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2. World Health Organization (WHO) International Classification of Functioning, Disability and Health (ICF) Core Set Development for Interstitial Lung Disease.

Author

Saketkoo, Lesley, Escorpizo, Reuben, Varga, Janos, Keen, Kevin, Fligelstone, Kim, Birring, Surinder, Alexanderson, Helene, Pettersson, Henrik, Chaudhry, Humza, Poole, Janet, Regardt, Malin, LeSage, Daphne, Sarver, Catherine, Lanario, Joseph, Renzoni, Elisabetta, Scholand, Mary, Lammi, Matthew, Kowal-Bielecka, Otylia, Distler, Oliver, Frech, Tracy, Shapiro, Lee, Varju, Cecilia, Volkmann, Elizabeth, Bernstein, Elana, Drent, Marjolein, Obi, Ogugua, Patterson, Karen, and Russell, Anne-Marie

Subjects
CTD-ILD, ICD-11, connective tissue, cough, fibrosis, patient-reported outcomes
Abstract

Background: The World Health Organization (WHO) introduced the International Classification of Functioning, Disability, and Health (ICF) as a scientific method of disability data collection comprised of >1,200 categories describing the spectrum of impairment types (functional, symptoms-based and anatomical) under the bio-psycho-social model with consideration of environmental and personal factors (pf). ICF Core Sets and ICF Checklists are streamlined disease-specific resources for clinical use, service provision, and for use in health economics and health policy. ICF can disclose strengths and weaknesses across multiple patient-reported outcome measures (PROMs) and help consolidate best-fitting question-items from multiple PROMs. Interstitial lung diseases (ILDs), are generally progressive, with restrictive physiology sometimes occurring in the context of multi-organ autoimmunity/inflammatory conditions such as connective tissue diseases (CTDs). In spite of significant associated morbidity and potential disability, ILD has yet to be linked to the ICF. Methods: Each instrument and their question-items within the consensus-recommended core sets for clinical trials in ILD were deconstructed to single concept units, and then linked per updated ICF linkage rules. Inter-linker agreement was established. Three additional subsequently validated measures were also included. Results: One-hundred-eleven ICF categories were identified for ten PROMs and three traditional objective measures that were amenable to ICF linkage. The proportion of agreement ranged from 0.79 (95% CI: 0.62, 0.91) to 0.93 (0.76, 0.99) with the overall proportion of inter-linker agreement being very high 0.86 (0.82, 0.89) for the initial instruments, with 94-100% for the three additional PROMs. Thirty-four new Personal Factors emerged to capture disease-specific qualities not elsewhere described in ICF, e.g. pf_embarrassed by cough or pf_panic/afraid when cant get a breath. Conclusion: This first known effort in ICF linkage of ILD has provided important revelations on the current utility of the ICF in lung disease. Results have indicated areas for meaningful assessment of ICF descriptors for lung impairment. The mapping across PROMs provides insight into possibilities of developing more streamline and precise instrumentation. Finally, familiarity with the ICF in ILD may enable clinicians to experience a smoother transition with the imminent harmonization of ICD and ICF, ICD-11.

Published
2022

3. A comprehensive framework for navigating patient care in systemic sclerosis: A global response to the need for improving the practice of diagnostic and preventive strategies in SSc.

Author

Saketkoo, Lesley, Frech, Tracy, Varjú, Cecília, Domsic, Robyn, Farrell, Jessica, Gordon, Jessica, Mihai, Carina, Sandorfi, Nora, Shapiro, Lee, Poole, Janet, Volkmann, Elizabeth, Lammi, Monika, McAnally, Kendra, Alexanderson, Helene, Pettersson, Henrik, Hant, Faye, Kuwana, Masataka, Shah, Ami, Smith, Vanessa, Hsu, Vivien, Kowal-Bielecka, Otylia, Assassi, Shervin, Cutolo, Maurizio, Kayser, Cristiane, Shanmugam, Victoria, Vonk, Madelon, Fligelstone, Kim, Baldwin, Nancy, Connolly, Kerri, Ronnow, Anneliese, Toth, Beata, Suave, Maureen, Farrington, Sue, Bernstein, Elana, Crofford, Leslie, Czirják, László, Jensen, Kelly, Hinchclif, Monique, Hudson, Marie, Lammi, Matthew, Mansour, Jennifer, Morgan, Nadia, Mendoza, Fabian, Nikpour, Mandana, Pauling, John, Riemekasten, Gabriela, Russell, Anne-Marie, Scholand, Mary, Seigart, Elise, Rodriguez-Reyna, Tatiana, Hummers, Laura, Walker, Ulrich, and Steen, Virginia

Subjects
Disability, Interstitial lung disease, Pulmonary fibrosis, Pulmonary hypertension, Quality of life, Renal crisis, Scleroderma, Symptom burden, Systemic sclerosis, Humans, Hypertension, Pulmonary, Lung, Lung Diseases, Interstitial, Patient Care, Scleroderma, Systemic
Abstract

Systemic sclerosis (SSc), the most lethal of rheumatologic conditions, is the cause of death in >50% of SSc cases, led by pulmonary fibrosis followed by pulmonary hypertension and then scleroderma renal crisis (SRC). Multiple other preventable and treatable SSc-related vascular, cardiac, gastrointestinal, nutritional and musculoskeletal complications can lead to disability and death. Vascular injury with subsequent inflammation transforming to irreversible fibrosis and permanent damage characterizes SSc. Organ involvement is often present early in the disease course of SSc, but requires careful history-taking and vigilance in screening to detect. Inflammation is potentially reversible provided that treatment intensity quells inflammation and other immune mechanisms. In any SSc phenotype, opportunities for early treatment are prone to be under-utilized, especially in slowly progressive phenotypes that, in contrast to severe progressive ILD, indolently accrue irreversible organ damage resulting in later-stage life-limiting complications such as pulmonary hypertension, cardiac involvement, and malnutrition. A single SSc patient visit often requires much more physician and staff time, organization, vigilance, and direct management for multiple organ systems compared to other rheumatic or pulmonary diseases. Efficiency and efficacy of comprehensive SSc care enlists trending of symptoms and bio-data. Financial sustainability of SSc care benefits from understanding insurance reimbursement and health system allocation policies for complex patients. Sharing care between recognised SSc centers and local cardiology/pulmonary/rheumatology/gastroenterology colleagues may prevent complications and poor outcomes, while providing support to local specialists. As scleroderma specialists, we offer a practical framework with tools to facilitate an optimal, comprehensive and sustainable approach to SSc care. Improved health outcomes in SSc relies upon recogntion, management and, to the extent possible, prevention of SSc and treatment-related complications.

Published
2021

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

Author

Vacca, Alessandra, Giollo, Alessandro, Balbir-Gurman, Alexandra, Gheorghiu, Ana Maria, Marcoccia, Antonella, Herrick, Ariane, Radic, Mislav, Stamenkovic, Bojana, Anic, Branimir, Granel, Brigitte, Ribi, Camillo, Selmi, Carlo Francesco, Carlos de la Puente, Milano, de Souza Müller, Carolina, Denton, Christopher, Kayser, Cristiane, Tanaseanu, Cristina-Mihaela, Majewski, Dominik, Rimar, Doron, Krasowska, Dorota, Veale, Douglas, Walker, Ulrich, Kerzberg, Eduardo, Rezus, Elena, Zanatta, Elisabetta, Siegert, Elise, De Langhe, Ellen, Oksel, Fahrettin, Ingegnoli, Francesca, Cantatore, Francesco Paolo, Szücs, Gabriela, Cuomo, Giovanna, Seskute, Goda, Litinsky, VilniusIra, Castellví, Ivan, Morovic-Vergles, Jadranka, Sibilia, Jean, Henes, Jörg, Solanki, Kamal, Perdan-Pirkmajer, Katja, Herrmann, Kristine, Saketkoo, Lesley Ann, Stamp, Lisa, Mouthon, Luc, Salvador, Maria João, Pozzi, Maria Rosa, Üprus, Maria, Vanthuyne, Marie, Engelhart, Merete, Köhm, Michaela, Iudici, Michele, Inanc, Murat, Fathi, Nihal, Pamuk, Nuri, García de la Peña Lefebv, Paloma, Carreira, Patricia E., Bancel, Dominique Farge, Moroncini, Luca, Montecucco, C., Ancuta, Codrina, Sunderkötter, Cord, Müller-Ladner, Ulf, Rosato, Edoardo, Kucharz, Eugene J., Iannone, Florenzo, Del Galdo, Francesco, Poormoghim, Hadi, Kötter, Ina, Distler, Jörg, Cutolo, Maurizio, Tikly, Mohammed, Damjanov, Nemanja, Hunzelmann, Nicolas, Vlachoyiannopoulos, P., Hasler, Paul, Sarzi Puttini, Piercarlo, Wiland, Piotr, Becvar, Radim, Yavuz, Sule, Zdrojewski, Zbigniew, Pellerito, Raffaele, Foti, Rosario, Ionescu, Ruxandra Maria, Adler, Sabine, Kahl, Sarah, Moiseev, Sergey, Stebbings, Simon, Rednic, Simona, Negrini, Simone, Heitmann, Stefan, Ullman, Susanne, Agachi, Svetlana, Martin, Thierry, Schmeiser, Tim, Riccieri, Valeria, Smith, Vanessa, Bernardino, Vera, Ortiz-Santamaria, Vera, Hsu, Vivien M., Abdel Atty Mohamed, Walid Ahmed, Hoffmann-Vold, Anna-Maria, Brunborg, Cathrine, Airò, Paolo, Ananyeva, Lidia P., Czirják, László, Guiducci, Serena, Hachulla, Eric, Li, Mengtao, Mihai, Carina, Riemekasten, Gabriela, Sfikakis, Petros P., Valentini, Gabriele, Kowal-Bielecka, Otylia, Allanore, Yannick, and Distler, Oliver

Published
2023
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5. Stratification in systemic sclerosis according to autoantibody status versus skin involvement: a study of the prospective EUSTAR cohort

Author

Matucci Cerinic, Marco, Walker, Ulrich, Iannone, Florenzo, Jordan, Suzana, Becvar, Radim, Kowal Bielecka, Otylia, Cutolo, Maurizio, Cuomo, Giovanna, Kedor, Claudia, Rednic, Simona, Avouac, Jérome, Vlachoyiannopoulos, P., Montecucco, C., Stork, Jiri, Inanc, Murat, Carreira, Patricia E., Novak, Srdan, Czirják, László, Iudici, Michele, Kucharz, Eugene J., Zanatta, Elisabetta, Perdan-Pirkmajer, Katja, Coleiro, Bernard, Moroncini, Gianluca, Farge Bancel, Dominique, Airò, Paolo, Hesselstrand, Roger, Radic, Mislav, Braun-Moscovici, Yolanda, Lo Monaco, Andrea, Hunzelmann, Nicolas, Pellerito, Raffaele, Giollo, Alessandro, Morovic-Vergles, Jadranka, Denton, Christopher, Vonk, Madelon, Damjanov, Nemanja, Henes, Jörg, Ortiz Santamaria, Vera, Heitmann, Stefan, Krasowska, Dorota, Hasler, Paul, Kohm, Michaela, Foeldvari, Ivan, Bajocchi, Gianluigi, Salvador, Maria João, Stamenkovic, Bojana, Selmi, Carlo Francesco, Tikly, Mohammed, Ananieva, Lidia P., Herrick, Ariane, Müller-Ladner, Ulf, De Palma, Raffaele, Engelhart, Merete, Szücs, Gabriela, Sobrino Grande, Cristina, Midtvedt, Øyvind, Launay, David, Riccieri, Valeria, Ionescu, Ruxandra Maria, Sha, Ami, Gheorghiu, Ana Maria, Sunderkötter, Cord, Ingegnoli, Francesca, Mouthon, Luc, Smith, Vanessa, Cantatore, Francesco Paolo, Ullman, Susanne, Alberto von Mühlen, Carlos, Pozzi, Maria Rosa, Eyerich, Kilian, Wiland, Piotr, Vanthuyne, Marie, Alegre-Sancho, Juan Jose, Herrmann, Kristine, De Langhe, Ellen, Anic, Branimir, Üprus, Maria, Yavuz, Sule, Granel, Brigitte, de Souza Müller, Carolina, Busquets, Joanna, Agachi, Svetlana, Stebbings, Simon, Mathieu, D'Alessandro, Sampaio-Barros, Percival D., Stamp, Lisa, Solanki, Kamal, Veale, Douglas, Loyo, Esthela, Li, Mengtao, Abdel Atty Mohamed, Walid Ahmed, Gigante, Antonietta, Oksel, Fahrettin, Tanaseanu, Cristina-Mihaela, Foti, Rosario, Ancuta, Codrina, Maurer, Britta, van Laar, Jacob, Kayser, Cristiane, Fathi, Nihal, García de la Peña Lefebvre, Paloma, Sibilia, Jean, Litinsky, Ira, Abignano, Giuseppina, Seskute, Goda, Saketkoo, Lesley Ann, Kerzberg, Eduardo, Bianchi, Washington, Castellví, Ivan, Limonta, Massimiliano, Rimar, Doron, Couto, Maura, Spertini, François, Marcoccia, Antonella, Kahl, Sarah, Hsu, Ivien M., Martin, Thierry, Moiseev, Sergey, Chung, Lorinda S., Schmeiser, Tim, Majewski, Dominik, Zdrojewski, Zbigniew, Martínez-Barrio, Julia, Bernardino, Vera, Sommerlatte, Sabine, Levy, Yair, Rezus, Elena, Nuri Pamuk, Omer, Sarzi Puttini, Piercarlo, Poormoghim, Hadi, Kötter, Ina, Gaches, Francis, Belloli, Laura, Sfikakis, Petros, Markus, Juliana, Feldman, Gary R, Ramazan, Ana-Maria, Scherer, H.U., Truchetet, Marie-Elise, Lescoat, Alain, Dagna, Lorenzo, van Laar, J.M., Rudnicka, Lidia, Oliveira, Susana, Atzeni, Fabiola, Kuwana, Masataka, Mekinian, Arsene, Martin, Mickaël, Tanaka, Yoshiya, Elhai, Muriel, Sritharan, Nanthara, Boubaya, Marouane, Balbir-Gurman, Alexandra, Siegert, Elise, Hachulla, Eric, de Vries-Bouwstra, Jeska, Riemekasten, Gabriela, Distler, Jörg H W, Rosato, Edoardo, Del Galdo, Francesco, Mendoza, Fabian A, Furst, Daniel E, de la Puente, Carlos, Hoffmann-Vold, Anna-Maria, Gabrielli, Armando, Distler, Oliver, Bloch-Queyrat, Coralie, and Allanore, Yannick

Published
2022
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6. Outcomes of limited cutaneous systemic sclerosis patients: Results on more than 12,000 patients from the EUSTAR database

Author

Frantz, Camelia, Huscher, Dorte, Avouac, Jérôme, Hachulla, Eric, Balbir-Gurman, Alexandra, Riemekasten, Gabriela, Siegert, Elise, Lazzaroni, Maria-Grazia, Carreira, Patricia E., Vettori, Serena, Zanatta, Elisabetta, Ullman, Susanne, Czirjàk, Laszlo, Kowal-Bielecka, Otylia, Distler, Oliver, Matucci-Cerinic, Marco, and Allanore, Yannick

Published
2020
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8. Connective tissue disease related interstitial lung diseases and idiopathic pulmonary fibrosis: provisional core sets of domains and instruments for use in clinical trials.

Author

Saketkoo, Lesley Ann, Mittoo, Shikha, Huscher, Dörte, Khanna, Dinesh, Dellaripa, Paul F, Distler, Oliver, Flaherty, Kevin R, Frankel, Sid, Oddis, Chester V, Denton, Christopher P, Fischer, Aryeh, Kowal-Bielecka, Otylia M, LeSage, Daphne, Merkel, Peter A, Phillips, Kristine, Pittrow, David, Swigris, Jeffrey, Antoniou, Katerina, Baughman, Robert P, Castelino, Flavia V, Christmann, Romy B, Christopher-Stine, Lisa, Collard, Harold R, Cottin, Vincent, Danoff, Sonye, Highland, Kristin B, Hummers, Laura, Shah, Ami A, Kim, Dong Soon, Lynch, David A, Miller, Frederick W, Proudman, Susanna M, Richeldi, Luca, Ryu, Jay H, Sandorfi, Nora, Sarver, Catherine, Wells, Athol U, Strand, Vibeke, Matteson, Eric L, Brown, Kevin K, Seibold, James R, and CTD-ILD Special Interest Group

Subjects
CTD-ILD Special Interest Group, Humans, Lung Diseases, Interstitial, Connective Tissue Diseases, Registries, Consensus, International Cooperation, Societies, Medical, Congresses as Topic, Randomized Controlled Trials as Topic, Idiopathic Pulmonary Fibrosis, Connective tissue disease associated lung disease, Idiopathic pulmonary fibrosis, Interstitial Fibrosis, Rheumatoid lung disease, Systemic disease and lungs, Lung Diseases, Interstitial, Societies, Medical, Respiratory System, Clinical Sciences
Abstract

RationaleClinical trial design in interstitial lung diseases (ILDs) has been hampered by lack of consensus on appropriate outcome measures for reliably assessing treatment response. In the setting of connective tissue diseases (CTDs), some measures of ILD disease activity and severity may be confounded by non-pulmonary comorbidities.MethodsThe Connective Tissue Disease associated Interstitial Lung Disease (CTD-ILD) working group of Outcome Measures in Rheumatology-a non-profit international organisation dedicated to consensus methodology in identification of outcome measures-conducted a series of investigations which included a Delphi process including >248 ILD medical experts as well as patient focus groups culminating in a nominal group panel of ILD experts and patients. The goal was to define and develop a consensus on the status of outcome measure candidates for use in randomised controlled trials in CTD-ILD and idiopathic pulmonary fibrosis (IPF).ResultsA core set comprising specific measures in the domains of lung physiology, lung imaging, survival, dyspnoea, cough and health-related quality of life is proposed as appropriate for consideration for use in a hypothetical 1-year multicentre clinical trial for either CTD-ILD or IPF. As many widely used instruments were found to lack full validation, an agenda for future research is proposed.ConclusionIdentification of consensus preliminary domains and instruments to measure them was attained and is a major advance anticipated to facilitate multicentre RCTs in the field.

Published
2014

11. Ciąża a choroby przewlekłe

Author

Olesińska, Marzena, primary, Teliga-Czajkowska, Justyna, additional, Bazała-Bęćkowska, Katarzyna, additional, Bik, Emil, additional, Bobrowska-Starska, Danuta, additional, Ciupa, Agata, additional, Czajkowski, Krzysztof, additional, Czuszyńska, Zenobia, additional, Dizner-Gołąb, Anna, additional, Drozdowicz-Jastrzębska, Ewa, additional, Dziewit, Martyna, additional, Fijałkowska, Anna, additional, Frasuńska, Justyna, additional, Gietka, Piotr, additional, Gryka-Marton, Małgorzata, additional, Haładyj, Ewa, additional, Jankowska, Katarzyna, additional, Janowska, Ewa, additional, Jaszczyk, Bożena, additional, Kopacz, Dorota, additional, Kopeć, Izabella, additional, Kosowicz, Mariola, additional, Kowal-Bielecka, Otylia, additional, Kowalska, Katarzyna, additional, Kozakowski, Jarosław, additional, Kucharski, Marek, additional, Kulpa, Marta, additional, Kwiatkowska, Brygida, additional, Leszczyński, Piotr, additional, Lichodziejewska-Niemierko, Monika, additional, Maciejewicz, Piotr, additional, Madajczak, Dariusz, additional, Majdan, Maria, additional, Makowska, Joanna, additional, Masiak, Anna, additional, Mastalerz, Lucyna, additional, Maślińska, Maria, additional, Matusiewicz, Agata, additional, Milkiewicz, Piotr, additional, Nałęcz-Janik, Jolanta, additional, Nowakowska-Płaza, Anna, additional, Opoka-Winiarska, Violetta, additional, Ornowska, Sylwia, additional, Ostanek, Lidia, additional, Pawlak-Buś, Katarzyna, additional, Pazik, Joanna, additional, Płaza, Mateusz, additional, Przepiera-Będzak, Hanna, additional, Radziwoń-Zaleska, Maria, additional, Rekosz, Agnieszka, additional, Rogowska, Agnieszka, additional, Romanowska-Próchnicka, Katarzyna, additional, Smolarczyk, Roman, additional, Suchta, Katarzyna, additional, Targońska-Stępniak, Bożena, additional, Tarnacka, Beata, additional, Woroń, Jarosław, additional, Wronka, Karolina M., additional, Wudarski, Mariusz, additional, Wunsch, Ewa, additional, and Zagórowicz, Edyta, additional

Published
2023
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13. Cohort enrichment strategies for progressive interstitial lung disease in systemic sclerosis from EUSTAR

Author

Hoffmann-Vold, Anna-Maria; https://orcid.org/0000-0001-6467-7422, Brunborg, Cathrine, Airò, Paolo, Ananyeva, Lidia P, Czirják, László, Guiducci, Serena, Hachulla, Eric; https://orcid.org/0000-0001-7432-847X, Li, Mengtao; https://orcid.org/0000-0003-4252-2889, Mihai, Carina; https://orcid.org/0000-0002-8627-8817, Riemekasten, Gabriela, Sfikakis, Petros P; https://orcid.org/0000-0001-5484-2930, Valentini, Gabriele; https://orcid.org/0000-0002-7852-9137, Kowal-Bielecka, Otylia, Allanore, Yannick; https://orcid.org/0000-0002-6149-0002, Distler, Oliver; https://orcid.org/0000-0002-0546-8310, Hoffmann-Vold, Anna-Maria; https://orcid.org/0000-0001-6467-7422, Brunborg, Cathrine, Airò, Paolo, Ananyeva, Lidia P, Czirják, László, Guiducci, Serena, Hachulla, Eric; https://orcid.org/0000-0001-7432-847X, Li, Mengtao; https://orcid.org/0000-0003-4252-2889, Mihai, Carina; https://orcid.org/0000-0002-8627-8817, Riemekasten, Gabriela, Sfikakis, Petros P; https://orcid.org/0000-0001-5484-2930, Valentini, Gabriele; https://orcid.org/0000-0002-7852-9137, Kowal-Bielecka, Otylia, Allanore, Yannick; https://orcid.org/0000-0002-6149-0002, and Distler, Oliver; https://orcid.org/0000-0002-0546-8310

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, representativeness and feasibility in SSc-ILD patients from the European Scleroderma Trials and Research (EUSTAR) database? STUDY DESIGN AND METHODS: We applied the inclusion criteria of major recent SSc-ILD trials (focuSSced, SLS II and SENSCIS) and assessed progressive ILD, defined as absolute change in forced vital capacity (FVC) and as significant progression (FVC decline ≥10%). Data were compared to all patients and patients not fulfilling any inclusion criteria. RESULTS: In total, 2258 SSc-ILD patients were included, with 31.2% meeting SENSCIS, 5.8% SLS II, 1.6% focuSSced and 1529 (67.7%) not meeting any criteria. In the first 12+/-3 months, the absolute FVC decline in all and in patients fulfilling criteria from SENSCIS was -0.1%, from focuSSced -3.7%, and from SLS II 2.3%, with accompanying more progressors in focuSSced. The patient populations fulfilling the different study inclusion criteria significantly differed in various clinical parameters. In the second 12 months period, SENSCIS enriched patients had a further absolute FVC% decline as described for the total cohort. In contrast, patients fulfilling the focuSSced and SLS II criteria showed numerical 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, leading 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.

Published
2023

17. Stratification in systemic sclerosis according to autoantibody status versus skin involvement: a study of the prospective EUSTAR cohort

Author

Elhai, Muriel, Sritharan, Nanthara, Boubaya, Marouane, Balbir-Gurman, Alexandra, Siegert, Elise, Hachulla, Eric, de Vries-Bouwstra, Jeska, Riemekasten, Gabriela, Distler, Jörg H W, Rosato, Edoardo, Del Galdo, Francesco, Mendoza, Fabian A, Furst, Daniel E, de la Puente, Carlos, Hoffmann-Vold, Anna-Maria, Gabrielli, Armando, Distler, Oliver, Bloch-Queyrat, Coralie, Allanore, Yannick, Matucci Cerinic, Marco, Walker, Ulrich, Iannone, Florenzo, Jordan, Suzana, Becvar, Radim, Kowal Bielecka, Otylia, Cutolo, Maurizio, Cuomo, Giovanna, Kedor, Claudia, Rednic, Simona, Avouac, Jérome, et al, University of Zurich, and Allanore, Yannick

Subjects
2403 Immunology, Rheumatology, 2745 Rheumatology, Immunology, 10051 Rheumatology Clinic and Institute of Physical Medicine, 2723 Immunology and Allergy, Immunology and Allergy, 610 Medicine & health
Published
2022
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18. World Health Organization (WHO) International Classification of Functioning, Disability and Health (ICF) Core Set Development for Interstitial Lung Disease

Author

Saketkoo, Lesley Ann, Escorpizo, Reuben, Varga, Janos, Keen, Kevin John, Fligelstone, Kim, Birring, Surinder S., Alexanderson, Helene, Pettersson, Henrik, Chaudhry, Humza Ahmad, Poole, Janet L., Regardt, Malin, LeSage, Daphne, Sarver, Catherine, Lanario, Joseph, Renzoni, Elisabetta, Scholand, Mary Beth, Lammi, Matthew R., Kowal-Bielecka, Otylia, Distler, Oliver, Frech, Tracy, Shapiro, Lee, Varju, Cecilia, Volkmann, Elizabeth R., Bernstein, Elana J., Drent, Marjolein, Obi, Ogugua Ndili, Patterson, Karen C., Russell, Anne-Marie, Faculteit FHML Centraal, and RS: FHML non-thematic output

Subjects
CTD-ILD, JOINT USE, REHABILITATION, Pharmacology, DOMAINS, fibrosis, INSTRUMENTS, PATIENT, VALIDATION, RHEUMATOID-ARTHRITIS, ICD-11, patient-reported outcomes, cough, QUALITY-OF-LIFE, UPDATE, IDIOPATHIC PULMONARY-FIBROSIS, Pharmacology (medical), connective tissue
Abstract

Background: The World Health Organization (WHO) introduced the International Classification of Functioning, Disability, and Health (ICF) as a scientific method of disability data collection comprised of >1,200 categories describing the spectrum of impairment types (functional, symptoms-based and anatomical) under the bio-psycho-social model with consideration of environmental and personal factors (pf). ICF Core Sets and ICF Checklists are streamlined disease-specific resources for clinical use, service provision, and for use in health economics and health policy. ICF can disclose strengths and weaknesses across multiple patient-reported outcome measures (PROMs) and help consolidate best-fitting question-items from multiple PROMs. Interstitial lung diseases (ILDs), are generally progressive, with restrictive physiology sometimes occurring in the context of multi-organ autoimmunity/inflammatory conditions such as connective tissue diseases (CTDs). In spite of significant associated morbidity and potential disability, ILD has yet to be linked to the ICF.Methods: Each instrument and their question-items within the consensus-recommended core sets for clinical trials in ILD were deconstructed to single concept units, and then linked per updated ICF linkage rules. Inter-linker agreement was established. Three additional subsequently validated measures were also included.Results: One-hundred-eleven ICF categories were identified for ten PROMs and three traditional objective measures that were amenable to ICF linkage. The proportion of agreement ranged from 0.79 (95% CI: 0.62, 0.91) to 0.93 (0.76, 0.99) with the overall proportion of inter-linker agreement being very high 0.86 (0.82, 0.89) for the initial instruments, with 94–100% for the three additional PROMs. Thirty-four new ‘Personal Factors’ emerged to capture disease-specific qualities not elsewhere described in ICF, e.g. ‘pf_embarrassed by cough’ or ‘pf_panic/afraid when can’t get a breath’.Conclusion: This first known effort in ICF linkage of ILD has provided important revelations on the current utility of the ICF in lung disease. Results have indicated areas for meaningful assessment of ICF descriptors for lung impairment. The mapping across PROMs provides insight into possibilities of developing more streamline and precise instrumentation. Finally, familiarity with the ICF in ILD may enable clinicians to experience a smoother transition with the imminent harmonization of ICD and ICF, ICD-11.

Published
2022
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19. Stratification in systemic sclerosis according to autoantibody status versus skin involvement: a study of the prospective EUSTAR cohort

Author

Elhai, Muriel, primary, Sritharan, Nanthara, additional, Boubaya, Marouane, additional, Balbir-Gurman, Alexandra, additional, Siegert, Elise, additional, Hachulla, Eric, additional, de Vries-Bouwstra, Jeska, additional, Riemekasten, Gabriela, additional, Distler, Jörg H W, additional, Rosato, Edoardo, additional, Del Galdo, Francesco, additional, Mendoza, Fabian A, additional, Furst, Daniel E, additional, de la Puente, Carlos, additional, Hoffmann-Vold, Anna-Maria, additional, Gabrielli, Armando, additional, Distler, Oliver, additional, Bloch-Queyrat, Coralie, additional, Allanore, Yannick, additional, Matucci Cerinic, Marco, additional, Walker, Ulrich, additional, Iannone, Florenzo, additional, Jordan, Suzana, additional, Becvar, Radim, additional, Kowal Bielecka, Otylia, additional, Cutolo, Maurizio, additional, Cuomo, Giovanna, additional, Kedor, Claudia, additional, Rednic, Simona, additional, Avouac, Jérome, additional, Vlachoyiannopoulos, P., additional, Montecucco, C., additional, Stork, Jiri, additional, Inanc, Murat, additional, Carreira, Patricia E., additional, Novak, Srdan, additional, Czirják, László, additional, Iudici, Michele, additional, Kucharz, Eugene J., additional, Zanatta, Elisabetta, additional, Perdan-Pirkmajer, Katja, additional, Coleiro, Bernard, additional, Moroncini, Gianluca, additional, Farge Bancel, Dominique, additional, Airò, Paolo, additional, Hesselstrand, Roger, additional, Radic, Mislav, additional, Braun-Moscovici, Yolanda, additional, Lo Monaco, Andrea, additional, Hunzelmann, Nicolas, additional, Pellerito, Raffaele, additional, Giollo, Alessandro, additional, Morovic-Vergles, Jadranka, additional, Denton, Christopher, additional, Vonk, Madelon, additional, Damjanov, Nemanja, additional, Henes, Jörg, additional, Ortiz Santamaria, Vera, additional, Heitmann, Stefan, additional, Krasowska, Dorota, additional, Hasler, Paul, additional, Kohm, Michaela, additional, Foeldvari, Ivan, additional, Bajocchi, Gianluigi, additional, Salvador, Maria João, additional, Stamenkovic, Bojana, additional, Selmi, Carlo Francesco, additional, Tikly, Mohammed, additional, Ananieva, Lidia P., additional, Herrick, Ariane, additional, Müller-Ladner, Ulf, additional, De Palma, Raffaele, additional, Engelhart, Merete, additional, Szücs, Gabriela, additional, Sobrino Grande, Cristina, additional, Midtvedt, Øyvind, additional, Launay, David, additional, Riccieri, Valeria, additional, Ionescu, Ruxandra Maria, additional, Sha, Ami, additional, Gheorghiu, Ana Maria, additional, Sunderkötter, Cord, additional, Ingegnoli, Francesca, additional, Mouthon, Luc, additional, Smith, Vanessa, additional, Cantatore, Francesco Paolo, additional, Ullman, Susanne, additional, Alberto von Mühlen, Carlos, additional, Pozzi, Maria Rosa, additional, Eyerich, Kilian, additional, Wiland, Piotr, additional, Vanthuyne, Marie, additional, Alegre-Sancho, Juan Jose, additional, Herrmann, Kristine, additional, De Langhe, Ellen, additional, Anic, Branimir, additional, Üprus, Maria, additional, Yavuz, Sule, additional, Granel, Brigitte, additional, de Souza Müller, Carolina, additional, Busquets, Joanna, additional, Agachi, Svetlana, additional, Stebbings, Simon, additional, Mathieu, D'Alessandro, additional, Sampaio-Barros, Percival D., additional, Stamp, Lisa, additional, Solanki, Kamal, additional, Veale, Douglas, additional, Loyo, Esthela, additional, Li, Mengtao, additional, Abdel Atty Mohamed, Walid Ahmed, additional, Gigante, Antonietta, additional, Oksel, Fahrettin, additional, Tanaseanu, Cristina-Mihaela, additional, Foti, Rosario, additional, Ancuta, Codrina, additional, Maurer, Britta, additional, van Laar, Jacob, additional, Kayser, Cristiane, additional, Fathi, Nihal, additional, García de la Peña Lefebvre, Paloma, additional, Sibilia, Jean, additional, Litinsky, Ira, additional, Abignano, Giuseppina, additional, Seskute, Goda, additional, Saketkoo, Lesley Ann, additional, Kerzberg, Eduardo, additional, Bianchi, Washington, additional, Castellví, Ivan, additional, Limonta, Massimiliano, additional, Rimar, Doron, additional, Couto, Maura, additional, Spertini, François, additional, Marcoccia, Antonella, additional, Kahl, Sarah, additional, Hsu, Ivien M., additional, Martin, Thierry, additional, Moiseev, Sergey, additional, Chung, Lorinda S., additional, Schmeiser, Tim, additional, Majewski, Dominik, additional, Zdrojewski, Zbigniew, additional, Martínez-Barrio, Julia, additional, Bernardino, Vera, additional, Sommerlatte, Sabine, additional, Levy, Yair, additional, Rezus, Elena, additional, Nuri Pamuk, Omer, additional, Sarzi Puttini, Piercarlo, additional, Poormoghim, Hadi, additional, Kötter, Ina, additional, Gaches, Francis, additional, Belloli, Laura, additional, Sfikakis, Petros, additional, Markus, Juliana, additional, Feldman, Gary R, additional, Ramazan, Ana-Maria, additional, Scherer, H.U., additional, Truchetet, Marie-Elise, additional, Lescoat, Alain, additional, Dagna, Lorenzo, additional, van Laar, J.M., additional, Rudnicka, Lidia, additional, Oliveira, Susana, additional, Atzeni, Fabiola, additional, Kuwana, Masataka, additional, Mekinian, Arsene, additional, Martin, Mickaël, additional, and Tanaka, Yoshiya, additional

Published
2022
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20. Guidelines of the Polish Respiratory Society on the Diagnosis and Treatment of Progressive Fibrosing Interstitial Lung Diseases Other than Idiopathic Pulmonary Fibrosis

Author

Piotrowski, Wojciech, primary, Martusewicz-Boros, Magdalena, additional, Białas, Adam, additional, Barczyk, Adam, additional, Batko, Bogdan, additional, Błasińska, Katarzyna, additional, Boros, Piotr, additional, Górska, Katarzyna, additional, Grzanka, Piotr, additional, Jassem, Ewa, additional, Jastrzębski, Dariusz, additional, Kaczyńska, Janina, additional, Kowal-Bielecka, Otylia, additional, Kucharz, Eugeniusz, additional, Kuś, Jan, additional, Kuźnar-Kamińska, Barbara, additional, Kwiatkowska, Brygida, additional, Langfort, Renata, additional, Lewandowska, Katarzyna, additional, Mackiewicz, Barbara, additional, Majewski, Sebastian, additional, Makowska, Joanna, additional, Miłkowska-Dymanowska, Joanna, additional, Puścińska, Elżbieta, additional, Siemińska, Alicja, additional, Sobiecka, Małgorzata, additional, Soroka-Dąda, Renata, additional, Szołkowska, Małgorzata, additional, Wiatr, Elżbieta, additional, Ziora, Dariusz, additional, and Śliwiński, Paweł, additional

Published
2022
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21. Cohort Enrichment Strategies for Progressive Interstitial Lung Disease in Systemic Sclerosis From European Scleroderma Trials and Research

Author

Hoffmann-Vold, Anna-Maria, primary, Brunborg, Cathrine, additional, Airò, Paolo, additional, Ananyeva, Lidia P., additional, Czirják, László, additional, Guiducci, Serena, additional, Hachulla, Eric, additional, Li, Mengtao, additional, Mihai, Carina, additional, Riemekasten, Gabriela, additional, Sfikakis, P., additional, Valentini, Gabriele, additional, Kowal-Bielecka, Otylia, additional, Allanore, Yannick, additional, and Distler, Oliver, additional

Published
2022
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23. Cohort enrichment strategies for progressive interstitial lung disease in systemic sclerosis from EUSTAR

Author

Hoffmann-Vold, Anna-Maria, Brunborg, Cathrine, Airò, Paolo, Ananyeva, Lidia P, Czirják, László, Guiducci, Serena, Hachulla, Eric, Li, Mengtao, Mihai, Carina, Riemekasten, Gabriela, Sfikakis, Petros P, Valentini, Gabriele, Kowal-Bielecka, Otylia, Allanore, Yannick, Distler, Oliver, and University of Zurich

Subjects
10051 Rheumatology Clinic and Institute of Physical Medicine, 610 Medicine & health
Published
2022
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25. Prediction of improvement in skin fibrosis in diffuse cutaneous systemic sclerosis: a EUSTAR analysis

Author

Dobrota, Rucsandra, Maurer, Britta, Graf, Nicole, Jordan, Suzana, Mihai, Carina, Kowal-Bielecka, Otylia, Allanore, Yannick, Distler, Oliver, Cerinic, Marco Matucci, Guiducci, Serena, Walker, Ulrich, Lapadula, Giovanni, Iannone, Florenzo, Becvar, Radim, Sierakowsky, Stanislaw, Cutolo, Maurizio, Sulli, Alberto, Valentini, Gabriele, Cuomo, Giovanna, Vettori, Serena, Riemekasten, Gabriela, Siegert, Elise, Rednic, Simona, Nicoara, Ileana, Kahan, André, Vlachoyiannopoulos, P., Montecucco, C., Caporali, Roberto, Carreira, Patricia E., Novak, Srdan, Czirják, László, Varju, Cecilia, Chizzolini, Carlo, Kucharz, Eugene J., Kotulska, Anna, Kopec-Medrek, Magdalena, Widuchowska, Malgorzata, Cozzi, Franco, Rozman, Blaz, Mallia, Carmel, Coleiro, Bernard, Gabrielli, Armando, Farge, Dominique, Wu, Chen, Marjanovic, Zora, Faivre, Helene, Hij, Darin, Dhamadi, Roza, Airò, Paolo, Hesselstrand, Roger, Wollheim, Frank, Wuttge, Dirk M, Andréasson, Kristofer, Martinovic, Duska, Balbir-Gurman, Alexandra, Braun-Moscovici, Yolanda, Trotta, F., Lo Monaco, Andrea, Hunzelmann, Nicolas, Pellerito, Raffaele, Mauriziano, Ospedale, Bambara, Lisa Maria, Caramaschi, Paola, Black, Carol, Denton, Christopher, Damjanov, Nemanja, Henes, Jörg, Ortiz Santamaria, Vera, Heitmann, Stefan, Krasowska, Dorota, Seidel, Matthias, Burkhardt, Harald, Himsel, Andrea, Salvador, Maria J., Silva, José Antonio Pereira Da, Stamenkovic, Bojana, Stankovic, Aleksandra, Banja, Niska, Tikly, Mohammed, Ananieva, Lidia P., Denisov, Lev N., Müller-Ladner, Ulf, Frerix, Marc, Tarner, Ingo, Scorza, Raffaella, Clinica, U.O. Immunologia, Engelhart, Merete, Strauss, Gitte, Nielsen, Henrik, Damgaard, Kirsten, Mendoza, Antonio Zea, de la Puente, Carlos, Giraldo, Walter A. Sifuentes, Midtvedt, Øyvind, Reiseter, Silje, Hachulla, Eric, Launay, David, Valesini, Guido, Riccieri, Valeria, Ionescu, Ruxandra Maria, Opris, Daniela, Groseanu, Laura, Cornateanu, Roxana Sfrent, Ionitescu, Razvan, Gherghe, Ana Maria, Soare, Alina, Gorga, Marilena, Bojinca, Mihai, Schett, Georg, Distler, Jörg HW, Beyer, Christian, Meroni, Pierluigi, Ingegnoli, Francesca, Mouthon, Luc, De Keyser, Filip, Smith, Vanessa, Cantatore, Francesco P., Corrado, Ada, Pozzi, Maria R., Eyerich, Kilian, Hein, Rüdiger, Knott, Elisabeth, Wiland, Piotr, Szmyrka-Kaczmarek, Magdalena, Sokolik, Renata, Morgiel, Ewa, Madej, Marta, Krummel-Lorenz, Brigitte, Aringer, Martin, Günther, Claudia, Westhovens, Rene, de Langhe, Ellen, Lenaerts, Jan, Anic, Branimir, Baresic, Marko, Mayer, Miroslav, Radominski, Sebastião C., de Souza Müller, Carolina, Azevedo, Valderílio F., Agachi, Svetlana, Groppa, Liliana, Chiaburu, Lealea, Russu, Eugen, Popa, Sergei, Zenone, Thierry, Highton, John, Stamp, Lisa, Chapman, Peter, O’Donnell, John, Solanki, Kamal, Veale, Douglas, OʼRourke, Marie, Loyo, Esthela, Li, Mengtao, Rosato, Edoardo, Amoroso, Antonio, Tanaseanu, Cristina-Mihaela, Popescu, Monica, Dumitrascu, Alina, Tiglea, Isabela, Foti, Rosario, Chirieac, Rodica, Ancuta, Codrina, Villiger, Peter, Adler, Sabine, Sibilia, Jean, Chatelus, Emmanuel, Gottenberg, Jacques Eric, Chifflot, Hélène, Litinsky, Ira, Venalis, Algirdas, Butrimiene, Irena, Venalis, Paulius, Rugiene, Rita, Karpec, Diana, Saketkoo, Lesley Ann, Lasky, Joseph A., Kerzberg, Eduardo, Montoya, Fabiana, Cosentino, Vanesa, Limonta, Massimiliano, Brucato, Antonio Luca, Lupi, Elide, Spertini, François, Ribi, Camillo, Buss, Guillaume, Pasquali, Jean Louis, Martin, Thierry, and Gorse, Audrey

Published
2016
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26. Mapping and predicting mortality from systemic sclerosis

Author

Elhai, Muriel, Meune, Christophe, Boubaya, Marouane, Avouac, Jérôme, Hachulla, Eric, Balbir-Gurman, Alexandra, Riemekasten, Gabriela, Airò, Paolo, Joven, Beatriz, Vettori, Serena, Cozzi, Franco, Ullman, Susanne, Czirják, László, Tikly, Mohammed, Müller-Ladner, Ulf, Caramaschi, Paola, Distler, Oliver, Iannone, Florenzo, Ananieva, Lidia P, Hesselstrand, Roger, Becvar, Radim, Gabrielli, Armando, Damjanov, Nemanja, Salvador, Maria J, Riccieri, Valeria, Mihai, Carina, Szücs, Gabriella, Walker, Ulrich A, Hunzelmann, Nicolas, Martinovic, Duska, Smith, Vanessa, Müller, Carolina de Souza, Montecucco, Carlo Maurizio, Opris, Daniela, Ingegnoli, Francesca, Vlachoyiannopoulos, Panayiotis G, Stamenkovic, Bojana, Rosato, Edoardo, Heitmann, Stefan, Distler, Jörg H W, Zenone, Thierry, Seidel, Matthias, Vacca, Alessandra, Langhe, Ellen De, Novak, Srdan, Cutolo, Maurizio, Mouthon, Luc, Henes, Jörg, Chizzolini, Carlo, Mühlen, Carlos Alberto von, Solanki, Kamal, Rednic, Simona, Stamp, Lisa, Anic, Branimir, Santamaria, Vera Ortiz, Santis, Maria De, Yavuz, Sule, Sifuentes-Giraldo, Walter Alberto, Chatelus, Emmanuel, Stork, Jiri, Laar, Jacob van, Loyo, Esthela, García de la Peña Lefebvre, Paloma, Eyerich, Kilian, Cosentino, Vanesa, Alegre-Sancho, Juan Jose, Kowal-Bielecka, Otylia, Rey, Grégoire, Matucci-Cerinic, Marco, and Allanore, Yannick

Published
2017
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27. The European Scleroderma Trials and Research group (EUSTAR) task force for the development of revised activity criteria for systemic sclerosis: derivation and validation of a preliminarily revised EUSTAR activity index

Author

Valentini, Gabriele, Iudici, Michele, Walker, Ulrich A, Jaeger, Veronika K, Baron, Murray, Carreira, Patricia, Czirják, László, Denton, Christopher P, Distler, Oliver, Hachulla, Eric, Herrick, Ariane L, Kowal-Bielecka, Otylia, Pope, Janet, Müller-Ladner, Ulf, Riemekasten, Gabriela, Avouac, Jerome, Frerix, Marc, Jordan, Suzana, Minier, Tünde, Siegert, Elise, Ong, Voon H, Vettori, Serena, and Allanore, Yannick

Published
2017
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28. Progressive interstitial lung disease in patients with systemic sclerosis-associated interstitial lung disease in the EUSTAR database

Author

Hoffmann-Vold, Anna-Maria, Allanore, Yannick, Alves, Margarida, Brunborg, Cathrine, Airó, Paolo, Ananieva, Lidia P, Czirják, László, Guiducci, Serena, Hachulla, Eric, Li, Mengtao, Mihai, Carina, Riemekasten, Gabriela, Sfikakis, Petros P, Kowal-Bielecka, Otylia, Riccardi, Antonella, Distler, Oliver, Smith, Vanessa, and on behalf of the EUSTAR consortium, [ missing ]

Subjects
Male, Vital capacity, Databases, Factual, Vital Capacity, Severity of Illness Index, Pulmonary function testing, 0302 clinical medicine, QUALITY-OF-LIFE, Risk Factors, Pulmonary fibrosis, Prevalence, Immunology and Allergy, FIBROSIS, scleroderma, Prospective Studies, 610 Medicine & health, PREDICTORS, Lung, Interstitial lung disease, respiratory system, Middle Aged, Dysphagia, Europe, Performing Arts, Cohort, Disease Progression, Female, medicine.symptom, Adult, medicine.medical_specialty, Immunology, Genetics and Molecular Biology, Systemic Sclerosis, behavioral disciplines and activities, General Biochemistry, Genetics and Molecular Biology, 03 medical and health sciences, FEV1/FVC ratio, Rheumatology, Internal medicine, medicine, Humans, COHORT, autoimmune diseases, 030203 arthritis & rheumatology, DECLINE, Scleroderma, Systemic, pulmonary fibrosis, business.industry, MORTALITY, PULMONARY-FUNCTION, systemic, medicine.disease, respiratory tract diseases, body regions, 030228 respiratory system, General Biochemistry, EULAR SCLERODERMA TRIALS, business, Lung Diseases, Interstitial, FORCED VITAL CAPACITY
Abstract

ObjectivesTo identify overall disease course, progression patterns and risk factors predictive for progressive interstitial lung disease (ILD) in patients with systemic sclerosis-associated ILD (SSc-ILD), using data from the European Scleroderma Trials And Research (EUSTAR) database over long-term follow-up.MethodsEligible patients with SSc-ILD were registered in the EUSTAR database and had measurements of forced vital capacity (FVC) at baseline and after 12±3 months. Long-term progressive ILD and progression patterns were assessed in patients with multiple FVC measurements. Potential predictors of ILD progression were analysed using multivariable mixed-effect models.Results826 patients with SSc-ILD were included. Over 12±3 months, 219 (27%) showed progressive ILD: either moderate (FVC decline 5% to 10%) or significant (FVC decline >10%). A total of 535 (65%) patients had multiple FVC measurements available over mean 5-year follow-up. In each 12-month period, 23% to 27% of SSc-ILD patients showed progressive ILD, but only a minority of patients showed progression in consecutive periods. Most patients with progressive ILD (58%) had a pattern of slow lung function decline, with more periods of stability/improvement than decline, whereas only 8% showed rapid, continuously declining FVC; 178 (33%) experienced no episode of FVC decline. The strongest predictive factors for FVC decline over 5 years were male sex, higher modified Rodnan skin score and reflux/dysphagia symptoms.ConclusionSSc-ILD shows a heterogeneous and variable disease course, and thus monitoring all patients closely is important. Novel treatment concepts, with treatment initiation before FVC decline occurs, should aim for prevention of progression to avoid irreversible organ damage.

Published
2020
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30. Cohort Enrichment Strategies for Progressive Interstitial Lung Disease in Systemic Sclerosis From European Scleroderma Trials and Research.

Author

Hoffmann-Vold, Anna-Maria, Brunborg, Cathrine, Airò, Paolo, Ananyeva, Lidia P., Czirják, László, Guiducci, Serena, Hachulla, Eric, Li, Mengtao, Mihai, Carina, Riemekasten, Gabriela, Sfikakis, Petros P., Valentini, Gabriele, Kowal-Bielecka, Otylia, Allanore, Yannick, Distler, Oliver, and Sfikakis, P

Subjects
INTERSTITIAL lung diseases, SYSTEMIC scleroderma, EXPERIMENTAL design, DATABASE design
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, representativeness, 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. [ABSTRACT FROM AUTHOR]

Published
2023
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31. Use of platelet inhibitors for digital ulcers related to systemic sclerosis: EUSTAR study on derivation and validation of the DU-VASC model.

Author

Garaiman, Alexandru, Steigmiller, Klaus, Gebhard, Catherine, Mihai, Carina, Dobrota, Rucsandra, Bruni, Cosimo, Matucci-Cerinic, Marco, Henes, Joerg, Vries-Bouwstra, Jeska de, Smith, Vanessa, Doria, Andrea, Allanore, Yannick, Dagna, Lorenzo, Anić, Branimir, Montecucco, Carlomaurizio, Kowal-Bielecka, Otylia, Martin, Mickael, Tanaka, Yoshiya, Hoffmann-Vold, Anna-Maria, and Held, Ulrike

Subjects
EXPERIMENTAL design, CONFIDENCE intervals, RESEARCH methodology evaluation, RESEARCH methodology, SYSTEMIC scleroderma, PLATELET aggregation inhibitors, DESCRIPTIVE statistics, PREDICTION models, LOGISTIC regression analysis, SKIN ulcers, LONGITUDINAL method, DISEASE complications
Abstract

Objective To develop and validate the prognostic prediction model DU-VASC to assist the clinicians in decision-making regarding the use of platelet inhibitors (PIs) for the management of digital ulcers in patients with systemic sclerosis. Secondly, to assess the incremental value of PIs as predictor. Methods We analysed patient data from the European Scleroderma Trials and Research group registry (one time point assessed). Three sets of derivation/validation cohorts were obtained from the original cohort. Using logistic regression, we developed a model for prediction of digital ulcers (DUs). C -Statistics and calibration plots were calculated to evaluate the prediction performance. Variable importance plots and the decrease in C -statistics were used to address the importance of the predictors. Results Of 3710 patients in the original cohort, 487 had DUs and 90 were exposed to PIs. For the DU-VASC model, which includes 27 predictors, we observed good calibration and discrimination in all cohorts (C -statistic = 81.1% [95% CI: 78.9%, 83.4%] for the derivation and 82.3% [95% CI: 779.3%, 85.3%] for the independent temporal validation cohort). Exposure to PIs was associated with absence of DUs and was the most important therapeutic predictor. Further important factors associated with absence of DUs were lower modified Rodnan skin score, anti-Scl-70 negativity and normal CRP. Conversely, the exposure to phosphodiesterase-5 inhibitor, prostacyclin analogues or endothelin receptor antagonists seemed to be associated with the occurrence of DUs. Nonetheless, previous DUs remains the most impactful predictor of DUs. Conclusion The DU-VASC model, with good calibration and discrimination ability, revealed that PI treatment was the most important therapy-related predictor associated with reduced DU occurrence. [ABSTRACT FROM AUTHOR]

Published
2023
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34. Development and validation of a patient-reported outcome measure for systemic sclerosis: the EULAR Systemic Sclerosis Impact of Disease (ScleroID) questionnaire

Author

Becker, Mike O, primary, Dobrota, Rucsandra, additional, Garaiman, Alexandru, additional, Debelak, Rudolf, additional, Fligelstone, Kim, additional, Tyrrell Kennedy, Ann, additional, Roennow, Annelise, additional, Allanore, Yannick, additional, Carreira, Patricia E, additional, Czirják, László, additional, Denton, Christopher P, additional, Hesselstrand, Roger, additional, Sandqvist, Gunnel, additional, Kowal-Bielecka, Otylia, additional, Bruni, Cosimo, additional, Matucci-Cerinic, Marco, additional, Mihai, Carina, additional, Gheorghiu, Ana Maria, additional, Mueller-Ladner, Ulf, additional, Sexton, Joseph, additional, Kvien, Tore K, additional, Heiberg, Turid, additional, and Distler, Oliver, additional

Published
2021
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35. An expert opinion of the Polish Cardiac Society Working Group on Pulmonary Circulation and the Polish Society for Rheumatology on the diagnosis and treatment of pulmonary hypertension in patients with connective tissue disease

Author

Mularek-Kubzdela, Tatiana, primary, Ciurzyński, Michał, additional, Kowal Bielecka, Otylia, additional, Kasprzak, Jarosław D, additional, Kopeć, Grzegorz, additional, Mizia-Stec, Katarzyna, additional, Mroczek, Ewa, additional, Lewicka, Ewa, additional, Skoczylas, Ilona, additional, Grabka, Marek, additional, Furdal, Michał, additional, Florczyk, Michał, additional, Brzosko, Marek, additional, Batko, Bogdan, additional, Przepiera-Będzak, Hanna, additional, Samborski, Włodzimierz, additional, and Kucharz, Eugeniusz J, additional

Published
2021
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36. Progressive interstitial lung disease in patients with systemic sclerosis-associated interstitial lung disease in the EUSTAR database

Author

Hoffmann-Vold, Anna-Maria, Allanore, Yannick, Alves, Margarida, Brunborg, Cathrine, Airó, Paolo, Ananieva, Lidia P, Czirják, László, Guiducci, Serena, Hachulla, Eric, Li, Mengtao, Mihai, Carina, Riemekasten, Gabriela, Sfikakis, Petros P, Kowal-Bielecka, Otylia, Riccardi, Antonella, Distler, Oliver, EUSTAR collaborators, University of Zurich, and Distler, Oliver

Subjects
2403 Immunology, 1300 General Biochemistry, Genetics and Molecular Biology, 2745 Rheumatology, 10051 Rheumatology Clinic and Institute of Physical Medicine, 2723 Immunology and Allergy, 610 Medicine & health
Published
2021
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37. Progressive interstitial lung disease in patients with systemic sclerosis-associated interstitial lung disease in the EUSTAR database

Author

Hoffmann-Vold, Anna-Maria; https://orcid.org/0000-0001-6467-7422, Allanore, Yannick; https://orcid.org/0000-0002-6149-0002, Alves, Margarida, Brunborg, Cathrine, Airó, Paolo, Ananieva, Lidia P, Czirják, László, Guiducci, Serena, Hachulla, Eric; https://orcid.org/0000-0001-7432-847X, Li, Mengtao; https://orcid.org/0000-0003-4252-2889, Mihai, Carina; https://orcid.org/0000-0002-8627-8817, Riemekasten, Gabriela, Sfikakis, Petros P; https://orcid.org/0000-0001-5484-2930, Kowal-Bielecka, Otylia, Riccardi, Antonella, Distler, Oliver; https://orcid.org/0000-0002-0546-8310, EUSTAR collaborators, Hoffmann-Vold, Anna-Maria; https://orcid.org/0000-0001-6467-7422, Allanore, Yannick; https://orcid.org/0000-0002-6149-0002, Alves, Margarida, Brunborg, Cathrine, Airó, Paolo, Ananieva, Lidia P, Czirják, László, Guiducci, Serena, Hachulla, Eric; https://orcid.org/0000-0001-7432-847X, Li, Mengtao; https://orcid.org/0000-0003-4252-2889, Mihai, Carina; https://orcid.org/0000-0002-8627-8817, Riemekasten, Gabriela, Sfikakis, Petros P; https://orcid.org/0000-0001-5484-2930, Kowal-Bielecka, Otylia, Riccardi, Antonella, Distler, Oliver; https://orcid.org/0000-0002-0546-8310, and EUSTAR collaborators

Abstract

OBJECTIVES: To identify overall disease course, progression patterns and risk factors predictive for progressive interstitial lung disease (ILD) in patients with systemic sclerosis-associated ILD (SSc-ILD), using data from the European Scleroderma Trials And Research (EUSTAR) database over long-term follow-up. METHODS: Eligible patients with SSc-ILD were registered in the EUSTAR database and had measurements of forced vital capacity (FVC) at baseline and after 12±3 months. Long-term progressive ILD and progression patterns were assessed in patients with multiple FVC measurements. Potential predictors of ILD progression were analysed using multivariable mixed-effect models. RESULTS: 826 patients with SSc-ILD were included. Over 12±3 months, 219 (27%) showed progressive ILD: either moderate (FVC decline 5% to 10%) or significant (FVC decline >10%). A total of 535 (65%) patients had multiple FVC measurements available over mean 5-year follow-up. In each 12-month period, 23% to 27% of SSc-ILD patients showed progressive ILD, but only a minority of patients showed progression in consecutive periods. Most patients with progressive ILD (58%) had a pattern of slow lung function decline, with more periods of stability/improvement than decline, whereas only 8% showed rapid, continuously declining FVC; 178 (33%) experienced no episode of FVC decline. The strongest predictive factors for FVC decline over 5 years were male sex, higher modified Rodnan skin score and reflux/dysphagia symptoms. CONCLUSION: SSc-ILD shows a heterogeneous and variable disease course, and thus monitoring all patients closely is important. Novel treatment concepts, with treatment initiation before FVC decline occurs, should aim for prevention of progression to avoid irreversible organ damage.

Published
2021

38. Expert consensus for performing right heart catheterisation for suspected pulmonary arterial hypertension in systemic sclerosis: a Delphi consensus study with cluster analysis

Author

Avouac, Jérôme, Huscher, Dörte, Furst, Daniel E, Opitz, Christian F, Distler, Oliver, Allanore, Yannick, Ahmadi-Simab, Keihan, Albera, Carlo, Behrens, Frank, Bolster, Marcy B., Burger, Charles, Chatterjee, Soumya, Clements, Philip, Confalonieri, Marco, Ellen Csuka, Mary, Denton, Christopher P., Farber, Harrison, Fessler, Barri, Foeldvari, Ivan, Foley, Raymond, Frantz, Robert, Tore Gran, Jan, Highland, Kristin, Hsu, Vivien, Humbert, Marc, Inanc, Murat, Johnson, Sindhu, Kahaleh, Bashar, Kawut, Steven M, Khanna, Dinesh, Köhler, Christian M., Kowal-Bielecka, Otylia, Lang, Irene, Mahmud, Tafazzul H., Mandel, Jess, Cerinic, Marco Matucci, Mayes, Maureen, McHugh, Neil, McLaughlin, Vallerie, Medsger, Thomas A., Mehta, Sanjay, Merkel, Peter A., Mubarak, Kamal, Nash, Peter, Nathan, Steven, Oudiz, Ronald, Palevsky, Harold, Park, Myung, Pittrow, David, Pope, Janet, Ralph, David, Rothfield, Naomi, Rubenfire, Melvyn, Rubin, Lewis J., Saketkoo, Lesley Ann, Scorza, Sak Raffaella, Seibold, James R., Senecal, Jean-Luc, Silver, Richard, Staehler, Gerd, Steen, Virginia, strand, Vibeke, Strange, Charlie, Sweiss, Nadera, Taichman, Darren, Voskuyl, Alexandre, Wigley, Fredrick, Williamson, Tim, and Wollheim, Frank

Published
2014
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40. ACE inhibitors in SSc patients display a risk factor for scleroderma renal crisis-a EUSTAR analysis

Author

Bütikofer, Lukas, Varisco, Pierre A, Distler, Oliver, Kowal-Bielecka, Otylia, Allanore, Yannick, Riemekasten, Gabriela, Villiger, Peter M, Adler, Sabine, EUSTAR collaborators, and University of Zurich

Subjects
2403 Immunology, 2745 Rheumatology, 10051 Rheumatology Clinic and Institute of Physical Medicine, 2723 Immunology and Allergy, 610 Medicine & health
Published
2020
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41. 2013 Classification Criteria for Systemic Sclerosis: An American College of Rheumatology/European League Against Rheumatism Collaborative Initiative

Author

van den Hoogen, Frank, Khanna, Dinesh, Fransen, Jaap, Johnson, Sindhu R., Baron, Murray, Tyndall, Alan, Matucci-Cerinic, Marco, Naden, Raymond P., Medsger, Thomas A., Jr., Carreira, Patricia E., Riemekasten, Gabriela, Clements, Philip J., Denton, Christopher P., Distler, Oliver, Allanore, Yannick, Furst, Daniel E., Gabrielli, Armando, Mayes, Maureen D., van Laar, Jacob M., Seibold, James R., Czirjak, Laszlo, Steen, Virginia D., Inanc, Murat, Kowal-Bielecka, Otylia, Müller-Ladner, Ulf, Valentini, Gabriele, Veale, Douglas J., Vonk, Madelon C., Walker, Ulrich A., Chung, Lorinda, Collier, David H., Csuka, Mary Ellen, Fessler, Barri J., Guiducci, Serena, Herrick, Ariane, Hsu, Vivien M., Jimenez, Sergio, Kahaleh, Bashar, Merkel, Peter A., Sierakowski, Stanislav, Silver, Richard M., Simms, Robert W., Varga, John, and Pope, Janet E.

Published
2013
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42. Development and validation of a patient-reported outcome measure for systemic sclerosis: the EULAR Systemic Sclerosis Impact of Disease (ScleroID) questionnaire.

Author

Becker, Mike O., Dobrota, Rucsandra, Garaiman, Alexandru, Debelak, Rudolf, Fligelstone, Kim, Kennedy, Ann Tyrrell, Roennow, Annelise, Allanore, Yannick, Carreira, Patricia E., Czirják, László, Denton, Christopher P., Hesselstrand, Roger, Sandqvist, Gunnel, Kowal-Bielecka, Otylia, Bruni, Cosimo, Matucci-Cerinic, Marco, Mihai, Carina, Gheorghiu, Ana Maria, Mueller-Ladner, Ulf, and Sexton, Joseph

Subjects
RESEARCH, RESEARCH evaluation, RHEUMATOLOGY, RESEARCH methodology, SYSTEMIC scleroderma, DISABILITY evaluation, EVALUATION research, HEALTH surveys, SEVERITY of illness index, COMPARATIVE studies, QUALITY of life, QUESTIONNAIRES, SCLERODERMA (Disease), LONGITUDINAL method, DISEASE complications
Abstract

Objectives: Patient-reported outcome measures (PROMs) are important for clinical practice and research. Given the high unmet need, our aim was to develop a comprehensive PROM for systemic sclerosis (SSc), jointly with patient experts.Methods: This European Alliance of Associations for Rheumatology (EULAR)-endorsed project involved 11 European SSc centres. Relevant health dimensions were chosen and prioritised by patients. The resulting Systemic Sclerosis Impact of Disease (ScleroID) questionnaire was subsequently weighted and validated by Outcome Measures in Rheumatology criteria in an observational cohort study, cross-sectionally and longitudinally. As comparators, SSc-Health Assessment Questionnaire (HAQ), EuroQol Five Dimensional (EQ-5D), Short Form-36 (SF-36) were included.Results: Initially, 17 health dimensions were selected and prioritised. The top 10 health dimensions were selected for the ScleroID questionnaire. Importantly, Raynaud's phenomenon, impaired hand function, pain and fatigue had the highest patient-reported disease impact. The validation cohort study included 472 patients with a baseline visit, from which 109 had a test-retest reliability visit and 113 had a follow-up visit (85% female, 38% diffuse SSc, mean age 58 years, mean disease duration 9 years). The total ScleroID score showed strong Pearson correlation coefficients with comparators (SSc-HAQ, 0.73; Patient's global assessment, Visual Analogue Scale 0.77; HAQ-Disability Index, 0.62; SF-36 physical score, -0.62; each p<0.001). The internal consistency was strong: Cronbach's alpha was 0.87, similar to SSc-HAQ (0.88) and higher than EQ-5D (0.77). The ScleroID had excellent reliability and good sensitivity to change, superior to all comparators (intraclass correlation coefficient 0.84; standardised response mean 0.57).Conclusions: We have developed and validated the EULAR ScleroID, which is a novel, brief, disease-specific, patient-derived, disease impact PROM, suitable for research and clinical use in SSc. [ABSTRACT FROM AUTHOR]

Published
2022
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43. TGFβ receptor gene variants in systemic sclerosis-related pulmonary arterial hypertension: results from a multicentre EUSTAR study of European Caucasian patients

Author

Koumakis, Eugénie, Wipff, Julien, Dieudé, Philippe, Ruiz, Barbara, Bouaziz, Matthieu, Revillod, Lucile, Guedj, Mickaël, Distler, Jörg H W, Matucci-Cerinic, Marco, Humbert, Marc, Riemekasten, Gabriella, Airo, Paolo, Melchers, Inga, Hachulla, Eric, Cusi, Daniele, Wichmann, H- Erich, Hunzelmann, Nicolas, Tiev, Kiet, Caramaschi, Paola, Diot, Elisabeth, Kowal-Bielecka, Otylia, Cuomo, Giovanna, Walker, Ulrich, Czirják, László, Damjanov, Nemanja, Lupoli, Sara, Conti, Costanza, Müller-Nurasyid, Martina, Müller-Ladner, Ulf, Riccieri, Valeria, Cracowski, Jean-Luc, Cozzi, Franco, Bournia, Vasiliki Kalliopi, Vlachoyiannopoulos, P, Chiocchia, Gilles, Boileau, Catherine, and Allanore, Yannick

Published
2012
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44. EUSTAR biobanking: recommendations for the collection, storage and distribution of biospecimens in scleroderma research

Author

Beyer, Christian, Distler, Jörg H W, Allanore, Yannick, Aringer, Martin, Avouac, Jérôme, Czirják, László, Cutolo, Maurizio, Damjanov, Nemanja, Del Galdo, Francesco, Fligelstone, Kim, Guiducci, Serena, Kowal-Bielecka, Otylia, van Laar, Jacob M, Martucci-Cerinic, Marco, Müller-Ladner, Ulf, Riemekasten, Gabriela, Tarner, Ingo H, Tyndall, Alan, Kennedy, Ann Tyrrell, Valentini, Gabriele, Vettori, Serena, Walker, Ulrich A, Denton, Christopher, and Distler, Oliver

Published
2011
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