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1. Machine learning identifies right index finger tenderness as key signal of DAS28-CRP based psoriatic arthritis activity

Author

Samuel Rischke, Sorwe Mojtahed Poor, Robert Gurke, Lisa Hahnefeld, Michaela Köhm, Alfred Ultsch, Gerd Geisslinger, Frank Behrens, and Jörn Lötsch

Subjects
Medicine, Science
Abstract

Abstract Psoriatic arthritis (PsA) is a chronic inflammatory systemic disease whose activity is often assessed using the Disease Activity Score 28 (DAS28-CRP). The present study was designed to investigate the significance of individual components within the score for PsA activity. A cohort of 80 PsA patients (44 women and 36 men, aged 56.3 ± 12 years) with a range of disease activity from remission to moderate was analyzed using unsupervised and supervised methods applied to the DAS28-CRP components. Machine learning-based permutation importance identified tenderness in the metacarpophalangeal joint of the right index finger as the most informative item of the DAS28-CRP for PsA activity staging. This symptom alone allowed a machine learned (random forests) classifier to identify PsA remission with 67% balanced accuracy in new cases. Projection of the DAS28-CRP data onto an emergent self-organizing map of artificial neurons identified outliers, which following augmentation of group sizes by emergent self-organizing maps based generative artificial intelligence (AI) could be defined as subgroups particularly characterized by either tenderness or swelling of specific joints. AI-assisted re-evaluation of the DAS28-CRP for PsA has narrowed the score items to a most relevant symptom, and generative AI has been useful for identifying and characterizing small subgroups of patients whose symptom patterns differ from the majority. These findings represent an important step toward precision medicine that can address outliers.

Published
2023
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2. An objective, automated and robust scoring using fluorescence optical imaging to evaluate changes in micro-vascularisation indicating early arthritis

Author

Lukas Zerweck, Michaela Köhm, Phuong-Ha Nguyen, Gerd Geißlinger, Frank Behrens, and Andreas Pippow

Subjects
Medicine, Science
Abstract

Fluorescence optical imaging technique (FOI) is a well-established and valid method for visualization of changes in micro vascularization at different organ systems. As increased vascularization is an early feature of joint inflammation, FOI is a promising method to assess arthritis of the hands. But usability of the method is limited to the assessors experience as the measurement of FOI is semi-quantitative using an individual grading system such as the fluorescence optical imaging activity score (FOIAS). The goal of the study was to automatically and thus, objectively analyze the measured fluorescence intensity generated by FOI to evaluate the amount of inflammation of each of the subject’s joints focusing on the distinction between normal joint status or arthritis in psoriatic arthritis patients compared to healthy volunteers. Due to the heterogeneity of the pathophysiological perfusion of the hands, a method to overcome the absoluteness of the data by extracting heatmaps out of the image stacks is developed. To calculate a heatmap for one patient, firstly the time series for each pixel is extracted, which is then represented by a feature value. Secondly, all feature values are clustered. The calculated cluster values represent the relativity between the different pixels and enable a comparison of multiple patients. As a metric to quantify the conspicuousness of a joint a score is calculated based on the extracted cluster values. These steps are repeated for a total number of three features. With this method a tendency towards a classification into unaffected and inflamed joints can be achieved. However, further research is necessary to transform the tendency into a robust classification model.

Published
2022

3. Phenotype of limited cutaneous systemic sclerosis patients with positive anti-topoisomerase I antibodies: data from the EUSTAR cohort

Author

Elisabetta Zanatta 1, Dörte Huscher 2, Augusta Ortolan 1, Jérôme Avouac 3, Paolo Airò 4, Alexandra Balbir-Gurman 5, Elise Siegert 6, Marco Matucci Cerinic 7, Franco Cozzi 8, Gabriela Riemekasten 9, Anna-Maria Hoffmann-Vold 10, Oliver Distler 11, Armando Gabrielli 12, Stefan Heitmann 13, Nicolas Hunzelmann 14, Carlomaurizio Montecucco 15, Jadranka Morovic-Vergles 16, Camillo Ribi 17, Andrea Doria 1, Yannick Allanore 3, EUSTAR collaborators, Giovanna Cuomo, Gianluca Moroncini, Jiri Stork, Fiorenzo Iannone, Ulrich Walker, Eugenia Bertoldo, Dorota Krasowska, Maria João Salvador, Mohammed Tikly, Eric Hachulla, Valeria Riccieri, Ami Sha, Ana Maria Gheorghiu, Cord Sunderkötter, Francesca Ingegnoli, Luc Mouthon, Vanessa Smith, Francesco Paolo Cantatore, Kilian Eyerich, Piotr Wiland, Marie Vanthuyne, Branimir Anic, Maria Üprus, Brigitte Granel, Alessandra Vacca, Cristina-Mihaela Tanaseanu, Paloma García de la Peña Lefebvre, Jean Sibilia, Ira Litinsky, Lesley Ann Saketkoo, Eduardo Kerzberg, Massimiliano Limonta, Doron Rimar, Petros Sfikakis, Maurizio Cutolo, Patricia E Carreira, Rosario Foti, Srdan Novak, Michele Iudici, Mislav Radic, Raffaele Pellerito, Carlo Francesco Selmi, Lidia P Ananieva, Gabriela Szücs, Carlos de la Puente, Ruxandra Maria Ionescu, Jörg Distler, Maria Rosa Pozzi, Juan Jose Alegre-Sancho, Kristine Herrmann, Ellen De Langhe, Sule Yavuz, Carolina de Souza Müller, Svetlana Agachi, Douglas Veale, Esthela Loyo, Mengtao Li, Edoardo Rosato, Britta Maurer, Ivan Castellví, François Spertini, Kamal Solanki, Nicoletta Del Papa, Gerard Espinosa, László Czirják, Bernard Coleiro, Dominique Farge Bancel, Christopher Denton, Nemanja Damjanov, Jörg Henes, Vera Ortiz Santamaria, Michaela Kohm, Bojana Stamenkovic, 1, Elisabetta Zanatta, 2, Dörte Huscher, 1, Augusta Ortolan, 3, Jérôme Avouac, 4, Paolo Airò, 5, Alexandra Balbir-Gurman, 6, Elise Siegert, 7, Marco Matucci Cerinic, 8, Franco Cozzi, 9, Gabriela Riemekasten, Hoffmann-Vold 10, Anna-Maria, Distler 11, Oliver, Gabrielli 12, Armando, Heitmann 13, Stefan, Hunzelmann 14, Nicola, Montecucco 15, Carlomaurizio, Morovic-Vergles 16, Jadranka, Ribi 17, Camillo, 1, Andrea Doria, 3, Yannick Allanore, Collaborators, Eustar, Cuomo, Giovanna, Moroncini, Gianluca, Stork, Jiri, Iannone, Fiorenzo, Walker, Ulrich, Bertoldo, Eugenia, Krasowska, Dorota, João Salvador, Maria, Tikly, Mohammed, Hachulla, Eric, Riccieri, Valeria, Sha, Ami, Maria Gheorghiu, Ana, Sunderkötter, Cord, Ingegnoli, Francesca, Mouthon, Luc, Smith, Vanessa, Paolo Cantatore, Francesco, Eyerich, Kilian, Wiland, Piotr, Vanthuyne, Marie, Anic, Branimir, Üprus, Maria, Granel, Brigitte, Vacca, Alessandra, Tanaseanu, Cristina-Mihaela, García de la Peña Lefebvre, Paloma, Sibilia, Jean, Litinsky, Ira, Ann Saketkoo, Lesley, Kerzberg, Eduardo, Limonta, Massimiliano, Rimar, Doron, Sfikakis, Petro, Cutolo, Maurizio, E Carreira, Patricia, Foti, Rosario, Novak, Srdan, Iudici, Michele, Radic, Mislav, Pellerito, Raffaele, Francesco Selmi, Carlo, P Ananieva, Lidia, Szücs, Gabriela, de la Puente, Carlo, Maria Ionescu, Ruxandra, Distler, Jörg, Rosa Pozzi, Maria, Jose Alegre-Sancho, Juan, Herrmann, Kristine, De Langhe, Ellen, Yavuz, Sule, de Souza Müller, Carolina, Agachi, Svetlana, Veale, Dougla, Loyo, Esthela, Li, Mengtao, Rosato, Edoardo, Maurer, Britta, Castellví, Ivan, Spertini, Françoi, Solanki, Kamal, Del Papa, Nicoletta, Espinosa, Gerard, Czirják, László, Coleiro, Bernard, Farge Bancel, Dominique, Denton, Christopher, Damjanov, Nemanja, Henes, Jörg, Ortiz Santamaria, Vera, Kohm, Michaela, and Stamenkovic, Bojana

Subjects
interstitial lung disease, Scleroderma, Systemic, Hypertension, Pulmonary, disease subset, anti-topoisomerase I, Scleroderma, Systemic sclerosis, cutaneous form, outcome, Phenotype, Rheumatology, Scleroderma, Limited, Antibodies, Antinuclear, Scleroderma, Diffuse, Humans, Pharmacology (medical), Lung Diseases, Interstitial
Abstract

Objectives To characterize patients with positive anti-topoisomerase I (ATA) in lcSSc. Methods SSc patients enrolled in the EUSTAR cohort with a disease duration of ≤3 years at database entry were considered. We assessed the risk of major organ involvement in the following groups: ATA-lcSSc vs ACA-lcSSc and vs ANA without specificity (ANA)-lcSSc, and ATA-lcSSc vs ATA-dcSSc. Cox regression models with time-dependent covariates were performed with the following outcomes: new-onset interstitial lung disease (ILD), ILD progression [forced vital capacity (FVC) decline ≥10% and ≥5% vs values at ILD diagnosis), primary myocardial involvement (PMI), pulmonary hypertension (PH), any organ involvement and all-cause mortality. Results We included 1252 patients [194 ATA-lcSSc (15.5%)], with 7.7 years (s.d. 3.5) of follow-up. ILD risk was higher in ATA-lcSSc vs ACA- and ANA-lcSSc and similar to ATA-dcSSc, although with less frequent restrictive lung disease. The risk of FVC decline ≥10% (35% of ATA-lcSSc) was lower in ATA-lcSSc than in ATA-dcSSc, whereas FVC decline ≥5% occurs similarly between ATA-lcSSc (58% of patients) and other SSc subsets, including ATA-dcSSc. The risk of PMI was similar in ATA-lcSSc and ANA-lcSSc but lower than in ACA-lcSSc; no difference in PH and mortality risk was observed among lcSSc subsets. The risk of any organ involvement, PMI and PH was lower and the mortality tended to be lower in ATA-lcSSc vs ATA-dcSSc. Conclusion ATA-lcSSc patients have a high risk of ILD, albeit with a lower risk of progression compared with ATA-dcSSc, supporting careful screening for ILD in this subgroup.

Published
2022

4. Glucocorticoids prescribing practices in systemic sclerosis: an analysis of the EUSTAR database

Author

Iudici, Michele, Mongin, Denis, Siegert, Elise, Carreira, Patricia E, Distler, Jörg, Henes, Jörg, Zanatta, Elisabetta, Hachulla, Eric, De Luca, Giacomo, Müller, Carolina de Souza, Santiago, Tânia, Tandaipan, José-Luis, Bianchi, Breno Valdetaro, De Santis, Maria, Hoffmann-Vold, Anna-Maria, Gabrielli, Armando, Distler, Oliver, Courvoisier, Delphine Sophie, Giovanna Cuomo, Gianluca Moroncini, Jiri Stork, Fiorenzo Iannone, Ulrich Walker, Eugenia Bertoldo, Dorota Krasowska, Maria João Salvador, Mohammed Tikly, Valeria Riccieri, Ami Sha, Ana Maria Gheorghiu, Cord Sunderkötter, Francesca Ingegnoli, Luc Mouthon, Vanessa Smith, Francesco Paolo Cantatore, Kilian Eyerich, Piotr Wiland, Marie Vanthuyne, Branimir Anic, Maria Üprus, Brigitte Granel, Alessandra Vacca, Cristina-Mihaela Tanaseanu, Paloma García de la Peña Lefebvre, Jean Sibilia, Ira Litinsky, Lesley Ann Saketkoo, Eduardo Kerzberg, Massimiliano Limonta, Doron Rimar, Petros Sfikakis, Maurizio Cutolo, Rosario Foti, Srdan Novak, Mislav Radic, Raffaele Pellerito, Carlo Francesco Selmi Rozzano, Lidia P Ananieva, Gabriela Szűcs, Carlos de la Puente, Ruxandra Maria Ionescu, Maria Rosa Pozzi, Juan Jose Alegre-Sancho, Kristine Herrmann, Ellen De Langhe, Sule Yavuz Altunizade, Svetlana Agachi, Douglas Veale, Esthela Loyo, Mengtao Li, Edoardo Rosato, Britta Maurer, Iván Castellví, François Spertini, Kamal Solanki, Nicoletta Del Papa, Gerard Espinosa, László Czirják, Bernard Coleiro, Dominique Farge Bancel, Christopher Denton, Nemanja Damjanov, Vera Ortiz Santamaria Granollers, Michaela Kohm, Bojana Stamenkovic, Yannick Allanore, Paolo Airo, Alexandra Balbir-Gurman, Marco Matucci Cerinic, Gabriela Riemekasten, Stefan Heitmann, Nicolas Hunzelmann, Carlomaurizio Montecucco, Jadranka Morovic-Vergles, Camillo Ribi, Michele, Iudici, Denis, Mongin, Elise, Siegert, Patricia E, Carreira, Jörg, Distler, Jörg, Hene, Elisabetta, Zanatta, Eric, Hachulla, Giacomo, De Luca, Carolina de Souza, Müller, Tânia, Santiago, José-Luis, Tandaipan, Breno Valdetaro, Bianchi, Maria, De Santi, Anna-Maria, Hoffmann-Vold, Armando, Gabrielli, Oliver, Distler, Courvoisier, Sophie, Delphine, Cuomo, Giovanna, Moroncini, Gianluca, Stork, Jiri, Iannone, Fiorenzo, Walker, Ulrich, Bertoldo, Eugenia, Krasowska, Dorota, João Salvador, Maria, Tikly, Mohammed, Riccieri, Valeria, Sha, Ami, Maria Gheorghiu, Ana, Sunderkötter, Cord, Ingegnoli, Francesca, Mouthon, Luc, Smith, Vanessa, Paolo Cantatore, Francesco, Eyerich, Kilian, Wiland, Piotr, Vanthuyne, Marie, Anic, Branimir, Üprus, Maria, Granel, Brigitte, Vacca, Alessandra, Tanaseanu, Cristina-Mihaela, García de la Peña Lefebvre, Paloma, Sibilia, Jean, Litinsky, Ira, Ann Saketkoo, Lesley, Kerzberg, Eduardo, Limonta, Massimiliano, Rimar, Doron, Sfikakis, Petro, Cutolo, Maurizio, Foti, Rosario, Novak, Srdan, Radic, Mislav, Pellerito, Raffaele, Francesco Selmi Rozzano, Carlo, P Ananieva, Lidia, Szűcs, Gabriela, de la Puente, Carlo, Maria Ionescu, Ruxandra, Rosa Pozzi, Maria, Jose Alegre-Sancho, Juan, Herrmann, Kristine, De Langhe, Ellen, Yavuz Altunizade, Sule, Agachi, Svetlana, Veale, Dougla, Loyo, Esthela, Li, Mengtao, Rosato, Edoardo, Maurer, Britta, Castellví, Iván, Spertini, Françoi, Solanki, Kamal, Del Papa, Nicoletta, Espinosa, Gerard, Czirják, László, Coleiro, Bernard, Farge Bancel, Dominique, Denton, Christopher, Damjanov, Nemanja, Ortiz Santamaria Granollers, Vera, Kohm, Michaela, Stamenkovic, Bojana, Allanore, Yannick, Airo, Paolo, Balbir-Gurman, Alexandra, Matucci Cerinic, Marco, Riemekasten, Gabriela, Heitmann, Stefan, Hunzelmann, Nicola, Montecucco, Carlomaurizio, Morovic-Vergles, Jadranka, and Ribi, Camillo

Subjects
Rheumatology, systemic sclerosis, Pharmacology (medical), epidemiology, glucocorticoid
Abstract

Objectives To estimate the prevalence of long-term exposure to glucocorticoids (GCs) and to identify factors associated with, and variations in prescribing practices over time and across recruiting countries. Methods We included patients with SSc having a visit recorded in the EUSTAR database from January 2013 onward. We analysed the prevalence and the main features of GCs users, their exposure to GCs over time, and their GCs dosages. Multivariable linear regression was used to analyse the factors identified as associated with GCs intake duration. Time trends, and variations in GCs utilization across recruiting countries were explored. Missing data were imputed using multiple imputation with chained equations. Results The 9819 patients included were mostly females (85%), the majority had lcSSc (73%), and the median age was 58 years. At baseline, 34% of patients (n = 2769/8109) (48% dcSSc vs 29% lcSSc) were on GCs, and the median dose was 7.5 mg/day. GCs users were more frequently males and anti-Scl70 positive, and more commonly had dcSSc and more severe disease. On average, GCs users spent 25% of their follow-up time (median 33.2 months) on GCs, with no significant between-subsets difference. Notably, 33% (n = 971/2959) and 22% (n = 647/2959) of patients followed up for >1 year had received GCs for >6 and >12 months, respectively. Multivariable analysis showed that patient and disease characteristics poorly explained the variability in GCs exposure (adjusted-R2 = 0.06, P Conclusions GCs are widely and long-term prescribed in SSc, with significant between-countries and within-country differences. A gradual decrease in their utilization has been observed.

Published
2022

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

Author

Muriel Elhai, Nanthara Sritharan, Marouane Boubaya, Alexandra Balbir-Gurman, Elise Siegert, Eric Hachulla, Jeska de Vries-Bouwstra, Gabriela Riemekasten, Jörg H W Distler, Edoardo Rosato, Francesco Del Galdo, Fabian A Mendoza, Daniel E Furst, Carlos de la Puente, Anna-Maria Hoffmann-Vold, Armando Gabrielli, Oliver Distler, Coralie Bloch-Queyrat, Yannick Allanore, Marco Matucci Cerinic, Ulrich Walker, Florenzo Iannone, Suzana Jordan, Radim Becvar, Otylia Kowal Bielecka, Maurizio Cutolo, Giovanna Cuomo, Claudia Kedor, Simona Rednic, Jérome Avouac, P. Vlachoyiannopoulos, C. Montecucco, Jiri Stork, Murat Inanc, Patricia E. Carreira, Srdan Novak, László Czirják, Michele Iudici, Eugene J. Kucharz, Elisabetta Zanatta, Katja Perdan-Pirkmajer, Bernard Coleiro, Gianluca Moroncini, Dominique Farge Bancel, Paolo Airò, Roger Hesselstrand, Mislav Radic, Yolanda Braun-Moscovici, Andrea Lo Monaco, Nicolas Hunzelmann, Raffaele Pellerito, Alessandro Giollo, Jadranka Morovic-Vergles, Christopher Denton, Madelon Vonk, Nemanja Damjanov, Jörg Henes, Vera Ortiz Santamaria, Stefan Heitmann, Dorota Krasowska, Paul Hasler, Michaela Kohm, Ivan Foeldvari, Gianluigi Bajocchi, Maria João Salvador, Bojana Stamenkovic, Carlo Francesco Selmi, Mohammed Tikly, Lidia P. Ananieva, Ariane Herrick, Ulf Müller-Ladner, Raffaele De Palma, Merete Engelhart, Gabriela Szücs, Cristina Sobrino Grande, Øyvind Midtvedt, David Launay, Valeria Riccieri, Ruxandra Maria Ionescu, Ami Sha, Ana Maria Gheorghiu, Cord Sunderkötter, Francesca Ingegnoli, Luc Mouthon, Vanessa Smith, Francesco Paolo Cantatore, Susanne Ullman, Carlos Alberto von Mühlen, Maria Rosa Pozzi, Kilian Eyerich, Piotr Wiland, Marie Vanthuyne, Juan Jose Alegre-Sancho, Kristine Herrmann, Ellen De Langhe, Branimir Anic, Maria Üprus, Sule Yavuz, Brigitte Granel, Carolina de Souza Müller, Joanna Busquets, Svetlana Agachi, Simon Stebbings, D'Alessandro Mathieu, Percival D. Sampaio-Barros, Lisa Stamp, Kamal Solanki, Douglas Veale, Esthela Loyo, Mengtao Li, Walid Ahmed Abdel Atty Mohamed, Antonietta Gigante, Fahrettin Oksel, Cristina-Mihaela Tanaseanu, Rosario Foti, Codrina Ancuta, Britta Maurer, Jacob van Laar, Cristiane Kayser, Nihal Fathi, Paloma García de la Peña Lefebvre, Jean Sibilia, Ira Litinsky, Giuseppina Abignano, Goda Seskute, Lesley Ann Saketkoo, Eduardo Kerzberg, Washington Bianchi, Ivan Castellví, Massimiliano Limonta, Doron Rimar, Maura Couto, François Spertini, Antonella Marcoccia, Sarah Kahl, Ivien M. Hsu, Thierry Martin, Sergey Moiseev, Lorinda S. Chung, Tim Schmeiser, Dominik Majewski, Zbigniew Zdrojewski, Julia Martínez-Barrio, Vera Bernardino, Sabine Sommerlatte, Yair Levy, Elena Rezus, Omer Nuri Pamuk, Piercarlo Sarzi Puttini, Hadi Poormoghim, Ina Kötter, Francis Gaches, Laura Belloli, Petros Sfikakis, Juliana Markus, Gary R Feldman, Ana-Maria Ramazan, H.U. Scherer, Marie-Elise Truchetet, Alain Lescoat, Lorenzo Dagna, J.M. van Laar, Lidia Rudnicka, Susana Oliveira, Fabiola Atzeni, Masataka Kuwana, Arsene Mekinian, Mickaël Martin, Yoshiya Tanaka, Elhai, M., Sritharan, N., Boubaya, M., Balbir-Gurman, A., Siegert, E., Hachulla, E., de Vries-Bouwstra, J., Riemekasten, G., Distler, J. H. W., Rosato, E., Del Galdo, F., Mendoza, F. A., Furst, D. E., de la Puente, C., Hoffmann-Vold, A. -M., Gabrielli, A., Distler, O., Bloch-Queyrat, C., Allanore, Y., Matucci Cerinic, M., Walker, U., Iannone, F., Jordan, S., Becvar, R., Kowal Bielecka, O., Cutolo, M., Cuomo, G., Kedor, C., Rednic, S., Avouac, J., Vlachoyiannopoulos, P., Montecucco, C., Stork, J., Inanc, M., Carreira, P. E., Novak, S., Czirjak, L., Iudici, M., Kucharz, E. J., Zanatta, E., Perdan-Pirkmajer, K., Coleiro, B., Moroncini, G., Farge Bancel, D., Airo, P., Hesselstrand, R., Radic, M., Braun-Moscovici, Y., Lo Monaco, A., Hunzelmann, N., Pellerito, R., Giollo, A., Morovic-Vergles, J., Denton, C., Vonk, M., Damjanov, N., Henes, J., Ortiz Santamaria, V., Heitmann, S., Krasowska, D., Hasler, P., Kohm, M., Foeldvari, I., Bajocchi, G., Salvador, M. J., Stamenkovic, B., Selmi, C. F., Tikly, M., Ananieva, L. P., Herrick, A., Muller-Ladner, U., De Palma, R., Engelhart, M., Szucs, G., Sobrino Grande, C., Midtvedt, O., Launay, D., Riccieri, V., Ionescu, R. M., Sha, A., Gheorghiu, A. M., Sunderkotter, C., Ingegnoli, F., Mouthon, L., Smith, V., Cantatore, F. P., Ullman, S., Alberto von Muhlen, C., Pozzi, M. R., Eyerich, K., Wiland, P., Vanthuyne, M., Alegre-Sancho, J. J., Herrmann, K., De Langhe, E., Anic, B., Uprus, M., Yavuz, S., Granel, B., de Souza Muller, C., Busquets, J., Agachi, S., Stebbings, S., Mathieu, D. A., Sampaio-Barros, P. D., Stamp, L., Solanki, K., Veale, D., Loyo, E., Li, M., Abdel Atty Mohamed, W. A., Gigante, A., Oksel, F., Tanaseanu, C. -M., Foti, R., Ancuta, C., Maurer, B., van Laar, J., Kayser, C., Fathi, N., Garcia de la Pena Lefebvre, P., Sibilia, J., Litinsky, I., Abignano, G., Seskute, G., Saketkoo, L. A., Kerzberg, E., Bianchi, W., Castellvi, I., Limonta, M., Rimar, D., Couto, M., Spertini, F., Marcoccia, A., Kahl, S., Hsu, I. M., Martin, T., Moiseev, S., Chung, L. S., Schmeiser, T., Majewski, D., Zdrojewski, Z., Martinez-Barrio, J., Bernardino, V., Sommerlatte, S., Levy, Y., Rezus, E., Nuri Pamuk, O., Sarzi Puttini, P., Poormoghim, H., Kotter, I., Gaches, F., Belloli, L., Sfikakis, P., Markus, J., Feldman, G. R., Ramazan, A. -M., Scherer, H. U., Truchetet, M. -E., Lescoat, A., Dagna, L., van Laar, J. M., Rudnicka, L., Oliveira, S., Atzeni, F., Kuwana, M., Mekinian, A., Martin, M., and Tanaka, Y.

Subjects
Rheumatology, Immunology, Immunology and Allergy
Abstract

Background: The current subclassification of systemic sclerosis into cutaneous subtypes does not fully capture the heterogeneity of the disease. We aimed to compare the performances of stratification into LeRoy's cutaneous subtypes versus stratification by autoantibody status in systemic sclerosis. Methods: For this cohort study, we assessed people with systemic sclerosis in the multicentre international European Scleroderma Trials and Research (EUSTAR) database. Individuals positive for systemic-sclerosis autoantibodies of two specificities were excluded, and remaining individuals were classified by cutaneous subtype, according to their systemic sclerosis-specific autoantibodies, or both. We assessed the performance of each model to predict overall survival, progression-free survival, disease progression, and different organ involvement. The three models were compared by use of the area under the curve (AUC) of the receiver operating characteristic and the net reclassification improvement (NRI). Missing data were imputed. Findings: We assessed the database on July 26, 2019. Of 16 939 patients assessed for eligibility, 10 711 patients were included: 1647 (15·4%) of 10 709 were male, 9062 (84·6%) were female, mean age was 54·4 (SD 13·8) years, and mean disease duration was 7·9 (SD 8·2) years. Information regarding cutaneous subtype was available for 10 176 participants and antibody data were available for 9643 participants. In the prognostic analysis, there was no difference in AUC for overall survival (0·82, 95% CI 0·81–0·84 for cutaneous only vs 0·84, 0·82–0·85 for antibody only vs 0·84, 0·83–0·86 for combined) or for progression-free survival (0·70, 0·69–0·71 vs 0·71, 0·70–0·72 vs 0·71, 0·70–0·72). However, at 4 years the NRI showed substantial improvement for the antibody-only model compared with the cutaneous-only model in prediction of overall survival (0·57, 0·46–0·71 for antibody only vs 0·29, 0·19–0·39 for cutaneous only) and disease progression (0·36, 0·29–0·46 vs 0·21, 0·14–0·28). The antibody-only model did better than the cutaneous-only model in predicting renal crisis (AUC 0·72, 0·70–0·74 for antibody only vs 0·66, 0·64–0·69 for cutaneous only) and lung fibrosis leading to restrictive lung function (AUC 0·76, 0·75–0·77 vs 0·71, 0·70–0·72). The combined model improved the prediction of digital ulcers and elevated systolic pulmonary artery pressure, but did poorly for cardiac involvement. Interpretation: The autoantibody-only model outperforms cutaneous-only subsetting for risk stratifying people with systemic sclerosis in the EUSTAR cohort. Physicians should be aware of these findings at the time of decision making for patient management. Funding: World Scleroderma Foundation.

Published
2022

6. Significant weight loss in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database

Author

Michael Hughes, Calvin Heal, Elise Siegert, Eric Hachulla, Paolo Airó, Antonella Riccardi, Oliver Distler, Marco Matucci-Cerinic, Andrea Doria, Lorenzo Baretta, Alexandra Balbir-Gurman, Patricia E Carreira, Vanessa Smith, Carlos Alberto, Jörg Distler von Mühlen, Ulf Müller-Ladner, Lidia P Ananieva, László Czirják, Jörg Henes, Jeska de Vries-Bouwstra, Mengtao Li, Fabian Mendoza, Nemanja Damjanov, Ivan Castellví, Alessandro Giollo, Stefan Heitmann, Edoardo Rosato, Lorenzo Dagna, Christopher P Denton, Marie Vanthuyne, Fabio Cacciapaglia, Valeria Riccieri, Nicolas Hunzelmann, Ami Shah, Carlomaurizio Montecucco, Raffaele Pellerito, Ruxandra Maria Ionescu, Simona Rednic, Ulrich Walker, Maria Rosa Pozzi, Anna-Maria Hoffmann-Vold, Marie-Elise Truchetet, Susanne Ullman, Carolina de Souza Müller, Juan Jose Alegre-Sancho, Eduardo Kerzberg, Francesco Del Galdo, Gabriela Riemekasten, Branimir Anic, Marko Baresic, Miroslav Mayer, Fahrettin Oksel, Figen Yargucu, Ellen De Langhe, Ina Kötter, Mohammed Tikly, Radim Becvar, Douglas Veale, Dorota Krasowska, Andrea Lo Monaco, Lidia Rudnicka, Ana Maria Gheorghiu, Piercarlo Sarzi Puttini, Mislav Radic, Armando Gabrielli, Maria João Salvador, Carlos de la Puente, Gabriela Szücs, Sule Yavuz, Rosario Foti, Otylia Kowal Bielecka, Codrina Ancuta, Peter Villiger, Sabine Adler, Patrick Jego, Michaela Kohm, Eugene J Kucharz, Dominique Farge Bancel, Tim Schmeiser, Alberto Cauli, Alessandra Vacca, Kamal Solanki, Piotr Wiland, Paloma García de la Peña Lefebvre, Jorge Juan Gonzalez Martin, Sergio Jimenez, Lesley Ann Saketkoo, Roger Hesselstrand, Francesca Ingegnoli, Jean Sibilia, Merete Engelhart, Esthela Loyo, Carmen Tineo, Francesco Paolo Cantatore, Brigitte Krummel-Lorenz, Petros Sfikakis, Cristiane Kayser, Vera Ortiz Santamaria, Bojana Stamenkovic, Giovanna Cuomo, Francesco Puppo, Thierry Zenone, Nihal Fathi, Ira Litinsky, Carlo Chizzolini, Monika Swacha, Washington Bianchi, Breno Valdetaro Bianchi, Maria Üprus, Kati Otsa, Masataka Kuwana, Panayiotis Vlachoyiannopoulos, Sarah Kahl, Bernard Coleiro, François Spertini, Walid Ahmed Abdel Atty Mohamed, Sergey Moiseev, Pavel Novikov, Dominik Majewski, Simon Stebbings, Svetlana Agachi, Massimiliano Limonta, Carlo Francesco Selmi, Elena Rezus, Kristine Herrmann, Brigitte Granel, Goda Seskute, Matthias Seidel, Paul Hasler, Maurizio Cutolo Vera Bernardino, Carmen Pizzorni, Jadranka Morovic-Vergles, Daniel Furst, Ana-Maria Ramazan, Gianluigi Bajocchi, Lisa Stamp, Doron Rimar, Antonella Marcoccia, Srdan Novak, Luc Mouthon, Jiri Stork, Lorinda S Chung, Hadi Poormoghim, Francis Gaches, Laura Belloli, Cristina-Mihaela Tanaseanu, Fabiola Atzeni, Kilian Eyerich, Ivien M Hsu, Jacob van Laar, Mary Ellen Csuka, Omer Nuri Pamuk, Maura Couto, Arsene Mekinian, Murat Inanc, Ivan Foeldvari, Julia Martínez-Barrio, Yair Levy, Juliana Markus, Susana Oliveira, Hughes, Michael, Heal, Calvin, Siegert, Elise, Hachulla, Eric, Airó, Paolo, Riccardi, Antonella, Distler, Oliver, Matucci-Cerinic, Marco, Doria, Andrea, Baretta, Lorenzo, Balbir-Gurman, Alexandra, E Carreira, Patricia, Smith, Vanessa, Alberto, Carlo, Distler von Mühlen, Jörg, Müller-Ladner, Ulf, P Ananieva, Lidia, Czirják, László, Henes, Jörg, de Vries-Bouwstra, Jeska, Li, Mengtao, Mendoza, Fabian, Damjanov, Nemanja, Castellví, Ivan, Giollo, Alessandro, Heitmann, Stefan, Rosato, Edoardo, Dagna, Lorenzo, P Denton, Christopher, Vanthuyne, Marie, Cacciapaglia, Fabio, Riccieri, Valeria, Hunzelmann, Nicola, Shah, Ami, Montecucco, Carlomaurizio, Pellerito, Raffaele, Maria Ionescu, Ruxandra, Rednic, Simona, Walker, Ulrich, Rosa Pozzi, Maria, Hoffmann-Vold, Anna-Maria, Truchetet, Marie-Elise, Ullman, Susanne, de Souza Müller, Carolina, Jose Alegre-Sancho, Juan, Kerzberg, Eduardo, Del Galdo, Francesco, Riemekasten, Gabriela, Anic, Branimir, Baresic, Marko, Mayer, Miroslav, Oksel, Fahrettin, Yargucu, Figen, De Langhe, Ellen, Kötter, Ina, Tikly, Mohammed, Becvar, Radim, Veale, Dougla, Krasowska, Dorota, Lo Monaco, Andrea, Rudnicka, Lidia, Maria Gheorghiu, Ana, Sarzi Puttini, Piercarlo, Radic, Mislav, Gabrielli, Armando, João Salvador, Maria, de la Puente, Carlo, Szücs, Gabriela, Yavuz, Sule, Foti, Rosario, Kowal Bielecka, Otylia, Ancuta, Codrina, Villiger, Peter, Adler, Sabine, Jego, Patrick, Kohm, Michaela, J Kucharz, Eugene, Farge Bancel, Dominique, Schmeiser, Tim, Cauli, Alberto, Vacca, Alessandra, Solanki, Kamal, Wiland, Piotr, García de la Peña Lefebvre, Paloma, Juan Gonzalez Martin, Jorge, Jimenez, Sergio, Ann Saketkoo, Lesley, Hesselstrand, Roger, Ingegnoli, Francesca, Sibilia, Jean, Engelhart, Merete, Loyo, Esthela, Tineo, Carmen, Paolo Cantatore, Francesco, Krummel-Lorenz, Brigitte, Sfikakis, Petro, Kayser, Cristiane, Ortiz Santamaria, Vera, Stamenkovic, Bojana, Cuomo, Giovanna, Puppo, Francesco, Zenone, Thierry, Fathi, Nihal, Litinsky, Ira, Chizzolini, Carlo, Swacha, Monika, Bianchi, Washington, Valdetaro Bianchi, Breno, Üprus, Maria, Otsa, Kati, Kuwana, Masataka, Vlachoyiannopoulos, Panayioti, Kahl, Sarah, Coleiro, Bernard, Spertini, Françoi, Ahmed Abdel Atty Mohamed, Walid, Moiseev, Sergey, Novikov, Pavel, Majewski, Dominik, Stebbings, Simon, Agachi, Svetlana, Limonta, Massimiliano, Francesco Selmi, Carlo, Rezus, Elena, Herrmann, Kristine, Granel, Brigitte, Seskute, Goda, Seidel, Matthia, Hasler, Paul, Cutolo Vera Bernardino, Maurizio, Pizzorni, Carmen, Morovic-Vergles, Jadranka, Furst, Daniel, Ramazan, Ana-Maria, Bajocchi, Gianluigi, Stamp, Lisa, Rimar, Doron, Marcoccia, Antonella, Novak, Srdan, Mouthon, Luc, Stork, Jiri, S Chung, Lorinda, Poormoghim, Hadi, Gaches, Franci, Belloli, Laura, Tanaseanu, Cristina-Mihaela, Atzeni, Fabiola, Eyerich, Kilian, M Hsu, Ivien, van Laar, Jacob, Ellen Csuka, Mary, Nuri Pamuk, Omer, Couto, Maura, Mekinian, Arsene, Inanc, Murat, Foeldvari, Ivan, Martínez-Barrio, Julia, Levy, Yair, Markus, Juliana, and Oliveira, Susana

Subjects
Adult, Male, 0301 basic medicine, medicine.medical_specialty, weight loss, systemic sclerosis, nutrition, Immunology, Disease, computer.software_genre, General Biochemistry, Genetics and Molecular Biology, Scleroderma, 03 medical and health sciences, 0302 clinical medicine, Rheumatology, Risk Factors, Weight loss, Internal medicine, Weight Loss, Epidemiology, medicine, Humans, Immunology and Allergy, 610 Medicine & health, Aged, 030203 arthritis & rheumatology, Scleroderma, Systemic, Database, business.industry, Middle Aged, medicine.disease, 030104 developmental biology, Mood, Databases as Topic, Female, Outcomes research, medicine.symptom, business, computer, Rheumatism
Abstract

Gastrointestinal (GI) involvement is almost universal in patients with systemic sclerosis (SSc) and is associated with significant disease-related morbidity and mortality.1 The entire GI tract can be involved and other disease features (eg, low mood, terminal organ failure and functional hand impairment) can result in significant nutritional impairment. Severe GI involvement has been reported to occur in ~10% of patients with SSc and often occurs early in the course of the disease.2 However, identification of patients at high risk of clinically significant weight loss is extremely challenging, including from the high prevalence of GI symptoms in patients with SSc. Therefore, there is a need to understand high-risk patients including potentially modifiable risk factors, with a view to early intervention strategies. Against this background, the aim of this study was to examine potential clinical risk factors of significant weight loss in patients with SSc. We performed an analysis of patients with SSc enrolled in the multinational, longitudinal European League Against Rheumatism (EULAR) Scleroderma Trials and Research (EUSTAR) database. In our study, we defined significant weight loss as 4.5 kg and/or least 5% of their body weight at 5 months onwards.3 Patients with a recorded second visit after 3 months and before 12 months were included in the analysis. We adopted a pragmatic approach (relevant to clinical practice) in …

Published
2020
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7. Genetic variants in FBLIM1 gene do not contribute to SAPHO syndrome and chronic recurrent multifocal osteomyelitis in typical patient groups

Author

Gunter Assmann, Michaela Köhm, Volker Schuster, Frank Behrens, Rotraut Mössner, Nina Magnolo, Vinzenz Oji, Harald Burkhardt, and Ulrike Hüffmeier

Subjects
Chronic recurrent multifocal osteomyelitis (CRMO), Chronic non-bacterial osteomyelitis (CNO), Syndrome of synovitis acne pustulosis hyperostosis osteitis (SAPHO (syndrome)), Coding variants, Association, Internal medicine, RC31-1245, Genetics, QH426-470
Abstract

Abstract Background Syndrome of synovitis acne pustulosis hyperostosis osteitis (SAPHO) and chronic recurrent multifocal osteomyelitis (CRMO) present two diseases of a dermatologic and rheumatologic spectrum that are variable in manifestation und therapeutic response. Genetic risk factors have long been assumed in both diseases, but no single reliable factor has been identified yet. Therefore, we aimed to clinically characterize a patient group with syndrome of synovitis acne pustulosis hyperostosis osteitis (SAPHO) (n = 47) and chronic recurrent multifocal osteomyelitis (CRMO)/ chronic non-bacterial osteomyelitis (CNO) (n = 9) and analyze a CRMO candidate gene. Methods Clinical data of all patients were collected and assessed for different combinations of clinical symptoms. SAPHO patients were grouped into categories according to the acronym; disease-contribution by pathogens was evaluated. We sequenced coding exons of FBLIM1. Results Palmoplantar pustular psoriasis (PPP) was the most common skin manifestation in CRMO/CNO and SAPHO patients; most SAPHO patients had sterno-costo-clavicular hyperostosis. The most common clinical category of the acronym was S_PHO (n = 26). Lack of pathogen detection from bone biopsies was more common than microbial isolation. We did not identify autosomal-recessive FBLIM1 variants. Conclusions S_PHO is the most common combination of symptoms of its acronym. Genetic analyses of FBLIM1 did not provide evidence that this gene is relevant in our patient group. Our study indicates the need to elucidate SAPHO’s and CRMO/CNO’s pathogenesis.

Published
2020
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8. Innovative Imaging Technique for Visualization of Vascularization and Established Methods for Detection of Musculoskeletal Inflammation in Psoriasis Patients

Author

Michaela Köhm, Lukas Zerweck, Phuong-Ha Ngyuen, Harald Burkhardt, and Frank Behrens

Subjects
psoriasis, psoriatic arthritis, early detection, imaging, musculoskeletal inflammation, Medicine (General), R5-920
Abstract

Psoriasis (PsO) is one of the common chronic inflammatory skin diseases. Approximately 3% of the European Caucasian population is affected. Psoriatic arthritis (PsA) is a chronic immune-mediated disease associated with PsO characterized by distinct musculoskeletal inflammation. Due to its heterogeneous clinical manifestations (e.g., oligo- or polyarthritis, enthesitis, dactylitis, and axial inflammation), early diagnosis of PsA is often difficult and delayed. Approximately 30% of PsO patients will develop PsA. The responsible triggers for the transition from PsO only to PsA are currently unclear, and the impacts of different factors (e.g., genetic, environmental) on disease development are currently discussed. There is a high medical need, recently unmet, to specifically detect those patients with an increased risk for the development of clinically evident PsA early to initiate sufficient treatment to inhibit disease progression and avoid structural damage and loss of function or even intercept disease development. Increased neoangiogenesis and enthesial inflammation are hypothesized to be early pathological findings in PsO patients with PsA development. Different disease states describe the transition from PsO to PsA. Two of those phases are of value for early detection of PsA at-risk patients to prevent later development of PsA as changes in biomarker profiles are detectable: the subclinical phase (soluble and imaging biomarkers detectable, no clinical symptoms) and the prodromal phase (imaging biomarkers detectable, unspecific musculoskeletal symptoms such as arthralgia and fatigue). To target the unmet need for early detection of this at-risk population and to identify the subgroup of patients who will transition from PsO to PsA, imaging plays an important role in characterizing patients precisely. Imaging techniques such as ultrasound (US), magnetic resonance imaging (MRI), and computerized tomography (CT) are advanced techniques to detect sensitively inflammatory changes or changes in bone structure. With the use of these techniques, anatomic structures involved in inflammatory processes can be identified. These techniques are complemented by fluorescence optical imaging as a sensitive method for detection of changes in vascularization, especially in longitudinal measures. Moreover, high-resolution peripheral quantitative CT (HR-pQCT) and dynamic contrast-enhanced MRI (DCE-MRI) may give the advantage to identify PsA-related early characteristics in PsO patients reflecting transition phases of the disease.

Published
2020
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