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1. The added value of a European Reference Network on rare and complex connective tissue and musculoskeletal diseases: insights after the first 5 years of the ERN ReCONNET

Author: Talarico, R, Aguilera, S, Alexander, T, Amoura, Z, Andersen, J, Arnaud, L, Avcin, T, Marsal Barril, S, Beretta, L, Bombardieri, S, Bortoluzzi, A, Bouillot, C, Bulina, I, Burmester, G, Cannizzo, S, Cavagna, L, Chaigne, B, Cornet, A, Corti, P, Costedoat-Chalumeau, N, Davidsone, Z, Doria, A, Fenech, C, Ferraris, A, Fischer-Betz, R, Fonseca, J, Frank, C, Gaglioti, A, Galetti, I, Guimaraes, V, Hachulla, E, Holmner, M, Houssiau, F, Iaccarino, L, Jacobsen, S, Limper, M, Malfait, F, Mariette, X, Marinello, D, Martin, T, Matthews, L, Matucci-Cerinic, M, Meyer, A, Milas-Ahic, J, Moinzadeh, P, Montecucco, C, Mouthon, L, Muller-Ladner, U, Nagy, G, Patarata, E, Pileckyte, M, Pruunsild, C, Rednic, S, Romao, V, Schneider, M, Scire, C, Smith, V, Sulli, A, Tamirou, F, Tani, C, Taruscio, D, Taulaigo, A, Tincani, A, Ticciati, S, Turchetti, G, van Hagen, P, van Laar, J, Vieira, A, de Vries-Bouwstra, J, Zschocke, J, Cutolo, M, Mosca, M, Talarico R., Aguilera S., Alexander T., Amoura Z., Andersen J., Arnaud L., Avcin T., Marsal Barril S., Beretta L., Bombardieri S., Bortoluzzi A., Bouillot C., Bulina I., Burmester G. R., Cannizzo S., Cavagna L., Chaigne B., Cornet A., Corti P., Costedoat-Chalumeau N., Davidsone Z., Doria A., Fenech C., Ferraris A., Fischer-Betz R., Fonseca J. E., Frank C., Gaglioti A., Galetti I., Guimaraes V., Hachulla E., Holmner M., Houssiau F., Iaccarino L., Jacobsen S., Limper M., Malfait F., Mariette X., Marinello D., Martin T., Matthews L., Matucci-Cerinic M., Meyer A., Milas-Ahic J., Moinzadeh P., Montecucco C., Mouthon L., Muller-Ladner U., Nagy G., Patarata E., Pileckyte M., Pruunsild C., Rednic S., Romao V. C., Schneider M., Scire C. A., Smith V., Sulli A., Tamirou F., Tani C., Taruscio D., Taulaigo A. V., Tincani A., Ticciati S., Turchetti G., van Hagen P. M., van Laar J. M., Vieira A., de Vries-Bouwstra J. K., Zschocke J., Cutolo M., Mosca M., Talarico, R, Aguilera, S, Alexander, T, Amoura, Z, Andersen, J, Arnaud, L, Avcin, T, Marsal Barril, S, Beretta, L, Bombardieri, S, Bortoluzzi, A, Bouillot, C, Bulina, I, Burmester, G, Cannizzo, S, Cavagna, L, Chaigne, B, Cornet, A, Corti, P, Costedoat-Chalumeau, N, Davidsone, Z, Doria, A, Fenech, C, Ferraris, A, Fischer-Betz, R, Fonseca, J, Frank, C, Gaglioti, A, Galetti, I, Guimaraes, V, Hachulla, E, Holmner, M, Houssiau, F, Iaccarino, L, Jacobsen, S, Limper, M, Malfait, F, Mariette, X, Marinello, D, Martin, T, Matthews, L, Matucci-Cerinic, M, Meyer, A, Milas-Ahic, J, Moinzadeh, P, Montecucco, C, Mouthon, L, Muller-Ladner, U, Nagy, G, Patarata, E, Pileckyte, M, Pruunsild, C, Rednic, S, Romao, V, Schneider, M, Scire, C, Smith, V, Sulli, A, Tamirou, F, Tani, C, Taruscio, D, Taulaigo, A, Tincani, A, Ticciati, S, Turchetti, G, van Hagen, P, van Laar, J, Vieira, A, de Vries-Bouwstra, J, Zschocke, J, Cutolo, M, Mosca, M, Talarico R., Aguilera S., Alexander T., Amoura Z., Andersen J., Arnaud L., Avcin T., Marsal Barril S., Beretta L., Bombardieri S., Bortoluzzi A., Bouillot C., Bulina I., Burmester G. R., Cannizzo S., Cavagna L., Chaigne B., Cornet A., Corti P., Costedoat-Chalumeau N., Davidsone Z., Doria A., Fenech C., Ferraris A., Fischer-Betz R., Fonseca J. E., Frank C., Gaglioti A., Galetti I., Guimaraes V., Hachulla E., Holmner M., Houssiau F., Iaccarino L., Jacobsen S., Limper M., Malfait F., Mariette X., Marinello D., Martin T., Matthews L., Matucci-Cerinic M., Meyer A., Milas-Ahic J., Moinzadeh P., Montecucco C., Mouthon L., Muller-Ladner U., Nagy G., Patarata E., Pileckyte M., Pruunsild C., Rednic S., Romao V. C., Schneider M., Scire C. A., Smith V., Sulli A., Tamirou F., Tani C., Taruscio D., Taulaigo A. V., Tincani A., Ticciati S., Turchetti G., van Hagen P. M., van Laar J. M., Vieira A., de Vries-Bouwstra J. K., Zschocke J., Cutolo M., and Mosca M.
Abstract: In order to address the main challenges related to the rare diseases (RDs) the European Commission launched the European Reference Networks (ERNs), virtual networks involving healthcare providers (HCPs) across Europe. The mission of the ERNs is to tackle low prevalence and RDs that require highly specialised treatment and a concentration of knowledge and resources. In fact, ERNs offer the potential to give patients and healthcare professionals across the EU access to the best expertise and timely exchange of lifesaving knowledge, trying to make the knowledge travelling more than patients. For this reason, ERNs were established as concrete European infrastructures, and this is particularly crucial in the framework of rare and complex diseases in which no country alone has the whole knowledge and capacity to treat all types of patients. It has been five years since their kick-off launch in Vilnius in 2017. The 24 ERNs have been intensively working on different transversal areas, including patient management, education, clinical practice guidelines, patients' care pathways and many other fundamental topics. The present work is therefore aimed not only at reporting a summary of the main activities and milestones reached so far, but also at celebrating the first 5 years of the ERN on Rare and Complex Connective Tissue and Musculo-skeletal Diseases (ReCONNET), in which the members of the network built together one of the 24 infrastructures that are hopefully going to change the scenario of rare diseases across the EU.
Published: 2022

2. Impact of systemic sclerosis-associated interstitial lung disease with and without pulmonary hypertension on survival in over 5,000 patients

Author: Moinzadeh, P, Sunderkötter, C, Ehrchen, J, Kötter, I, Korsten, P, Lorenz, HM, Müller-Ladner, U, Oberste, M, Kreuter, A, Susok, L, Hunzelmann, N, Siegert, E, Worm, M, Pfeiffer, C, Riemekasten, G, Jandova, I, Schmalzing, M, Kreuter, M, Ramming, A, Bonella, F, Henes, J, Schmeiser, T, Zeidler, G, Görl, N, Juche, A, Günther, C, Keyßer, G, Blank, N, Moinzadeh, P, Sunderkötter, C, Ehrchen, J, Kötter, I, Korsten, P, Lorenz, HM, Müller-Ladner, U, Oberste, M, Kreuter, A, Susok, L, Hunzelmann, N, Siegert, E, Worm, M, Pfeiffer, C, Riemekasten, G, Jandova, I, Schmalzing, M, Kreuter, M, Ramming, A, Bonella, F, Henes, J, Schmeiser, T, Zeidler, G, Görl, N, Juche, A, Günther, C, Keyßer, G, and Blank, N
Published: 2023

3. Impact of systemic sclerosis-associated interstitial lung disease with and without pulmonary hypertension on survival in over 5,000 patients

Author: Moinzadeh, P, Sunderkötter, C, Ehrchen, J, Kötter, I, Korsten, P, Lorenz, HM, Müller-Ladner, U, Oberste, M, Kreuter, A, Susok, L, Hunzelmann, N, Siegert, E, Worm, M, Pfeiffer, C, Riemekasten, G, Jandova, I, Schmalzing, M, Kreuter, M, Ramming, A, Bonella, F, Henes, J, Schmeiser, T, Zeidler, G, Görl, N, Juche, A, Günther, C, Keyßer, G, Blank, N, Moinzadeh, P, Sunderkötter, C, Ehrchen, J, Kötter, I, Korsten, P, Lorenz, HM, Müller-Ladner, U, Oberste, M, Kreuter, A, Susok, L, Hunzelmann, N, Siegert, E, Worm, M, Pfeiffer, C, Riemekasten, G, Jandova, I, Schmalzing, M, Kreuter, M, Ramming, A, Bonella, F, Henes, J, Schmeiser, T, Zeidler, G, Görl, N, Juche, A, Günther, C, Keyßer, G, and Blank, N
Published: 2023

4. The added value of a European Reference Network on rare and complex connective tissue and musculoskeletal diseases: insights after the first 5 years of the ERN ReCONNET.

Author: UCL - SSS/IREC/RUMA - Pôle de Pathologies rhumatismales, UCL - (SLuc) Service de rhumatologie, Talarico, Rosaria, Aguilera, Silvia, Alexander, Tobias, Amoura, Zahir, Andersen, Janette, Arnaud, Laurent, Avcin, Tadej, Marsal Barril, Sara, Beretta, Lorenzo, Bombardieri, Stefano, Bortoluzzi, Alessandra, Bouillot, Coralie, Bulina, Inita, Burmester, Gerd R, Cannizzo, Sara, Cavagna, Lorenzo, Chaigne, Benjamin, Cornet, Alain, Corti, Paolo, Costedoat-Chalumeau, Nathalie, Dāvidsone, Zane, Doria, Andrea, Fenech, Carol, Ferraris, Alessandro, Fischer-Betz, Rebecca, Fonseca, João Eurico, Frank, Charissa, Gaglioti, Andrea, Galetti, Ilaria, Guimarães, Vera, Hachulla, Eric, Holmner, Monica, Houssiau, Frederic, Iaccarino, Luca, Jacobsen, Søren, Limper, Maarten, Malfait, Fransiska, Mariette, Xavier, Marinello, Diana, Martin, Thierry, Matthews, Lisa, Matucci-Cerinic, Marco, Meyer, Alain, Milas-Ahić, Jasminka, Moinzadeh, Pia, Montecucco, Carlomaurizio, Mouthon, Luc, Müller-Ladner, Ulf, Nagy, György, Patarata, Eunice, Pileckyte, Margarita, Pruunsild, Chris, Rednic, Simona, Romão, Vasco C, Schneider, Matthias, Scirè, Carlo Alberto, Smith, Vanessa, Sulli, Alberto, Tamirou, Farah, Tani, Chiara, Taruscio, Domenica, Taulaigo, Anna V, Tincani, Angela, Ticciati, Simone, Turchetti, Giuseppe, van Hagen, P Martin, van Laar, Jacob M, Viera, Ana, de Vries-Bouwstra, Jeska K, Zschocke, Johannes, Cutolo, Maurizio, Mosca, Marta, UCL - SSS/IREC/RUMA - Pôle de Pathologies rhumatismales, UCL - (SLuc) Service de rhumatologie, Talarico, Rosaria, Aguilera, Silvia, Alexander, Tobias, Amoura, Zahir, Andersen, Janette, Arnaud, Laurent, Avcin, Tadej, Marsal Barril, Sara, Beretta, Lorenzo, Bombardieri, Stefano, Bortoluzzi, Alessandra, Bouillot, Coralie, Bulina, Inita, Burmester, Gerd R, Cannizzo, Sara, Cavagna, Lorenzo, Chaigne, Benjamin, Cornet, Alain, Corti, Paolo, Costedoat-Chalumeau, Nathalie, Dāvidsone, Zane, Doria, Andrea, Fenech, Carol, Ferraris, Alessandro, Fischer-Betz, Rebecca, Fonseca, João Eurico, Frank, Charissa, Gaglioti, Andrea, Galetti, Ilaria, Guimarães, Vera, Hachulla, Eric, Holmner, Monica, Houssiau, Frederic, Iaccarino, Luca, Jacobsen, Søren, Limper, Maarten, Malfait, Fransiska, Mariette, Xavier, Marinello, Diana, Martin, Thierry, Matthews, Lisa, Matucci-Cerinic, Marco, Meyer, Alain, Milas-Ahić, Jasminka, Moinzadeh, Pia, Montecucco, Carlomaurizio, Mouthon, Luc, Müller-Ladner, Ulf, Nagy, György, Patarata, Eunice, Pileckyte, Margarita, Pruunsild, Chris, Rednic, Simona, Romão, Vasco C, Schneider, Matthias, Scirè, Carlo Alberto, Smith, Vanessa, Sulli, Alberto, Tamirou, Farah, Tani, Chiara, Taruscio, Domenica, Taulaigo, Anna V, Tincani, Angela, Ticciati, Simone, Turchetti, Giuseppe, van Hagen, P Martin, van Laar, Jacob M, Viera, Ana, de Vries-Bouwstra, Jeska K, Zschocke, Johannes, Cutolo, Maurizio, and Mosca, Marta
Abstract: In order to address the main challenges related to the rare diseases (RDs) the European Commission launched the European Reference Networks (ERNs), virtual networks involving healthcare providers (HCPs) across Europe. The mission of the ERNs is to tackle low prevalence and RDs that require highly specialised treatment and a concentration of knowledge and resources. In fact, ERNs offer the potential to give patients and healthcare professionals across the EU access to the best expertise and timely exchange of lifesaving knowledge, trying to make the knowledge travelling more than patients. For this reason, ERNs were established as concrete European infrastructures, and this is particularly crucial in the framework of rare and complex diseases in which no country alone has the whole knowledge and capacity to treat all types of patients.It has been five years since their kick-off launch in Vilnius in 2017. The 24 ERNs have been intensively working on different transversal areas, including patient management, education, clinical practice guidelines, patients' care pathways and many other fundamental topics. The present work is therefore aimed not only at reporting a summary of the main activities and milestones reached so far, but also at celebrating the first 5 years of the ERN on Rare and Complex Connective Tissue and Musculo-skeletal Diseases (ReCONNET), in which the members of the network built together one of the 24 infrastructures that are hopefully going to change the scenario of rare diseases across the EU.
Published: 2022

5. Scleroderma: the hard skin

Author: Speidel, Jil, Hunzelmann, Nicolas, Moinzadeh, Pia, Speidel, Jil, Hunzelmann, Nicolas, and Moinzadeh, Pia
Abstract: The term scleroderma covers a heterogeneous group of diseases sharing the characteristic feature of cutaneous sclerosis. The individual diseases vary in the involvement of affected structures such as skin, adipose tissue, muscles, joint structures, bones, internal organs and the associated disease expression. Basically, two different subtypes can be distinguished: localised scleroderma (LoS) and systemic sclerosis (SSc), each of which are subdivided into further subgroups. These are two distinct diseases that differ in the extent of skin sclerosis, involvement of internal organs, antibody profile, disease management and prognosis. In addition, there are clinical pictures that mimic scleroderma and are therefore referred to as scleroderma-like diseases or pseudoscleroderma. In order to make the correct diagnosis, a standardised diagnostic procedure is significant. Early diagnosis is important in order to initiate early treatment and thus minimise functional cutaneous and extracutaneous impairment as well as cosmetic damage.
Published: 2022

6. NON-SURGICAL LOCAL TREATMENTS FOR DIGITAL ULCERS IN SYSTEMIC SCLEROSIS: A SYSTEMATIC LITERATURE REVIEW

Author: Campochiaro, C., Suliman, Y. A., Hughes, M., Schoones, J., Giuggioli, D., Moinzadeh, P., Maltez, N., Ross, L., Baron, M., Chung, L., Allanore, Y., Denton, C. P., Distler, O., Frech, T., Furst, D., Khanna, D., Krieg, T., Kuwana, M., Matucci-Cerinic, M., Pope, J., Alunno, A., Campochiaro, C., Suliman, Y. A., Hughes, M., Schoones, J., Giuggioli, D., Moinzadeh, P., Maltez, N., Ross, L., Baron, M., Chung, L., Allanore, Y., Denton, C. P., Distler, O., Frech, T., Furst, D., Khanna, D., Krieg, T., Kuwana, M., Matucci-Cerinic, M., Pope, J., and Alunno, A.
Published: 2022

7. SYSTEMIC PHARMACOLOGICAL TREATMENT OF DIGITAL ULCERS IN SYSTEMIC SCLEROSIS: A SYSTEMATIC LITERATURE REVIEW

Author: Maltez, N., Ross, L., Hughes, M., Schoones, J., Baron, M., Chung, L., Campochiaro, C., Suliman, Y. A., Giuggioli, D., Moinzadeh, P., Allanore, Y., Denton, C. P., Distler, O., Frech, T., Furst, D., Khanna, D., Krieg, T., Kuwana, M., Matucci-Cerinic, M., Pope, J., Alunno, A., Maltez, N., Ross, L., Hughes, M., Schoones, J., Baron, M., Chung, L., Campochiaro, C., Suliman, Y. A., Giuggioli, D., Moinzadeh, P., Allanore, Y., Denton, C. P., Distler, O., Frech, T., Furst, D., Khanna, D., Krieg, T., Kuwana, M., Matucci-Cerinic, M., Pope, J., and Alunno, A.
Published: 2022

8. SURGICAL MANAGEMENT OF DIGITAL ULCERS IN SYSTEMIC SCLEROSIS: A SYSTEMATIC LITERATURE REVIEW.

Author: Suliman, Y. A., Campochiaro, C., Hughes, M., Schoones, J., Giuggioli, D., Maltez, N., Moinzadeh, P., Ross, L., Chung, L., Allanore, Y., Baron, M., Denton, C. P., Distler, O., Frech, T., Furst, D., Khanna, D., Krieg, T., Kuwana, M., Matucci-Cerinic, M., Pope, J., Alunno, A., Suliman, Y. A., Campochiaro, C., Hughes, M., Schoones, J., Giuggioli, D., Maltez, N., Moinzadeh, P., Ross, L., Chung, L., Allanore, Y., Baron, M., Denton, C. P., Distler, O., Frech, T., Furst, D., Khanna, D., Krieg, T., Kuwana, M., Matucci-Cerinic, M., Pope, J., and Alunno, A.
Published: 2022

9. Autologous hematopoietic stem cell transplantation improves long-term survival-data from a national registry

Author: Blank, Norbert, Schmalzing, Marc, Moinzadeh, Pia, Oberste, Max, Siegert, Elise, Mueller-Ladner, Ulf, Riemekasten, Gabriela, Guenther, Claudia, Koetter, Ina, Zeidler, Gabriele, Pfeiffer, Christiane, Juche, Aaron, Jandova, Ilona, Ehrchen, Jan, Susok, Laura, Schmeiser, Tim, Sunderkoetter, Cord, Distler, Jorg H. W., Worm, Margitta, Kreuter, Alexander, Keysser, Gernot, Lorenz, Hanns-Martin, Krieg, Thomas, Hunzelmann, Nicolas, Henes, Jorg, Blank, Norbert, Schmalzing, Marc, Moinzadeh, Pia, Oberste, Max, Siegert, Elise, Mueller-Ladner, Ulf, Riemekasten, Gabriela, Guenther, Claudia, Koetter, Ina, Zeidler, Gabriele, Pfeiffer, Christiane, Juche, Aaron, Jandova, Ilona, Ehrchen, Jan, Susok, Laura, Schmeiser, Tim, Sunderkoetter, Cord, Distler, Jorg H. W., Worm, Margitta, Kreuter, Alexander, Keysser, Gernot, Lorenz, Hanns-Martin, Krieg, Thomas, Hunzelmann, Nicolas, and Henes, Jorg
Abstract: Background Current recommendations on the management of systemic sclerosis (SSc) suggest that autologous hematopoietic stem cell therapy (HSCT) can be a rescue therapy for patients with rapidly progressive SSc. Objectives To assess the safety and efficacy of HSCT for patients with SSc and to compare these with non-HSCT patients in a control cohort with adjusted risk factors. Methods A retrospective analysis of data from the multicentric German network for systemic scleroderma (DNSS) with 5000 patients with SSc. Control groups consisted of all patients with diffuse cutaneous (dc)-SSc (group A) and an adjusted high-risk cohort of male patients with Scl70-positive dc-SSc (group B). Results Eighty SSc patients received an HSCT 4.1 +/- 4.8 years after SSc diagnosis. Among them, 86.3% had dc-SSc, 43.5% were males, and 71.3% were positive for Scl70 antibodies. The control group A (n=1513) showed a significant underrepresentation of these risk factors for mortality. When the survival of the control group B (n=240) was compared with the HSCT group, a lower mortality of the latter was observed instead. Within 5 years after HSCT, we observed an improvement of the mRSS from 17.6 +/- 11.5 to 11.0 +/- 8.5 (p=0.001) and a stabilization of the DLCO. We did not see differences in transplant-related mortality between patients who received HSCT within 3 years after SSc diagnosis or later. Conclusion Our analysis of real-life data show that the distribution of risk factors for mortality is critical when HSCT cohorts are compared with non-HSCT control groups.
Published: 2022

10. The added value of a European Reference Network on rare and complex connective tissue and musculoskeletal diseases:insights after the first 5 years of the ERN ReCONNET

Author: Talarico, R., Aguilera, S., Alexander, T., Amoura, Z., Andersen, J., Arnaud, L., Avcin, T., Marsal Barril, S., Beretta, L., Bombardieri, S., Bortoluzzi, A., Bouillot, C., Bulina, I., Burmester, G. R., Cannizzo, S., Cavagna, L., Chaigne, B., Cornet, A., Corti, P., Costedoat-Chalumeau, N., Dāvidsone, Z., Doria, A., Fenech, C., Ferraris, A., Fischer-Betz, R., Fonseca, J. E., Frank, C., Gaglioti, A., Galetti, I., Guimarães, V., Hachulla, E., Holmner, M., Houssiau, F., Iaccarino, L., Jacobsen, S., Limper, M., Malfait, F., Mariette, X., Marinello, D., Martin, T., Matthews, L., Matucci-Cerinic, M., Meyer, A., Milas-Ahić, J., Moinzadeh, P., Montecucco, C., Mouthon, L., Müller-Ladner, U., Nagy, G., Patarata, E., Pileckyte, M., Pruunsild, C., Rednic, S., Romão, V. C., Schneider, M., Scirè, C. A., Smith, V., Sulli, A., Tamirou, F., Tani, C., Taruscio, D., Taulaigo, A. V., Tincani, A., Ticciati, S., Turchetti, G., van Hagen, P. M., van Laar, J. M., Vieira, A., de Vries-Bouwstra, J. K., Zschocke, J., Cutolo, M., Mosca, Marta, Talarico, R., Aguilera, S., Alexander, T., Amoura, Z., Andersen, J., Arnaud, L., Avcin, T., Marsal Barril, S., Beretta, L., Bombardieri, S., Bortoluzzi, A., Bouillot, C., Bulina, I., Burmester, G. R., Cannizzo, S., Cavagna, L., Chaigne, B., Cornet, A., Corti, P., Costedoat-Chalumeau, N., Dāvidsone, Z., Doria, A., Fenech, C., Ferraris, A., Fischer-Betz, R., Fonseca, J. E., Frank, C., Gaglioti, A., Galetti, I., Guimarães, V., Hachulla, E., Holmner, M., Houssiau, F., Iaccarino, L., Jacobsen, S., Limper, M., Malfait, F., Mariette, X., Marinello, D., Martin, T., Matthews, L., Matucci-Cerinic, M., Meyer, A., Milas-Ahić, J., Moinzadeh, P., Montecucco, C., Mouthon, L., Müller-Ladner, U., Nagy, G., Patarata, E., Pileckyte, M., Pruunsild, C., Rednic, S., Romão, V. C., Schneider, M., Scirè, C. A., Smith, V., Sulli, A., Tamirou, F., Tani, C., Taruscio, D., Taulaigo, A. V., Tincani, A., Ticciati, S., Turchetti, G., van Hagen, P. M., van Laar, J. M., Vieira, A., de Vries-Bouwstra, J. K., Zschocke, J., Cutolo, M., and Mosca, Marta
Abstract: In order to address the main challenges related to the rare diseases (RDs) the European Commission launched the European Reference Networks (ERNs), virtual networks involving healthcare providers (HCPs) across Europe. The mission of the ERNs is to tackle low prevalence and RDs that require highly specialised treatment and a concentration of knowledge and resources. In fact, ERNs offer the potential to give patients and healthcare professionals across the EU access to the best expertise and timely exchange of lifesaving knowledge, trying to make the knowledge travelling more than patients. For this reason, ERNs were established as concrete European infrastructures, and this is particularly crucial in the framework of rare and complex diseases in which no country alone has the whole knowledge and capacity to treat all types of patients. It has been five years since their kick-off launch in Vilnius in 2017. The 24 ERNs have been intensively working on different transversal areas, including patient management, education, clinical practice guidelines, patients' care pathways and many other fundamental topics. The present work is therefore aimed not only at reporting a summary of the main activities and milestones reached so far, but also at celebrating the first 5 years of the ERN on Rare and Complex Connective Tissue and Musculo-skeletal Diseases (ReCONNET), in which the members of the network built together one of the 24 infrastructures that are hopefully going to change the scenario of rare diseases across the EU.
Published: 2022

11. The added value of a European Reference Network on rare and complex connective tissue and musculoskeletal diseases:insights after the first 5 years of the ERN ReCONNET

Author: Talarico, R., Aguilera, S., Alexander, T., Amoura, Z., Andersen, J., Arnaud, L., Avcin, T., Marsal Barril, S., Beretta, L., Bombardieri, S., Bortoluzzi, A., Bouillot, C., Bulina, I., Burmester, G. R., Cannizzo, S., Cavagna, L., Chaigne, B., Cornet, A., Corti, P., Costedoat-Chalumeau, N., Dāvidsone, Z., Doria, A., Fenech, C., Ferraris, A., Fischer-Betz, R., Fonseca, J. E., Frank, C., Gaglioti, A., Galetti, I., Guimarães, V., Hachulla, E., Holmner, M., Houssiau, F., Iaccarino, L., Jacobsen, S., Limper, M., Malfait, F., Mariette, X., Marinello, D., Martin, T., Matthews, L., Matucci-Cerinic, M., Meyer, A., Milas-Ahić, J., Moinzadeh, P., Montecucco, C., Mouthon, L., Müller-Ladner, U., Nagy, G., Patarata, E., Pileckyte, M., Pruunsild, C., Rednic, S., Romão, V. C., Schneider, M., Scirè, C. A., Smith, V., Sulli, A., Tamirou, F., Tani, C., Taruscio, D., Taulaigo, A. V., Tincani, A., Ticciati, S., Turchetti, G., van Hagen, P. M., van Laar, J. M., Vieira, A., de Vries-Bouwstra, J. K., Zschocke, J., Cutolo, M., Mosca, Marta, Talarico, R., Aguilera, S., Alexander, T., Amoura, Z., Andersen, J., Arnaud, L., Avcin, T., Marsal Barril, S., Beretta, L., Bombardieri, S., Bortoluzzi, A., Bouillot, C., Bulina, I., Burmester, G. R., Cannizzo, S., Cavagna, L., Chaigne, B., Cornet, A., Corti, P., Costedoat-Chalumeau, N., Dāvidsone, Z., Doria, A., Fenech, C., Ferraris, A., Fischer-Betz, R., Fonseca, J. E., Frank, C., Gaglioti, A., Galetti, I., Guimarães, V., Hachulla, E., Holmner, M., Houssiau, F., Iaccarino, L., Jacobsen, S., Limper, M., Malfait, F., Mariette, X., Marinello, D., Martin, T., Matthews, L., Matucci-Cerinic, M., Meyer, A., Milas-Ahić, J., Moinzadeh, P., Montecucco, C., Mouthon, L., Müller-Ladner, U., Nagy, G., Patarata, E., Pileckyte, M., Pruunsild, C., Rednic, S., Romão, V. C., Schneider, M., Scirè, C. A., Smith, V., Sulli, A., Tamirou, F., Tani, C., Taruscio, D., Taulaigo, A. V., Tincani, A., Ticciati, S., Turchetti, G., van Hagen, P. M., van Laar, J. M., Vieira, A., de Vries-Bouwstra, J. K., Zschocke, J., Cutolo, M., and Mosca, Marta
Abstract: In order to address the main challenges related to the rare diseases (RDs) the European Commission launched the European Reference Networks (ERNs), virtual networks involving healthcare providers (HCPs) across Europe. The mission of the ERNs is to tackle low prevalence and RDs that require highly specialised treatment and a concentration of knowledge and resources. In fact, ERNs offer the potential to give patients and healthcare professionals across the EU access to the best expertise and timely exchange of lifesaving knowledge, trying to make the knowledge travelling more than patients. For this reason, ERNs were established as concrete European infrastructures, and this is particularly crucial in the framework of rare and complex diseases in which no country alone has the whole knowledge and capacity to treat all types of patients. It has been five years since their kick-off launch in Vilnius in 2017. The 24 ERNs have been intensively working on different transversal areas, including patient management, education, clinical practice guidelines, patients' care pathways and many other fundamental topics. The present work is therefore aimed not only at reporting a summary of the main activities and milestones reached so far, but also at celebrating the first 5 years of the ERN on Rare and Complex Connective Tissue and Musculo-skeletal Diseases (ReCONNET), in which the members of the network built together one of the 24 infrastructures that are hopefully going to change the scenario of rare diseases across the EU.
Published: 2022

12. Idiopathic inflammatory myopathies: Narrative review of unmet needs in clinical practice guidelines

Author: Meyer, A, Scire, C, Talarico, R, Alexander, T, Amoura, Z, Avcin, T, Barsotti, S, Beretta, L, Blagojevic, J, Burmester, G, Cavazzana, I, Cherrin, P, Damian, L, Doria, A, Fonseca, J, Furini, F, Galetti, I, Houssiau, F, Krieg, T, Maddalena, L, Launay, D, Campanilho-Marques, R, Martin, T, Matucci-Cerinic, M, Moinzadeh, P, Montecucco, C, Moraes-Fontes, M, Mouthon, L, Neri, R, Paolino, S, Piette, Y, Rednic, S, Tamirou, F, Tincani, A, Toplak, N, Bombardieri, S, Hachulla, E, Mueller-Ladner, U, Schneider, M, Smith, V, Vieira, A, Cutolo, M, Mosca, M, Cavagna, L, Meyer A., Scire C. A., Talarico R., Alexander T., Amoura Z., Avcin T., Barsotti S., Beretta L., Blagojevic J., Burmester G., Cavazzana I., Cherrin P., Damian L., Doria A., Fonseca J. E., Furini F., Galetti I., Houssiau F., Krieg T., Maddalena L., Launay D., Campanilho-Marques R., Martin T., Matucci-Cerinic M., Moinzadeh P., Montecucco C., Moraes-Fontes M. F., Mouthon L., Neri R., Paolino S., Piette Y., Rednic S., Tamirou F., Tincani A., Toplak N., Bombardieri S., Hachulla E., Mueller-Ladner U., Schneider M., Smith V., Vieira A., Cutolo M., Mosca M., Cavagna L., Meyer, A, Scire, C, Talarico, R, Alexander, T, Amoura, Z, Avcin, T, Barsotti, S, Beretta, L, Blagojevic, J, Burmester, G, Cavazzana, I, Cherrin, P, Damian, L, Doria, A, Fonseca, J, Furini, F, Galetti, I, Houssiau, F, Krieg, T, Maddalena, L, Launay, D, Campanilho-Marques, R, Martin, T, Matucci-Cerinic, M, Moinzadeh, P, Montecucco, C, Moraes-Fontes, M, Mouthon, L, Neri, R, Paolino, S, Piette, Y, Rednic, S, Tamirou, F, Tincani, A, Toplak, N, Bombardieri, S, Hachulla, E, Mueller-Ladner, U, Schneider, M, Smith, V, Vieira, A, Cutolo, M, Mosca, M, Cavagna, L, Meyer A., Scire C. A., Talarico R., Alexander T., Amoura Z., Avcin T., Barsotti S., Beretta L., Blagojevic J., Burmester G., Cavazzana I., Cherrin P., Damian L., Doria A., Fonseca J. E., Furini F., Galetti I., Houssiau F., Krieg T., Maddalena L., Launay D., Campanilho-Marques R., Martin T., Matucci-Cerinic M., Moinzadeh P., Montecucco C., Moraes-Fontes M. F., Mouthon L., Neri R., Paolino S., Piette Y., Rednic S., Tamirou F., Tincani A., Toplak N., Bombardieri S., Hachulla E., Mueller-Ladner U., Schneider M., Smith V., Vieira A., Cutolo M., Mosca M., and Cavagna L.
Abstract: Idiopathic inflammatory myopathies (IIMs) encompass a heterogeneous group of rare autoimmune diseases characterised by muscle weakness and inflammation, but in antisynthetase syndrome arthritis and interstitial lung disease are more frequent and often inaugurate the disease. Clinical practice guidelines (CPGs) have been proposed for IIMs, but they are sparse and heterogeneous. This work aimed at identifying: i) current available CPGs for IIMs, ii) patients ' and clinicians' unmet needs not covered by CPGs. It has been performed in the framework of the European Reference Network on rare and complex connective tissue and musculoskeletal diseases (ReCONNET), a network of centre of expertise and patients funded by the European Union's Health Programme. Fourteen original CPGs were identified, notably recommending that: i) extra-muscular involvements should be assessed; ii) corticosteroids and methotrexate or azathioprine are first-line therapies of IIMs. ii) IVIG is a treatment of resistant-DM that may be also used in other resistant-IIMs; iii) physical therapy and sun protection (in DM patients) are part of the treatment; v) tumour screening for patients with DM include imaging of chest, abdomen, pelvis and breast (in woman) along with colonoscopy (in patients over 50 years); vi) disease activity and damages should be monitor using standardised and validated tools. Yet, only half of these CPGs were evidence-based. Crucial unmet needs were identified both by patients and clinicians. In particular, there was a lack of large multidisciplinary working group and of patients ' preferences. The following fields were not or inappropriately targeted: diagnosis; management of extra-muscular involvements other than skin; co-morbidities and severe manifestations.
Published: 2019

13. Idiopathic inflammatory myopathies: Narrative review of unmet needs in clinical practice guidelines

Author: Meyer, A, Scire, C, Talarico, R, Alexander, T, Amoura, Z, Avcin, T, Barsotti, S, Beretta, L, Blagojevic, J, Burmester, G, Cavazzana, I, Cherrin, P, Damian, L, Doria, A, Fonseca, J, Furini, F, Galetti, I, Houssiau, F, Krieg, T, Maddalena, L, Launay, D, Campanilho-Marques, R, Martin, T, Matucci-Cerinic, M, Moinzadeh, P, Montecucco, C, Moraes-Fontes, M, Mouthon, L, Neri, R, Paolino, S, Piette, Y, Rednic, S, Tamirou, F, Tincani, A, Toplak, N, Bombardieri, S, Hachulla, E, Mueller-Ladner, U, Schneider, M, Smith, V, Vieira, A, Cutolo, M, Mosca, M, Cavagna, L, Meyer A., Scire C. A., Talarico R., Alexander T., Amoura Z., Avcin T., Barsotti S., Beretta L., Blagojevic J., Burmester G., Cavazzana I., Cherrin P., Damian L., Doria A., Fonseca J. E., Furini F., Galetti I., Houssiau F., Krieg T., Maddalena L., Launay D., Campanilho-Marques R., Martin T., Matucci-Cerinic M., Moinzadeh P., Montecucco C., Moraes-Fontes M. F., Mouthon L., Neri R., Paolino S., Piette Y., Rednic S., Tamirou F., Tincani A., Toplak N., Bombardieri S., Hachulla E., Mueller-Ladner U., Schneider M., Smith V., Vieira A., Cutolo M., Mosca M., Cavagna L., Meyer, A, Scire, C, Talarico, R, Alexander, T, Amoura, Z, Avcin, T, Barsotti, S, Beretta, L, Blagojevic, J, Burmester, G, Cavazzana, I, Cherrin, P, Damian, L, Doria, A, Fonseca, J, Furini, F, Galetti, I, Houssiau, F, Krieg, T, Maddalena, L, Launay, D, Campanilho-Marques, R, Martin, T, Matucci-Cerinic, M, Moinzadeh, P, Montecucco, C, Moraes-Fontes, M, Mouthon, L, Neri, R, Paolino, S, Piette, Y, Rednic, S, Tamirou, F, Tincani, A, Toplak, N, Bombardieri, S, Hachulla, E, Mueller-Ladner, U, Schneider, M, Smith, V, Vieira, A, Cutolo, M, Mosca, M, Cavagna, L, Meyer A., Scire C. A., Talarico R., Alexander T., Amoura Z., Avcin T., Barsotti S., Beretta L., Blagojevic J., Burmester G., Cavazzana I., Cherrin P., Damian L., Doria A., Fonseca J. E., Furini F., Galetti I., Houssiau F., Krieg T., Maddalena L., Launay D., Campanilho-Marques R., Martin T., Matucci-Cerinic M., Moinzadeh P., Montecucco C., Moraes-Fontes M. F., Mouthon L., Neri R., Paolino S., Piette Y., Rednic S., Tamirou F., Tincani A., Toplak N., Bombardieri S., Hachulla E., Mueller-Ladner U., Schneider M., Smith V., Vieira A., Cutolo M., Mosca M., and Cavagna L.
Abstract: Idiopathic inflammatory myopathies (IIMs) encompass a heterogeneous group of rare autoimmune diseases characterised by muscle weakness and inflammation, but in antisynthetase syndrome arthritis and interstitial lung disease are more frequent and often inaugurate the disease. Clinical practice guidelines (CPGs) have been proposed for IIMs, but they are sparse and heterogeneous. This work aimed at identifying: i) current available CPGs for IIMs, ii) patients ' and clinicians' unmet needs not covered by CPGs. It has been performed in the framework of the European Reference Network on rare and complex connective tissue and musculoskeletal diseases (ReCONNET), a network of centre of expertise and patients funded by the European Union's Health Programme. Fourteen original CPGs were identified, notably recommending that: i) extra-muscular involvements should be assessed; ii) corticosteroids and methotrexate or azathioprine are first-line therapies of IIMs. ii) IVIG is a treatment of resistant-DM that may be also used in other resistant-IIMs; iii) physical therapy and sun protection (in DM patients) are part of the treatment; v) tumour screening for patients with DM include imaging of chest, abdomen, pelvis and breast (in woman) along with colonoscopy (in patients over 50 years); vi) disease activity and damages should be monitor using standardised and validated tools. Yet, only half of these CPGs were evidence-based. Crucial unmet needs were identified both by patients and clinicians. In particular, there was a lack of large multidisciplinary working group and of patients ' preferences. The following fields were not or inappropriately targeted: diagnosis; management of extra-muscular involvements other than skin; co-morbidities and severe manifestations.
Published: 2019

14. LONG-TERM OUTCOME OF SSC ASSOCIATED ILD: IMPROVED SURVIVAL IN PPI TREATED PATIENTS

Author: Kreuter, M., Bonella, F., Kathrin, K., Henes, J., Siegert, E., Riemekasten, G., Blank, N., Pfeiffer, C., Mueller-Ladner, U., Kreuter, A., Korsten, P., Juche, A., Schmalzing, M., Worm, M., Jandova, I., Susok, L., Schmeiser, T., Guenther, C., Keyszer, G., Ehrchen, J., Ramming, A., Koetter, I., Lorenz, H. M., Moinzadeh, P., Hunzelmann, N., Kreuter, M., Bonella, F., Kathrin, K., Henes, J., Siegert, E., Riemekasten, G., Blank, N., Pfeiffer, C., Mueller-Ladner, U., Kreuter, A., Korsten, P., Juche, A., Schmalzing, M., Worm, M., Jandova, I., Susok, L., Schmeiser, T., Guenther, C., Keyszer, G., Ehrchen, J., Ramming, A., Koetter, I., Lorenz, H. M., Moinzadeh, P., and Hunzelmann, N.
Published: 2021

15. This is how I treat Calcinosis cutis in the Context of Collagenosis ...

Author: Haeger, C., Moinzadeh, P., Hunzelmann, N., Haeger, C., Moinzadeh, P., and Hunzelmann, N.
Published: 2021

16. This is how I treat Calcinosis cutis in the Context of Collagenosis ...

Author: Haeger, C., Moinzadeh, P., Hunzelmann, N., Haeger, C., Moinzadeh, P., and Hunzelmann, N.
Published: 2021

17. LONG-TERM OUTCOME OF SSC ASSOCIATED ILD: IMPROVED SURVIVAL IN PPI TREATED PATIENTS

Author: Kreuter, M., Bonella, F., Kathrin, K., Henes, J., Siegert, E., Riemekasten, G., Blank, N., Pfeiffer, C., Mueller-Ladner, U., Kreuter, A., Korsten, P., Juche, A., Schmalzing, M., Worm, M., Jandova, I., Susok, L., Schmeiser, T., Guenther, C., Keyszer, G., Ehrchen, J., Ramming, A., Koetter, I., Lorenz, H. M., Moinzadeh, P., Hunzelmann, N., Kreuter, M., Bonella, F., Kathrin, K., Henes, J., Siegert, E., Riemekasten, G., Blank, N., Pfeiffer, C., Mueller-Ladner, U., Kreuter, A., Korsten, P., Juche, A., Schmalzing, M., Worm, M., Jandova, I., Susok, L., Schmeiser, T., Guenther, C., Keyszer, G., Ehrchen, J., Ramming, A., Koetter, I., Lorenz, H. M., Moinzadeh, P., and Hunzelmann, N.
Published: 2021

18. Scleroderma Renal Crisis: Risk Factors for an Increasingly Rare Organ Complication

Author: Moinzadeh, Pia, Kuhr, Kathrin, Siegert, Elise, Blank, Norbert, Sunderkoetter, Cord, Henes, Joerg, Krusche, Martin, Schmalzing, Marc, Worm, Margitta, Schmeiser, Tim, Guenther, Claudia, Aberer, Elisabeth, Susok, Laura, Riemekasten, Gabriela, Kreuter, Alexander, Zeidler, Gabriele, Juche, Aaron, Hadjiski, Denitsa, Mueller-Ladner, Ulf, Gaebelein-Wissing, Noemi, Distler, Joerg H. W., Sardy, Miklos, Krieg, Thomas, Hunzelmann, Nicolas, Moinzadeh, Pia, Kuhr, Kathrin, Siegert, Elise, Blank, Norbert, Sunderkoetter, Cord, Henes, Joerg, Krusche, Martin, Schmalzing, Marc, Worm, Margitta, Schmeiser, Tim, Guenther, Claudia, Aberer, Elisabeth, Susok, Laura, Riemekasten, Gabriela, Kreuter, Alexander, Zeidler, Gabriele, Juche, Aaron, Hadjiski, Denitsa, Mueller-Ladner, Ulf, Gaebelein-Wissing, Noemi, Distler, Joerg H. W., Sardy, Miklos, Krieg, Thomas, and Hunzelmann, Nicolas
Abstract: Objective. Scleroderma renal crisis (SRC) is a severe life-threatening manifestation in patients with systemic sclerosis (SSc). However, the knowledge about risk factors for SRC is limited. We determined here the frequency of SRC and identified risk factors for the prediction of SRC. Methods. Based on regular followup data from the German Network for Systemic Scleroderma, we used univariate and multivariate generalized estimating equations to analyze the association between clinical variables, SSc subsets, therapy [i.e., angiotensin-converting enzyme inhibitors (ACEi), corticosteroids], and the occurrence of SRC. Results. Data of 2873 patients with 10,425 visits were available for analysis with a mean number of registry visits of 3.6 +/- 2.8 and a mean time of followup of 3.6 +/- 3.8 years. In total, 70 patients developed SRC (70/ 2873, 2.4%). Of these patients, 57.1% (40/70) were diagnosed with diffuse cutaneous SSc, 31.4% (22/70) with limited cutaneous SSc, and 11.4% (8/70) with SSc- overlap syndromes. Predictive independent factors with the highest probability for SRC were positive anti-RNA polymerase antibodies (RNAP), a history of proteinuria prior to SRC onset, diminished DLCO, and a history of hypertension. Interestingly, positive antitopoisomerase autoantibodies did not predict a higher risk for SRC. Further, patients with SRC were significantly more frequently treated with ACEi and corticosteroids without being independently associated with SRC. Conclusion. In this cohort, SRC has become a rare complication. By far the highest risk for SRC was associated with the detection of anti-RNAP and proteinuria.
Published: 2020

19. Long term outcomes of immunmodulatory drugs in SSc-ILD - data rom the German SSc network

Author: Kreuter, M., Bonella, F., Blank, N., Siegert, E., Henes, J., Worm, M., Sunderkoetter, C., Schmalzing, M., Kreuter, A., Guenther, C., Susok, L., Zeidler, G., Koetter, I, Mueller-Ladner, U., Krieg, T., Juche, A., Schmeiser, T., Riemekasten, G., Aberer, E., Gaebelein-Wissing, N., Distler, J. H. W., Sardy, M., Pfeiffer, C., Kuhr, K., Lorenz, H. M., Moinzadeh, P., Hunzelmann, N., Kreuter, M., Bonella, F., Blank, N., Siegert, E., Henes, J., Worm, M., Sunderkoetter, C., Schmalzing, M., Kreuter, A., Guenther, C., Susok, L., Zeidler, G., Koetter, I, Mueller-Ladner, U., Krieg, T., Juche, A., Schmeiser, T., Riemekasten, G., Aberer, E., Gaebelein-Wissing, N., Distler, J. H. W., Sardy, M., Pfeiffer, C., Kuhr, K., Lorenz, H. M., Moinzadeh, P., and Hunzelmann, N.
Published: 2020

20. Whole blood gene expression profiling distinguishes systemic sclerosis-overlap syndromes from other subsets

Author: Moinzadeh, P., Frommolt, P., Franitza, M., Toliat, M. R., Becker, K., Nuernberg, P., Nihtyanova, S. I., Ahrazoglu, M., Belz, D., Hunzelmann, N., Abraham, D., Ong, V. H., Mouthon, L., Hesselstrand, R., Denton, C. P., Krieg, T., Moinzadeh, P., Frommolt, P., Franitza, M., Toliat, M. R., Becker, K., Nuernberg, P., Nihtyanova, S. I., Ahrazoglu, M., Belz, D., Hunzelmann, N., Abraham, D., Ong, V. H., Mouthon, L., Hesselstrand, R., Denton, C. P., and Krieg, T.
Published: 2020

21. Does anti-acid treatment influence disease progression in SSc-ILD? Data form the German SSc-network

Author: Kreuter, M., Bonella, F., Blank, N., Siegert, E., Henes, J., Worm, M., Sunderkoetter, C., Schmalzing, M., Kreuter, A., Guenther, C., Susok, L., Zeidler, G., Koetter, I, Mueller-Ladner, U., Krieg, T., Juche, A., Schmeiser, T., Riemekasten, G., Aberer, E., Gaebelein-Wissing, N., Distler, J. H. W., Sardy, M., Pfeiffer, C., Kuhr, K., Lorenz, H. M., Moinzadeh, P., Hunzelmann, N., Kreuter, M., Bonella, F., Blank, N., Siegert, E., Henes, J., Worm, M., Sunderkoetter, C., Schmalzing, M., Kreuter, A., Guenther, C., Susok, L., Zeidler, G., Koetter, I, Mueller-Ladner, U., Krieg, T., Juche, A., Schmeiser, T., Riemekasten, G., Aberer, E., Gaebelein-Wissing, N., Distler, J. H. W., Sardy, M., Pfeiffer, C., Kuhr, K., Lorenz, H. M., Moinzadeh, P., and Hunzelmann, N.
Published: 2020

22. Large Variability of Frequency and Type of Physical Therapy in Patients in the German Network for Systemic Sclerosis

Author: Belz, D., Moinzadeh, P., Riemekasten, G., Henes, J., Mueller-Ladner, U., Blank, N., Koetter, I, Siegert, E., Pfeiffer, C., Schmalzing, M., Zeidler, G., Schmeiser, T., Worm, M., Guenther, C., Susok, L., Kreuter, A., Sunderkoetter, C., Juche, A., Aberer, E., Gaebelein-Wissing, N., Ramming, A., Kuhr, K., Hunzelmann, N., Belz, D., Moinzadeh, P., Riemekasten, G., Henes, J., Mueller-Ladner, U., Blank, N., Koetter, I, Siegert, E., Pfeiffer, C., Schmalzing, M., Zeidler, G., Schmeiser, T., Worm, M., Guenther, C., Susok, L., Kreuter, A., Sunderkoetter, C., Juche, A., Aberer, E., Gaebelein-Wissing, N., Ramming, A., Kuhr, K., and Hunzelmann, N.
Abstract: Objective To determine the type and frequency of physical therapy (PT) prescribed by physicians for patients in the registry of the German Network for Systemic Sclerosis. Methods The data for 4,252 patients were analyzed using descriptive statistics, chi-square tests, and odds ratios (ORs). Results Overall, 37.4% of patients (1,590 of 4,252) receivedPTat the end of a yearly follow-up. The most frequently used type ofPTwas lymphatic drainage (n = 1,061, 36.8%), followed by exercise therapy (n = 1,047, 36.3%) and heat therapy (n = 689, 23.9%). More than three-fourths of treated patients (82%) received 1 or 2 different forms ofPTsimultaneously. The prescription ofPTwas associated with the extent of skin fibrosis as measured by the modified Rodnan skin thickness score (<10 [41.8% of patients], 11-20 [55.8% of patients], and >21 [63.9% of patients];P< 0.001). Patients with musculoskeletal involvement (e.g., arthritis, muscle weakness, joint contractures, tendon friction rubs) had a higher chance of receivingPTthan patients without these symptoms, with correspondingORs ranging from 1.96 (95% confidence interval [95%CI] 1.69-2.28) for joint contractures to 3.83 (95%CI2.89-5.08) for arthritis. When comparing the type ofPTprescription across the initial and all follow-up visits from 2003 to 2017, significant alterations with a decreasing frequency of patients receivingPTcould be observed (P= 0.001). Conclusion To our knowledge, this is the first study reporting the use ofPTin patients with systemic sclerosis (SSc) in a large cohort. AlthoughSSc is characterized by considerable disability and restriction of motion, PT.
Published: 2020

23. DOES ANTI-ACID TREATMENT INFLUENCE DISEASE PROGRESSION IN SYSTEMIC SCLEROSIS INTERSTITIAL LUNG DISEASE (SSC-ILD)? DATA FROM THE GERMAN SSC-NETWORK

Author: Kreuter, M., Bonella, F., Riemekasten, G., Mueller-Ladner, U., Henes, J., Siegert, E., Guenther, C., Koetter, I., Blank, N., Pfeiffer, C., Schmalzing, M., Zeidler, G., Korsten, P., Susok, L., Juche, A., Worm, M., Jandova, I., Ehrchen, J., Sunderkoetter, C., Keyszer, G., Ramming, A., Schmeiser, T., Kreuter, A., Kuhr, K., Lorenz, H. M., Moinzadeh, P., Hunzelmann, N., Kreuter, M., Bonella, F., Riemekasten, G., Mueller-Ladner, U., Henes, J., Siegert, E., Guenther, C., Koetter, I., Blank, N., Pfeiffer, C., Schmalzing, M., Zeidler, G., Korsten, P., Susok, L., Juche, A., Worm, M., Jandova, I., Ehrchen, J., Sunderkoetter, C., Keyszer, G., Ramming, A., Schmeiser, T., Kreuter, A., Kuhr, K., Lorenz, H. M., Moinzadeh, P., and Hunzelmann, N.
Published: 2020

24. Older age onset of systemic sclerosis - accelerated disease progression in all disease subsets

Author: Moinzadeh, Pia, Kuhr, Kathrin, Siegert, Elise, Mueller-Ladner, Ulf, Riemekasten, Gabriela, Guenther, Claudia, Koetter, Ina, Henes, Joerg, Blank, Norbert, Zeidler, Gabriele, Pfeiffer, Christiane, Juche, Aaron, Jandova, Ilona, Ehrchen, Jan, Schmalzing, Marc, Susok, Laura, Schmeiser, Tim, Sunderkoetter, Cord, Distler, Joerg H. W., Worm, Margitta, Kreuter, Alexander, Krieg, Thomas, Hunzelman, Nicolas, Moinzadeh, Pia, Kuhr, Kathrin, Siegert, Elise, Mueller-Ladner, Ulf, Riemekasten, Gabriela, Guenther, Claudia, Koetter, Ina, Henes, Joerg, Blank, Norbert, Zeidler, Gabriele, Pfeiffer, Christiane, Juche, Aaron, Jandova, Ilona, Ehrchen, Jan, Schmalzing, Marc, Susok, Laura, Schmeiser, Tim, Sunderkoetter, Cord, Distler, Joerg H. W., Worm, Margitta, Kreuter, Alexander, Krieg, Thomas, and Hunzelman, Nicolas
Abstract: Objectives. Systemic sclerosis is a heterogeneous, multisystem disease. It can occur at any age, but most patients develop the disease between the age of 40 to 50 years. There is controversial evidence on whether/how the age at disease onset affects their clinical phenotype. We here investigate the relationship between age at disease onset and symptoms in a large cohort of SSc patients (IcSSc, dcSSc and SSc-overlap syndromes). Methods. Clinical data of the registry of the German Network for Systemic Scleroderma including 3281 patients were evaluated and subdivided into three age groups at disease onset (<40years, 40-60 years, >60 years). Results. Among all SSc patients, 24.5% developed their first non-Raynaud phenomenon symptoms at the age <40years, and 22.5% were older than 60 years of age. In particular, older patients at onset developed the IcSSc subset significantly more often. Furthermore, they had pulmonary hypertension more often, but digital ulcerations less often. Remarkably, the course of the disease was more rapidly progressing in the older cohort (>60 years), except for gastrointestinal and musculoskeletal involvement. No significant difference was found for the use of corticosteroids. However, significantly, fewer patients older than 60 years received immunosuppressive treatment. Conclusion. In this large registry, similar to 25% of patients developed SSc at an age above 60years with an increased frequency of IcSSc. In this age group, an onset of internal organ involvement was significantly accelerated across all three subsets. These findings suggest that, in the elderly cohort, more frequent follow-up examinations are required for an earlier detection of organ complications.
Published: 2020

25. Whole blood gene expression profiling distinguishes systemic sclerosis‐overlap syndromes from other subsets

Author: Moinzadeh, P., Frommolt, Peter, Franitza, M., Toliat, Mohammad Reza, Becker, K., Nürnberg, Peter, Nihtyanova, Svetlana I., Ahrazoglu, M., Belz, Doreen, Hunzelmann, N., Abraham, D., Ong, V. H., Mouthon, L., Hesselstrand, R., Denton, C. P., Krieg, Thomas M., Moinzadeh, P., Frommolt, Peter, Franitza, M., Toliat, Mohammad Reza, Becker, K., Nürnberg, Peter, Nihtyanova, Svetlana I., Ahrazoglu, M., Belz, Doreen, Hunzelmann, N., Abraham, D., Ong, V. H., Mouthon, L., Hesselstrand, R., Denton, C. P., and Krieg, Thomas M.
Published: 2020

26. Scleroderma Renal Crisis: Risk Factors for an Increasingly Rare Organ Complication

Author: Moinzadeh, Pia, Kuhr, Kathrin, Siegert, Elise, Blank, Norbert, Sunderkoetter, Cord, Henes, Joerg, Krusche, Martin, Schmalzing, Marc, Worm, Margitta, Schmeiser, Tim, Guenther, Claudia, Aberer, Elisabeth, Susok, Laura, Riemekasten, Gabriela, Kreuter, Alexander, Zeidler, Gabriele, Juche, Aaron, Hadjiski, Denitsa, Mueller-Ladner, Ulf, Gaebelein-Wissing, Noemi, Distler, Joerg H. W., Sardy, Miklos, Krieg, Thomas, Hunzelmann, Nicolas, Moinzadeh, Pia, Kuhr, Kathrin, Siegert, Elise, Blank, Norbert, Sunderkoetter, Cord, Henes, Joerg, Krusche, Martin, Schmalzing, Marc, Worm, Margitta, Schmeiser, Tim, Guenther, Claudia, Aberer, Elisabeth, Susok, Laura, Riemekasten, Gabriela, Kreuter, Alexander, Zeidler, Gabriele, Juche, Aaron, Hadjiski, Denitsa, Mueller-Ladner, Ulf, Gaebelein-Wissing, Noemi, Distler, Joerg H. W., Sardy, Miklos, Krieg, Thomas, and Hunzelmann, Nicolas
Abstract: Objective. Scleroderma renal crisis (SRC) is a severe life-threatening manifestation in patients with systemic sclerosis (SSc). However, the knowledge about risk factors for SRC is limited. We determined here the frequency of SRC and identified risk factors for the prediction of SRC. Methods. Based on regular followup data from the German Network for Systemic Scleroderma, we used univariate and multivariate generalized estimating equations to analyze the association between clinical variables, SSc subsets, therapy [i.e., angiotensin-converting enzyme inhibitors (ACEi), corticosteroids], and the occurrence of SRC. Results. Data of 2873 patients with 10,425 visits were available for analysis with a mean number of registry visits of 3.6 +/- 2.8 and a mean time of followup of 3.6 +/- 3.8 years. In total, 70 patients developed SRC (70/ 2873, 2.4%). Of these patients, 57.1% (40/70) were diagnosed with diffuse cutaneous SSc, 31.4% (22/70) with limited cutaneous SSc, and 11.4% (8/70) with SSc- overlap syndromes. Predictive independent factors with the highest probability for SRC were positive anti-RNA polymerase antibodies (RNAP), a history of proteinuria prior to SRC onset, diminished DLCO, and a history of hypertension. Interestingly, positive antitopoisomerase autoantibodies did not predict a higher risk for SRC. Further, patients with SRC were significantly more frequently treated with ACEi and corticosteroids without being independently associated with SRC. Conclusion. In this cohort, SRC has become a rare complication. By far the highest risk for SRC was associated with the detection of anti-RNAP and proteinuria.
Published: 2020

27. Whole blood gene expression profiling distinguishes systemic sclerosis-overlap syndromes from other subsets

Author: Moinzadeh, P., Frommolt, P., Franitza, M., Toliat, M. R., Becker, K., Nuernberg, P., Nihtyanova, S. I., Ahrazoglu, M., Belz, D., Hunzelmann, N., Abraham, D., Ong, V. H., Mouthon, L., Hesselstrand, R., Denton, C. P., Krieg, T., Moinzadeh, P., Frommolt, P., Franitza, M., Toliat, M. R., Becker, K., Nuernberg, P., Nihtyanova, S. I., Ahrazoglu, M., Belz, D., Hunzelmann, N., Abraham, D., Ong, V. H., Mouthon, L., Hesselstrand, R., Denton, C. P., and Krieg, T.
Published: 2020

28. Long term outcomes of immunmodulatory drugs in SSc-ILD - data rom the German SSc network

Author: Kreuter, M., Bonella, F., Blank, N., Siegert, E., Henes, J., Worm, M., Sunderkoetter, C., Schmalzing, M., Kreuter, A., Guenther, C., Susok, L., Zeidler, G., Koetter, I, Mueller-Ladner, U., Krieg, T., Juche, A., Schmeiser, T., Riemekasten, G., Aberer, E., Gaebelein-Wissing, N., Distler, J. H. W., Sardy, M., Pfeiffer, C., Kuhr, K., Lorenz, H. M., Moinzadeh, P., Hunzelmann, N., Kreuter, M., Bonella, F., Blank, N., Siegert, E., Henes, J., Worm, M., Sunderkoetter, C., Schmalzing, M., Kreuter, A., Guenther, C., Susok, L., Zeidler, G., Koetter, I, Mueller-Ladner, U., Krieg, T., Juche, A., Schmeiser, T., Riemekasten, G., Aberer, E., Gaebelein-Wissing, N., Distler, J. H. W., Sardy, M., Pfeiffer, C., Kuhr, K., Lorenz, H. M., Moinzadeh, P., and Hunzelmann, N.
Published: 2020

29. Does anti-acid treatment influence disease progression in SSc-ILD? Data form the German SSc-network

Author: Kreuter, M., Bonella, F., Blank, N., Siegert, E., Henes, J., Worm, M., Sunderkoetter, C., Schmalzing, M., Kreuter, A., Guenther, C., Susok, L., Zeidler, G., Koetter, I, Mueller-Ladner, U., Krieg, T., Juche, A., Schmeiser, T., Riemekasten, G., Aberer, E., Gaebelein-Wissing, N., Distler, J. H. W., Sardy, M., Pfeiffer, C., Kuhr, K., Lorenz, H. M., Moinzadeh, P., Hunzelmann, N., Kreuter, M., Bonella, F., Blank, N., Siegert, E., Henes, J., Worm, M., Sunderkoetter, C., Schmalzing, M., Kreuter, A., Guenther, C., Susok, L., Zeidler, G., Koetter, I, Mueller-Ladner, U., Krieg, T., Juche, A., Schmeiser, T., Riemekasten, G., Aberer, E., Gaebelein-Wissing, N., Distler, J. H. W., Sardy, M., Pfeiffer, C., Kuhr, K., Lorenz, H. M., Moinzadeh, P., and Hunzelmann, N.
Published: 2020

30. DOES ANTI-ACID TREATMENT INFLUENCE DISEASE PROGRESSION IN SYSTEMIC SCLEROSIS INTERSTITIAL LUNG DISEASE (SSC-ILD)? DATA FROM THE GERMAN SSC-NETWORK

Author: Kreuter, M., Bonella, F., Riemekasten, G., Mueller-Ladner, U., Henes, J., Siegert, E., Guenther, C., Koetter, I., Blank, N., Pfeiffer, C., Schmalzing, M., Zeidler, G., Korsten, P., Susok, L., Juche, A., Worm, M., Jandova, I., Ehrchen, J., Sunderkoetter, C., Keyszer, G., Ramming, A., Schmeiser, T., Kreuter, A., Kuhr, K., Lorenz, H. M., Moinzadeh, P., Hunzelmann, N., Kreuter, M., Bonella, F., Riemekasten, G., Mueller-Ladner, U., Henes, J., Siegert, E., Guenther, C., Koetter, I., Blank, N., Pfeiffer, C., Schmalzing, M., Zeidler, G., Korsten, P., Susok, L., Juche, A., Worm, M., Jandova, I., Ehrchen, J., Sunderkoetter, C., Keyszer, G., Ramming, A., Schmeiser, T., Kreuter, A., Kuhr, K., Lorenz, H. M., Moinzadeh, P., and Hunzelmann, N.
Published: 2020

31. Large Variability of Frequency and Type of Physical Therapy in Patients in the German Network for Systemic Sclerosis

Author: Belz, D., Moinzadeh, P., Riemekasten, G., Henes, J., Mueller-Ladner, U., Blank, N., Koetter, I, Siegert, E., Pfeiffer, C., Schmalzing, M., Zeidler, G., Schmeiser, T., Worm, M., Guenther, C., Susok, L., Kreuter, A., Sunderkoetter, C., Juche, A., Aberer, E., Gaebelein-Wissing, N., Ramming, A., Kuhr, K., Hunzelmann, N., Belz, D., Moinzadeh, P., Riemekasten, G., Henes, J., Mueller-Ladner, U., Blank, N., Koetter, I, Siegert, E., Pfeiffer, C., Schmalzing, M., Zeidler, G., Schmeiser, T., Worm, M., Guenther, C., Susok, L., Kreuter, A., Sunderkoetter, C., Juche, A., Aberer, E., Gaebelein-Wissing, N., Ramming, A., Kuhr, K., and Hunzelmann, N.
Abstract: Objective To determine the type and frequency of physical therapy (PT) prescribed by physicians for patients in the registry of the German Network for Systemic Sclerosis. Methods The data for 4,252 patients were analyzed using descriptive statistics, chi-square tests, and odds ratios (ORs). Results Overall, 37.4% of patients (1,590 of 4,252) receivedPTat the end of a yearly follow-up. The most frequently used type ofPTwas lymphatic drainage (n = 1,061, 36.8%), followed by exercise therapy (n = 1,047, 36.3%) and heat therapy (n = 689, 23.9%). More than three-fourths of treated patients (82%) received 1 or 2 different forms ofPTsimultaneously. The prescription ofPTwas associated with the extent of skin fibrosis as measured by the modified Rodnan skin thickness score (<10 [41.8% of patients], 11-20 [55.8% of patients], and >21 [63.9% of patients];P< 0.001). Patients with musculoskeletal involvement (e.g., arthritis, muscle weakness, joint contractures, tendon friction rubs) had a higher chance of receivingPTthan patients without these symptoms, with correspondingORs ranging from 1.96 (95% confidence interval [95%CI] 1.69-2.28) for joint contractures to 3.83 (95%CI2.89-5.08) for arthritis. When comparing the type ofPTprescription across the initial and all follow-up visits from 2003 to 2017, significant alterations with a decreasing frequency of patients receivingPTcould be observed (P= 0.001). Conclusion To our knowledge, this is the first study reporting the use ofPTin patients with systemic sclerosis (SSc) in a large cohort. AlthoughSSc is characterized by considerable disability and restriction of motion, PT.
Published: 2020

32. Older age onset of systemic sclerosis - accelerated disease progression in all disease subsets

Author: Moinzadeh, Pia, Kuhr, Kathrin, Siegert, Elise, Mueller-Ladner, Ulf, Riemekasten, Gabriela, Guenther, Claudia, Koetter, Ina, Henes, Joerg, Blank, Norbert, Zeidler, Gabriele, Pfeiffer, Christiane, Juche, Aaron, Jandova, Ilona, Ehrchen, Jan, Schmalzing, Marc, Susok, Laura, Schmeiser, Tim, Sunderkoetter, Cord, Distler, Joerg H. W., Worm, Margitta, Kreuter, Alexander, Krieg, Thomas, Hunzelman, Nicolas, Moinzadeh, Pia, Kuhr, Kathrin, Siegert, Elise, Mueller-Ladner, Ulf, Riemekasten, Gabriela, Guenther, Claudia, Koetter, Ina, Henes, Joerg, Blank, Norbert, Zeidler, Gabriele, Pfeiffer, Christiane, Juche, Aaron, Jandova, Ilona, Ehrchen, Jan, Schmalzing, Marc, Susok, Laura, Schmeiser, Tim, Sunderkoetter, Cord, Distler, Joerg H. W., Worm, Margitta, Kreuter, Alexander, Krieg, Thomas, and Hunzelman, Nicolas
Abstract: Objectives. Systemic sclerosis is a heterogeneous, multisystem disease. It can occur at any age, but most patients develop the disease between the age of 40 to 50 years. There is controversial evidence on whether/how the age at disease onset affects their clinical phenotype. We here investigate the relationship between age at disease onset and symptoms in a large cohort of SSc patients (IcSSc, dcSSc and SSc-overlap syndromes). Methods. Clinical data of the registry of the German Network for Systemic Scleroderma including 3281 patients were evaluated and subdivided into three age groups at disease onset (<40years, 40-60 years, >60 years). Results. Among all SSc patients, 24.5% developed their first non-Raynaud phenomenon symptoms at the age <40years, and 22.5% were older than 60 years of age. In particular, older patients at onset developed the IcSSc subset significantly more often. Furthermore, they had pulmonary hypertension more often, but digital ulcerations less often. Remarkably, the course of the disease was more rapidly progressing in the older cohort (>60 years), except for gastrointestinal and musculoskeletal involvement. No significant difference was found for the use of corticosteroids. However, significantly, fewer patients older than 60 years received immunosuppressive treatment. Conclusion. In this large registry, similar to 25% of patients developed SSc at an age above 60years with an increased frequency of IcSSc. In this age group, an onset of internal organ involvement was significantly accelerated across all three subsets. These findings suggest that, in the elderly cohort, more frequent follow-up examinations are required for an earlier detection of organ complications.
Published: 2020

33. A late onset of systemic sclerosis correlates with a more rapidly progressing clinical phenotype in lcSSc patients - Data of the German Network for Systemic Sclerosis

Author: Moinzadeh, P, Müller-Ladner, U, Siegert, E, Riemekasten, G, Henes, J, Blank, N, Sunderkötter, C, Schmalzing, M, Kötter, I, Schmeiser, T, Zeidler, G, Kreuter, A, Worm, M, Günther, C, Susok, L, Juche, A, Böhm, M, Jandova, I, Pfeiffer, C, Homayoon, D, Kuhr, K, Hunzelmann, N, Moinzadeh, P, Müller-Ladner, U, Siegert, E, Riemekasten, G, Henes, J, Blank, N, Sunderkötter, C, Schmalzing, M, Kötter, I, Schmeiser, T, Zeidler, G, Kreuter, A, Worm, M, Günther, C, Susok, L, Juche, A, Böhm, M, Jandova, I, Pfeiffer, C, Homayoon, D, Kuhr, K, and Hunzelmann, N
Published: 2019

34. A late onset of systemic sclerosis correlates with a more rapidly progressing clinical phenotype in lcSSc patients - Data of the German Network for Systemic Sclerosis

Author: Moinzadeh, P, Müller-Ladner, U, Siegert, E, Riemekasten, G, Henes, J, Blank, N, Sunderkötter, C, Schmalzing, M, Kötter, I, Schmeiser, T, Zeidler, G, Kreuter, A, Worm, M, Günther, C, Susok, L, Juche, A, Böhm, M, Jandova, I, Pfeiffer, C, Homayoon, D, Kuhr, K, Hunzelmann, N, Moinzadeh, P, Müller-Ladner, U, Siegert, E, Riemekasten, G, Henes, J, Blank, N, Sunderkötter, C, Schmalzing, M, Kötter, I, Schmeiser, T, Zeidler, G, Kreuter, A, Worm, M, Günther, C, Susok, L, Juche, A, Böhm, M, Jandova, I, Pfeiffer, C, Homayoon, D, Kuhr, K, and Hunzelmann, N
Published: 2019

35. Idiopathic inflammatory myopathies: state of the art on clinical practice guidelines [corrected].

Author: UCL - (SLuc) Service de rhumatologie, UCL - SSS/IREC/RUMA - Pôle de Pathologies rhumatismales, Meyer, Alain, Scirè, Carlo Alberto, Talarico, Rosaria, Alexander, Tobias, Amoura, Zahir, Avcin, Tadej, Barsotti, Simone, Beretta, Lorenzo, Blagojevic, Jelena, Burmester, Gerd, Cavazzana, Ilaria, Cherrin, Patrick, Damian, Laura, Doria, Andrea, Fonseca, João Eurico, Furini, Federica, Galetti, Ilaria, Houssiau, Frédéric, Krieg, Thomas, Maddalena, Larosa, Launay, David, Campanilho-Marques, Raquel, Martin, Thierry, Matucci-Cerinic, Marco, Moinzadeh, Pia, Montecucco, Carlomaurizio, Moraes-Fontes, Maria Francisca, Mouthon, Luc, Neri, Rossella, Paolino, Sabrina, Piette, Yves, Rednic, Simona, Tamirou, Farah, Tincani, Angela, Toplak, Natasa, Bombardieri, Stefano, Hachulla, Eric, Mueller-Ladner, Ulf, Schneider, Matthias, Smith, Vanessa, Vieira, Ana, Cutolo, Maurizio, Mosca, Marta, Cavagna, Lorenzo, UCL - (SLuc) Service de rhumatologie, UCL - SSS/IREC/RUMA - Pôle de Pathologies rhumatismales, Meyer, Alain, Scirè, Carlo Alberto, Talarico, Rosaria, Alexander, Tobias, Amoura, Zahir, Avcin, Tadej, Barsotti, Simone, Beretta, Lorenzo, Blagojevic, Jelena, Burmester, Gerd, Cavazzana, Ilaria, Cherrin, Patrick, Damian, Laura, Doria, Andrea, Fonseca, João Eurico, Furini, Federica, Galetti, Ilaria, Houssiau, Frédéric, Krieg, Thomas, Maddalena, Larosa, Launay, David, Campanilho-Marques, Raquel, Martin, Thierry, Matucci-Cerinic, Marco, Moinzadeh, Pia, Montecucco, Carlomaurizio, Moraes-Fontes, Maria Francisca, Mouthon, Luc, Neri, Rossella, Paolino, Sabrina, Piette, Yves, Rednic, Simona, Tamirou, Farah, Tincani, Angela, Toplak, Natasa, Bombardieri, Stefano, Hachulla, Eric, Mueller-Ladner, Ulf, Schneider, Matthias, Smith, Vanessa, Vieira, Ana, Cutolo, Maurizio, Mosca, Marta, and Cavagna, Lorenzo
Abstract: Idiopathic inflammatory myopathies (IIMs) encompass a heterogeneous group of rare autoimmune diseases characterised by muscle weakness and inflammation, but in antisynthetase syndrome arthritis and interstitial lung disease are more frequent and often inaugurate the disease. Clinical practice guidelines (CPGs) have been proposed for IIMs, but they are sparse and heterogeneous. This work aimed at identifying: i) current available CPGs for IIMs, ii) patients ' and clinicians' unmet needs not covered by CPGs. It has been performed in the framework of the European Reference Network on rare and complex connective tissue and musculoskeletal diseases (ReCONNET), a network of centre of expertise and patients funded by the European Union's Health Programme. Fourteen original CPGs were identified, notably recommending that: i) extra-muscular involvements should be assessed; ii) corticosteroids and methotrexate or azathioprine are first-line therapies of IIMs. ii) IVIG is a treatment of resistant-DM that may be also used in other resistant-IIMs; iii) physical therapy and sun protection (in DM patients) are part of the treatment; v) tumour screening for patients with DM include imaging of chest, abdomen, pelvis and breast (in woman) along with colonoscopy (in patients over 50 years); vi) disease activity and damages should be monitor using standardised and validated tools. Yet, only half of these CPGs were evidence-based. Crucial unmet needs were identified both by patients and clinicians. In particular, there was a lack of large multidisciplinary working group and of patients ' preferences. The following fields were not or inappropriately targeted: diagnosis; management of extra-muscular involvements other than skin; co-morbidities and severe manifestations.
Published: 2019

36. SSC in older age: Frequent and with a different phenotype. Data of the German Network for Systemic Sclerosis

Author: Moinzadeh, P, Riemekasten, G, Blank, H, Henes, J, Kötter, I, Siegert, E, Pfeiffer, C, Zeidler, G, Schmalzing, M, Günther, C, Susok, L, Worm, M, Kreuter, A, Sunderkötter, C, Müller-Ladner, U, Juche, A, Aberer, E, Schmeiser, T, Krieg, T, Kuhr, K, Hunzelmann, N, Moinzadeh, P, Riemekasten, G, Blank, H, Henes, J, Kötter, I, Siegert, E, Pfeiffer, C, Zeidler, G, Schmalzing, M, Günther, C, Susok, L, Worm, M, Kreuter, A, Sunderkötter, C, Müller-Ladner, U, Juche, A, Aberer, E, Schmeiser, T, Krieg, T, Kuhr, K, and Hunzelmann, N
Published: 2019

37. Proteomic Analysis of Human Scleroderma Fibroblasts Response to Transforming Growth Factor-ss

Author: Chaigne, Benjamin, Clary, Guilhem, Le Gall, Morgane, Dumoitier, Nicolas, Fernandez, Claire, Lofek, Sebastien, Chafey, Philippe, Moinzadeh, Pia, Krieg, Thomas, Denton, Christopher P., Mouthon, Luc, Chaigne, Benjamin, Clary, Guilhem, Le Gall, Morgane, Dumoitier, Nicolas, Fernandez, Claire, Lofek, Sebastien, Chafey, Philippe, Moinzadeh, Pia, Krieg, Thomas, Denton, Christopher P., and Mouthon, Luc
Abstract: Purpose Systemic sclerosis (SSc) is characterized by autoimmunity, vasculopathy and fibrosis. Fibrosis is due to an activation of fibroblasts by the transforming growth factor-ss (TGF-ss). This study investigates the proteomic response of SSc fibroblasts to TGF-ss. Experimental design Skin fibroblasts from diffuse SSc patients and healthy controls (HC) are cultured with or without TGF-ss. Two-dimensional differential in-gel electrophoresis and mass spectrometry (MS) combined with Ingenuity Pathway analysis (IPA) and Panther/David software analyze proteins differentially expressed between groups. Real-time cell analyzer (RTCA) assesses fibroblast proliferation and viability. Results Two-hundred-and-seventy-nine proteins are differentially expressed between groups. Principal component analysis shows significant differences between groups. IPA shows specific process networks such as actin cytoskeleton and integrin signaling. Panther and David software show predominant biological processes such as cellular and metabolic processes. TGF-ss enhances protein synthesis and protein pathways. IPA and RTCA suggest the involvement of epidermal growth factor receptor (EGFR) and phosphatidylinositol 3 kinase (Pi3K). Conclusions and clinical relevance That the proteome of fibroblasts differs between SSc patients and HC is confirmed, and it is demonstrated that fibroblasts exacerbate their proteomic phenotype upon stimulation with TGF-ss. EGFR and Pi3K are highlighted as proteins of interest in SSc fibroblasts.
Published: 2019

38. SSC in older age: Frequent and with a different phenotype. Data of the German Network for Systemic Sclerosis

Author: Moinzadeh, P, Riemekasten, G, Blank, H, Henes, J, Kötter, I, Siegert, E, Pfeiffer, C, Zeidler, G, Schmalzing, M, Günther, C, Susok, L, Worm, M, Kreuter, A, Sunderkötter, C, Müller-Ladner, U, Juche, A, Aberer, E, Schmeiser, T, Krieg, T, Kuhr, K, Hunzelmann, N, Moinzadeh, P, Riemekasten, G, Blank, H, Henes, J, Kötter, I, Siegert, E, Pfeiffer, C, Zeidler, G, Schmalzing, M, Günther, C, Susok, L, Worm, M, Kreuter, A, Sunderkötter, C, Müller-Ladner, U, Juche, A, Aberer, E, Schmeiser, T, Krieg, T, Kuhr, K, and Hunzelmann, N
Published: 2019

39. Significance of pulmonary involvement in systemic sclerosis (SSc) - data from the German SSc-network

Author: Kreuter, M, Bonella, F, Blank, N, Henes, J, Worm, M, Sunderkötter, C, Schmalzing, M, Kreuter, A, Günther, C, Susok, L, Zeidler, G, Kötter, I, Müller-Ladner, U, Krieg, T, Juche, A, Schmeiser, T, Riemekasten, G, Aberer, E, Gäbelein-Wissing, N, Distler, JHW, Sardy, M, Pfeiffer, C, Kuhr, K, Lorenz, HM, Moinzadeh, P, Hunzelmann, N, Kreuter, M, Bonella, F, Blank, N, Henes, J, Worm, M, Sunderkötter, C, Schmalzing, M, Kreuter, A, Günther, C, Susok, L, Zeidler, G, Kötter, I, Müller-Ladner, U, Krieg, T, Juche, A, Schmeiser, T, Riemekasten, G, Aberer, E, Gäbelein-Wissing, N, Distler, JHW, Sardy, M, Pfeiffer, C, Kuhr, K, Lorenz, HM, Moinzadeh, P, and Hunzelmann, N
Published: 2019

40. Proteomic Analysis of Human Scleroderma Fibroblasts Response to Transforming Growth Factor-ss

Author: Chaigne, Benjamin, Clary, Guilhem, Le Gall, Morgane, Dumoitier, Nicolas, Fernandez, Claire, Lofek, Sebastien, Chafey, Philippe, Moinzadeh, Pia, Krieg, Thomas, Denton, Christopher P., Mouthon, Luc, Chaigne, Benjamin, Clary, Guilhem, Le Gall, Morgane, Dumoitier, Nicolas, Fernandez, Claire, Lofek, Sebastien, Chafey, Philippe, Moinzadeh, Pia, Krieg, Thomas, Denton, Christopher P., and Mouthon, Luc
Abstract: Purpose Systemic sclerosis (SSc) is characterized by autoimmunity, vasculopathy and fibrosis. Fibrosis is due to an activation of fibroblasts by the transforming growth factor-ss (TGF-ss). This study investigates the proteomic response of SSc fibroblasts to TGF-ss. Experimental design Skin fibroblasts from diffuse SSc patients and healthy controls (HC) are cultured with or without TGF-ss. Two-dimensional differential in-gel electrophoresis and mass spectrometry (MS) combined with Ingenuity Pathway analysis (IPA) and Panther/David software analyze proteins differentially expressed between groups. Real-time cell analyzer (RTCA) assesses fibroblast proliferation and viability. Results Two-hundred-and-seventy-nine proteins are differentially expressed between groups. Principal component analysis shows significant differences between groups. IPA shows specific process networks such as actin cytoskeleton and integrin signaling. Panther and David software show predominant biological processes such as cellular and metabolic processes. TGF-ss enhances protein synthesis and protein pathways. IPA and RTCA suggest the involvement of epidermal growth factor receptor (EGFR) and phosphatidylinositol 3 kinase (Pi3K). Conclusions and clinical relevance That the proteome of fibroblasts differs between SSc patients and HC is confirmed, and it is demonstrated that fibroblasts exacerbate their proteomic phenotype upon stimulation with TGF-ss. EGFR and Pi3K are highlighted as proteins of interest in SSc fibroblasts.
Published: 2019

41. Disability, fatigue, pain and their associates in early diffuse cutaneous systemic sclerosis:the European Scleroderma Observational Study

Author: Peytrignet, Sébastien, Denton, Christopher P, Lunt, Mark, Hesselstrand, Roger, Mouthon, Luc, Silman, Alan, Pan, Xiaoyan, Brown, Edith, Czirják, László, Distler, Jörg H W, Distler, Oliver, Fligelstone, Kim, Gregory, William J, Ochiel, Rachel, Vonk, Madelon, Ancuta, Codrina, Ong, Voon H, Farge, Dominique, Hudson, Marie, Matucci-Cerinic, Marco, Balbir-Gurman, Alexandra, Midtvedt, Øyvind, Jordan, Alison C, Stevens, Wendy, Moinzadeh, Pia, Hall, Frances C, Agard, Christian, Anderson, Marina E, Diot, Elisabeth, Madhok, Rajan, Akil, Mohammed, Buch, Maya H, Chung, Lorinda, Damjanov, Nemanja, Gunawardena, Harsha, Lanyon, Peter, Ahmad, Yasmeen, Chakravarty, Kuntal, Jacobsen, Søren, MacGregor, Alexander J, McHugh, Neil, Müller-Ladner, Ulf, Riemekasten, Gabriela, Becker, Michael, Roddy, Janet, Carreira, Patricia E, Fauchais, Anne Laure, Hachulla, Eric, Hamilton, Jennifer, Inanç, Murat, McLaren, John S, van Laar, Jacob M, Pathare, Sanjay, Proudman, Susanna, Rudin, Anna, Sahhar, Joanne, Coppere, Brigitte, Serratrice, Christine, Sheeran, Tom, Veale, Douglas J, Grange, Claire, Trad, Georges-Selim, Herrick, Ariane L, Peytrignet, Sébastien, Denton, Christopher P, Lunt, Mark, Hesselstrand, Roger, Mouthon, Luc, Silman, Alan, Pan, Xiaoyan, Brown, Edith, Czirják, László, Distler, Jörg H W, Distler, Oliver, Fligelstone, Kim, Gregory, William J, Ochiel, Rachel, Vonk, Madelon, Ancuta, Codrina, Ong, Voon H, Farge, Dominique, Hudson, Marie, Matucci-Cerinic, Marco, Balbir-Gurman, Alexandra, Midtvedt, Øyvind, Jordan, Alison C, Stevens, Wendy, Moinzadeh, Pia, Hall, Frances C, Agard, Christian, Anderson, Marina E, Diot, Elisabeth, Madhok, Rajan, Akil, Mohammed, Buch, Maya H, Chung, Lorinda, Damjanov, Nemanja, Gunawardena, Harsha, Lanyon, Peter, Ahmad, Yasmeen, Chakravarty, Kuntal, Jacobsen, Søren, MacGregor, Alexander J, McHugh, Neil, Müller-Ladner, Ulf, Riemekasten, Gabriela, Becker, Michael, Roddy, Janet, Carreira, Patricia E, Fauchais, Anne Laure, Hachulla, Eric, Hamilton, Jennifer, Inanç, Murat, McLaren, John S, van Laar, Jacob M, Pathare, Sanjay, Proudman, Susanna, Rudin, Anna, Sahhar, Joanne, Coppere, Brigitte, Serratrice, Christine, Sheeran, Tom, Veale, Douglas J, Grange, Claire, Trad, Georges-Selim, and Herrick, Ariane L
Abstract: Objectives: Our aim was to describe the burden of early dcSSc in terms of disability, fatigue and pain in the European Scleroderma Observational Study cohort, and to explore associated clinical features.Methods: Patients completed questionnaires at study entry, 12 and 24 months, including the HAQ disability index (HAQ-DI), the Cochin Hand Function Scale (CHFS), the Functional Assessment of Chronic Illness Therapy-fatigue and the Short Form 36 (SF36). Associates examined included the modified Rodnan skin score (mRSS), current digital ulcers and internal organ involvement. Correlations between 12-month changes were also examined.Results: The 326 patients recruited (median disease duration 11.9 months) displayed high levels of disability [mean (s.d.) HAQ-DI 1.1 (0.83)], with 'grip' and 'activity' being most affected. Of the 18 activities assessed in the CHFS, those involving fine finger movements were most affected. High HAQ-DI and CHFS scores were both associated with high mRSS (ρ = 0.34, P < 0.0001 and ρ = 0.35, P < 0.0001, respectively). HAQ-DI was higher in patients with digital ulcers (P = 0.004), pulmonary fibrosis (P = 0.005), cardiac (P = 0.005) and muscle involvement (P = 0.002). As anticipated, HAQ-DI, CHFS, the Functional Assessment of Chronic Illness Therapy and SF36 scores were all highly correlated, in particular the HAQ-DI with the CHFS (ρ = 0.84, P < 0.0001). Worsening HAQ-DI over 12 months was strongly associated with increasing mRSS (ρ = 0.40, P < 0.0001), decreasing hand function (ρ = 0.57, P < 0.0001) and increasing fatigue (ρ = -0.53, P < 0.0001).Conclusion: The European Scleroderma Observational Study highlights the burden of disability in early dcSSc, with high levels of disability and fatigue, associating with the degree of skin thickening (mRSS). Impaired hand function is a major contributor to overall disability.
Published: 2018

42. Patterns and predictors of skin score change in early diffuse systemic sclerosis from the European Scleroderma Observational Study

Author: Herrick, A.L., Peytrignet, S., Lunt, M., Pan, X., Hesselstrand, R., Mouthon, L., Silman, A.J., Dinsdale, G., Brown, E., Czirjak, L., Distler, J.H., Distler, O., Fligelstone, K., Gregory, W.J., Ochiel, R., Vonk, M.C., Ancuta, C., Ong, V.H., Farge, D., Hudson, M., Matucci-Cerinic, M., Balbir-Gurman, A., Midtvedt, O., Jobanputra, P., Jordan, A.C., Stevens, W.B.C., Moinzadeh, P., Hall, F.C., Agard, C., Anderson, M.E., Diot, E., Madhok, R., Akil, M., Buch, M.H., Chung, L., Damjanov, N.S., Gunawardena, H., Lanyon, P., Ahmad, Y., Chakravarty, K., Jacobsen, S., MacGregor, A.J., McHugh, N., Muller-Ladner, U., Riemekasten, G., Becker, M., Roddy, J., Carreira, P.E., Fauchais, A.L., Hachulla, E., Hamilton, J., Inanc, M., McLaren, J.S., Laar, J.M. van, Pathare, S., Proudman, S.M., Rudin, A., Sahhar, J., Coppere, B., Serratrice, C., Sheeran, T., Veale, D.J., Grange, C., Trad, G.S., Denton, C.P., Herrick, A.L., Peytrignet, S., Lunt, M., Pan, X., Hesselstrand, R., Mouthon, L., Silman, A.J., Dinsdale, G., Brown, E., Czirjak, L., Distler, J.H., Distler, O., Fligelstone, K., Gregory, W.J., Ochiel, R., Vonk, M.C., Ancuta, C., Ong, V.H., Farge, D., Hudson, M., Matucci-Cerinic, M., Balbir-Gurman, A., Midtvedt, O., Jobanputra, P., Jordan, A.C., Stevens, W.B.C., Moinzadeh, P., Hall, F.C., Agard, C., Anderson, M.E., Diot, E., Madhok, R., Akil, M., Buch, M.H., Chung, L., Damjanov, N.S., Gunawardena, H., Lanyon, P., Ahmad, Y., Chakravarty, K., Jacobsen, S., MacGregor, A.J., McHugh, N., Muller-Ladner, U., Riemekasten, G., Becker, M., Roddy, J., Carreira, P.E., Fauchais, A.L., Hachulla, E., Hamilton, J., Inanc, M., McLaren, J.S., Laar, J.M. van, Pathare, S., Proudman, S.M., Rudin, A., Sahhar, J., Coppere, B., Serratrice, C., Sheeran, T., Veale, D.J., Grange, C., Trad, G.S., and Denton, C.P.
Abstract: Contains fulltext : 191335.pdf (publisher's version ) (Open Access), OBJECTIVES: Our aim was to use the opportunity provided by the European Scleroderma Observational Study to (1) identify and describe those patients with early diffuse cutaneous systemic sclerosis (dcSSc) with progressive skin thickness, and (2) derive prediction models for progression over 12 months, to inform future randomised controlled trials (RCTs). METHODS: The modified Rodnan skin score (mRSS) was recorded every 3 months in 326 patients. 'Progressors' were defined as those experiencing a 5-unit and 25% increase in mRSS score over 12 months (+/-3 months). Logistic models were fitted to predict progression and, using receiver operating characteristic (ROC) curves, were compared on the basis of the area under curve (AUC), accuracy and positive predictive value (PPV). RESULTS: 66 patients (22.5%) progressed, 227 (77.5%) did not (33 could not have their status assessed due to insufficient data). Progressors had shorter disease duration (median 8.1 vs 12.6 months, P=0.001) and lower mRSS (median 19 vs 21 units, P=0.030) than non-progressors. Skin score was highest, and peaked earliest, in the anti-RNA polymerase III (Pol3+) subgroup (n=50). A first predictive model (including mRSS, duration of skin thickening and their interaction) had an accuracy of 60.9%, AUC of 0.666 and PPV of 33.8%. By adding a variable for Pol3 positivity, the model reached an accuracy of 71%, AUC of 0.711 and PPV of 41%. CONCLUSIONS: Two prediction models for progressive skin thickening were derived, for use both in clinical practice and for cohort enrichment in RCTs. These models will inform recruitment into the many clinical trials of dcSSc projected for the coming years. TRIAL REGISTRATION NUMBER: NCT02339441.
Published: 2018

43. Patterns and predictors of skin score change in early diffuse systemic sclerosis from the European Scleroderma Observational Study.

Author: Midtvedt O., Veale D.J., Grange C., Trad G.-S., Denton C.P., Herrick A.L., Peytrignet S., Lunt M., Pan X., Hesselstrand R., Mouthon L., Silman A.J., DInsdale G., Brown E., Czirjak L., DIstler J.H.W., DIstler O., Fligelstone K., Gregory W.J., Ochiel R., Vonk M.C., Ancu C., Ong V.H., Farge D., Hudson M., Matucci-Cerinic M., Balbir-Gurman A., Jobanputra P., Jordan A.C., Stevens W., Moinzadeh P., Hall F.C., Agard C., Anderson M.E., DIot E., Madhok R., Akil M., Buch M.H., Chung L., Damjanov N.S., Gunawardena H., Lanyon P., Ahmad Y., Chakravarty K., Jacobsen S., MacGregor A.J., McHugh N., Muller-Ladner U., Riemekasten G., Becker M., Roddy J., Carreira P.E., Fauchais A.L., Hachulla E., Hamilton J., Inanc M., McLaren J.S., Van Laar J.M., Pathare S., Proudman S.M., Rudin A., Sahhar J., Coppere B., Serratrice C., Sheeran T., Midtvedt O., Veale D.J., Grange C., Trad G.-S., Denton C.P., Herrick A.L., Peytrignet S., Lunt M., Pan X., Hesselstrand R., Mouthon L., Silman A.J., DInsdale G., Brown E., Czirjak L., DIstler J.H.W., DIstler O., Fligelstone K., Gregory W.J., Ochiel R., Vonk M.C., Ancu C., Ong V.H., Farge D., Hudson M., Matucci-Cerinic M., Balbir-Gurman A., Jobanputra P., Jordan A.C., Stevens W., Moinzadeh P., Hall F.C., Agard C., Anderson M.E., DIot E., Madhok R., Akil M., Buch M.H., Chung L., Damjanov N.S., Gunawardena H., Lanyon P., Ahmad Y., Chakravarty K., Jacobsen S., MacGregor A.J., McHugh N., Muller-Ladner U., Riemekasten G., Becker M., Roddy J., Carreira P.E., Fauchais A.L., Hachulla E., Hamilton J., Inanc M., McLaren J.S., Van Laar J.M., Pathare S., Proudman S.M., Rudin A., Sahhar J., Coppere B., Serratrice C., and Sheeran T.
Abstract: Objectives Our aim was to use the opportunity provided by the European Scleroderma Observational Study to (1) identify and describe those patients with early diffuse cutaneous systemic sclerosis (dcSSc) with progressive skin thickness, and (2) derive prediction models for progression over 12 months, to inform future randomised controlled trials (RCTs). Methods The modified Rodnan skin score (mRSS) was recorded every 3 months in 326 patients. 'Progressors' were defined as those experiencing a 5-unit and 25% increase in mRSS score over 12 months (+/-3 months). Logistic models were fitted to predict progression and, using receiver operating characteristic (ROC) curves, were compared on the basis of the area under curve (AUC), accuracy and positive predictive value (PPV). Results 66 patients (22.5%) progressed, 227 (77.5%) did not (33 could not have their status assessed due to insufficient data). Progressors had shorter disease duration (median 8.1 vs 12.6 months, P=0.001) and lower mRSS (median 19 vs 21 units, P=0.030) than non-progressors. Skin score was highest, and peaked earliest, in the anti-RNA polymerase III (Pol3+) subgroup (n=50). A first predictive model (including mRSS, duration of skin thickening and their interaction) had an accuracy of 60.9%, AUC of 0.666 and PPV of 33.8%. By adding a variable for Pol3 positivity, the model reached an accuracy of 71%, AUC of 0.711 and PPV of 41%. Conclusions Two prediction models for progressive skin thickening were derived, for use both in clinical practice and for cohort enrichment in RCTs. These models will inform recruitment into the many clinical trials of dcSSc projected for the coming years. Trial registration number NCT02339441.Copyright © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.
Published: 2018

44. Disability, fatigue, pain and their associates in early diffuse cutaneous systemic sclerosis: the European Scleroderma Observational Study

Author: Peytrignet, Sebastien, Denton, Christopher P., Lunt, Mark, Hesselstrand, Roger, Mouthon, Luc, Silman, Alan, Pan, Xiaoyan, Brown, Edith, Czirjak, Laszlo, Distler, Jorg H. W., Distler, Oliver, Fligelstone, Kim, Gregory, William J., Ochiel, Rachel, Vonk, Madelon, Ancuta, Codrina, Ong, Voon H., Farge, Dominique, Hudson, Marie, Matucci-Cerinic, Marco, Balbir-Gurman, Alexandra, Midtvedt, Oyvind, Jordan, Alison C., Stevens, Wendy, Moinzadeh, Pia, Hall, Frances C., Agard, Christian, Anderson, Marina E., Diot, Elisabeth, Madhok, Rajan, Akil, Mohammed, Buch, Maya H., Chung, Lorinda, Damjanov, Nemanja, Gunawardena, Harsha, Lanyon, Peter, Ahmad, Yasmeen, Chakravarty, Kuntal, Jacobsen, Soren, MacGregor, Alexander J., McHugh, Neil, Muller-Ladner, Ulf, Riemekasten, Gabriela, Becker, Michael, Roddy, Janet, Carreira, Patricia E., Fauchais, Anne Laure, Hachulla, Eric, Hamilton, Jennifer, Inanc, Murat, McLaren, John S., van Laar, Jacob M., Pathare, Sanjay, Proudman, Susanna, Rudin, Anna, Sahhar, Joanne, Coppere, Brigitte, Serratrice, Christine, Sheeran, Tom, Veale, Douglas J., Grange, Claire, Trad, Georges-Selim, Herrick, Ariane L., Peytrignet, Sebastien, Denton, Christopher P., Lunt, Mark, Hesselstrand, Roger, Mouthon, Luc, Silman, Alan, Pan, Xiaoyan, Brown, Edith, Czirjak, Laszlo, Distler, Jorg H. W., Distler, Oliver, Fligelstone, Kim, Gregory, William J., Ochiel, Rachel, Vonk, Madelon, Ancuta, Codrina, Ong, Voon H., Farge, Dominique, Hudson, Marie, Matucci-Cerinic, Marco, Balbir-Gurman, Alexandra, Midtvedt, Oyvind, Jordan, Alison C., Stevens, Wendy, Moinzadeh, Pia, Hall, Frances C., Agard, Christian, Anderson, Marina E., Diot, Elisabeth, Madhok, Rajan, Akil, Mohammed, Buch, Maya H., Chung, Lorinda, Damjanov, Nemanja, Gunawardena, Harsha, Lanyon, Peter, Ahmad, Yasmeen, Chakravarty, Kuntal, Jacobsen, Soren, MacGregor, Alexander J., McHugh, Neil, Muller-Ladner, Ulf, Riemekasten, Gabriela, Becker, Michael, Roddy, Janet, Carreira, Patricia E., Fauchais, Anne Laure, Hachulla, Eric, Hamilton, Jennifer, Inanc, Murat, McLaren, John S., van Laar, Jacob M., Pathare, Sanjay, Proudman, Susanna, Rudin, Anna, Sahhar, Joanne, Coppere, Brigitte, Serratrice, Christine, Sheeran, Tom, Veale, Douglas J., Grange, Claire, Trad, Georges-Selim, and Herrick, Ariane L.
Abstract: Objectives. Our aim was to describe the burden of early dcSSc in terms of disability, fatigue and pain in the European Scleroderma Observational Study cohort, and to explore associated clinical features. Methods. Patients completed questionnaires at study entry, 12 and 24 months, including the HAQ disability index (HAQ-DI), the Cochin Hand Function Scale (CHFS), the Functional Assessment of Chronic Illness Therapy-fatigue and the Short Form 36 (SF36). Associates examined included the modified Rodnan skin score (mRSS), current digital ulcers and internal organ involvement. Correlations between 12-month changes were also examined. Results. The 326 patients recruited (median disease duration 11.9 months) displayed high levels of disability [mean (s.d.) HAQ-DI 1.1 (0.83)], with 'grip' and 'activity' being most affected. Of the 18 activities assessed in the CHFS, those involving fine finger movements were most affected. High HAQ-DI and CHFS scores were both associated with high mRSS (rho = 0.34, P < 0.0001 and rho = 0.35, P < 0.0001, respectively). HAQ-DI was higher in patients with digital ulcers (P = 0.004), pulmonary fibrosis (P = 0.005), cardiac (P = 0.005) and muscle involvement (P = 0.002). As anticipated, HAQ-DI, CHFS, the Functional Assessment of Chronic Illness Therapy and SF36 scores were all highly correlated, in particular the HAQ-DI with the CHFS (rho = 0.84, P < 0.0001). Worsening HAQ-DI over 12 months was strongly associated with increasing mRSS (rho = 0.40, P < 0.0001), decreasing hand function (rho = 0.57, P < 0.0001) and increasing fatigue (rho = -0.53, P < 0.0001). Conclusion.The European Scleroderma Observational Study highlights the burden of disability in early dcSSc, with high levels of disability and fatigue, associating with the degree of skin thickening (mRSS). Impaired hand function is a major contributor to overall disability.
Published: 2018

45. Patterns and predictors of skin score change in early diffuse systemic sclerosis from the European Scleroderma Observational Study

Author: Herrick, Ariane L., Peytrignet, Sebastien, Lunt, Mark, Pan, Xiaoyan, Hesselstrand, Roger, Mouthon, Luc, Silman, Alan J., Dinsdale, Graham, Brown, Edith, Czirjak, Laszlo, Distler, Joerg H. W., Distler, Oliver, Fligelstone, Kim, Gregory, William J., Ochiel, Rachel, Vonk, Madelon C., Ancuta, Codrina, Ong, Voon H., Farge, Dominique, Hudson, Marie, Matucci-Cerinic, Marco, Balbir-Gurman, Alexandra, Midtvedt, Oyvind, Jobanputra, Paresh, Jordan, Alison C., Stevens, Wendy, Moinzadeh, Pia, Hall, Frances C., Agard, Christian, Anderson, Marina E., Diot, Elisabeth, Madhok, Rajan, Akil, Mohammed, Buch, Maya H., Chung, Lorinda, Damjanov, Nemanja S., Gunawardena, Harsha, Lanyon, Peter, Ahmad, Yasmeen, Chakravarty, Kuntal, Jacobsen, Soren, MacGregor, Alexander J., McHugh, Neil, Mueller-Ladner, Ulf, Riemekasten, Gabriela, Becker, Michael, Roddy, Janet, Carreira, Patricia E., Fauchais, Anne Laure, Hachulla, Eric, Hamilton, Jennifer, Inanc, Murat, McLaren, John S., van Laar, Jacob M., Pathare, Sanjay, Proudman, Susanna M., Rudin, Anna, Sahhar, Joanne, Coppere, Brigitte, Serratrice, Christine, Sheeran, Tom, Veale, Douglas J., Grange, Claire, Trad, Georges-Selim, Denton, Christopher P., Herrick, Ariane L., Peytrignet, Sebastien, Lunt, Mark, Pan, Xiaoyan, Hesselstrand, Roger, Mouthon, Luc, Silman, Alan J., Dinsdale, Graham, Brown, Edith, Czirjak, Laszlo, Distler, Joerg H. W., Distler, Oliver, Fligelstone, Kim, Gregory, William J., Ochiel, Rachel, Vonk, Madelon C., Ancuta, Codrina, Ong, Voon H., Farge, Dominique, Hudson, Marie, Matucci-Cerinic, Marco, Balbir-Gurman, Alexandra, Midtvedt, Oyvind, Jobanputra, Paresh, Jordan, Alison C., Stevens, Wendy, Moinzadeh, Pia, Hall, Frances C., Agard, Christian, Anderson, Marina E., Diot, Elisabeth, Madhok, Rajan, Akil, Mohammed, Buch, Maya H., Chung, Lorinda, Damjanov, Nemanja S., Gunawardena, Harsha, Lanyon, Peter, Ahmad, Yasmeen, Chakravarty, Kuntal, Jacobsen, Soren, MacGregor, Alexander J., McHugh, Neil, Mueller-Ladner, Ulf, Riemekasten, Gabriela, Becker, Michael, Roddy, Janet, Carreira, Patricia E., Fauchais, Anne Laure, Hachulla, Eric, Hamilton, Jennifer, Inanc, Murat, McLaren, John S., van Laar, Jacob M., Pathare, Sanjay, Proudman, Susanna M., Rudin, Anna, Sahhar, Joanne, Coppere, Brigitte, Serratrice, Christine, Sheeran, Tom, Veale, Douglas J., Grange, Claire, Trad, Georges-Selim, and Denton, Christopher P.
Abstract: Objectives Our aim was to use the opportunity provided by the European Scleroderma Observational Study to (1) identify and describe those patients with early diffuse cutaneous systemic sclerosis (dcSSc) with progressive skin thickness, and (2) derive prediction models for progression over 12 months, to inform future randomised controlled trials (RCTs). Methods The modified Rodnan skin score (mRSS) was recorded every 3months in 326 patients. Progressors' were defined as those experiencing a 5-unit and 25% increase in mRSS score over 12 months (3 months). Logistic models were fitted to predict progression and, using receiver operating characteristic (ROC) curves, were compared on the basis of the area under curve (AUC), accuracy and positive predictive value (PPV). Results 66 patients (22.5%) progressed, 227 (77.5%) did not (33 could not have their status assessed due to insufficient data). Progressors had shorter disease duration (median 8.1 vs 12.6 months, P=0.001) and lower mRSS (median 19 vs 21 units, P=0.030) than non-progressors. Skin score was highest, and peaked earliest, in the anti-RNA polymerase III (Pol3+) subgroup (n=50). A first predictive model (including mRSS, duration of skin thickening and their interaction) had an accuracy of 60.9%, AUC of 0.666 and PPV of 33.8%. By adding a variable for Pol3 positivity, the model reached an accuracy of 71%, AUC of 0.711 and PPV of 41%. Conclusions Two prediction models for progressive skin thickening were derived, for use both in clinical practice and for cohort enrichment in RCTs. These models will inform recruitment into the many clinical trials of dcSSc projected for the coming years. Trial registration number NCT02339441.
Published: 2018

46. Localized Scleroderma of the Head and Face Area: A Retrospective Cross- sectional Study of 96 Patients from 5 German Tertiary Referral Centres

Author: Kreuter, Alexander, Mitrakos, Georg, Hofmann, Silke C., Lehmann, Percy, Sticherling, Michael, Krieg, Thomas, Lahner, Nina, Tigges, Christian, Hunzelmann, Nicolas, Moinzadeh, Pia, Kreuter, Alexander, Mitrakos, Georg, Hofmann, Silke C., Lehmann, Percy, Sticherling, Michael, Krieg, Thomas, Lahner, Nina, Tigges, Christian, Hunzelmann, Nicolas, and Moinzadeh, Pia
Published: 2018

47. SSC IN OLDER AGE: FREQUENT AND WITH A DIFFERENT PHENOTYPE. DATA OF THE GERMAN NETWORK FOR SYSTEMIC SCLEROSIS

Author: Moinzadeh, P., Riemekasten, G., Blank, N., Henes, J., Koetter, I., Siegert, E., Pfeiffer, C., Zeidler, G., Schmalzing, M., Guenther, C., Susok, L., Worm, M., Kreuter, A., Sunderkoetter, C., Mueller-Ladner, U., Juche, A., Aberer, E., Schmeiser, T., Krieg, T., Kuhr, K., Hunzelmann, N., Moinzadeh, P., Riemekasten, G., Blank, N., Henes, J., Koetter, I., Siegert, E., Pfeiffer, C., Zeidler, G., Schmalzing, M., Guenther, C., Susok, L., Worm, M., Kreuter, A., Sunderkoetter, C., Mueller-Ladner, U., Juche, A., Aberer, E., Schmeiser, T., Krieg, T., Kuhr, K., and Hunzelmann, N.
Published: 2018

48. Significance of pulmonary involvement in systemic sclerosis (SSc)- data from the German SSc-network

Author: Kreuter, Michael, Bonella, Francesco, Blank, Nobert, Siegert, Elise, Henes, Joerg, Worm, Margitta, Sunderkoetter, Cord, Schmalzing, Marc, Kreuter, Alexander, Guenther, Claudia, Susok, Laura, Zeidler, Gabriele, Koetter, Ina, Mueller-Ladner, Ulf, Krieg, Thomas, Juche, Aaron, Schmeiser, Tim, Riemekasten, Gabriele, Aberer, Elisabeth, Gaebelein-Wissing, Noemi, Distler, Joerg H. W., Sardy, Miklos, Pfeiffer, Christiane, Kuhr, Kathrin, Lorenz, Hanns-Martin, Moinzadeh, Pia, Hunzelmann, Nicolas, Kreuter, Michael, Bonella, Francesco, Blank, Nobert, Siegert, Elise, Henes, Joerg, Worm, Margitta, Sunderkoetter, Cord, Schmalzing, Marc, Kreuter, Alexander, Guenther, Claudia, Susok, Laura, Zeidler, Gabriele, Koetter, Ina, Mueller-Ladner, Ulf, Krieg, Thomas, Juche, Aaron, Schmeiser, Tim, Riemekasten, Gabriele, Aberer, Elisabeth, Gaebelein-Wissing, Noemi, Distler, Joerg H. W., Sardy, Miklos, Pfeiffer, Christiane, Kuhr, Kathrin, Lorenz, Hanns-Martin, Moinzadeh, Pia, and Hunzelmann, Nicolas
Published: 2018

49. Mixed connective tissue disease: state of the art on clinical practice guidelines

Author: Benjamin, Chaigne, Alberto, Scire Carlo, Rosaria, Talarico, Tobias, Alexander, Zahir, Amoura, Tadej, Avcin, Lorenzo, Beretta, Andrea, Doria, Aurelien, Guffroy, Vera, Guimaraes, Eric, Hachulla, Thomas, Krieg, David, Launay, Gemma, Lepri, Pia, Moinzadeh, Ulf, Mueller-Ladner, Simona, Rednic, Ana, Rodrigues, Sander, Tas W., Van Vollenhoven, R. F., Ana, Vieira, Stefano, Bombardieri, Eurico, Fonseca Joao, Ilaria, Galetti, Matthias, Schneider, Vanessa, Smith, Maurizio, Cutolo, Marta, Mosca, Rebecca, Fischer-Betz, Benjamin, Chaigne, Alberto, Scire Carlo, Rosaria, Talarico, Tobias, Alexander, Zahir, Amoura, Tadej, Avcin, Lorenzo, Beretta, Andrea, Doria, Aurelien, Guffroy, Vera, Guimaraes, Eric, Hachulla, Thomas, Krieg, David, Launay, Gemma, Lepri, Pia, Moinzadeh, Ulf, Mueller-Ladner, Simona, Rednic, Ana, Rodrigues, Sander, Tas W., Van Vollenhoven, R. F., Ana, Vieira, Stefano, Bombardieri, Eurico, Fonseca Joao, Ilaria, Galetti, Matthias, Schneider, Vanessa, Smith, Maurizio, Cutolo, Marta, Mosca, and Rebecca, Fischer-Betz
Abstract: Mixed connective tissue disease (MCTD) is a complex overlap disease with features of different autoimmune connective tissue diseases (CTDs) namely systemic sclerosis, poly/dermatomyositis and systemic lupus erythematous in patients with antibodies targeting the U1 small nuclear ribonucleoprotein particle. In this narrative review, we summarise the results of a systematic literature research which was performed as part of the European Reference Network on Rare and Complex Connective Tissue and Musculoskeletal Diseases project, aimed at evaluating existing clinical practice guidelines (CPGs) or recommendations. Since no specific CPGs on MCTD were found, other CPGs developed for other CTDs were taken into consideration in order to discuss what can be applied to MCTD even if designed for other diseases. Three major objectives were proposed for the future development of CPGs: MCTD diagnosis (diagnostic criteria), MCTD initial and follow-up evaluations, MCTD treatment. Early diagnosis, epidemiological data, assessment of burden of disease and QOL aspects are among the unmet needs identified by patients.
Published: 2018

50. Idiopathic inflammatory myopathies: state of the art on clinical practice guidelines

Author: Meyer, Alain, Scire, Carlo Alberto, Talarico, Rosaria, Alexander, Tobias, Amoura, Zahir, Avcin, Tadej, Barsotti, Simone, Beretta, Lorenzo, Blagojevic, Jelena, Burmester, Gerd, Cavazzana, Ilaria, Cherrin, Patrick, Damian, Laura, Doria, Andrea, Fonseca, Joao Eurico, Furini, Federica, Galetti, Ilaria, Houssiau, Frederic, Krieg, Thomas, Larosa, Maddalena, Launay, David, Campanilho-Marques, Raquel, Martin, Thierry, Matucci-Cerinic, Marco, Moinzadeh, Pia, Montecucco, Carlomaurizio, Moraes-Fontes, Maria Francisca, Mouthon, Luc, Neri, Rossella, Paolino, Sabrina, Piette, Yves, Rednic, Simona, Tamirou, Farah, Tincani, Angela, Toplak, Natasa, Bombardieri, Stefano, Hachulla, Eric, Mueller-Ladner, Ulf, Schneider, Matthias, Smith, Vanessa, Vieira, Ana, Cutolo, Maurizio, Mosca, Marta, Cavagna, Lorenzo, Meyer, Alain, Scire, Carlo Alberto, Talarico, Rosaria, Alexander, Tobias, Amoura, Zahir, Avcin, Tadej, Barsotti, Simone, Beretta, Lorenzo, Blagojevic, Jelena, Burmester, Gerd, Cavazzana, Ilaria, Cherrin, Patrick, Damian, Laura, Doria, Andrea, Fonseca, Joao Eurico, Furini, Federica, Galetti, Ilaria, Houssiau, Frederic, Krieg, Thomas, Larosa, Maddalena, Launay, David, Campanilho-Marques, Raquel, Martin, Thierry, Matucci-Cerinic, Marco, Moinzadeh, Pia, Montecucco, Carlomaurizio, Moraes-Fontes, Maria Francisca, Mouthon, Luc, Neri, Rossella, Paolino, Sabrina, Piette, Yves, Rednic, Simona, Tamirou, Farah, Tincani, Angela, Toplak, Natasa, Bombardieri, Stefano, Hachulla, Eric, Mueller-Ladner, Ulf, Schneider, Matthias, Smith, Vanessa, Vieira, Ana, Cutolo, Maurizio, Mosca, Marta, and Cavagna, Lorenzo
Abstract: Idiopathic inflammatory myopathies (IIMs) encompass a heterogeneous group of rare autoimmune diseases characterised by muscle weakness and inflammation, but in antisynthetase syndrome arthritis and interstitial lung disease are more frequent and often inaugurate the disease. Clinical practice guidelines (CPGs) have been proposed for IIMs, but they are sparse and heterogeneous. This work aimed at identifying: i) current available CPGs for IIMs, ii) patients' and clinicians' unmet needs not covered by CPGs. It has been performed in the framework of the European Reference Network on rare and complex connective tissue and musculoskeletal diseases (ReCONNET), a network of centre of expertise and patients funded by the European Union's Health Programme. Fourteen original CPGs were identified, notably recommending that: i) extra-muscular involvements should be assessed; ii) corticosteroids and methotrexate or azathioprine are first-line therapies of IIMs. iii) IVIG is a treatment of resistant-DM that may be also used in other resistant-IIMs; iv) physical therapy and sun protection (in DM patients) are part of the treatment; v) tumour screening for patients with DM include imaging of chest, abdomen, pelvis and breast (in woman) along with colonoscopy (in patients over 50 years); vi) disease activity and damages should be monitor using standardised and validated tools. Yet, only half of these CPGs were evidence-based. Crucial unmet needs were identified both by patients and clinicians. In particular, there was a lack of large multidisciplinary working group and of patients' preferences. The following fields were not or inappropriately targeted: diagnosis; management of extra-muscular involvements other than skin; co-morbidities and severe manifestations.
Published: 2018

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