Sciascia, S, Scire, C, Simonini, G, Smith, V, Sulli, A, Tani, C, Tas, S, Tincani, A, Vonk, M, Tektonidou, M, Mosca, M, Talarico R., Ramirez G. A., Barreira S. C., Cardamone C., Triggianese P., Aguilera S., Andersen J., Avcin T., Benistan K., Bertsias G., Bortoluzzi A., Bouillot C., Bulina I., Burmester G. R., Callens S., Carreira P. E., Cervera R., Cutolo M., Damian L., Della-Torre E., Faria R., Fonseca J. E., Galetti I., Hachulla E., Iaccarino L., Jacobsen S., Khmelinskii N., Limper M., Marinello D., Meyer A., Moroncini G., Nagy G., Olesinska M., Pamfil C., Pileckyte M., Pistello M., Rednic S., Richez C., Romao V. C., Schneider M., Sciascia S., Scire C. A., Simonini G., Smith V., Sulli A., Tani C., Tas S. W., Tincani A., Vonk M. C., Tektonidou M., Mosca M., Talarico, R, Ramirez, G, Barreira, S, Cardamone, C, Triggianese, P, Aguilera, S, Andersen, J, Avcin, T, Benistan, K, Bertsias, G, Bortoluzzi, A, Bouillot, C, Bulina, I, Burmester, G, Callens, S, Carreira, P, Cervera, R, Cutolo, M, Damian, L, Della-Torre, E, Faria, R, Fonseca, J, Galetti, I, Hachulla, E, Iaccarino, L, Jacobsen, S, Khmelinskii, N, Limper, M, Marinello, D, Meyer, A, Moroncini, G, Nagy, G, Olesinska, M, Pamfil, C, Pileckyte, M, Pistello, M, Rednic, S, Richez, C, Romao, V, Schneider, M, Sciascia, S, Scire, C, Simonini, G, Smith, V, Sulli, A, Tani, C, Tas, S, Tincani, A, Vonk, M, Tektonidou, M, Mosca, M, Talarico R., Ramirez G. A., Barreira S. C., Cardamone C., Triggianese P., Aguilera S., Andersen J., Avcin T., Benistan K., Bertsias G., Bortoluzzi A., Bouillot C., Bulina I., Burmester G. R., Callens S., Carreira P. E., Cervera R., Cutolo M., Damian L., Della-Torre E., Faria R., Fonseca J. E., Galetti I., Hachulla E., Iaccarino L., Jacobsen S., Khmelinskii N., Limper M., Marinello D., Meyer A., Moroncini G., Nagy G., Olesinska M., Pamfil C., Pileckyte M., Pistello M., Rednic S., Richez C., Romao V. C., Schneider M., Sciascia S., Scire C. A., Simonini G., Smith V., Sulli A., Tani C., Tas S. W., Tincani A., Vonk M. C., Tektonidou M., and Mosca M.
- Abstract
Recent studies have shown that people who are immunocompromised may inadvertently play a role in spurring the mutations of the virus that create new variants. This is because some immunocompromised individuals remain at risk of getting COVID-19 despite vaccination, experience more severe disease, are susceptible to being chronically infected and remain contagious for longer if they become infected and considering that immunocompromised individuals represent approximately 2% of the overall population, this aspect should be carefully considered. So far, some autoimmune rheumatic disease (ARD) patients with COVID-19 have been treated with antiviral therapies or anti-SARS-CoV-2 antibody products. However, there is no homogeneous approach to these treatment strategies. This issue was addressed within the European Reference Network (ERN) on Rare and Complex Connective Tissue and Musculoskeletal Diseases (ReCONNET) in a discussion among experts and patient's representatives in the context of the rare and complex connective tissue diseases (rCTDs) covered by the Network. ERN ReCONNET is one of the 24 ERNs launched by the European Commission in 2017 with the aim of tackling low prevalence and rare diseases that require highly specialised treatment and promoting concentration of knowledge and resources through virtual networks involving healthcare providers (HCPs) across the European Union (EU). Considering the urgent need to provide guidance not only to the rCTDs community, but also to the whole ARDs community, a multidisciplinary Task Force, including expert clinicians and European Patient Advocacy Group (ePAG) Advocates, was created in the framework of ERN ReCONNET with the aim of developing overarching principles (OP) and points-to-consider (PtC) on a homogenous approach to treat immunocompromised patients with ARDs (with a particular focus on CTDs) affected by COVID-19 using antiviral therapies and anti-SARS-CoV-2 antibody products. The present work reports the final OP
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- 2023
8. ERN ReCONNET points to consider for treating patients living with autoimmune rheumatic diseases with antiviral therapies and anti-SARS-CoV-2 antibody products
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Talarico, Rosaria R., Ramirez, G. A., Barreira, S. C., Cardamone, C., Triggianese, P., Aguilera, S., Andersen, J., Avcin, T., Benistan, K., Bertsias, G., Bortoluzzi, A., Bouillot, C., Bulina, I., Burmester, G. R., Callens, S., Carreira, P. E., Cervera, R., Cutolo, M., Damian, L., Torre, E. Della, Faria, R., Fonseca, J. E., Galetti, I., Hachulla, E., Iaccarino, L., Jacobsen, S., Khmelinskii, N., Limper, M., Marinello, D., Meyer, A., Moroncini, G., Nagy, G., Olesinska, M., Pamfil, C., Pileckyte, M., Pistello, M., Rednic, S., Richez, C., Romão, V. C., Schneider, M., Sciascia, S., Scirè, C. A., Simonini, G., Smith, V., Sulli, A., Tani, C., Tas, S. W., Tincani, A., Vonk, M. C., Tektonidou, M., Mosca, M., Talarico, Rosaria R., Ramirez, G. A., Barreira, S. C., Cardamone, C., Triggianese, P., Aguilera, S., Andersen, J., Avcin, T., Benistan, K., Bertsias, G., Bortoluzzi, A., Bouillot, C., Bulina, I., Burmester, G. R., Callens, S., Carreira, P. E., Cervera, R., Cutolo, M., Damian, L., Torre, E. Della, Faria, R., Fonseca, J. E., Galetti, I., Hachulla, E., Iaccarino, L., Jacobsen, S., Khmelinskii, N., Limper, M., Marinello, D., Meyer, A., Moroncini, G., Nagy, G., Olesinska, M., Pamfil, C., Pileckyte, M., Pistello, M., Rednic, S., Richez, C., Romão, V. C., Schneider, M., Sciascia, S., Scirè, C. A., Simonini, G., Smith, V., Sulli, A., Tani, C., Tas, S. W., Tincani, A., Vonk, M. C., Tektonidou, M., and Mosca, M.
- Abstract
Recent studies have shown that people who are immunocompromised may inadvertently play a role in spurring the mutations of the virus that create new variants. This is because some immunocompromised individuals remain at risk of getting COVID-19 despite vaccination, experience more severe disease, are susceptible to being chronically infected and remain contagious for longer if they become infected and considering that immunocompromised individuals represent approximately 2% of the overall population, this aspect should be carefully considered. So far, some autoimmune rheumatic disease (ARD) patients with COVID-19 have been treated with antiviral therapies or anti-SARS-CoV-2 antibody products. However, there is no homogeneous approach to these treatment strategies. This issue was addressed within the European Reference Network (ERN) on Rare and Complex Connective Tissue and Musculoskeletal Diseases (ReCONNET) in a discussion among experts and patient’s representatives in the context of the rare and complex connective tissue diseases (rCTDs) covered by the Network. ERN ReCONNET is one of the 24 ERNs launched by the European Commission in 2017 with the aim of tackling low prevalence and rare diseases that require highly specialised treatment and promoting concentration of knowledge and resources through virtual networks involving healthcare providers (HCPs) across the European Union (EU). Considering the urgent need to provide guidance not only to the rCTDs community, but also to the whole ARDs community, a multidisciplinary Task Force, including expert clinicians and European Patient Advocacy Group (ePAG) Advocates, was created in the framework of ERN ReCONNET with the aim of developing overarching principles (OP) and points-to-consider (PtC) on a homogenous approach to treat immunocompromised patients with ARDs (with a particular focus on CTDs) affected by COVID-19 using antiviral therapies and anti-SARS-CoV-2 antibody products. The present work reports the final OP, Recent studies have shown that people who are immunocompromised may inadvertently play a role in spurring the mutations of the virus that create new variants. This is because some immunocompromised individuals remain at risk of getting COVID-19 despite vaccination, experience more severe disease, are susceptible to being chronically infected and remain contagious for longer if they become infected and considering that immunocompromised individuals represent approximately 2% of the overall population, this aspect should be carefully considered. So far, some autoimmune rheumatic disease (ARD) patients with COVID-19 have been treated with antiviral therapies or anti-SARS-CoV-2 antibody products. However, there is no homogeneous approach to these treatment strategies. This issue was addressed within the European Reference Network (ERN) on Rare and Complex Connective Tissue and Musculoskeletal Diseases (ReCONNET) in a discussion among experts and patient's representatives in the context of the rare and complex connective tissue diseases (rCTDs) covered by the Network. ERN ReCONNET is one of the 24 ERNs launched by the European Commission in 2017 with the aim of tackling low prevalence and rare diseases that require highly specialised treatment and promoting concentration of knowledge and resources through virtual networks involving healthcare providers (HCPs) across the European Union (EU). Considering the urgent need to provide guidance not only to the rCTDs community, but also to the whole ARDs community, a multidisciplinary Task Force, including expert clinicians and European Patient Advocacy Group (ePAG) Advocates, was created in the framework of ERN ReCONNET with the aim of developing overarching principles (OP) and points-to-consider (PtC) on a homogenous approach to treat immunocompromised patients with ARDs (with a particular focus on CTDs) affected by COVID-19 using antiviral therapies and anti-SARS-CoV-2 antibody products. The present work reports the final
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- 2023
9. Does the Impact of COVID‐19 on Patients With Systemic Sclerosis Change Over Time?
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Deibel, Elisabeth, Carreira, Patricia E., Vonk, Madelon, del Papa, Nicoletta, Bečvář, Radim, Guillén‐Del‐Castillo, Alfredo, Campochiaro, Corrado, Poormoghim, Hadi, Liem, Sophie, Lazzaroni, Maria‐Grazia, Giollo, Alessandro, Mekinian, Arsène, Vries‐Bouwstra, Jeska, De Santis, Maria, Balbir‐Gurman, Alexandra, Mihai, Carina, De Luca, Giacomo, Moiseev, Sergey, Zanatta, Elisabetta, Foti, Rosario, Rednic, Simona, Denton, Christopher, Cutolo, Maurizio, Belloli, Laura, Airo, Paolo, Garzanova, Liudmila, Moroncini, Gianluca, İnanç, Murat, Panopoulos, Stylianos, Tandaipan, Jose‐Luis, Chatelus, Emmanuel, Rosato, Edoardo, Kuwana, Masataka, Yavuz, Sule, Alegre‐Sancho, Juan J., Smith, Vanessa, Szűcs, Gabriella, Henes, Joerg, Rodríguez‐Pintó, Ignasi, Atzeni, Fabiola, Spierings, Julia, Truchetet, Marie‐Elise, Milchert, Marcin, Brito de Araujo, Daniel, Riemekasten, Gabriela, Bernardino, Vera, Martin, Thierry, del Galdo, Francesco, Vacca, Alessandra, Mendoza, Fabian, Midtvedt, Øyvind, Murdaca, Giuseppe, Santiago, Tânia, Codullo, Veronica, Cacciapaglia, Fabio, Walker, Ulrich, Brunborg, Cathrine, Tirelli, Francesca, Allanore, Yannick, Furst, Daniel E., Matucci, Marco, Gabrielli, Armando, Distler, Oliver, and Hoffmann‐Vold, Anna‐Maria
- Abstract
The outcome of patients with COVID‐19 improved over the pandemic, including patients with systemic rheumatic diseases. However, data on patients with systemic sclerosis (SSc) are lacking. This study aimed to assess the outcome of patients with both SSc and COVID‐19 over several waves. Patients with both SSc and COVID‐19 who were registered in the European Scleroderma Trials and Research group (EUSTAR) were collected between April 2020 and April 2021. Patients were assigned to waves 1, 2, or 3 depending on the date of their COVID‐19 diagnosis. Primary endpoints were death, intensive care unit stay, or ventilatory support (severe outcome). Subgroup analyses of patients who were hospitalized or died were conducted. General and SSc‐specific characteristics and treatment were compared over the waves. Descriptive statistics and multivariate logistic regression were applied. A total of 333 patients were included; 57 patients (17%) had a severe outcome, and 30 patients (9%) died. Compared to wave 1, significantly fewer patients with SSc suffered from severe COVID‐19 in waves 2 and 3 (28.2% vs 9.8% and 12.7%; P< 0.001), fewer patients required hospitalization (46.7% vs 19.6% and 25.5%; P< 0.001) or ventilatory support (24.0% vs 8.7% and 10.9%; P =0.001), and fewer patients died (15.7% vs 5.0% and 7.5%; P =0.011). Patients were significantly younger, more often men, had less frequent arterial hypertension, and less SSc cardiac involvement over waves 1 to 3. Patients received significantly less medium to high doses of corticosteroids as they did SSc treatment. The outcome of patients with both SSc and COVID‐19 improved significantly over time because of intrinsic and extrinsic factors.
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- 2024
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10. Mortality and prognostic factors in idiopathic inflammatory myositis: a retrospective analysis of a large multicenter cohort of Spain
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Nuño-Nuño, Laura, Joven, Beatriz Esther, Carreira, Patricia E., Maldonado-Romero, Valentina, Larena-Grijalba, Carmen, Cubas, Irene Llorente, Tomero, Eva Gloria, Barbadillo-Mateos, María Carmen, De la Peña Lefebvre, Paloma García, Ruiz-Gutiérrez, Lucía, López-Robledillo, Juan Carlos, Moruno-Cruz, Henry, Pérez, Ana, Cobo-Ibáñez, Tatiana, Almodóvar González, Raquel, Lojo, Leticia, García De Yébenes, María Jesús, and López-Longo, Francisco Javier
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- 2017
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11. Risk stratification approaches perform differently in SSc-associated PAH in EUSTAR
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Bjørkekjær, H J, primary, Bruni, C, additional, Carreira, P E, additional, Airò, P, additional, Simeón-Aznar, C, additional, Truchetet, M, additional, Giollo, A, additional, Balbir-Gurman, A, additional, Martin, M, additional, Denton, C, additional, Gabrielli, A, additional, Fretheim, H, additional, Barua, I, additional, Bitter, H, additional, Midtvedt, Ø, additional, Broch, K, additional, Andreassen, A K, additional, Tanaka, Y, additional, Riemekasten, G, additional, Müller-Ladner, U, additional, Matucci Cerinic, M, additional, Castellví, I, additional, Siegert, E, additional, Hachulla, E, additional, Distler, O, additional, and Hoffmann-Vold, A, additional
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- 2022
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12. Cutaneous Manifestations, Clinical Characteristics, and Prognosis of Patients With Systemic Sclerosis Sine Scleroderma: Data From the International EUSTAR Database
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Lescoat, Alain, Huang, Suiyuan, Carreira, Patricia E., Siegert, Elise, de Vries-Bouwstra, Jeska, Distler, Jörg H. W., Smith, Vanessa, Del Galdo, Francesco, Anic, Branimir, Damjanov, Nemanja, Rednic, Simona, Ribi, Camillo, Bancel, Dominique Farge, Hoffmann-Vold, Anna-Maria, Gabrielli, Armando, Distler, Oliver, Khanna, Dinesh, and Allanore, Yannick
- Abstract
IMPORTANCE: Systemic sclerosis (SSc) sine scleroderma (ssSSc) is a subset of SSc defined by the absence of skin fibrosis. Little is known about the natural history and skin manifestations among patients with ssSSc. OBJECTIVE: To characterize the clinical phenotype of patients with ssSSc compared with patients with limited cutaneous SSc (lcSSc) and diffuse cutaneous SSc (dcSSc) within the EUSTAR database. DESIGN, SETTING, AND PARTICIPANTS: This longitudinal observational cohort study based on the international EUSTAR database included all patients fulfilling the classification criteria for SSc assessed by the modified Rodnan Skin score (mRSS) at inclusion and with at least 1 follow-up visit; ssSSc was defined by the absence of skin fibrosis (mRSS = 0 and no sclerodactyly) at all available visits. Data extraction was performed in November 2020, and data analysis was performed from April 2021 to April 2023. MAIN OUTCOMES AND MEASURES: Main outcomes were survival and skin manifestations (onset of skin fibrosis, digital ulcers, telangiectasias, puffy fingers). RESULTS: Among the 4263 patients fulfilling the inclusion criteria, 376 (8.8%) were classified as having ssSSc (mean [SD] age, 55.3 [13.9] years; 345 [91.8%] were female). At last available visit, in comparison with 708 patients with lcSSc and 708 patients with dcSSc with the same disease duration, patients with ssSSc had a lower prevalence of previous or current digital ulcers (28.2% vs 53.1% in lcSSc; P < .001; and 68.3% in dcSSc; P < .001) and puffy fingers (63.8% vs 82.4% in lcSSc; P < .001; and 87.6% in dcSSc; P < .001). By contrast, the prevalence of interstitial lung disease was similar in ssSSc and lcSSc (49.8% and 57.1%; P = .03) but significantly higher in dcSSc (75.0%; P < .001). Skin telangiectasias were associated with diastolic dysfunction in patients with ssSSc (odds ratio, 4.778; 95% CI, 2.060-11.081; P < .001). The only independent factor for the onset of skin fibrosis in ssSSc was the positivity for anti–Scl-70 antibodies (odds ratio, 3.078; 95% CI, 1.227-7.725; P = .02). Survival rate was higher in patients with ssSSc (92.4%) compared with lcSSc (69.4%; P = .06) and dcSSc (55.5%; P < .001) after up to 15 years of follow-up. CONCLUSIONS AND RELEVANCE: Systemic sclerosis sine scleroderma should not be neglected considering the high prevalence of interstitial lung disease (>40%) and SSc renal crisis (almost 3%). Patients with ssSSc had a higher survival than other subsets. Dermatologists should be aware that cutaneous findings in this subgroup may be associated with internal organ dysfunction. In particular, skin telangiectasias in ssSSc were associated with diastolic heart dysfunction.
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- 2023
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13. Outcomes of limited cutaneous systemic sclerosis patients: Results on more than 12,000 patients from the EUSTAR database
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Frantz C., Huscher D., Avouac J., Hachulla E., Balbir-Gurman A., Riemekasten G., Siegert E., Lazzaroni M. -G., Carreira P. E., Vettori S., Zanatta E., Ullman S., Czirjak L., Kowal-Bielecka O., Distler O., Matucci-Cerinic M., Allanore Y, Cuomo Giovanna, University of Zurich, Allanore, Yannick, Frantz, C., Huscher, D., Avouac, J., Hachulla, E., Balbir-Gurman, A., Riemekasten, G., Siegert, E., Lazzaroni, M. -G., Carreira, P. E., Vettori, S., Zanatta, E., Ullman, S., Czirjak, L., Kowal-Bielecka, O., Distler, O., Matucci-Cerinic, M., Allanore, Y, and Cuomo, Giovanna
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0301 basic medicine ,Skin score ,Limited cutaneous systemic sclerosis ,Male ,Databases, Factual ,Fibrosi ,Limited cutaneous systemic sclerosi ,Immunology ,Digital ulcer ,610 Medicine & health ,Interstitial lung disease ,Disease ,Outcomes ,computer.software_genre ,Scleroderma ,03 medical and health sciences ,FEV1/FVC ratio ,Databases ,0302 clinical medicine ,Scleroderma, Limited ,medicine ,Immunology and Allergy ,Humans ,In patient ,Limited ,Lung ,Factual ,Skin ,030203 arthritis & rheumatology ,Peripheral Vascular Diseases ,2403 Immunology ,integumentary system ,Database ,business.industry ,10051 Rheumatology Clinic and Institute of Physical Medicine ,Digital ulcers ,Middle Aged ,medicine.disease ,Fibrosis ,Female ,Clinical trial ,030104 developmental biology ,2723 Immunology and Allergy ,business ,computer - Abstract
Objectives: Limited cutaneous systemic sclerosis (LcSSc) is the most common subset of SSc but it has been overlooked in the past years. At a time at which clinical trials focus on diffuse cutaneous SSc (DcSSc) we aimed at clarifying the outcomes of LcSSc and at evaluating whether potential drug positioned in DcSSc may also be used in LcSSc. Methods: The EUSTAR database was used to investigate skin, lung and peripheral vasculopathy outcomes in LcSSc. Worsening of skin fibrosis, ILD and peripheral vasculopathy were defined by an increase in modified Rodnan skin score (mRSS) > 3.5 points, a decrease of FVC > 10% in patients with ILD at baseline, and by the development of new digital ulcers (DU) in patients without DU at baseline. Results: 8013 LcSSc and 4786 DcSSc patients were included. In contrast to DcSSc, skin disease was remarkably stable in the majority of LcSSc patients with >80% having a change lower than ±4 units of mRSS at 12, 24 and 36 months follow-up. Conversely, FVC changes over time were very similar between LcSSc and DcSSc. Regarding DU, numbers of patients with new DU over time seemed to be almost similar between the two subsets. Conclusions: LcSSc patients have a low mRSS at baseline with marginal changes with time. Conversely, SSc-ILD can be as progressive as in DcSSc supporting the inclusion of LcSSc patients in SSc-ILD trials and suggesting potential benefit of any anti-ILD drugs. Similarly, although slightly less common, DU should receive the same attention in the two subsets.
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- 2020
14. Health Assessment Questionnaire-Disability Index (HAQ-DI) use in modelling disease progression in diffuse cutaneous systemic sclerosis: an analysis from the EUSTAR database
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Allanore Y., Bozzi S., Terlinden A., Huscher D., Amand C., Soubrane C., Siegert E., Czirjak L., Carreira P. E., Hachulla E., Zanatta E., Li M., Airo P., Mendoza F. A., Rosato E., Distler O. Svetlana Agachi, Walid Ahmed Abdel Atty Mohamed, Paolo Airò, Sabine Adler, Juan Jose Alegre-Sancho, Yannick Allanore, Lidia P. Ananieva, Codrina Ancuta, Branimir Anic, Fabiola Atzeni, Gianluigi Bajocchi, Alexandra Balbir-Gurman, Marko Baresic, Radim Becvar, Laura Belloli, Vera Bernardino, Washington Bianchi, Breno Valdetaro Bianchi, Otylia Kowal Bielecka, Francesco Paolo Cantatore, Patricia E. Carreira, Ivan Castellví, Marco Matucci Cerinic, Carlo Chizzolini, Lorinda S. Chung, Bernard Coleiro, Maura Couto, Mary Ellen Csuka, Giovanna Cuomo, Maurizio Cutolo, László Czirják, Lorenzo Dagna, Nemanja Damjanov, Ellen De Langhe, Francesco Del Galdo, Carlos de la Puente, Christopher Denton, Y., Allanore, S., Bozzi, A., Terlinden, D., Huscher, C., Amand, C., Soubrane, E., Siegert, L., Czirjak, P. E., Carreira, E., Hachulla, E., Zanatta, M., Li, P., Airo, F. A., Mendoza, E., Rosato, Svetlana Agachi, Distler O., Ahmed Abdel Atty Mohamed, Walid, Airò, Paolo, Adler, Sabine, Jose Alegre-Sancho, Juan, Allanore, Yannick, Ananieva, Lidia P., Ancuta, Codrina, Anic, Branimir, Atzeni, Fabiola, Bajocchi, Gianluigi, Balbir-Gurman, Alexandra, Baresic, Marko, Becvar, Radim, Belloli, Laura, Bernardino, Vera, Bianchi, Washington, Valdetaro Bianchi, Breno, Kowal Bielecka, Otylia, Paolo Cantatore, Francesco, Carreira, Patricia E., Castellví, Ivan, Matucci Cerinic, Marco, Chizzolini, Carlo, Chung, Lorinda S., Coleiro, Bernard, Couto, Maura, Ellen Csuka, Mary, Cuomo, Giovanna, Cutolo, Maurizio, Czirják, László, Dagna, Lorenzo, Damjanov, Nemanja, De Langhe, Ellen, Del Galdo, Francesco, de la Puente, Carlo, Denton, Christopher, Publica, and University of Zurich
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0301 basic medicine ,Longitudinal study ,lcsh:Diseases of the musculoskeletal system ,medicine.medical_treatment ,2745 Rheumatology ,HAQ-DI score ,computer.software_genre ,Severity of Illness Index ,0302 clinical medicine ,Quality of life ,Surveys and Questionnaires ,Medicine and Health Sciences ,Longitudinal Studies ,610 Medicine & health ,skin and connective tissue diseases ,Database ,Mortality rate ,10051 Rheumatology Clinic and Institute of Physical Medicine ,EUSTAR registry, Diffuse cutaneous systemic sclerosis, Health Assessment Questionnaire-Disability Index, HAQ-DI score ,2723 Immunology and Allergy ,Disease Progression ,Hemodialysis ,ARTHRITIS ,CLINICAL-TRIALS ,Research Article ,musculoskeletal diseases ,medicine.medical_specialty ,Diffuse cutaneous systemic sclerosis ,PREDICTING MORTALITY ,EUSTAR registry ,CLASSIFICATION ,03 medical and health sciences ,Internal medicine ,SCORE ,medicine ,Humans ,030203 arthritis & rheumatology ,2403 Immunology ,Scleroderma, Systemic ,business.industry ,Proportional hazards model ,Diffuse cutaneous systemic sclerosi ,Health Assessment Questionnaire-Disability Index ,Rheumatology ,Clinical trial ,030104 developmental biology ,Scleroderma, Diffuse ,Quality of Life ,FUNCTIONAL DISABILITY ,Observational study ,lcsh:RC925-935 ,Mitoxantrone ,business ,computer - Abstract
BackgroundPatients with diffuse cutaneous systemic sclerosis (dcSSc) have a poor prognosis. The importance of monitoring subjective measures of functioning and disability, such as the Health Assessment Questionnaire-Disability Index (HAQ-DI), is important as dcSSc is rated by patients as worse than diabetes or hemodialysis for quality of life impairment. This European Scleroderma Trials and Research (EUSTAR) database analysis was undertaken to examine the importance of impaired functionality in dcSSc prognosis. The primary objectives were to identify predictors of death and HAQ-DI score progression over 1 year. HAQ-DI score, major advanced organ involvement, and death rate were also used to develop a comprehensive model to predict lifetime dcSSc progression.MethodsThis was an observational, longitudinal study in patients with dcSSc registered in EUSTAR. Death and HAQ-DI scores were, respectively, analyzed by Cox regression and linear regression analyses in relation to baseline covariates. A microsimulation Markov model was developed to estimate/predict natural progression of dcSSc over a patient’s lifetime.ResultsThe analysis included dcSSc patients with (N = 690) and without (N = 4132) HAQ-DI score assessments from the EUSTAR database. Baseline HAQ-DI score, corticosteroid treatment, and major advanced organ involvement were predictive of death on multivariable analysis; a 1-point increase in baseline HAQ-DI score multiplied the risk of death by 2.7 (p p p ConclusionsHAQ-DI score and major advanced organ involvement were comparable predictors of mortality risk in dcSSc. Baseline mRSS and baseline HAQ-DI score were predictive of HAQ-DI score progression at 1 year, indicating a correlation between these endpoints in monitoring disease progression. It is hoped that this EUSTAR analysis may change physician perception about the importance of the HAQ-DI score in dcSSc.
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- 2020
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15. Evidence that deletion at FCGR3B is a risk factor for systemic sclerosis
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McKinney, C, Broen, J C A, Vonk, M C, Beretta, L, Hesselstrand, R, Hunzelmann, N, Riemekasten, G, Scorza, R, Simeon, C P, Fonollosa, V, Carreira, P E, Ortego-Centeno, N, Gonzalez-Gay, M A, Airo, P, Coenen, M, Martin, J, Radstake, T R D J, and Merriman, T R
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- 2012
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16. Nailfold capillaroscopy in Behçet’s disease, analysis of 128 patients
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Movasat, Atusa, Shahram, Farhad, Carreira, Patricia E., Nadji, Abdolhadi, Akhlaghi, Maassoomeh, Naderi, Nassim, and Davatchi, Fereydoun
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- 2009
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17. An open-label pilot study of infliximab therapy in diffuse cutaneous systemic sclerosis
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Denton, C P, Engelhart, M, Tvede, N, Wilson, H, Khan, K, Shiwen, X, Carreira, P E, Gonzalez, Diaz F, Black, C M, and van den Hoogen, F H
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- 2009
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18. ‘Quality of pain’ in systemic sclerosis
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Carreira, P. E.
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- 2006
19. Update of EULAR recommendations for the treatment of systemic sclerosis
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Kowal-Bielecka O., Fransen J., Avouac J., Becker M., Kulak A., Allanore Y., Distler O., Clements P., Cutolo M., Czirjak L., Damjanov N., Del Galdo F., Denton C. P., Distler J. H. W., Foeldvari I., Figelstone K., Frerix M., Furst D. E., Guiducci S., Hunzelmann N., Khanna D., Matucci-Cerinic M., Herrick A. L., Van Den Hoogen F., Van Laar J. M., Riemekasten G., Silver R., Smith V., Sulli A., Tarner I., Tyndall A., Welling J., Wigley F., Valentini G., Walker U. A., Zulian F., Muller-Ladner U., Daikeler T., Lanciano E., Becvar R., Tomcik M., Gindzienska-Sieskiewicz E., Cuomo G., Iudici M., Rednic S., Vlachoyiannopoulos P. G., Caporali R., Carreira P. E., Novak S., Minier T., Kucharz E. J., Gabrielli A., Moroncini G., Airo' P., Hesselstrand R., Martinovic D., Radic M., Marasovic-Krstulovic D., Braun-Moscovici Y., Balbir-Gurman A., Lo Monaco A., Caramaschi P., Morovic-Vergles J., Henes J., Ortiz Santamaria V., Heitmann S., Krasowska D., Seidel M. F., Hasler P., Pereira Da Silva J. A., Salvador M. J., Stamenkovic B., Stankovic A., Tikly M., Ananieva L. P., Beretta L., Szucs G., Szamosi S., de la Puente Bujidos C., Midtvedt O., Hoffmann-Vold A. -M., Launay D., Hachulla E., Riccieri V., Ionescu R., Opris D., Mihai C., Herrgott I., Beyer C., Ingegnoli F., von Muhlen C. A., Alegre-Sancho J. J., Beltran-Catalan E., Aringer M., Fantana J., Leuchten N., Tausche A. -K., De Langhe E., Vanthuyne M., Anic B., Baresic M., Mayer M., Uprus M., Otsa K., Yavuz S., Granel B., Azevedo V. F., Muller C., Jimenez S. A., Popa S., Agachi S., Zenone T., Stebbings S., Dockerty J., Vacca A., Schollum J., Veale D. J., Toloza S., Xu D., Olas J., Rosato E., Foti R., Adler S., Dan D., Wiesik-Szewczyk E., Olesinska M., Kayser C., Fathi N., de la Pena Lefebvre P. G., Imbert B., Kowal-Bielecka, O., Fransen, J., Avouac, J., Becker, M., Kulak, A., Allanore, Y., Distler, O., Clements, P., Cutolo, M., Czirjak, L., Damjanov, N., Del Galdo, F., Denton, C. P., Distler, J. H. W., Foeldvari, I., Figelstone, K., Frerix, M., Furst, D. E., Guiducci, S., Hunzelmann, N., Khanna, D., Matucci-Cerinic, M., Herrick, A. L., Van Den Hoogen, F., Van Laar, J. M., Riemekasten, G., Silver, R., Smith, V., Sulli, A., Tarner, I., Tyndall, A., Welling, J., Wigley, F., Valentini, G., Walker, U. A., Zulian, F., Muller-Ladner, U., Daikeler, T., Lanciano, E., Becvar, R., Tomcik, M., Gindzienska-Sieskiewicz, E., Cuomo, G., Iudici, M., Rednic, S., Vlachoyiannopoulos, P. G., Caporali, R., Carreira, P. E., Novak, S., Minier, T., Kucharz, E. J., Gabrielli, A., Moroncini, G., Airo', P., Hesselstrand, R., Martinovic, D., Radic, M., Marasovic-Krstulovic, D., Braun-Moscovici, Y., Balbir-Gurman, A., Lo Monaco, A., Caramaschi, P., Morovic-Vergles, J., Henes, J., Ortiz Santamaria, V., Heitmann, S., Krasowska, D., Seidel, M. F., Hasler, P., Pereira Da Silva, J. A., Salvador, M. J., Stamenkovic, B., Stankovic, A., Tikly, M., Ananieva, L. P., Beretta, L., Szucs, G., Szamosi, S., de la Puente Bujidos, C., Midtvedt, O., Hoffmann-Vold, A. -M., Launay, D., Hachulla, E., Riccieri, V., Ionescu, R., Opris, D., Mihai, C., Herrgott, I., Beyer, C., Ingegnoli, F., von Muhlen, C. A., Alegre-Sancho, J. J., Beltran-Catalan, E., Aringer, M., Fantana, J., Leuchten, N., Tausche, A. -K., De Langhe, E., Vanthuyne, M., Anic, B., Baresic, M., Mayer, M., Uprus, M., Otsa, K., Yavuz, S., Granel, B., Azevedo, V. F., Muller, C., Jimenez, S. A., Popa, S., Agachi, S., Zenone, T., Stebbings, S., Dockerty, J., Vacca, A., Schollum, J., Veale, D. J., Toloza, S., Xu, D., Olas, J., Rosato, E., Foti, R., Adler, S., Dan, D., Wiesik-Szewczyk, E., Olesinska, M., Kayser, C., Fathi, N., de la Pena Lefebvre, P. G., Imbert, B., UCL - SSS/IREC/SLUC - Pôle St.-Luc, UCL - (MGD) Service de rhumatologie, Kowal Bielecka, Otylia, Fransen, Jaap, Avouac, Jerome, Becker, Mike, Kulak, Agnieszka, Allanore, Yannick, Distler, Oliver, Clements, Philip, Cutolo, Maurizio, Czirjak, Laszlo, Damjanov, Nemanja, del Galdo, Francesco, Denton, Christopher P., Distler, Jörg H. W., Foeldvari, Ivan, Figelstone, Kim, Frerix, Marc, Furst, Daniel E., Guiducci, Serena, Hunzelmann, Nicola, Khanna, Dinesh, Matucci Cerinic, Marco, Herrick, Ariane L., van den Hoogen, Frank, van Laar, Jacob M., Riemekasten, Gabriela, Silver, Richard, Smith, Vanessa, Sulli, Alberto, Tarner, Ingo, Tyndall, Alan, Welling, Joep, Wigley, Frederic, Valentini, Gabriele, Walker, Ulrich A., Zulian, Francesco, Müller Ladner, Ulf, Daikeler, Thoma, Lanciano, Elisabetta, Becvã¡r, Radim, Tomcik, Michal, Gindzienska Sieskiewicz, Ewa, Iudici, Michele, Rednic, Simona, Vlachoyiannopoulos, Panayiotis G., Caporali, Roberto, Carreira, Patricia E., Novak, Srdan, Minier, Tã¼nde, Kucharz, Eugene J., Gabrielli, Armando, Moroncini, Gianluca, Airo, Paolo, Hesselstrand, Roger, Martinovic, Duska, Radic, Mislav, Marasovic Krstulovic, Daniela, Braun Moscovici, Yolanda, Monaco, Andrea Lo, Morovic Vergles, Jadranka, Culo, Melanie I., Henes, Jã¶rg, Santamaria, Vera Ortiz, Heitmann, Stefan, Krasowska, Dorota, Michalska Jakubus, Malgorzata, Seidel, Matthias F., Klinik III, Medizinische, Hasler, Paul, Da Silva, José A. Pereira, Salvador, Maria J., Stamenkovic, Bojana, Stankovic, Aleksandra, Tikly, Mohammed, Ananieva, Lidia P., Beretta, Lorenzo, Szucs, Gabriella, Szamosi, Szilvia, de la Puente Bujidos, Carlo, Midtvedt, Øyvind, Hoffmann Vold, Anna Maria, Launay, David, Hachulla, Eric, Riccieri, Valeria, Ionescu, Ruxandra, Opris, Daniela, Mihai, Carina, Herrgott, Ilka, Beyer, Christian, Ingegnoli, Francesca, von Mühlen, Carlos Alberto, Alegre Sancho, Juan José, Beltran Catalan, Emma, Aringer, Martin, Fantana, Julia, Leuchten, Nicolai, Tausche, Anne Kathrin, Langhe, Ellen De, Vanthuyne, Marie, Anic, Branimir, Bareå¡ic, Marko, Mayer, Miroslav, Ãœprus, Maria, Otsa, Kati, Yavuz, Sule, Granel, Brigitte, Jimenez, Sergio A., Popa, Serghei, Agachi, Svetlana, Zenone, Thierry, Stebbings, Simon, Dockerty, Joanne, Vacca, Alessandra, Schollum, Joanna, Veale, Douglas J., Toloza, Sergio, Xu, Dong, Olas, Jacek, Rosato, Edoardo, Foti, Rosario, Adler, Sabine, Dan, Diana, Wiesik Szewczyk, Ewa, Olesinska, Marzena, Kayser, Cristiane, Fathi, Nihal, de la Peña Lefebvre, Paloma GarcÃa, Imbert, Bernard, and Cuomo, Giovanna
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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.
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- 2017
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20. What have multicentre registries across the world taught us about the disease features of systemic sclerosis?
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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.
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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.
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- 2017
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21. Cyclooxygenase-1 and -2 are expressed by human T cells
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PABLOS, J. L., SANTIAGO, B., CARREIRA, P. E., GALINDO, M., and GOMEZ-REINO, J. J.
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- 1999
22. Clinical Characteristics of Juvenile Idiopathic Inflammatory Myopathy and Comparison With Adult Patients
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Loarce-Martos, Jesús, Larena, Carmen, Blázquez, M. Ángeles, Joven, Beatriz E., Carreira, Patricia E., Martínez-Barrio, Julia, Monteagudo, Indalecio, López-Longo, Francisco Javier, Ruiz, Lucía, López-Robledillo, Juan Carlos, Almodóvar, Raquel, Llorente, Irene, Tomero, Eva, García-de la Peña, Paloma, Moruno, Henry, Pérez, Ana, Cobo-Ibáñez, Tatiana, Lojo Oliveira, Leticia, Barbadillo, María Carmen, García-De Yébenes, María Jesús, and Nuño-Nuño, Laura
- Abstract
Few studies have been published focusing on the differences between juvenile idiopathic inflammatory myopathy (JIIM) and adult IIM. This study aimed to describe the characteristics of JIIM main subgroups (juvenile dermatomyositis [JDM] and juvenile polymyositis [JPM]) and to compare their differences with adult IIM subgroups (adult DM and adult PM).
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- 2022
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23. There is a need for new systemic sclerosis subset criteria. A content analytic approach
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Johnson, S. R., Soowamber, M. L., Fransen, J., Khanna, D., Van den Hoogen, F., Baron, M., Matucci-Cerinic, M., Denton, C. P., Medsger, T. A., Jr., Carreira, P. E., Riemekasten, G., Distler, J., Gabrielli, A., Steen, V., Chung, L., Silver, R., Varga, J., Mueller-Ladner, U., Vonk, M. C., Walker, U. A., Wollheim, F. A., Herrick, A., Furst, D. E., Czirjak, L., Kowal-Bielecka, O., Del Galdo, F., Cutolo, M., Hunzelmann, N., Murray, C. D., Foeldvari, I., Mouthon, L., Damjanov, N., Kahaleh, B., Frech, T., Assassi, S., Saketkoo, L. A., Pope, J. E., Johnson, S. R., Soowamber, M. L., Fransen, J., Khanna, D., Van den Hoogen, F., Baron, M., Matucci-Cerinic, M., Denton, C. P., Medsger, T. A., Jr., Carreira, P. E., Riemekasten, G., Distler, J., Gabrielli, A., Steen, V., Chung, L., Silver, R., Varga, J., Mueller-Ladner, U., Vonk, M. C., Walker, U. A., Wollheim, F. A., Herrick, A., Furst, D. E., Czirjak, L., Kowal-Bielecka, O., Del Galdo, F., Cutolo, M., Hunzelmann, N., Murray, C. D., Foeldvari, I., Mouthon, L., Damjanov, N., Kahaleh, B., Frech, T., Assassi, S., Saketkoo, L. A., and Pope, J. E.
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Objectives. Systemic sclerosis (SSc) is heterogenous. The objectives of this study were to evaluate the purpose, strengths and limitations of existing SSc subset criteria, and identify ideas among experts about subsets. Methods. We conducted semi-structured interviews with randomly sampled international SSc experts. The interview transcripts underwent an iterative process with text deconstructed to single thought units until a saturated conceptual framework with coding was achieved and respondent occurrence tabulated. Serial cross-referential analyses of clusters were developed. Results. Thirty experts from 13 countries were included; 67% were male, 63% were from Europe and 37% from North America; median experience of 22.5years, with a median of 55 new SSc patients annually. Three thematic clusters regarding subsetting were identified: research and communication; management; and prognosis (prediction of internal organ involvement, survival). The strength of the limited/diffuse system was its ease of use, however 10% stated this system had marginal value. Shortcomings of the diffuse/limited classification were the risk of misclassification, predictions/generalizations did not always hold true, and that the elbow or knee threshold was arbitrary. Eighty-seven percent use more than 2 subsets including: SSc sine scleroderma, overlap conditions, antibody-determined subsets, speed of progression, and age of onset (juvenile, elderly). Conclusions. We have synthesized an international view of the construct of SSc subsets in the modern era. We found a number of factors underlying the construct of SSc subsets. Considerations for the next phase include rate of change and hierarchal clustering (e.g. limited/diffuse, then by antibodies).
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- 2018
24. NEW SYSTEMIC SCLEROSIS RISK LOCI IDENTIFIED THROUGH A META-GWAS STRATEGY
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Lopez-Isac, E., Acosta-Herrera, M., Assassi, S., Simeon, C. P., Carreira, P. E., Castellvi, I., Ortego-Centeno, N., Beretta, L., Lunardi, C., Gabrielli, A., Moroncini, G., Hunzelmann, N., Witte, T., Distler, J. H., Franke, A., Voskuyl, A. E., de Vries-Bouwstra, J., Wijmenga, C., Hesselstrand, R., Nordin, A., Hoffmann-Vold, A. -M., Herrick, A., Worthington, J., Denton, C. P., Brown, M. A., Allanore, Y., Radstake, T. R., Fonseca, C., Mayes, M. D., Martin, J., Lopez-Isac, E., Acosta-Herrera, M., Assassi, S., Simeon, C. P., Carreira, P. E., Castellvi, I., Ortego-Centeno, N., Beretta, L., Lunardi, C., Gabrielli, A., Moroncini, G., Hunzelmann, N., Witte, T., Distler, J. H., Franke, A., Voskuyl, A. E., de Vries-Bouwstra, J., Wijmenga, C., Hesselstrand, R., Nordin, A., Hoffmann-Vold, A. -M., Herrick, A., Worthington, J., Denton, C. P., Brown, M. A., Allanore, Y., Radstake, T. R., Fonseca, C., Mayes, M. D., and Martin, J.
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- 2018
25. Incidence and predictors of cutaneous manifestations during the early course of systemic sclerosis. a 10-year longitudinal study from the EUSTAR database
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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
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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.
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- 2016
26. Prediction of worsening of skin fibrosis in patients with diffuse cutaneous systemic sclerosis using the EUSTAR database
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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
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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.
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- 2014
27. Antibodies to extractable cellular antigens in Spanish systemic lupus erythematosus (SLE) patients
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Carreira, P. E., Mateo, I., Garcia, G., Garrido, G., and Gomez-Reino, J.
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- 1987
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28. C-reactive protein (CRP) levels in systemic lupus erythematosus (SLE)
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Linares, L. F., Gomez-Reino, J. J., Carreira, P. E., Morillas, L., and Ibero, I.
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- 1986
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29. Abatacept in patients with rheumatoid arthritis and interstitial lung disease: A national multicenter study of 63 patients.
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Fernández-Díaz, Carlos, Loricera, Javier, Castañeda, Santos, López-Mejías, Raquel, Ojeda-García, Clara, Olivé, Alejandro, Rodríguez-Muguruza, Samantha, Carreira, Patricia E., Pérez-Sandoval, Trinidad, Retuerto, Miriam, Cervantes-Pérez, Evelin C., Flores-Robles, Bryan J., Hernández-Cruz, Blanca, Urruticoechea, Ana, Maíz-Alonso, Olga, Arboleya, Luis, Bonilla, Gema, Hernández-Rodríguez, Íñigo, Palma, Desirée, and Delgado, Concepción
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Objective Interstitial lung disease (ILD) is one of the most serious complications of rheumatoid arthritis (RA). In the present study, we aimed to assess the efficacy of abatacept (ABA) in patients with ILD associated to RA. Methods National multicenter, non-controlled, open-label registry study of RA patients with ILD treated with ABA. Results 63 patients (36 women) with RA-associated ILD undergoing ABA therapy were studied. The mean ± standard deviation age at the time of the study was 63.2 ± 9.8 years. The median duration of RA and ILD from diagnosis were 6.8 and 1 year, respectively. RA was seropositive in 55 patients (87.3%). In 15 (23.8%) of 63 patients the development of ILD was closely related to the administration of synthetic or biologic disease modifying anti-rheumatic drugs. After a follow-up of 9.4 ± 3.2 months, two-thirds of patients remained stable whereas one-quarter experienced improvement in the Modified Medical Research Council scale. At that time forced vital capacity remained stable in almost two-thirds of patents and improved in one out of five patients assessed. Also, diffusing capacity of the lung for carbon monoxide remained stable in almost two-thirds and showed improvement in a quarter of the patients assessed. At 12 months, 50% of the 22 patients in whom chest HRCT scan was performed due persistence of respiratory symptoms showed stabilization, 8 (36.4%) improvement and 3 worsening of the HRCT scan pattern. Eleven of 63 patients had to discontinue ABA, mainly due to adverse events. Conclusion ABA appears to be an effective in RA-associated ILD. [ABSTRACT FROM AUTHOR]
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- 2018
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30. 2013 ACR/EULAR systemic sclerosis classification criteria in patients with associated pulmonary arterial hypertension.
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Joven, Beatriz E., Escribano, Pilar, Andreu, Jose Luis, Loza, Estibaliz, Jimenez, Carmen, de Yebenes, M. Jesus Garcia, Ruiz-Cano, M. Jose, Carmona, Loreto, and Carreira, Patricia E.
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Objective To analyze the performance of the 1980 ACR and new 2013 ACR/EULAR criteria for systemic sclerosis (SSc) in cutaneous SSc (lcSSc) patients, especially those affected by lcSSc and pulmonary arterial hypertension (PAH). Methods All patients with a clinical lcSSc diagnosis from a prospective observational SSc cohort were included. Sociodemographic and disease-related variables were collected, and PAH confirmed by right heart catheterization (RHC). Performance of the 2013 and 1980 SSc criteria was analyzed in terms of clinical diagnosis. Descriptive and between-group analyses were performed as to the fulfillment of criterion sets, including comparison of survival. Results Overall, 321 patients were included, 63% of whom fulfilled the 1980 ACR and 93% the 2013 ACR/EULAR criteria. Agreement between both criteria sets proved poor ( κ = 0.23). LcSSC patients fulfilling both criterion sets were significantly younger at diagnosis, whilst presenting organ involvement, calcinosis, fingertip digital ulcers, and pitting scars more frequently than those who met the 2013 criteria only. Patients who fulfilled the 2013 but not the 1980 criteria presented a higher degree of ACA positivity and PAH. Nearly 12% of patients developed PAH. Patients who did not meet the 1980 criteria were affected by a milder disease from but demonstrated higher pulmonary vascular resistance and lower cardiac index than those fulfilling both criterion sets. Whereas patients with PAH met the 2013 criteria, only 47% fulfilled the 1980 criteria. Regardless of criterion set fulfillment, high mortality was observed in PAH patients, with no significant between-patient difference based on criterion set. Conclusion The new 2013 ARC/EULAR criteria prove more accurate than the former 1980 ACR criteria in identifying and differentiating patients with lcSSc, especially those with associated PAH. Since PAH exhibits a better prognosis if treated early, all SSc patients should undergo PAH screening. [ABSTRACT FROM AUTHOR]
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- 2018
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31. Items for developing revised classification criteria in systemic sclerosis: Results of a consensus exercise with the ACR/EULAR working committee for classification criteria in systemic sclerosis
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Fransen, J, Johnson, S R, van den Hoogen, F, Baron, M, Allanore, Y, Carreira, P E, Czirják, L, Denton, C P, Distler, O, Furst, D E, Gabrielli, A, Herrick, A, Inanc, M, Kahaleh, B, Kowal-Bielecka, O, Medsger, T A, Mueller-Ladner, U, Riemekasten, G, Sierakowski, S, Valentini, G, Veale, D, Vonk, M C, Walker, U, Chung, L, Clements, P J, Collier, D H, Csuka, M E, Jimenez, S, Merkel, P A, Seibold, J R, Silver, R, Steen, V, Tyndall, A, Matucci-Cerinic, M, Pope, J E, Khanna, D, Fransen, J, Johnson, Sr, van den Hoogen, F, Baron, M, Allanore, Y, Carreira, Pe, Czirják, L, Denton, Cp, Distler, O, Furst, De, Gabrielli, A, Herrick, A, Inanc, M, Kahaleh, B, Kowal Bielecka, O, Medsger TA, Jr, Mueller Ladner, U, Riemekasten, G, Sierakowski, S, Valentini, Gabriele, Veale, D, Vonk, Mc, Walker, U, Chung, L, Clements, Pj, Collier, Dh, Csuka, Me, Jimenez, S, Merkel, Pa, Seibold, Jr, Silver, R, Steen, V, Tyndall, A, Matucci Cerinic, M, Pope, Je, Khanna, D., University of Zurich, and Khanna, D
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2745 Rheumatology ,10051 Rheumatology Clinic and Institute of Physical Medicine ,610 Medicine & health ,Delphi technique, nominal group technique, systemic sclerosis, scleroderma, classification, classification criteria - Abstract
Background Classification criteria for systemic sclerosis (SSc) are being updated. Objective To select a set of items potentially useful for the classification of SSc using consensus procedures including the Delphi and nominal group techniques (NGT). Methods Items were identified through two independent consensus exercises performed by the Scleroderma Clinical Trials Consortium (SCTC) and the EULAR Scleroderma Trials and Research Group (EUSTAR). The first-round items from both exercises were collated and redundancies were removed leaving 168 items. A 3-round Delphi exercise was performed using a 1–9 scale (1=completely inappropriate and 9=completely appropriate) and a consensus meeting using NGT. During the last Delphi, the items were ranked on a 1–10 scale. Results Round 1: 106 experts rated the 168 items. Those with a median score
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- 2012
32. Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database
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Tyndall, A. J., Bannert, B., Vonk, M., Airo, P., Cozzi, F., Carreira, P. E., Bancel, D. F., Allanore, Y., Muller Ladner, U., Distler, O., Iannone, F., Pellerito, R., Pileckyte, M., Miniati, I., Ananieva, L., Gurman, A. B., Damjanov, N., Mueller, A., Valentini, G., Riemekasten, G., Tikly, M., Hummers, L., Henriques, M. J. S., Caramaschi, P., Scheja, A., Rozman, B., Ton, E., Kumanovics, G., Coleiro, B., Feierl, E., Szucs, G., Von Muhlen, C. A., Riccieri, Valeria, Novak, S., Chizzolini, C., Kotulska, A., Denton, C., Coelho, P. C., Kotter, I., Simsek, I., La Pena, D. E., Lefebvre, P. G. D. L. P., Hachulla, E., Seibold, J. R., Rednic, S., Stork, J., Morovic Vergles, J., Walker, U. A., Tyndall, Aj, Bannert, B, Vonk, M, Airò, P, Cozzi, F, Carreira, Pe, Bancel, Df, Allanore, Y, MÜLLER LADNER, U, Distler, O, Iannone, F, Pellerito, R, Pileckyte, M, Miniati, I, Ananieva, L, Gurman, Ab, Damjanov, N, Mueller, A, Valentini, Gabriele, Riemekasten, G, Tikly, M, Hummers, L, Henriques, Mj, Caramaschi, P, Scheja, A, Rozman, B, Ton, E, Kumánovics, G, Coleiro, B, Feierl, E, Szucs, G, VON MÜHLEN, Ca, Riccieri, V, Novak, S, Chizzolini, C, Kotulska, A, Denton, C, Coelho, Pc, Kötter, I, Simsek, I, DE LA PENA LEFEBVRE, Pg, Hachulla, E, Seibold, Jr, Rednic, S, Stork, J, MOROVIC VERGLES, J, and Walker, Ua
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Lung Diseases ,systemic sclreosis ,risk factors ,Male ,Lung Diseases/mortality ,Comorbidity ,030204 cardiovascular system & hematology ,0302 clinical medicine ,Neoplasms ,Epidemiology ,Pulmonary fibrosis ,Immunology and Allergy ,skin and connective tissue diseases ,Cause of death ,ddc:616 ,integumentary system ,Interstitial lung disease ,Sepsis/mortality ,Middle Aged ,Prognosis ,3. Good health ,Pneumonia/mortality ,Cohort ,Female ,Neoplasms/mortality ,Gastrointestinal Hemorrhage ,Scleroderma, Systemic/*mortality ,Adult ,medicine.medical_specialty ,Heart Diseases ,Immunology ,Auto-immunity, transplantation and immunotherapy [N4i 4] ,General Biochemistry, Genetics and Molecular Biology ,03 medical and health sciences ,Rheumatology ,Sepsis ,Internal medicine ,medicine ,Humans ,Gastrointestinal Hemorrhage/mortality ,Risk factor ,Health care ethics [NCEBP 5] ,Aged ,030203 arthritis & rheumatology ,Scleroderma, Systemic ,business.industry ,Proportional hazards model ,Heart Diseases/mortality ,Pneumonia ,medicine.disease ,Surgery ,Evaluation of complex medical interventions [NCEBP 2] ,Heart failure ,business ,Epidemiologic Methods - Abstract
Item does not contain fulltext OBJECTIVES: To determine the causes and predictors of mortality in systemic sclerosis (SSc). METHODS: Patients with SSc (n=5860) fulfilling the American College of Rheumatology criteria and prospectively followed in the EULAR Scleroderma Trials and Research (EUSTAR) cohort were analysed. EUSTAR centres completed a structured questionnaire on cause of death and comorbidities. Kaplan-Meier and Cox proportional hazards models were used to analyse survival in SSc subgroups and to identify predictors of mortality. RESULTS: Questionnaires were obtained on 234 of 284 fatalities. 55% of deaths were attributed directly to SSc and 41% to non-SSc causes; in 4% the cause of death was not assigned. Of the SSc-related deaths, 35% were attributed to pulmonary fibrosis, 26% to pulmonary arterial hypertension (PAH) and 26% to cardiac causes (mainly heart failure and arrhythmias). Among the non-SSc-related causes, infections (33%) and malignancies (31%) were followed by cardiovascular causes (29%). Of the non-SSc-related fatalities, 25% died of causes in which SSc-related complications may have participated (pneumonia, sepsis and gastrointestinal haemorrhage). Independent risk factors for mortality and their HR were: proteinuria (HR 3.34), the presence of PAH based on echocardiography (HR 2.02), pulmonary restriction (forced vital capacity below 80% of normal, HR 1.64), dyspnoea above New York Heart Association class II (HR 1.61), diffusing capacity of the lung (HR 1.20 per 10% decrease), patient age at onset of Raynaud's phenomenon (HR 1.30 per 10 years) and the modified Rodnan skin score (HR 1.20 per 10 score points). CONCLUSION: Disease-related causes, in particular pulmonary fibrosis, PAH and cardiac causes, accounted for the majority of deaths in SSc. 01 oktober 2010
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- 2010
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33. Validation of the 2013 American College of Rheumatology/European League Against Rheumatism classification criteria for systemic sclerosis in patients from a capillaroscopy clinic.
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Melchor, Sheila, Joven, Beatriz E., Andreu, Jose Luis, Loza, Estibaliz, Garcia de Yebenes, M. Jesus, Carmona, Loreto, and Carreira, Patricia E.
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Objective To validate the 2013 ACR/EULAR classification criteria for systemic sclerosis (SSc) in patients from a capillaroscopy clinic. Methods All patients attended in a capillaroscopy clinic were included. Sociodemographic and SSc-related variables were collected. Using as gold standard for SSc the clinical judgement, the performance (sensitivity, specificity, predictive values, and likelihood ratios) of the 2013 ACR/EULAR criteria were analyzed. Receiver operating characteristic (ROC) curve and the area under the curve (AUC) were calculated for the global score and individual items, and the best cutoffs were obtained. Results We included 327 patients (84% women, mean age at capillaroscopy 48 years). Main reasons for capillaroscopy referral were Raynaud’s phenomenon (39%) and SSc evaluation (27%). The most frequent final clinical diagnosis were SSc (32.4%) and primary Raynaud’s phenomenon (25.7%). The 2013 ACR/EULAR SSc classification criteria were met by 116 patients (35.5%). Sensitivity and specificity of the new criteria were 98.1% and 94.6%, respectively, and positive and negative predictive values were 89.7% and 99.1%. The individual variables with the best sensitivity were Raynaud’s phenomenon (99.1%) and abnormal nailfold capillaries (81.1%). All the individual variables, except Raynaud’s phenomenon, puffy fingers and sclerodactily showed high specificity values, over 90%. The best cutoffs of the total score were ≥8, ≥9, and ≥10, and the AUC = 0.993. Conclusions We validated the new ACR/EULAR classification criteria for SSc in unselected patients from a capillaroscopy clinic. Global score and individual items included in the new criteria show high diagnostic accuracy and discriminatory capacity. [ABSTRACT FROM AUTHOR]
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- 2016
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34. L1 Retrotransposon Heterogeneity in Ovarian Tumor Cell Evolution
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Nguyen, Thu H.M., Carreira, Patricia E., Sanchez-Luque, Francisco J., Schauer, Stephanie N., Fagg, Allister C., Richardson, Sandra R., Davies, Claire M., Jesuadian, J. Samuel, Kempen, Marie-Jeanne H.C., Troskie, Robin-Lee, James, Cini, Beaven, Elizabeth A., Wallis, Tristan P., Coward, Jermaine I.G., Chetty, Naven P., Crandon, Alexander J., Venter, Deon J., Armes, Jane E., Perrin, Lewis C., Hooper, John D., Ewing, Adam D., Upton, Kyle R., and Faulkner, Geoffrey J.
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LINE-1 (L1) retrotransposons are a source of insertional mutagenesis in tumor cells. However, the clinical significance of L1 mobilization during tumorigenesis remains unclear. Here, we applied retrotransposon capture sequencing (RC-seq) to multiple single-cell clones isolated from five ovarian cancer cell lines and HeLa cells and detected endogenous L1 retrotransposition in vitro. We then applied RC-seq to ovarian tumor and matched blood samples from 19 patients and identified 88 tumor-specific L1 insertions. In one tumor, an intronic de novoL1 insertion supplied a novel cis-enhancer to the putative chemoresistance gene STC1. Notably, the tumor subclone carrying the STC1L1 mutation increased in prevalence after chemotherapy, further increasing STC1expression. We also identified hypomethylated donor L1s responsible for new L1 insertions in tumors and cultivated cancer cells. These congruent in vitroand in vivoresults highlight L1 insertional mutagenesis as a common component of ovarian tumorigenesis and cancer genome heterogeneity.
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- 2018
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35. L1 retrotransposition is a common feature of mammalian hepatocarcinogenesis
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Schauer, Stephanie N., Carreira, Patricia E., Shukla, Ruchi, Gerhardt, Daniel J., Gerdes, Patricia, Sanchez-Luque, Francisco J., Nicoli, Paola, Kindlova, Michaela, Ghisletti, Serena, Santos, Alexandre Dos, Rapoud, Delphine, Samuel, Didier, Faivre, Jamila, Ewing, Adam D., Richardson, Sandra R., and Faulkner, Geoffrey J.
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The retrotransposon Long Interspersed Element 1 (LINE-1 or L1) is a continuing source of germline and somatic mutagenesis in mammals. Deregulated L1 activity is a hallmark of cancer, and L1 mutagenesis has been described in numerous human malignancies. We previously employed retrotransposon capture sequencing (RC-seq) to analyze hepatocellular carcinoma (HCC) samples from patients infected with hepatitis B or hepatitis C virus and identified L1 variants responsible for activating oncogenic pathways. Here, we have applied RC-seq and whole-genome sequencing (WGS) to an Abcb4 (Mdr2)−/−mouse model of hepatic carcinogenesis and demonstrated for the first time that L1 mobilization occurs in murine tumors. In 12 HCC nodules obtained from 10 animals, we validated four somatic L1 insertions by PCR and capillary sequencing, including TFsubfamily elements, and one GFsubfamily example. One of the TFinsertions carried a 3′ transduction, allowing us to identify its donor L1 and to demonstrate that this full-length TFelement retained retrotransposition capacity in cultured cancer cells. Using RC-seq, we also identified eight tumor-specific L1 insertions from 25 HCC patients with a history of alcohol abuse. Finally, we used RC-seq and WGS to identify three tumor-specific L1 insertions among 10 intra-hepatic cholangiocarcinoma (ICC) patients, including one insertion traced to a donor L1 on Chromosome 22 known to be highly active in other cancers. This study reveals L1 mobilization as a common feature of hepatocarcinogenesis in mammals, demonstrating that the phenomenon is not restricted to human viral HCC etiologies and is encountered in murine liver tumors.
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- 2018
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36. An open-label pilot study of infliximab therapy in diffuse cutaneous systemic sclerosis
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Denton, C P, Engelhart, M, Tvede, N, Wilson, H, Khan, K, Shiwen, X, Carreira, P E, Diaz Gonzalez, F, Black, C M, van den Hoogen, F H, Denton, C P, Engelhart, M, Tvede, N, Wilson, H, Khan, K, Shiwen, X, Carreira, P E, Diaz Gonzalez, F, Black, C M, and van den Hoogen, F H
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Udgivelsesdato: 2009-Sep, AIM: The safety and potential efficacy of a chimaeric anti-tumour necrosis factor alpha monoclonal antibody (infliximab) were examined in diffuse cutaneous systemic sclerosis (dcSSc). METHODS: A 26-week open-label pilot study in which 16 cases of dcSSc received five infusions of infliximab (5 mg/kg). Clinical assessment included skin sclerosis score, scleroderma health assessment questionnaire, self-reported functional score and physician global visual analogue scale. Collagen turnover, skin biopsy analysis and full safety evaluation were performed. RESULTS: There was no significant change in skin score at 26 weeks but a trend for lower modified Rodnan skin score at 22 weeks (OR 17, 95% CI 6 to 46) compared with peak value (OR 29, 95% CI 11 to 44; p = 0.10). Serum aminoterminal propeptide of type III collagen level was significantly lower at week 26 compared with baseline (p = 0.03). Secretion of type I collagen by dermal fibroblasts was reduced at 26 weeks compared with baseline (p = 0.02). There were no deaths during the study and no suspected unexpected serious adverse reactions. 21 serious adverse events (AE) occurred in seven subjects, mostly attributable to dcSSc. 127 distinct AE occurred in 16 subjects. Of these, 19 AE (15%) were probably or definitely related to infliximab treatment. Eight (50%) patients prematurely discontinued infliximab. Anti-infliximab antibodies developed during the study in five subjects and were significantly associated with suspected infusion reactions (p = 0.025). CONCLUSION: In dcSSc infliximab did not show clear benefit at 26 weeks but was associated with clinical stabilisation and a fall in two laboratory markers of collagen synthesis. The frequency of suspected infusion reactions may warrant additional immunosuppression in any future studies in systemic sclerosis.
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- 2008
37. Heritable L1 retrotransposition in the mouse primordial germline and early embryo
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Richardson, Sandra R., Gerdes, Patricia, Gerhardt, Daniel J., Sanchez-Luque, Francisco J., Bodea, Gabriela-Oana, Muñoz-Lopez, Martin, Jesuadian, J. Samuel, Kempen, Marie-Jeanne H.C., Carreira, Patricia E., Jeddeloh, Jeffrey A., Garcia-Perez, Jose L., Kazazian, Haig H., Ewing, Adam D., and Faulkner, Geoffrey J.
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LINE-1 (L1) retrotransposons are a noted source of genetic diversity and disease in mammals. To expand its genomic footprint, L1 must mobilize in cells that will contribute their genetic material to subsequent generations. Heritable L1 insertions may therefore arise in germ cells and in pluripotent embryonic cells, prior to germline specification, yet the frequency and predominant developmental timing of such events remain unclear. Here, we applied mouse retrotransposon capture sequencing (mRC-seq) and whole-genome sequencing (WGS) to pedigrees of C57BL/6J animals, and uncovered an L1 insertion rate of ≥1 event per eight births. We traced heritable L1 insertions to pluripotent embryonic cells and, strikingly, to early primordial germ cells (PGCs). New L1 insertions bore structural hallmarks of target-site primed reverse transcription (TPRT) and mobilized efficiently in a cultured cell retrotransposition assay. Together, our results highlight the rate and evolutionary impact of heritable L1 retrotransposition and reveal retrotransposition-mediated genomic diversification as a fundamental property of pluripotent embryonic cells in vivo.
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- 2017
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38. An open-label pilot study of infliximab therapy in diffuse cutaneous systemic sclerosis
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Denton, C P, primary, Engelhart, M, additional, Tvede, N, additional, Wilson, H, additional, Khan, K, additional, Shiwen, X, additional, Carreira, P E, additional, Gonzalez, F Diaz, additional, Black, C M, additional, and van den Hoogen, F H, additional
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- 2008
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39. Expression of types I, III and IV collagen genes in fibrotic skin and nerve lesions of toxic oil syndrome patients
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GOMEZ-REINO, J. J., primary, SANDBERG, M., additional, CARREIRA, P. E., additional, and VUORIO, E., additional
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- 2008
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40. Prevalence, Correlates and Outcomes of Gastric Antral Vascular Ectasia in Systemic Sclerosis: A EUSTAR Case-control Study.
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Ghrénassia, Etienne, Avouac, Jérome, Khanna, Dinesh, Derk, Chris T., Distler, Oliver, Suliman, Yossra Atef, Airo, Paolo, Carreira, Patricia E., Foti, Rosario, Granel, Brigitte, Berezne, Alice, Cabane, Jean, Ingegnoli, Francesca, Rosato, Edoardo, Caramaschi, Paola, Hesselstrand, Roger, Walker, Ulrich A., Alegre-Sancho, Juan Jose, Zarrouk, Virginie, and Agard, Christian
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- 2014
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41. Detection of COX-1 and COX-2 isoforms in synovial fluid cells from inflammatory joint diseases
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Iniguez, M. A., primary, Pablos, J. L., additional, Carreira, P. E., additional, Cabre, F., additional, and Gomez-Reino, J. J., additional
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- 1998
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42. POLYMORPHISM OF THE HEAT-SHOCK PROTEIN GENE HSP70-2IN SYSTEMIC LUPUS ERYTHEMATOSUS
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PABLOS, J. L., primary, CARREIRA, P. E., additional, MARTÍN-VILLA, J. M., additional, MONTALVO, G., additional, ARNAIZ-VILLENA, A., additional, and GOMEZ-REINO, J. J., additional
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- 1995
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43. Subcutaneous Nodular Amyloidosis in Sjögren's Syndrome
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Pablos, J. L., primary, Cogolludo, V., additional, Pinedo, F., additional, and Carreira, P. E., additional
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- 1993
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44. Survival, Causes of Death, and Risk Factors Associated With Mortality in Spanish Systemic Sclerosis Patients: Results From a Single University Hospital.
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Joven, Beatriz E., Almodovar, Raquel, Carmona, Loreto, and Carreira, Patricia E.
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Objective: To analyze the causes of death, survival, and risk factors for mortality in a large series of Spanish systemic sclerosis (SSc) patients followed over the last 25 years in a tertiary care university hospital. Methods: Demographic, clinical, and outcome data from all SSc patients followed in the rheumatology department were included in a database created in 1989. ANOVA, Kruskal-Wallis, or χ
2 tests were used to identify differences among groups; Kaplan-Meier analysis was used to estimate survival, and Cox proportional hazards regression analysis was used to identify factors associated with mortality. Results: A total of 204 patients were included, of whom 182 (89%) were women. Mean age at diagnosis was 49 ± 17 years, and mean follow-up was 8 years. Over 1635 patient-years, 36 of 44 deaths were attributable to SSc: 28 related to cardiorespiratory involvement, 4 to peripheral vascular disease, 3 to gastrointestinal, and 1 to renal involvement. The main SSc-unrelated causes of death were malignancy (3 cases) and infections (2 cases). Survival rates from disease onset were 85, 75, and 55% at 5, 10, and 20 years, respectively, with poorer survival in patients with renal disease and pulmonary hypertension (PH). Independent prognostic factors for mortality were older age at diagnosis, diffuse skin involvement, proteinuria, PH, and elevated erythrocyte sedimentation rate. Conclusions: Ten-year survival is over 70% in Spanish SSc patients. The main causes of death are lung and cardiac involvement, and to a lesser extent, peripheral vascular disease and coexisting malignancy. Diffuse subset, proteinuria, PH, elevated erythrocyte sedimentation rate and older age at diagnosis are the main risk factors for mortality. [Copyright &y& Elsevier]- Published
- 2010
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45. Inmune-mediated inflammatory rheumatic diseases in transgender people: A scoping review.
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Salgado, Eva, Romera-Baurés, Montserrat, Beltran-Catalan, Emma, Naredo, Esperanza, Carreira, Patricia E, Garcia-Vivar, Mariluz, Moreno-Muelas, Jose V, Boteanu, Alina, Calvo-Penades, Inma, Sellas-Fernandez, Agusti, Valero, Marta, and Gomez-Reino, Juan J
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In immune-mediated inflammatory rheumatic diseases (IMIRD), there are differences between cis-men and cis-women in epidemiology, clinical feature, therapeutic approach, treatment response, and prognosis. In transgender individuals, information concerning IMIRD is not substantial. The assessment of information concerning rheumatic diseases in transgenders is crucial because transgenders may undergo treatments with potential impacts on IMIRD. We aim to collect and discuss current knowledge on IMIRD in transgender individuals, determine the coverage of the literature, identify the knowledge gaps, and highlight opportunities for future research. We did a scoping review of publications collected through a systematic search of transgender patients with any IMIRD. Data sources were Medline, Embase, and Web of Knowledge. Synthesis of results and qualitative review of data information was collected in tables. A semi-quantification of the quality of the articles reporting clinical studies was performed. There were 11 transwoman, and 3 transmen cases of systemic lupus erythematosus (5 cases), skin lupus erythematosus (2), systemic sclerosis (4), anti-synthetase syndrome (1), rheumatoid arthritis (1) and ankylosing spondylitis (1). Eleven were de novo cases and three had prior history of IMIRD and developed a comorbidity after starting hormone replacement therapy. The clinical expression of the disease was variable. Two transwomen and one transman developed thrombotic events. The lupus skin lesions in one transman improved following testosterone treatment. No clinical studies were identified. Quality of publications was disparate. Although the number of cases is small, most cases of IMIRD occur in transwomen. The absence of solid data warrants caution in establishing recommendations regarding hormone replacement therapy in transgenders with IMIRD. There is an essential need for the consideration of cisgender and transgender particularities in future research on IMIRD [Display omitted] • Transgenders may undergo treatments with potential impacts on immune-mediated rheumatic diseases (IMRD). • Assessing immune-mediated rheumatic diseases in transgenders is crucial. • We conducted a scoping review to collect and discuss current knowledge on IMRD in transgender individuals, determine the coverage of the literature, and identify the knowledge gaps. • In the absence of solid data, establishing recommendations regarding hormone replacement therapy in transgenders is not feasible. • There is a need for the consideration of cisgender and transgender particularities in future research on IMRD. [ABSTRACT FROM AUTHOR]
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- 2022
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46. Reconciling Healthcare Professional and Patient Perspectives in the Development of Disease Activity and Response Criteria in Connective Tissue Disease–related Interstitial Lung Diseases
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Saketkoo, Lesley Ann, Mittoo, Shikha, Frankel, Sid, LeSage, Daphne, Sarver, Catherine, Phillips, Kristine, Strand, Vibeke, Matteson, Eric L., Baughman, Robert P., Brown, Kevin K., Christmann, Romy B., Dellaripa, Paul, Denton, Christopher P., Distler, Oliver, Fischer, Aryeh, Flaherty, Kevin, Huscher, Dörte, Khanna, Dinesh, Kowal-Bielecka, Otylia, Merkel, Peter A., Oddis, Chester V., Pittrow, David, Sandorfi, Nora, Seibold, James R., Swigris, Jeffrey, Wells, Athol, Antoniou, Katerina, Castelino, Flavia V., Christopher-Stine, Lisa, Collard, Harold R., Cottin, Vincent, Danoff, Sonye, Hedlund, Robert, Highland, Kristin B., Hummers, Laura, Lynch, David A., Kim, Dong Soon, Ryu, Jay H., Christopher-Stine, Lisa, Miller, Frederick W., Nichols, Karen, Proudman, Susanna M., Richeldi, Luca, Shah, Ami A., van den Assum, Pieter, Aggarwal, Rohit, Ainslie, Gillian, Alkassab, Firas, Allanore, Yannick, Anderson, Marina E., Andonopoulos, Andrew P., Antin-Ozerkis, Danielle, Antoniou, Katerina, Arrobas, Ana, Ascherman, Dana P., Assassi, Shervin, Baron, Murray, Bathon, Joan M., Baughman, Robert P., Behr, Juergen, Beretta, Lorenzo, Bingham, Clifton O., Binnie, Matthew, Birring, Surinder S., Boin, Francesco, Bongartz, Tim, Bourdin, Arnaud, Bouros, Demosthenes, Brasington, Richard, Bresser, Paul, Brown, Kevin K., Buch, Maya H., Burge, P. Sherwood, Carmona, Loreto, Castelino, Flavia V., Carreira, Patricia E., Carvalho, Carlos R.R., Catoggio, Luis J., Chan, Kevin M., Chapman, Jeffrey, Chatterjee, Soumya, Christmann, Romy B., Christopher-Stine, Lisa, Chua, Felix, Chung, Lorinda, Conron, Matthew, Corte, Tamera, Cosgrove, Gregory, Costabel, Ulrich, Cottin, Vincent, Cox, Gerard, Crestani, Bruno, Crofford, Leslie J., Csuka, Mary E., Curbelo, Pablo, Czirják, László, Daniil, Zoe, Danoff, Sonye, D’Arsigny, Christine L., Davis, Gerald S., de Andrade, Joao A., Dellaripa, Paul F., De Vuyst, Paul, Dempsey, Owen J., Denton, Christopher P., Derk, Chris T., Distler, Jörg, Distler, Oliver, Dixon, William G., Downey, Gregory, Doyle, Mittie K., Drent, Marjolein, Durairaj, Lakshmi, Emery, Paul, Espinoza, Luis R., Farge, Dominique, Fathi, Maryam, Fell, Charlene D., Fessler, Barri J., Fischer, Aryeh, Fitzgerald, John E., Flaherty, Kevin R., Foeldvari, Ivan, Fox, George A., Frech, Tracy M., Freitas, Sara, Furst, Daniel E., Gabrielli, Armando, García-Vicuña, Rosario, Georgiev, Ognian B., Gerbino, Anthony, Gillisen, Adrian, Gladman, Dafna D., Glassberg, Marilyn, Gochuico, Bernadette R., Gogali, Athena, Goh, Nicole S., Goldberg, Avram, Goldberg, Hilary J., Gourley, Mark F., Griffing, Leroy, Grutters, Jan C., Gunnarsson, Ragnar, Hachulla, Eric, Hall, Frances C., Harari, Sergio, Herrick, Ariane L., Herzog, Erica L., Hesselstrand, Roger, Highland, Kristin, Hirani, Nikhil, Hodgson, Ulla, Hollingsworth, Helen M., Homer, Robert J., Hoyles, Rachel K., Hsu, Vivien M., Hubbard, Richard B., Hummers, Laura, Hunzelmann, Nicolas, Huscher, Dörte, Isasi, Maria Eloisa, Isasi, Elida Susana, Jacobsen, Soren, Jimenez, Sergio A., Johnson, Sindhu R., Jones, Christine H., Kahaleh, Bashar, Kairalla, Ronaldo A., Kalluri, Meena, Kalra, Sanjay, Kaner, Robert J., Khanna, Dinesh, Kim, Dong Soon, Kinder, Brent W., Kiter, Goksel, Klingsberg, Ross C., Kokosi, Maria, Kolb, Martin R.J., Kowal-Bielecka, Otylia M., Kur-Zalewska, Joanna, Kuwana, Masataka, Lake, Fiona R., Lally, Edward V., Lasky, Joseph A., Laurindo, Ileda M., Able, Lawrence, Lee, Peter, Leonard, Colm T., Lien, Dale C., Limper, Andrew H., Liossis, Stamatis-Nick C., Lohr, Kristine M., Loyd, James E., Lundberg, Ingrid E., Mageto, Yolanda N., Maher, Toby M., Mahmud, Tafazzul H., Manganas, Helene, Marie, Isabelle, Marras, Theodore K., Martinez, José Antônio Baddini, Martinez, Fernando J., Mathieu, Alessandro, Matucci-Cerinic, Marco, Mayes, Maureen D., McKown, Kevin M., Medsger, Thomas A., Meehan, Richard T., Mendes, Ana Cristina, Merkel, Peter A., Meyer, Keith C., Millar, Ann B., Miller, Frederick W., Moğulkoç, Nesrin, Molitor, Jerry A., Morais, António, Mouthon, Luc, Müller, Veronika, Müller-Quernheim, Joachim, Nadashkevich, Oleg, Nador, Roland, Nash, Peter, Nathan, Steven D., Navarro, Carmen, Neves, Sofia, Noth, Imre, Nunes, Hilario, Oddis, Chester V., Olson, Amy L., Opitz, Christian F., Padilla, Maria, Pappas, Dimitrios, Parfrey, Helen, Pego-Reigosa, José M., Pereira, Carlos A.C., Perez, Rafael, Phillips, Kristine, Pittrow, David, Pope, Janet E., Porter, Joanna C., Proudman, Susanna M., Renzoni, Elisabeth A., Richeldi, Luca, Riemekasten, Gabriela, Riley, David J., Rischmueller, Maureen, Rodriguez-Reyna, Tatiana S., Rojas-Serrano, J., Roman, Jesse, Rosen, Glenn D., Rossman, Milton, Rothfield, Naomi, Ryu, Jay H., Sahn, Steven A., Sandorfi, Nora, Sanduzzi, Alessandro, Scholand, Mary Beth, Seibold, James R., Selman, Moises, Senécal, Jean-Luc, Seo, Philip, Shah, Ami, Silver, Richard M., Solomon, Joshua J., Steen, Virginia, Stevens, Wendy, Strange, Charlie, Sussman, Robert, Sutton, Evelyn D., Sweiss, Nadera J., Swigris, Jeffrey, Tornling, Göran, Tzelepis, George E., Undurraga, Alvaro, Vacca, Allessandra, Vancheri, Carlo, Varga, Janos, Veale, Douglas J., Volkov, Suncica, Walker, Ulrich A., Wells, Athol U., Wencel, Mark, Wesselius, Lewis J., Wickremasinghe, Melissa, Wilcox, Pearce, Wilsher, Margaret L., Wollheim, Frank A., Wuyts, Wim A., Yung, Gordon, Zanon, Pietro, Zappala, Christopher J., Groshong, Steve D., Leslie, Kevin O., Myers, Jeffrey L., Padera, Robert F., Desai, Sujal R., Goldin, Jonathan, Kazerooni, Ella A., Klein, Jeffrey S., Lynch, David A., and Keen, Kevin J.
- Abstract
Interstitial lung diseases (ILD), including those related to connective tissue disease (CTD), and idiopathic pulmonary fibrosis (IPF) carry high morbidity and mortality. Great efforts are under way to develop and investigate meaningful treatments in the context of clinical trials. However, efforts have been challenged by a lack of validated outcome measures and by inconsistent use of measures in clinical trials. Lack of consensus has fragmented effective use of strategies in CTD-ILD and IPF, with a history of resultant difficulties in obtaining agency approval of treatment interventions. Until recently, the patient perspective to determine domains and outcome measures in CTD-ILD and IPF had never been applied. Efforts described here demonstrate unequivocally the value and influence of patient involvement on core set development. Regarding CTD-ILD, this is the first OMERACT working group to directly address a manifestation/comorbidity of a rheumatic disease (ILD) as well as a disease not considered rheumatic (IPF). The OMERACT 11 proceedings of the CTD-ILD Working Group describe the forward and lateral process to include both the medical and patient perspectives in the urgently needed identification of a core set of preliminary domains and outcome measures in CTD-ILD and IPF.
- Published
- 2014
- Full Text
- View/download PDF
47. Validation of potential classification criteria for systemic sclerosis
- Author
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Johnson, Sindhu R., Fransen, Jaap, Khanna, Dinesh, Baron, Murray, van den Hoogen, Frank, Medsger, Thomas A., Peschken, Christine A., Carreira, Patricia E., Riemekasten, Gabriela, Tyndall, Alan, Matucci‐Cerinic, Marco, and Pope, Janet E.
- Published
- 2012
- Full Text
- View/download PDF
48. Items for developing revised classification criteria in systemic sclerosis: Results of a consensus exercise
- Author
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Fransen, Jaap, Johnson, Sindhu R., van den Hoogen, Frank, Baron, Murray, Allanore, Yannick, Carreira, Patricia E., Czirják, László, Denton, Christopher P., Distler, Oliver, Furst, Daniel E., Gabrielli, Armando, Herrick, Ariane, Inanc, Murat, Kahaleh, Bashar, Kowal‐Bielecka, Otylia, Medsger, Thomas A., Mueller‐Ladner, Ulf, Riemekasten, Gabriela, Sierakowski, Stanislaw, Valentini, Gabriele, Veale, Doug, Vonk, Madelon C., Walker, Ulrich, Chung, Lorinda, Clements, Philip J., Collier, David H., Csuka, Mary E., Jimenez, Sergio, Merkel, Peter A., Seibold, James R., Silver, Richard, Steen, Virginia, Tyndall, Alan, Matucci‐Cerinic, Marco, Pope, Janet E., and Khanna, Dinesh
- Published
- 2012
- Full Text
- View/download PDF
49. Expression of types I, III and IV collagen genes in fibrotic skin and nerve lesions of toxic oil syndrome patients.
- Author
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Gomez-Reino, J. J., Sandberg, M., Carreira, P. E., and Vuorio, E.
- Subjects
COLLAGEN diseases ,DNA probes ,CIRCULAR DNA ,FIBROBLASTS ,EXTRACELLULAR matrix proteins ,DIAGNOSTIC use of in-situ hybridization ,NUCLEIC acid hybridization - Abstract
We have studied the skin and nerve fibrosis in toxie oil syndrome by in situ hybridization using specific cDNA probes for types I, III, and IV collagens. Fibroblasts with high levels of type I and III collagen mRNA were observed in biopsies from fibrotic skin areas. Similarly, type IV collagen mRNA was abundant in cells within the fibrotic process of the nerves. These results suggest that the excessive accumulation of collagen in toxic oil syndrome results from transcriptional activation of collagen genes in a subpopulation of fibroblasts. [ABSTRACT FROM AUTHOR]
- Published
- 1993
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50. Keratinocyte apoptosis and p53 expression in cutaneous lupus and dermatomyositis
- Author
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Pablos, Jose L., Santiago, Begoña, Galindo, Maria, Carreira, Patricia E., Ballestin, Claudio, and Gomez‐Reino, Juan J.
- Abstract
Keratinocyte apoptosis may be induced by ultraviolet‐B radiation and represents a potential source of fragmented autoantigens in autoimmune diseases. This study investigates whether excessive keratinocyte apoptosis occurs in the skin lesions of cutaneous lupus (CLE) and dermatomyositis (DM) and the potential mechanisms responsible for this phenomenon. Skin biopsies have been studied from 19 patients with CLE and DM, eight with scleroderma, and five healthy controls. Apoptosis was detected by in situend‐labelling of fragmented DNA. The expression of Bcl‐2, PCNA, p53, and Ki‐67 proteins was studied by immunohistochemistry. In DM and CLE skin, the number of apoptotic keratinocytes was significantly increased (p=0·008) compared with normal skin. In both diseases, a large accumulation of apoptotic keratinocytes and apoptotic bodies was present in the disrupted basal zone. Unlike normal skin, a large number of keratinocytes, particularly those morphologically apoptotic, expressed p53 protein. A significant increase in the number of proliferating Ki‐67 positive (p=0·0007) and PCNA‐positive (p=0·0008) nuclei was also observed. In both CLE and DM, exaggerated and inappropriate keratinocyte apoptosis occurs. It is associated with increased expression of p53 and PCNA. This suggests that normal solar radiation alone or in combination with additional local factors induces DNA damage and excessive keratinocyte apoptosis in these autoimmune diseases of the skin. Apoptosis can mediate the severe epidermal lesions observed in both diseases and the release of fragmented autoantigens into the dermis. Copyright © 1999 John Wiley & Sons, Ltd.
- Published
- 1999
- Full Text
- View/download PDF
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