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51 results on '"Bonella F"'

1. Defining anti-synthetase syndrome: a systematic literature review

Author: Zanframundo, G, Faghihi-Kashani, S, Scire, C, Bonella, F, Corte, T, Doyle, T, Fiorentino, D, Gonzalez-Gay, M, Hudson, M, Kuwana, M, Lundberg, I, Mammen, A, Mchugh, N, Miller, F, Monteccucco, C, Oddis, C, Rojas-Serrano, J, Schmidt, J, Selva-O'Callaghan, A, Werth, V, Sakellariou, G, Aggarwal, R, Cavagna, L, Zanframundo G., Faghihi-Kashani S., Scire C. A., Bonella F., Corte T. J., Doyle T. J., Fiorentino D., Gonzalez-Gay M. A., Hudson M., Kuwana M., Lundberg I. E., Mammen A., McHugh N., Miller F. W., Monteccucco C., Oddis C. V., Rojas-Serrano J., Schmidt J., Selva-O'Callaghan A., Werth V. P., Sakellariou G., Aggarwal R., Cavagna L., Zanframundo, G, Faghihi-Kashani, S, Scire, C, Bonella, F, Corte, T, Doyle, T, Fiorentino, D, Gonzalez-Gay, M, Hudson, M, Kuwana, M, Lundberg, I, Mammen, A, Mchugh, N, Miller, F, Monteccucco, C, Oddis, C, Rojas-Serrano, J, Schmidt, J, Selva-O'Callaghan, A, Werth, V, Sakellariou, G, Aggarwal, R, Cavagna, L, Zanframundo G., Faghihi-Kashani S., Scire C. A., Bonella F., Corte T. J., Doyle T. J., Fiorentino D., Gonzalez-Gay M. A., Hudson M., Kuwana M., Lundberg I. E., Mammen A., McHugh N., Miller F. W., Monteccucco C., Oddis C. V., Rojas-Serrano J., Schmidt J., Selva-O'Callaghan A., Werth V. P., Sakellariou G., Aggarwal R., and Cavagna L.
Abstract: Anti-synthetase syndrome (ASSD) is a heterogeneous autoimmune disease characterised by multi-system involvement with a wide variety of manifestations. Validated classification criteria are necessary to improve recognition and prevent misclassification, especially given the lack of reliable and standardised autoantibody testing. We systematically reviewed the literature to analyse proposed ASSD criteria, characteristics, and diagnostic performance. Methods We searched PubMed and Embase databases (01/01/1984 to 06/11/2018) and the ACR and EULAR meeting abstracts (2017-2018). Sensitivities, specificities, positive, negative likelihood ratios and risk of bias were calculated for ASSD criteria and key variables reported in the literature. We performed meta-analysis when appropriate. Results We retrieved 4,358 studies. We found 85 proposed ASSD criteria from a total of 82 studies. All but one study included anti-synthetase autoantibody (ARS) positivity in the ASSD criteria. Most studies required only one ASSD feature plus anti-ARS to define ASSD (n=64, 78%), whereas 16 studies required more than one ASSD variable plus anti-ARS. The only criteria not including anti-ARS positivity required 5 ASSD clinical features. We found limited data and wide variability in the diagnostic performance of each variable and definition proposed in the literature. Given these limitations we only meta-analysed the performance of individual muscle biopsy and clinical variables in diagnosing ASSD, which performed poorly. Conclusion The current ASSD criteria include a variety of serological, clinical, and histological features with wide variability amongst proposed definitions and the performance of these definitions has not been tested. This systematic literature review suggests the need for additional data and consensus-driven classification criteria for ASSD.
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. Continued treatment with Nintedanib in patients with progressive fibrosing autoimmune disease-related interstitial lung diseases: Data from INBUILD-ON

Author: Müller-Ladner, U, Matteson, EL, Antin-Ozerkis, D, Bonella, F, Chaudhuri, N, Cottin, V, Mueller, H, Coeck, C, Rohr, KB, Wuyts, WA, Müller-Ladner, U, Matteson, EL, Antin-Ozerkis, D, Bonella, F, Chaudhuri, N, Cottin, V, Mueller, H, Coeck, C, Rohr, KB, and Wuyts, WA
Published: 2022

5. Continued treatment with Nintedanib in patients with progressive fibrosing autoimmune disease-related interstitial lung diseases: Data from INBUILD-ON

Author: Müller-Ladner, U, Matteson, EL, Antin-Ozerkis, D, Bonella, F, Chaudhuri, N, Cottin, V, Mueller, H, Coeck, C, Rohr, KB, Wuyts, WA, Müller-Ladner, U, Matteson, EL, Antin-Ozerkis, D, Bonella, F, Chaudhuri, N, Cottin, V, Mueller, H, Coeck, C, Rohr, KB, and Wuyts, WA
Published: 2022

6. Communicating with patients with idiopathic pulmonary fibrosis: can we do it better?

Author: Wijsenbeek, M. S., Bonella, F., Orsatti, L., Russell, A. M., Valenzuela, C., Wuyts, W. A., Baile, W. F., Wijsenbeek, M. S., Bonella, F., Orsatti, L., Russell, A. M., Valenzuela, C., Wuyts, W. A., and Baile, W. F.
Abstract: Communications between clinicians and patients with idiopathic pulmonary fibrosis (IPF) have the potential to be challenging. The variable course and poor prognosis of IPF complicate discussions around life expectancy but should not prevent clinicians from having meaningful conversations about patients’ fears and needs, while acknowledging uncertainties. Patients want information about the course of their disease and management options, but the provision of information needs to be individualised to the needs and preferences of the patient. Communication from clinicians should be empathetic and take account of the patient’s perceptions and concerns. Models, tools and protocols are available that can help clinicians to improve their interactions with patients. In this article, we consider the difficulties inherent in discussions with patients with IPF and their loved ones, and how clinicians might communicate with patients more effectively, from breaking the news about the diagnosis to providing support throughout the course of the disease.
Published: 2022

7. Nailfold capillaroscopy characteristics of antisynthetase syndrome and possible clinical associations: Results of a multicenter international study

Author: Sebastiani, M, Triantafyllias, K, Manfredi, A, Gonzalez-Gay, M, Palmou-Fontana, N, Cassone, G, Drott, U, Delbruck, C, Rojas-Serrano, J, Bertolazzi, C, Nuno, L, Giannini, M, Iannone, F, Vicente, E, Castaneda, S, Selva-O'Callaghan, A, Araguas, E, Emmi, G, Iuliano, A, Bauhammer, J, Miehle, N, Parisi, S, Cavagna, L, Codullo, V, Montecucco, C, Lopez-Longo, F, Martinez-Barrio, J, Nieto-Gonzalez, J, Vichi, S, Confalonieri, M, Tomietto, P, Bergner, R, Sulli, A, Bonella, F, Furini, F, Scire, C, Bortoluzzi, A, Specker, C, Barsotti, S, Neri, R, Mosca, M, Caproni, M, Weinmann-Menke, J, Schwarting, A, Smith, V, Cutolo, M, Sebastiani M., Triantafyllias K., Manfredi A., Gonzalez-Gay M. A., Palmou-Fontana N., Cassone G., Drott U., Delbruck C., Rojas-Serrano J., Bertolazzi C., Nuno L., Giannini M., Iannone F., Vicente E. F., Castaneda S., Selva-O'Callaghan A., Araguas E. T., Emmi G., Iuliano A., Bauhammer J., Miehle N., Parisi S., Cavagna L., Codullo V., Montecucco C., Lopez-Longo F. J., Martinez-Barrio J., Nieto-Gonzalez J. C., Vichi S., Confalonieri M., Tomietto P., Bergner R., Sulli A., Bonella F., Furini F., Scire C. A., Bortoluzzi A., Specker C., Barsotti S., Neri R., Mosca M., Caproni M., Weinmann-Menke J., Schwarting A., Smith V., Cutolo M., Sebastiani, M, Triantafyllias, K, Manfredi, A, Gonzalez-Gay, M, Palmou-Fontana, N, Cassone, G, Drott, U, Delbruck, C, Rojas-Serrano, J, Bertolazzi, C, Nuno, L, Giannini, M, Iannone, F, Vicente, E, Castaneda, S, Selva-O'Callaghan, A, Araguas, E, Emmi, G, Iuliano, A, Bauhammer, J, Miehle, N, Parisi, S, Cavagna, L, Codullo, V, Montecucco, C, Lopez-Longo, F, Martinez-Barrio, J, Nieto-Gonzalez, J, Vichi, S, Confalonieri, M, Tomietto, P, Bergner, R, Sulli, A, Bonella, F, Furini, F, Scire, C, Bortoluzzi, A, Specker, C, Barsotti, S, Neri, R, Mosca, M, Caproni, M, Weinmann-Menke, J, Schwarting, A, Smith, V, Cutolo, M, Sebastiani M., Triantafyllias K., Manfredi A., Gonzalez-Gay M. A., Palmou-Fontana N., Cassone G., Drott U., Delbruck C., Rojas-Serrano J., Bertolazzi C., Nuno L., Giannini M., Iannone F., Vicente E. F., Castaneda S., Selva-O'Callaghan A., Araguas E. T., Emmi G., Iuliano A., Bauhammer J., Miehle N., Parisi S., Cavagna L., Codullo V., Montecucco C., Lopez-Longo F. J., Martinez-Barrio J., Nieto-Gonzalez J. C., Vichi S., Confalonieri M., Tomietto P., Bergner R., Sulli A., Bonella F., Furini F., Scire C. A., Bortoluzzi A., Specker C., Barsotti S., Neri R., Mosca M., Caproni M., Weinmann-Menke J., Schwarting A., Smith V., and Cutolo M.
Abstract: Objective. To describe nailfold videocapillaroscopy (NVC) features of patients with antisynthetase syndrome (AS) and to investigate possible correlations with clinical and serological features of the disease. Methods. We retrospectively analyzed NVC images of 190 patients with AS [females/males 3.63, mean age 49.7 ± 12.8 yrs, median disease duration 53.7 mos (interquartile range 82), 133 anti-Jo1 and 57 non-anti-Jo1-positive patients]. For each patient, we examined number of capillaries, giant capillaries, microhemorrhages, avascular areas, ramified capillaries, and the presence of systemic sclerosis (SSc)-like pattern. Finally, we correlated NVC features with clinical and serological findings of patients with AS. Concomitantly, a historical cohort of 75 patients with antinuclear antibody-negative primary Raynaud phenomenon (RP) and longterm followup was used as a control group (female/male ratio 4.13/1, mean age 53.9 ± 17.6 yrs) for NVC measures. Results. NVC abnormalities were observed in 62.1% of AS patients compared with 29.3% of primary RP group (p < 0.001). An SSc-like pattern was detected in 67 patients (35.3%) and it was associated with anti-Jo1 antibodies (p = 0.002) and also with a longer disease duration (p = 0.004). Interestingly, there was no significant correlation between the presence of SSc-like pattern and RP, and only 47% of patients with SSc-like pattern had RP. Conclusion. NVC abnormalities are commonly observed in AS, independently from the occurrence of RP. The presence of an SSc-like pattern could allow identification of a more defined AS subtype, and prospective studies could confirm the association with clinical and serological features of AS.
Published: 2019

8. Nailfold capillaroscopy characteristics of antisynthetase syndrome and possible clinical associations: Results of a multicenter international study

Author: Sebastiani, M, Triantafyllias, K, Manfredi, A, Gonzalez-Gay, M, Palmou-Fontana, N, Cassone, G, Drott, U, Delbruck, C, Rojas-Serrano, J, Bertolazzi, C, Nuno, L, Giannini, M, Iannone, F, Vicente, E, Castaneda, S, Selva-O'Callaghan, A, Araguas, E, Emmi, G, Iuliano, A, Bauhammer, J, Miehle, N, Parisi, S, Cavagna, L, Codullo, V, Montecucco, C, Lopez-Longo, F, Martinez-Barrio, J, Nieto-Gonzalez, J, Vichi, S, Confalonieri, M, Tomietto, P, Bergner, R, Sulli, A, Bonella, F, Furini, F, Scire, C, Bortoluzzi, A, Specker, C, Barsotti, S, Neri, R, Mosca, M, Caproni, M, Weinmann-Menke, J, Schwarting, A, Smith, V, Cutolo, M, Sebastiani M., Triantafyllias K., Manfredi A., Gonzalez-Gay M. A., Palmou-Fontana N., Cassone G., Drott U., Delbruck C., Rojas-Serrano J., Bertolazzi C., Nuno L., Giannini M., Iannone F., Vicente E. F., Castaneda S., Selva-O'Callaghan A., Araguas E. T., Emmi G., Iuliano A., Bauhammer J., Miehle N., Parisi S., Cavagna L., Codullo V., Montecucco C., Lopez-Longo F. J., Martinez-Barrio J., Nieto-Gonzalez J. C., Vichi S., Confalonieri M., Tomietto P., Bergner R., Sulli A., Bonella F., Furini F., Scire C. A., Bortoluzzi A., Specker C., Barsotti S., Neri R., Mosca M., Caproni M., Weinmann-Menke J., Schwarting A., Smith V., Cutolo M., Sebastiani, M, Triantafyllias, K, Manfredi, A, Gonzalez-Gay, M, Palmou-Fontana, N, Cassone, G, Drott, U, Delbruck, C, Rojas-Serrano, J, Bertolazzi, C, Nuno, L, Giannini, M, Iannone, F, Vicente, E, Castaneda, S, Selva-O'Callaghan, A, Araguas, E, Emmi, G, Iuliano, A, Bauhammer, J, Miehle, N, Parisi, S, Cavagna, L, Codullo, V, Montecucco, C, Lopez-Longo, F, Martinez-Barrio, J, Nieto-Gonzalez, J, Vichi, S, Confalonieri, M, Tomietto, P, Bergner, R, Sulli, A, Bonella, F, Furini, F, Scire, C, Bortoluzzi, A, Specker, C, Barsotti, S, Neri, R, Mosca, M, Caproni, M, Weinmann-Menke, J, Schwarting, A, Smith, V, Cutolo, M, Sebastiani M., Triantafyllias K., Manfredi A., Gonzalez-Gay M. A., Palmou-Fontana N., Cassone G., Drott U., Delbruck C., Rojas-Serrano J., Bertolazzi C., Nuno L., Giannini M., Iannone F., Vicente E. F., Castaneda S., Selva-O'Callaghan A., Araguas E. T., Emmi G., Iuliano A., Bauhammer J., Miehle N., Parisi S., Cavagna L., Codullo V., Montecucco C., Lopez-Longo F. J., Martinez-Barrio J., Nieto-Gonzalez J. C., Vichi S., Confalonieri M., Tomietto P., Bergner R., Sulli A., Bonella F., Furini F., Scire C. A., Bortoluzzi A., Specker C., Barsotti S., Neri R., Mosca M., Caproni M., Weinmann-Menke J., Schwarting A., Smith V., and Cutolo M.
Abstract: Objective. To describe nailfold videocapillaroscopy (NVC) features of patients with antisynthetase syndrome (AS) and to investigate possible correlations with clinical and serological features of the disease. Methods. We retrospectively analyzed NVC images of 190 patients with AS [females/males 3.63, mean age 49.7 ± 12.8 yrs, median disease duration 53.7 mos (interquartile range 82), 133 anti-Jo1 and 57 non-anti-Jo1-positive patients]. For each patient, we examined number of capillaries, giant capillaries, microhemorrhages, avascular areas, ramified capillaries, and the presence of systemic sclerosis (SSc)-like pattern. Finally, we correlated NVC features with clinical and serological findings of patients with AS. Concomitantly, a historical cohort of 75 patients with antinuclear antibody-negative primary Raynaud phenomenon (RP) and longterm followup was used as a control group (female/male ratio 4.13/1, mean age 53.9 ± 17.6 yrs) for NVC measures. Results. NVC abnormalities were observed in 62.1% of AS patients compared with 29.3% of primary RP group (p < 0.001). An SSc-like pattern was detected in 67 patients (35.3%) and it was associated with anti-Jo1 antibodies (p = 0.002) and also with a longer disease duration (p = 0.004). Interestingly, there was no significant correlation between the presence of SSc-like pattern and RP, and only 47% of patients with SSc-like pattern had RP. Conclusion. NVC abnormalities are commonly observed in AS, independently from the occurrence of RP. The presence of an SSc-like pattern could allow identification of a more defined AS subtype, and prospective studies could confirm the association with clinical and serological features of AS.
Published: 2019

9. 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

10. 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

11. Patient-reported outcomes and patient-reported outcome measures in interstitial lung disease: where to go from here?

Author: Kalluri, M, Luppi, F, Vancheri, A, Vancheri, C, Balestro, E, Varone, F, Mogulkoc, N, Cacopardo, G, Bargagli, E, Renzoni, E, Torrisi, S, Calvello, M, Libra, A, Pavone, M, Bonella, F, Cottin, V, Valenzuela, C, Wijsenbeek, M, Bendstrup, E, Kalluri, Meena, Luppi, Fabrizio, Vancheri, Ada, Vancheri, Carlo, Balestro, Elisabetta, Varone, Francesco, Mogulkoc, Nesrin, Cacopardo, Giulia, Bargagli, Elena, Renzoni, Elizabeth, Torrisi, Sebastiano, Calvello, Mariarosaria, Libra, Alessandro, Pavone, Mauro, Bonella, Francesco, Cottin, Vincent, Valenzuela, Claudia, Wijsenbeek, Marlies, Bendstrup, Elisabeth, Kalluri, M, Luppi, F, Vancheri, A, Vancheri, C, Balestro, E, Varone, F, Mogulkoc, N, Cacopardo, G, Bargagli, E, Renzoni, E, Torrisi, S, Calvello, M, Libra, A, Pavone, M, Bonella, F, Cottin, V, Valenzuela, C, Wijsenbeek, M, Bendstrup, E, Kalluri, Meena, Luppi, Fabrizio, Vancheri, Ada, Vancheri, Carlo, Balestro, Elisabetta, Varone, Francesco, Mogulkoc, Nesrin, Cacopardo, Giulia, Bargagli, Elena, Renzoni, Elizabeth, Torrisi, Sebastiano, Calvello, Mariarosaria, Libra, Alessandro, Pavone, Mauro, Bonella, Francesco, Cottin, Vincent, Valenzuela, Claudia, Wijsenbeek, Marlies, and Bendstrup, Elisabeth
Abstract: Patient-reported outcome measures (PROMs), tools to assess patient self-report of health status, are now increasingly used in research, care and policymaking. While there are two well-developed disease-specific PROMs for interstitial lung diseases (ILD) and idiopathic pulmonary fibrosis (IPF), many unmet and urgent needs remain. In December 2019, 64 international ILD experts convened in Erice, Italy to deliberate on many topics, including PROMs in ILD. This review summarises the history of PROMs in ILD, shortcomings of the existing tools, challenges of development, validation and implementation of their use in clinical trials, and the discussion held during the meeting. Development of disease-specific PROMs for ILD including IPF with robust methodology and validation in concordance with guidance from regulatory authorities have increased user confidence in PROMs. Minimal clinically important difference for bidirectional changes may need to be developed. Cross-cultural validation and linguistic adaptations are necessary in addition to robust psychometric properties for effective PROM use in multinational clinical trials. PROM burden of use should be reduced through appropriate use of digital technologies and computerised adaptive testing. Active patient engagement in all stages from development, testing, choosing and implementation of PROMs can help improve probability of success and further growth.
Published: 2021

12. 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

13. 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

14. 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

15. Treatment of systemic sclerosis-associated interstitial lung disease

Author: Prasse, A., Bonella, F., Mueller-Ladner, U., Witte, T., Hunzelmann, N., Distler, J., Prasse, A., Bonella, F., Mueller-Ladner, U., Witte, T., Hunzelmann, N., and Distler, J.
Abstract: Background Systemic sclerosis (SSc) is a fibrosing autoimmune disease of the connective tissue. In addition to skin fibrosis, pulmonary involvement and interstitial lung disease (ILD) in particular are the most common and severe manifestations of SSc. The disease is associated with a substantial risk of morbidity and mortality, especially in progressive ILD. In the last 5 years new treatment concepts for SSc-ILD have been investigated in numerous clinical studies. Material and methods This review is based on a literature search in PubMed, focusing on the most relevant papers published up to the end of 2018 with the keywords SSc and treatment. Results The treatment of SSc-ILD has changed over the last few years due to the results of many clinical studies. The updated guidelines of the European League Against Rheumatism (EULAR) recommend the use of cyclophosphamide or hematopoietic stem cell transplantation. Data for a positive influence on SSc-ILD are also available for mycophenolate, tocilizumab and anabasum. Because of the pathophysiological similarities to idiopathic pulmonary fibrosis, the use of the antifibrotic agents nintedanib and pirfenidone is currently being investigated in randomized, multicenter clinical trials and could be a novel and promising therapeutic strategy. Conclusion Current drug studies may provide innovative therapeutic perspectives for SSc-ILD and could significantly improve the prognosis of affected patients in the future.
Published: 2020

16. 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

17. 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

18. 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

19. Treatment of systemic sclerosis-associated interstitial lung disease

Author: Prasse, A., Bonella, F., Mueller-Ladner, U., Witte, T., Hunzelmann, N., Distler, J., Prasse, A., Bonella, F., Mueller-Ladner, U., Witte, T., Hunzelmann, N., and Distler, J.
Abstract: Background Systemic sclerosis (SSc) is a fibrosing autoimmune disease of the connective tissue. In addition to skin fibrosis, pulmonary involvement and interstitial lung disease (ILD) in particular are the most common and severe manifestations of SSc. The disease is associated with a substantial risk of morbidity and mortality, especially in progressive ILD. In the last 5 years new treatment concepts for SSc-ILD have been investigated in numerous clinical studies. Material and methods This review is based on a literature search in PubMed, focusing on the most relevant papers published up to the end of 2018 with the keywords SSc and treatment. Results The treatment of SSc-ILD has changed over the last few years due to the results of many clinical studies. The updated guidelines of the European League Against Rheumatism (EULAR) recommend the use of cyclophosphamide or hematopoietic stem cell transplantation. Data for a positive influence on SSc-ILD are also available for mycophenolate, tocilizumab and anabasum. Because of the pathophysiological similarities to idiopathic pulmonary fibrosis, the use of the antifibrotic agents nintedanib and pirfenidone is currently being investigated in randomized, multicenter clinical trials and could be a novel and promising therapeutic strategy. Conclusion Current drug studies may provide innovative therapeutic perspectives for SSc-ILD and could significantly improve the prognosis of affected patients in the future.
Published: 2020

20. Nintedanib in patients with progressive fibrosing interstitial lung diseases—subgroup analyses by interstitial lung disease diagnosis in the INBUILD trial: a randomised, double-blind, placebo-controlled, parallel-group trial

Author: Wells, A. U., Flaherty, K. R., Brown, K. K., Inoue, Y., Devaraj, A., Richeldi, Luca, Moua, T., Crestani, B., Wuyts, W. A., Stowasser, S., Quaresma, M., Goeldner, R. -G., Schlenker-Herceg, R., Kolb, M., Abe, S., Aburto, M., Acosta, O., Andrews, C., Antin-Ozerkis, D., Arce, G., Arias, M., Avdeev, S., Barczyk, A., Bascom, R., Bazdyrev, E., Beirne, P., Belloli, E., Bergna, M. A., Bergot, E., Bhatt, N., Blaas, S., Bondue, B., Bonella, F., Britt, E., Buch, K., Burk, J., Cai, H., Cantin, A., Castillo Villegas, D. M., Cazaux, A., Cerri, S., Chaaban, S., Chaudhuri, N., Cottin, V., Criner, G., Dahlqvist, C., Danoff, S., Dematte D'Amico, J., Dilling, D., Elias, P., Ettinger, N., Falk, J., Fernandez Perez, E. R., Gamez-Dubuis, A., Giessel, G., Gifford, A., Glassberg, M., Glazer, C., Golden, J., Gomez Carrera, L., Guiot, J., Hallowell, R., Hayashi, H., Hetzel, J., Hirani, N., Homik, L., Hope-Gill, B., Hotchkin, D., Ichikado, K., Ilkovich, M., Izumi, S., Jassem, E., Jones, L., Jouneau, S., Kaner, R., Kang, J., Kawamura, T., Kessler, R., Kim, Y., Kishi, K., Kitamura, H., Kondoh, Y., Kono, C., Koschel, D., Kreuter, M., Kulkarni, T., Kus, J., Lebargy, F., Leon Jimenez, A., Luo, Q., Mageto, Y., Maher, T. M., Makino, S., Marchand-Adam, S., Marquette, C., Martinez, Sara, Martinez, M., Maturana Rozas, R., Miyazaki, Y., Moiseev, S., Molina-Molina, M., Malcolm, Joan Morrison, Morrow, L., Nambiar, A., Nishioka, Y., Nunes, H., Okamoto, M., Oldham, J., Otaola, M., Padilla, M., Park, J. S., Patel, N., Pesci, Riccardo, Piotrowski, W., Pitts, L., Poonyagariyagorn, H., Prasse, A., Quadrelli, S., Randerath, W., Refini, R., Reynaud-Gaubert, M., Riviere, F., Rodriguez Portal, J. A., Rosas, I., Rossman, M., Safdar, Z., Saito, T., Sakamoto, N., Salinas Fenero, M., Sauleda, J., Schmidt, S., Scholand, M. B., Schwartz, M., Shapera, S., Shlobin, O., Sigal, B., Silva Orellana, A., Skowasch, D., Song, J. W., Stieglitz, S., Stone, H., Strek, M., Suda, T., Sugiura, H., Takahashi, H., Takaya, H., Takeuchi, T., Thavarajah, K., Tolle, L., Tomassetti, S., Tomii, K., Valenzuela, C., Vancheri, C., Varone, Francesco, Veeraraghavan, S., Villar, A., Weigt, S., Wemeau, L., Wuyts, W., Xu, Z., Yakusevich, V., Yamada, Y., Yamauchi, H., Ziora, D., Richeldi L. (ORCID:0000-0001-8594-1448), Martinez R., Morrison L., Pesci A., Varone F., Wells, A. U., Flaherty, K. R., Brown, K. K., Inoue, Y., Devaraj, A., Richeldi, Luca, Moua, T., Crestani, B., Wuyts, W. A., Stowasser, S., Quaresma, M., Goeldner, R. -G., Schlenker-Herceg, R., Kolb, M., Abe, S., Aburto, M., Acosta, O., Andrews, C., Antin-Ozerkis, D., Arce, G., Arias, M., Avdeev, S., Barczyk, A., Bascom, R., Bazdyrev, E., Beirne, P., Belloli, E., Bergna, M. A., Bergot, E., Bhatt, N., Blaas, S., Bondue, B., Bonella, F., Britt, E., Buch, K., Burk, J., Cai, H., Cantin, A., Castillo Villegas, D. M., Cazaux, A., Cerri, S., Chaaban, S., Chaudhuri, N., Cottin, V., Criner, G., Dahlqvist, C., Danoff, S., Dematte D'Amico, J., Dilling, D., Elias, P., Ettinger, N., Falk, J., Fernandez Perez, E. R., Gamez-Dubuis, A., Giessel, G., Gifford, A., Glassberg, M., Glazer, C., Golden, J., Gomez Carrera, L., Guiot, J., Hallowell, R., Hayashi, H., Hetzel, J., Hirani, N., Homik, L., Hope-Gill, B., Hotchkin, D., Ichikado, K., Ilkovich, M., Izumi, S., Jassem, E., Jones, L., Jouneau, S., Kaner, R., Kang, J., Kawamura, T., Kessler, R., Kim, Y., Kishi, K., Kitamura, H., Kondoh, Y., Kono, C., Koschel, D., Kreuter, M., Kulkarni, T., Kus, J., Lebargy, F., Leon Jimenez, A., Luo, Q., Mageto, Y., Maher, T. M., Makino, S., Marchand-Adam, S., Marquette, C., Martinez, Sara, Martinez, M., Maturana Rozas, R., Miyazaki, Y., Moiseev, S., Molina-Molina, M., Malcolm, Joan Morrison, Morrow, L., Nambiar, A., Nishioka, Y., Nunes, H., Okamoto, M., Oldham, J., Otaola, M., Padilla, M., Park, J. S., Patel, N., Pesci, Riccardo, Piotrowski, W., Pitts, L., Poonyagariyagorn, H., Prasse, A., Quadrelli, S., Randerath, W., Refini, R., Reynaud-Gaubert, M., Riviere, F., Rodriguez Portal, J. A., Rosas, I., Rossman, M., Safdar, Z., Saito, T., Sakamoto, N., Salinas Fenero, M., Sauleda, J., Schmidt, S., Scholand, M. B., Schwartz, M., Shapera, S., Shlobin, O., Sigal, B., Silva Orellana, A., Skowasch, D., Song, J. W., Stieglitz, S., Stone, H., Strek, M., Suda, T., Sugiura, H., Takahashi, H., Takaya, H., Takeuchi, T., Thavarajah, K., Tolle, L., Tomassetti, S., Tomii, K., Valenzuela, C., Vancheri, C., Varone, Francesco, Veeraraghavan, S., Villar, A., Weigt, S., Wemeau, L., Wuyts, W., Xu, Z., Yakusevich, V., Yamada, Y., Yamauchi, H., Ziora, D., Richeldi L. (ORCID:0000-0001-8594-1448), Martinez R., Morrison L., Pesci A., and Varone F.
Abstract: Background: The INBUILD trial investigated the efficacy and safety of nintedanib versus placebo in patients with progressive fibrosing interstitial lung diseases (ILDs) other than idiopathic pulmonary fibrosis (IPF). We aimed to establish the effects of nintedanib in subgroups based on ILD diagnosis. Methods: The INBUILD trial was a randomised, double-blind, placebo-controlled, parallel group trial done at 153 sites in 15 countries. Participants had an investigator-diagnosed fibrosing ILD other than IPF, with chest imaging features of fibrosis of more than 10% extent on high resolution CT (HRCT), forced vital capacity (FVC) of 45% or more predicted, and diffusing capacity of the lung for carbon monoxide (DLco) of at least 30% and less than 80% predicted. Participants fulfilled protocol-defined criteria for ILD progression in the 24 months before screening, despite management considered appropriate in clinical practice for the individual ILD. Participants were randomly assigned 1:1 by means of a pseudo-random number generator to receive nintedanib 150 mg twice daily or placebo for at least 52 weeks. Participants, investigators, and other personnel involved in the trial and analysis were masked to treatment assignment until after database lock. In this subgroup analysis, we assessed the rate of decline in FVC (mL/year) over 52 weeks in patients who received at least one dose of nintedanib or placebo in five prespecified subgroups based on the ILD diagnoses documented by the investigators: hypersensitivity pneumonitis, autoimmune ILDs, idiopathic non-specific interstitial pneumonia, unclassifiable idiopathic interstitial pneumonia, and other ILDs. The trial has been completed and is registered with ClinicalTrials.gov, number NCT02999178. Findings: Participants were recruited between Feb 23, 2017, and April 27, 2018. Of 663 participants who received at least one dose of nintedanib or placebo, 173 (26%) had chronic hypersensitivity pneumonitis, 170 (26%) an autoimmune IL
Published: 2020

21. The Burden of Sarcoidosis Symptoms from a Patient Perspective

Author: Voortman, M., Hendriks, C. M.R., Elfferich, M. D.P., Bonella, F., Møller, J., De Vries, J., Costabel, U., Drent, M., Voortman, M., Hendriks, C. M.R., Elfferich, M. D.P., Bonella, F., Møller, J., De Vries, J., Costabel, U., and Drent, M.
Published: 2019

22. Gaps in care of patients living with pulmonary fibrosis

Author: Moor, C.C. (Karen), Wijsenbeek-Lourens, M.S. (Marlies), Balestro, E. (Elisabetta), Biondini, D. (Davide), Bondue, B. (Benjamin), Cottin, V. (Vincent), Kreuter, M. (Michael), Bonella, F. (Francesco), Moor, C.C. (Karen), Wijsenbeek-Lourens, M.S. (Marlies), Balestro, E. (Elisabetta), Biondini, D. (Davide), Bondue, B. (Benjamin), Cottin, V. (Vincent), Kreuter, M. (Michael), and Bonella, F. (Francesco)
Abstract: Introduction: Pulmonary fibrosis (PF) and its most common form, idiopathic pulmonary fibrosis (IPF), are chronic, progressive diseases resulting in increasing loss of lung function and impaired quality of life and survival. The aim of this joint expert and patient statement was to highlight the most pressing common unmet needs of patients with PF/IPF, putting forward recommendations to improve the quality of life and health outcomes throughout the patient journey. Methods: Two online surveys for patients and healthcare professionals (HCPs) were conducted by the European Idiopathic Pulmonary Fibrosis and Related Disorders Federation (EU-IPFF) in 14 European countries. Results: The surveys were answered by 286 patients and 69 HCPs, including physicians and nurses. Delays in diagnosis and timely access to interstitial lung disease specialists and pharmacological treatment have been identified as important gaps in care. Additionally, patients and HCPs reported that a greater focus on symptomcentred management, adequate information, trial information and increasing awareness of PF/IPF is required. Conclusions: The surveys offer important insights into the current unmet needs of PF/IPF patients. Interventions at different points of the care pathway are needed to improve patient experience.
Published: 2019
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23. Cardiovascular safety of nintedanib in subgroups by cardiovascular risk at baseline in the TOMORROW and INPULSIS trials

Author: Noth, I. (Imre), Wijsenbeek-Lourens, M.S. (Marlies), Kolb, M. (Martin), Bonella, F. (Francesco), Moros, L. (Lizette), Wachtlin, D. (Daniel), Corte, T.J. (Tamera J.), Noth, I. (Imre), Wijsenbeek-Lourens, M.S. (Marlies), Kolb, M. (Martin), Bonella, F. (Francesco), Moros, L. (Lizette), Wachtlin, D. (Daniel), and Corte, T.J. (Tamera J.)
Abstract: Nintedanib is a tyrosine kinase inhibitor used to treat idiopathic pulmonary fibrosis (IPF). We investigated the cardiovascular safety of nintedanib using pooled data from the TOMORROW and INPULSIS trials.Cardiovascular events were assessed post hoc in patients with a history of atherosclerotic cardiovascular disease (CVD) and/or one or more cardiovascular risk factors at baseline ("higher cardiovascular risk") and patients with no history of atherosclerotic CVD and no cardiovascular risk factors at baseline ("lower cardiovascular risk").Incidence rates were calculated for 1231 patients (n=723 nintedanib and n=508 placebo), of whom 89.9% had higher cardiovascular risk. Incidence rates of major adverse cardiovascular events were similar in the nintedanib and placebo groups in patients with higher cardiovascular risk (3.88 (95% CI 2.58-5.84) and 3.49 (95% CI 2.10-5.79) per 100 patient-years, respectively) and lower cardiovascular risk (4.78 (95% CI 1.54-14.82) and 5.37 (95% CI 1.73-16.65) per 100 patient-years, respectively). Incidence rates of myocardial infarction in the nintedanib and placebo groups, respectively, were 3.03 (95% CI 1.91-4.81) and 1.16 (95% CI 0.48-2.79) per 100 patient-years in patients with higher cardiovascular risk and 1.59 (95% CI 0.22-11.29) and 1.78 (95% CI 0.25-12.64) per 100 patient-years in patients with lower cardiovascular risk. Incidence rates of other ischaemic heart disease in the nintedanib and placebo groups, respectively, were 1.85 (95% CI 1.02-3.34) and 3.28 (95% CI 1.94-5.54) per 100 patient-years in patients with higher cardiovascular risk and 0 and 1.80 (95% CI 0.25-12.78) per 100 patient-years in patients with lower cardiovascular risk.These data help to establish the cardiovascular safety profile of nintedanib in IPF.
Published: 2019
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24. Resequencing study confirms that host defense and cell senescence gene variants contribute to the risk of idiopathic pulmonary fibrosis

Author: Moore, C., Blumhagen, R.Z., Yang, I.V., Walts, A., Powers, J., Walker, T., Bishop, M., Russell, P., Vestal, B., Cardwell, J., Markin, C.R., Mathai, S.K., Schwarz, M.I., Steele, M.P., Lee, J., Brown, K.K., Loyd, J.E., Crapo, J.D., Silverman, E.K., Cho, M.H., James, J.A., Guthridge, J.M., Cogan, J.D., Kropski, J.A., Swigris, J.J., Bair, C., Kim, D.S., Ji, W., Kim, H., Song, J.W., Maier, L.A., Pacheco, K.A., Hirani, N., Poon, A.S., Li, F., Jenkins, R.G., Braybrooke, R., Saini, G., Maher, T.M., Molyneaux, P.L., Saunders, P., Zhang, Y., Gibson, K.F., Kass, D.J., Rojas, M., Sembrat, J., Wolters, P.J., Collard, H.R., Sundy, J.S., O’Riordan, T., Strek, M.E., Noth, I., Ma, S-F, Porteous, M.K., Kreider, M.E., Patel, N.B., Inoue, Y., Hirose, M., Arai, T., Akagawa, S., Eickelberg, O., Fernandez, I.E., Behr, J., Mogulkoc, N., Corte, T.J., Glaspole, I., Tomassetti, S., Ravaglia, C., Poletti, V., Crestani, B., Borie, R., Kannengiesser, C., Parfrey, H., Fiddler, C., Rassl, D., Molina-Molina, M., Machahua, C., Worboys, A.M., Gudmundsson, G., Isaksson, H.J., Lederer, D.J., Podolanczuk, A.J., Montesi, S.B., Bendstrup, E., Danchel, V., Selman, M., Pardo, A., Henry, M.T., Keane, M.P., Doran, P., Vašáková, M., Sterclova, M., Ryerson, C.J., Wilcox, P.G., Okamoto, T., Furusawa, H., Miyazaki, Y., Laurent, G., Baltic, S., Prêle, C., Moodley, Y., Shea, B.S., Ohta, K., Suzukawa, M., Narumoto, O., Nathan, S.D., Venuto, D.C., Woldehanna, M.L., Kokturk, N., de Andrade, J.A., Luckhardt, T., Kulkarni, T., Bonella, F., Donnelly, S.C., McElroy, A., Armstong, M.E., Aranda, A., Carbone, R.G., Puppo, F., Beckman, K.B., Nickerson, D.A., Fingerlin, T.E., Schwartz, D.A., Moore, C., Blumhagen, R.Z., Yang, I.V., Walts, A., Powers, J., Walker, T., Bishop, M., Russell, P., Vestal, B., Cardwell, J., Markin, C.R., Mathai, S.K., Schwarz, M.I., Steele, M.P., Lee, J., Brown, K.K., Loyd, J.E., Crapo, J.D., Silverman, E.K., Cho, M.H., James, J.A., Guthridge, J.M., Cogan, J.D., Kropski, J.A., Swigris, J.J., Bair, C., Kim, D.S., Ji, W., Kim, H., Song, J.W., Maier, L.A., Pacheco, K.A., Hirani, N., Poon, A.S., Li, F., Jenkins, R.G., Braybrooke, R., Saini, G., Maher, T.M., Molyneaux, P.L., Saunders, P., Zhang, Y., Gibson, K.F., Kass, D.J., Rojas, M., Sembrat, J., Wolters, P.J., Collard, H.R., Sundy, J.S., O’Riordan, T., Strek, M.E., Noth, I., Ma, S-F, Porteous, M.K., Kreider, M.E., Patel, N.B., Inoue, Y., Hirose, M., Arai, T., Akagawa, S., Eickelberg, O., Fernandez, I.E., Behr, J., Mogulkoc, N., Corte, T.J., Glaspole, I., Tomassetti, S., Ravaglia, C., Poletti, V., Crestani, B., Borie, R., Kannengiesser, C., Parfrey, H., Fiddler, C., Rassl, D., Molina-Molina, M., Machahua, C., Worboys, A.M., Gudmundsson, G., Isaksson, H.J., Lederer, D.J., Podolanczuk, A.J., Montesi, S.B., Bendstrup, E., Danchel, V., Selman, M., Pardo, A., Henry, M.T., Keane, M.P., Doran, P., Vašáková, M., Sterclova, M., Ryerson, C.J., Wilcox, P.G., Okamoto, T., Furusawa, H., Miyazaki, Y., Laurent, G., Baltic, S., Prêle, C., Moodley, Y., Shea, B.S., Ohta, K., Suzukawa, M., Narumoto, O., Nathan, S.D., Venuto, D.C., Woldehanna, M.L., Kokturk, N., de Andrade, J.A., Luckhardt, T., Kulkarni, T., Bonella, F., Donnelly, S.C., McElroy, A., Armstong, M.E., Aranda, A., Carbone, R.G., Puppo, F., Beckman, K.B., Nickerson, D.A., Fingerlin, T.E., and Schwartz, D.A.
Abstract: Rationale: Several common and rare genetic variants have been associated with idiopathic pulmonary fibrosis, a progressive fibrotic condition that is localized to the lung. Objectives: To develop an integrated understanding of the rare and common variants located in multiple loci that have been reported to contribute to the risk of disease. Methods: We performed deep targeted resequencing (3.69 Mb of DNA) in cases (n = 3,624) and control subjects (n = 4,442) across genes and regions previously associated with disease. We tested for associations between disease and 1) individual common variants via logistic regression and 2) groups of rare variants via sequence kernel association tests. Measurements and Main Results: Statistically significant common variant association signals occurred in all 10 of the regions chosen based on genome-wide association studies. The strongest risk variant is the MUC5B promoter variant rs35705950, with an odds ratio of 5.45 (95% confidence interval, 4.91–6.06) for one copy of the risk allele and 18.68 (95% confidence interval, 13.34–26.17) for two copies of the risk allele (P = 9.60 × 10−295). In addition to identifying for the first time that rare variation in FAM13A is associated with disease, we confirmed the role of rare variation in the TERT and RTEL1 gene regions in the risk of IPF, and found that the FAM13A and TERT regions have independent common and rare variant signals. Conclusions: A limited number of common and rare variants contribute to the risk of idiopathic pulmonary fibrosis in each of the resequencing regions, and these genetic variants focus on biological mechanisms of host defense and cell senescence.
Published: 2019

25. The Burden of Sarcoidosis Symptoms from a Patient Perspective

Author: Voortman, M., Hendriks, C. M.R., Elfferich, M. D.P., Bonella, F., Møller, J., De Vries, J., Costabel, U., Drent, M., Voortman, M., Hendriks, C. M.R., Elfferich, M. D.P., Bonella, F., Møller, J., De Vries, J., Costabel, U., and Drent, M.
Published: 2019

26. Influence of Antisynthetase Antibodies Specificities on Antisynthetase Syndrome Clinical Spectrum Time Course

Author: Cavagna, L, Trallero-Araguás, E, Meloni, F, Cavazzana, I, Rojas-Serrano, J, Feist, E, Zanframundo, G, Morandi, V, Meyer, A, Pereira da Silva, J, Matos Costa, C, Molberg, O, Andersson, H, Codullo, V, Mosca, M, Barsotti, S, Neri, R, Scirè, C, Govoni, M, Furini, F, Lopez-Longo, F, Martinez-Barrio, J, Schneider, U, Lorenz, H, Doria, A, Ghirardello, A, Ortego-Centeno, N, Confalonieri, M, Tomietto, P, Pipitone, N, Rodriguez Cambron, A, Blázquez Cañamero, M, Voll, R, Wendel, S, Scarpato, S, Maurier, F, Limonta, M, Colombelli, P, Giannini, M, Geny, B, Arrigoni, E, Bravi, E, Migliorini, P, Mathieu, A, Piga, M, Drott, U, Delbrueck, C, Bauhammer, J, Cagnotto, G, Vancheri, C, Sambataro, G, De Langhe, E, Sainaghi, P, Monti, C, Gigli Berzolari, F, Romano, M, Bonella, F, Specker, C, Schwarting, A, Villa Blanco, I, Selmi, C, Ceribelli, A, Nuno, L, Mera-Varela, A, Perez Gomez, N, Fusaro, E, Parisi, S, Sinigaglia, L, Del Papa, N, Benucci, M, Cimmino, M, Riccieri, V, Conti, F, Sebastiani, G, Iuliano, A, Emmi, G, Cammelli, D, Sebastiani, M, Manfredi, A, Bachiller-Corral, J, Sifuentes Giraldo, W, Paolazzi, G, Saketkoo, L, Giorgi, R, Salaffi, F, Cifrian, J, Caporali, R, Locatelli, F, Marchioni, E, Pesci, A, Dei, G, Pozzi, M, Claudia, L, Distler, J, Knitza, J, Schett, G, Iannone, F, Fornaro, M, Franceschini, F, Quartuccio, L, Gerli, R, Bartoloni, E, Bellando Randone, S, Zampogna, G, Gonzalez Perez, M, Mejia, M, Vicente, E, Triantafyllias, K, Lopez-Mejias, R, Matucci-Cerinic, M, Selva-O'Callaghan, A, Castañeda, S, Montecucco, C, Gonzalez-Gay, M, Cavagna, Lorenzo, Trallero-Araguás, Ernesto, Meloni, Federica, Cavazzana, Ilaria, Rojas-Serrano, Jorge, Feist, Eugen, Zanframundo, Giovanni, Morandi, Valentina, Meyer, Alain, Pereira da Silva, Jose Antonio, Matos Costa, Carlo Jorge, Molberg, Oyvind, Andersson, Helena, Codullo, Veronica, Mosca, Marta, Barsotti, Simone, Neri, Rossella, Scirè, Carlo, Govoni, Marcello, Furini, Federica, Lopez-Longo, Francisco Javier, Martinez-Barrio, Julia, Schneider, Udo, Lorenz, Hanns-Martin, Doria, Andrea, Ghirardello, Anna, Ortego-Centeno, Norberto, Confalonieri, Marco, Tomietto, Paola, Pipitone, Nicolò, Rodriguez Cambron, Ana Belen, Blázquez Cañamero, María Ángeles, Voll, Reinhard Edmund, Wendel, Sarah, Scarpato, Salvatore, Maurier, Francois, Limonta, Massimiliano, Colombelli, Paolo, Giannini, Margherita, Geny, Bernard, Arrigoni, Eugenio, Bravi, Elena, Migliorini, Paola, Mathieu, Alessandro, Piga, Matteo, Drott, Ulrich, Delbrueck, Christiane, Bauhammer, Jutta, Cagnotto, Giovanni, Vancheri, Carlo, Sambataro, Gianluca, De Langhe, Ellen, Sainaghi, Pier Paolo, Monti, Cristina, Gigli Berzolari, Francesca, Romano, Mariaeva, Bonella, Francesco, Specker, Christof, Schwarting, Andreas, Villa Blanco, Ignacio, Selmi, Carlo, Ceribelli, Angela, Nuno, Laura, Mera-Varela, Antonio, Perez Gomez, Nair, Fusaro, Enrico, Parisi, Simone, Sinigaglia, Luigi, Del Papa, Nicoletta, Benucci, Maurizio, Cimmino, Marco Amedeo, Riccieri, Valeria, Conti, Fabrizio, Sebastiani, Gian Domenico, Iuliano, Annamaria, Emmi, Giacomo, Cammelli, Daniele, Sebastiani, Marco, Manfredi, Andreina, Bachiller-Corral, Javier, Sifuentes Giraldo, Walter Alberto, Paolazzi, Giuseppe, Saketkoo, Lesley Ann, Giorgi, Roberto, Salaffi, Fausto, Cifrian, Jose, Caporali, Roberto, Locatelli, Francesco, Marchioni, Enrico, Pesci, Alberto, Dei, Giulia, Pozzi, Maria Rosa, Claudia, Lomater, Distler, Jorg, Knitza, Johannes, Schett, George, Iannone, Florenzo, Fornaro, Marco, Franceschini, Franco, Quartuccio, Luca, Gerli, Roberto, Bartoloni, Elena, Bellando Randone, Silvia, Zampogna, Giuseppe, Gonzalez Perez, Montserrat I, Mejia, Mayra, Vicente, Esther, Triantafyllias, Konstantinos, Lopez-Mejias, Raquel, Matucci-Cerinic, Marco, Selva-O'Callaghan, Albert, Castañeda, Santos, Montecucco, Carlomaurizio, Gonzalez-Gay, Miguel Angel, Cavagna, L, Trallero-Araguás, E, Meloni, F, Cavazzana, I, Rojas-Serrano, J, Feist, E, Zanframundo, G, Morandi, V, Meyer, A, Pereira da Silva, J, Matos Costa, C, Molberg, O, Andersson, H, Codullo, V, Mosca, M, Barsotti, S, Neri, R, Scirè, C, Govoni, M, Furini, F, Lopez-Longo, F, Martinez-Barrio, J, Schneider, U, Lorenz, H, Doria, A, Ghirardello, A, Ortego-Centeno, N, Confalonieri, M, Tomietto, P, Pipitone, N, Rodriguez Cambron, A, Blázquez Cañamero, M, Voll, R, Wendel, S, Scarpato, S, Maurier, F, Limonta, M, Colombelli, P, Giannini, M, Geny, B, Arrigoni, E, Bravi, E, Migliorini, P, Mathieu, A, Piga, M, Drott, U, Delbrueck, C, Bauhammer, J, Cagnotto, G, Vancheri, C, Sambataro, G, De Langhe, E, Sainaghi, P, Monti, C, Gigli Berzolari, F, Romano, M, Bonella, F, Specker, C, Schwarting, A, Villa Blanco, I, Selmi, C, Ceribelli, A, Nuno, L, Mera-Varela, A, Perez Gomez, N, Fusaro, E, Parisi, S, Sinigaglia, L, Del Papa, N, Benucci, M, Cimmino, M, Riccieri, V, Conti, F, Sebastiani, G, Iuliano, A, Emmi, G, Cammelli, D, Sebastiani, M, Manfredi, A, Bachiller-Corral, J, Sifuentes Giraldo, W, Paolazzi, G, Saketkoo, L, Giorgi, R, Salaffi, F, Cifrian, J, Caporali, R, Locatelli, F, Marchioni, E, Pesci, A, Dei, G, Pozzi, M, Claudia, L, Distler, J, Knitza, J, Schett, G, Iannone, F, Fornaro, M, Franceschini, F, Quartuccio, L, Gerli, R, Bartoloni, E, Bellando Randone, S, Zampogna, G, Gonzalez Perez, M, Mejia, M, Vicente, E, Triantafyllias, K, Lopez-Mejias, R, Matucci-Cerinic, M, Selva-O'Callaghan, A, Castañeda, S, Montecucco, C, Gonzalez-Gay, M, Cavagna, Lorenzo, Trallero-Araguás, Ernesto, Meloni, Federica, Cavazzana, Ilaria, Rojas-Serrano, Jorge, Feist, Eugen, Zanframundo, Giovanni, Morandi, Valentina, Meyer, Alain, Pereira da Silva, Jose Antonio, Matos Costa, Carlo Jorge, Molberg, Oyvind, Andersson, Helena, Codullo, Veronica, Mosca, Marta, Barsotti, Simone, Neri, Rossella, Scirè, Carlo, Govoni, Marcello, Furini, Federica, Lopez-Longo, Francisco Javier, Martinez-Barrio, Julia, Schneider, Udo, Lorenz, Hanns-Martin, Doria, Andrea, Ghirardello, Anna, Ortego-Centeno, Norberto, Confalonieri, Marco, Tomietto, Paola, Pipitone, Nicolò, Rodriguez Cambron, Ana Belen, Blázquez Cañamero, María Ángeles, Voll, Reinhard Edmund, Wendel, Sarah, Scarpato, Salvatore, Maurier, Francois, Limonta, Massimiliano, Colombelli, Paolo, Giannini, Margherita, Geny, Bernard, Arrigoni, Eugenio, Bravi, Elena, Migliorini, Paola, Mathieu, Alessandro, Piga, Matteo, Drott, Ulrich, Delbrueck, Christiane, Bauhammer, Jutta, Cagnotto, Giovanni, Vancheri, Carlo, Sambataro, Gianluca, De Langhe, Ellen, Sainaghi, Pier Paolo, Monti, Cristina, Gigli Berzolari, Francesca, Romano, Mariaeva, Bonella, Francesco, Specker, Christof, Schwarting, Andreas, Villa Blanco, Ignacio, Selmi, Carlo, Ceribelli, Angela, Nuno, Laura, Mera-Varela, Antonio, Perez Gomez, Nair, Fusaro, Enrico, Parisi, Simone, Sinigaglia, Luigi, Del Papa, Nicoletta, Benucci, Maurizio, Cimmino, Marco Amedeo, Riccieri, Valeria, Conti, Fabrizio, Sebastiani, Gian Domenico, Iuliano, Annamaria, Emmi, Giacomo, Cammelli, Daniele, Sebastiani, Marco, Manfredi, Andreina, Bachiller-Corral, Javier, Sifuentes Giraldo, Walter Alberto, Paolazzi, Giuseppe, Saketkoo, Lesley Ann, Giorgi, Roberto, Salaffi, Fausto, Cifrian, Jose, Caporali, Roberto, Locatelli, Francesco, Marchioni, Enrico, Pesci, Alberto, Dei, Giulia, Pozzi, Maria Rosa, Claudia, Lomater, Distler, Jorg, Knitza, Johannes, Schett, George, Iannone, Florenzo, Fornaro, Marco, Franceschini, Franco, Quartuccio, Luca, Gerli, Roberto, Bartoloni, Elena, Bellando Randone, Silvia, Zampogna, Giuseppe, Gonzalez Perez, Montserrat I, Mejia, Mayra, Vicente, Esther, Triantafyllias, Konstantinos, Lopez-Mejias, Raquel, Matucci-Cerinic, Marco, Selva-O'Callaghan, Albert, Castañeda, Santos, Montecucco, Carlomaurizio, and Gonzalez-Gay, Miguel Angel
Abstract: Antisynthetase syndrome (ASSD) is a rare clinical condition that is characterized by the occurrence of a classic clinical triad, encompassing myositis, arthritis, and interstitial lung disease (ILD), along with specific autoantibodies that are addressed to different aminoacyl tRNA synthetases (ARS). Until now, it has been unknown whether the presence of a different ARS might affect the clinical presentation, evolution, and outcome of ASSD. In this study, we retrospectively recorded the time of onset, characteristics, clustering of triad findings, and survival of 828 ASSD patients (593 anti-Jo1, 95 anti-PL7, 84 anti-PL12, 38 anti-EJ, and 18 anti-OJ), referring to AENEAS (American and European NEtwork of Antisynthetase Syndrome) collaborative group's cohort. Comparisons were performed first between all ARS cases and then, in the case of significance, while using anti-Jo1 positive patients as the reference group. The characteristics of triad findings were similar and the onset mainly began with a single triad finding in all groups despite some differences in overall prevalence. The "ex-novo" occurrence of triad findings was only reduced in the anti-PL12-positive cohort, however, it occurred in a clinically relevant percentage of patients (30%). Moreover, survival was not influenced by the underlying anti-aminoacyl tRNA synthetase antibodies' positivity, which confirmed that antisynthetase syndrome is a heterogeneous condition and that antibody specificity only partially influences the clinical presentation and evolution of this condition.
Published: 2019

27. The Burden of Sarcoidosis Symptoms from a Patient Perspective

Author: Voortman, M., Hendriks, C. M.R., Elfferich, M. D.P., Bonella, F., Møller, J., De Vries, J., Costabel, U., Drent, M., Voortman, M., Hendriks, C. M.R., Elfferich, M. D.P., Bonella, F., Møller, J., De Vries, J., Costabel, U., and Drent, M.
Published: 2019

28. Resequencing study confirms that host defense and cell senescence gene variants contribute to the risk of idiopathic pulmonary fibrosis

Author: Moore, C., Blumhagen, R.Z., Yang, I.V., Walts, A., Powers, J., Walker, T., Bishop, M., Russell, P., Vestal, B., Cardwell, J., Markin, C.R., Mathai, S.K., Schwarz, M.I., Steele, M.P., Lee, J., Brown, K.K., Loyd, J.E., Crapo, J.D., Silverman, E.K., Cho, M.H., James, J.A., Guthridge, J.M., Cogan, J.D., Kropski, J.A., Swigris, J.J., Bair, C., Kim, D.S., Ji, W., Kim, H., Song, J.W., Maier, L.A., Pacheco, K.A., Hirani, N., Poon, A.S., Li, F., Jenkins, R.G., Braybrooke, R., Saini, G., Maher, T.M., Molyneaux, P.L., Saunders, P., Zhang, Y., Gibson, K.F., Kass, D.J., Rojas, M., Sembrat, J., Wolters, P.J., Collard, H.R., Sundy, J.S., O’Riordan, T., Strek, M.E., Noth, I., Ma, S-F, Porteous, M.K., Kreider, M.E., Patel, N.B., Inoue, Y., Hirose, M., Arai, T., Akagawa, S., Eickelberg, O., Fernandez, I.E., Behr, J., Mogulkoc, N., Corte, T.J., Glaspole, I., Tomassetti, S., Ravaglia, C., Poletti, V., Crestani, B., Borie, R., Kannengiesser, C., Parfrey, H., Fiddler, C., Rassl, D., Molina-Molina, M., Machahua, C., Worboys, A.M., Gudmundsson, G., Isaksson, H.J., Lederer, D.J., Podolanczuk, A.J., Montesi, S.B., Bendstrup, E., Danchel, V., Selman, M., Pardo, A., Henry, M.T., Keane, M.P., Doran, P., Vašáková, M., Sterclova, M., Ryerson, C.J., Wilcox, P.G., Okamoto, T., Furusawa, H., Miyazaki, Y., Laurent, G., Baltic, S., Prêle, C., Moodley, Y., Shea, B.S., Ohta, K., Suzukawa, M., Narumoto, O., Nathan, S.D., Venuto, D.C., Woldehanna, M.L., Kokturk, N., de Andrade, J.A., Luckhardt, T., Kulkarni, T., Bonella, F., Donnelly, S.C., McElroy, A., Armstong, M.E., Aranda, A., Carbone, R.G., Puppo, F., Beckman, K.B., Nickerson, D.A., Fingerlin, T.E., Schwartz, D.A., Moore, C., Blumhagen, R.Z., Yang, I.V., Walts, A., Powers, J., Walker, T., Bishop, M., Russell, P., Vestal, B., Cardwell, J., Markin, C.R., Mathai, S.K., Schwarz, M.I., Steele, M.P., Lee, J., Brown, K.K., Loyd, J.E., Crapo, J.D., Silverman, E.K., Cho, M.H., James, J.A., Guthridge, J.M., Cogan, J.D., Kropski, J.A., Swigris, J.J., Bair, C., Kim, D.S., Ji, W., Kim, H., Song, J.W., Maier, L.A., Pacheco, K.A., Hirani, N., Poon, A.S., Li, F., Jenkins, R.G., Braybrooke, R., Saini, G., Maher, T.M., Molyneaux, P.L., Saunders, P., Zhang, Y., Gibson, K.F., Kass, D.J., Rojas, M., Sembrat, J., Wolters, P.J., Collard, H.R., Sundy, J.S., O’Riordan, T., Strek, M.E., Noth, I., Ma, S-F, Porteous, M.K., Kreider, M.E., Patel, N.B., Inoue, Y., Hirose, M., Arai, T., Akagawa, S., Eickelberg, O., Fernandez, I.E., Behr, J., Mogulkoc, N., Corte, T.J., Glaspole, I., Tomassetti, S., Ravaglia, C., Poletti, V., Crestani, B., Borie, R., Kannengiesser, C., Parfrey, H., Fiddler, C., Rassl, D., Molina-Molina, M., Machahua, C., Worboys, A.M., Gudmundsson, G., Isaksson, H.J., Lederer, D.J., Podolanczuk, A.J., Montesi, S.B., Bendstrup, E., Danchel, V., Selman, M., Pardo, A., Henry, M.T., Keane, M.P., Doran, P., Vašáková, M., Sterclova, M., Ryerson, C.J., Wilcox, P.G., Okamoto, T., Furusawa, H., Miyazaki, Y., Laurent, G., Baltic, S., Prêle, C., Moodley, Y., Shea, B.S., Ohta, K., Suzukawa, M., Narumoto, O., Nathan, S.D., Venuto, D.C., Woldehanna, M.L., Kokturk, N., de Andrade, J.A., Luckhardt, T., Kulkarni, T., Bonella, F., Donnelly, S.C., McElroy, A., Armstong, M.E., Aranda, A., Carbone, R.G., Puppo, F., Beckman, K.B., Nickerson, D.A., Fingerlin, T.E., and Schwartz, D.A.
Abstract: Rationale: Several common and rare genetic variants have been associated with idiopathic pulmonary fibrosis, a progressive fibrotic condition that is localized to the lung. Objectives: To develop an integrated understanding of the rare and common variants located in multiple loci that have been reported to contribute to the risk of disease. Methods: We performed deep targeted resequencing (3.69 Mb of DNA) in cases (n = 3,624) and control subjects (n = 4,442) across genes and regions previously associated with disease. We tested for associations between disease and 1) individual common variants via logistic regression and 2) groups of rare variants via sequence kernel association tests. Measurements and Main Results: Statistically significant common variant association signals occurred in all 10 of the regions chosen based on genome-wide association studies. The strongest risk variant is the MUC5B promoter variant rs35705950, with an odds ratio of 5.45 (95% confidence interval, 4.91–6.06) for one copy of the risk allele and 18.68 (95% confidence interval, 13.34–26.17) for two copies of the risk allele (P = 9.60 × 10−295). In addition to identifying for the first time that rare variation in FAM13A is associated with disease, we confirmed the role of rare variation in the TERT and RTEL1 gene regions in the risk of IPF, and found that the FAM13A and TERT regions have independent common and rare variant signals. Conclusions: A limited number of common and rare variants contribute to the risk of idiopathic pulmonary fibrosis in each of the resequencing regions, and these genetic variants focus on biological mechanisms of host defense and cell senescence.
Published: 2019

29. Video-kapillarmikroskopische Veränderungen bei Patienten mit Antisynthetase-Syndrom. Ergebnisse einer internationalen, multizentrischen Studie des American and European Network of Antisynthetase Syndrome (AENEAS)

Author: Triantafyllias, K, Sebastiani, M, Manfredi, A, Codullo, V, Schwarting, A, Drott, U, Delbrück, C, Weinmann-Menke, J, Bauhammer, J, Miehle, N, Bonella, F, Bergner, R, Specker, C, Lütgendorf, F, Cavagna, L, Triantafyllias, K, Sebastiani, M, Manfredi, A, Codullo, V, Schwarting, A, Drott, U, Delbrück, C, Weinmann-Menke, J, Bauhammer, J, Miehle, N, Bonella, F, Bergner, R, Specker, C, Lütgendorf, F, and Cavagna, L
Published: 2019

30. 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

31. Video-kapillarmikroskopische Veränderungen bei Patienten mit Antisynthetase-Syndrom. Ergebnisse einer internationalen, multizentrischen Studie des American and European Network of Antisynthetase Syndrome (AENEAS)

Author: Triantafyllias, K, Sebastiani, M, Manfredi, A, Codullo, V, Schwarting, A, Drott, U, Delbrück, C, Weinmann-Menke, J, Bauhammer, J, Miehle, N, Bonella, F, Bergner, R, Specker, C, Lütgendorf, F, Cavagna, L, Triantafyllias, K, Sebastiani, M, Manfredi, A, Codullo, V, Schwarting, A, Drott, U, Delbrück, C, Weinmann-Menke, J, Bauhammer, J, Miehle, N, Bonella, F, Bergner, R, Specker, C, Lütgendorf, F, and Cavagna, L
Published: 2019

32. The Burden of Sarcoidosis Symptoms from a Patient Perspective

Author: Longziekten, Voortman, M., Hendriks, C. M.R., Elfferich, M. D.P., Bonella, F., Møller, J., De Vries, J., Costabel, U., Drent, M., Longziekten, Voortman, M., Hendriks, C. M.R., Elfferich, M. D.P., Bonella, F., Møller, J., De Vries, J., Costabel, U., and Drent, M.
Published: 2019

33. Influence of Antisynthetase Antibodies Specificities on Antisynthetase Syndrome Clinical Spectrum Time Course

Author: Cavagna, L, Trallero-Araguás, E, Meloni, F, Cavazzana, I, Rojas-Serrano, J, Feist, E, Zanframundo, G, Morandi, V, Meyer, A, Pereira da Silva, J, Matos Costa, C, Molberg, O, Andersson, H, Codullo, V, Mosca, M, Barsotti, S, Neri, R, Scirè, C, Govoni, M, Furini, F, Lopez-Longo, F, Martinez-Barrio, J, Schneider, U, Lorenz, H, Doria, A, Ghirardello, A, Ortego-Centeno, N, Confalonieri, M, Tomietto, P, Pipitone, N, Rodriguez Cambron, A, Blázquez Cañamero, M, Voll, R, Wendel, S, Scarpato, S, Maurier, F, Limonta, M, Colombelli, P, Giannini, M, Geny, B, Arrigoni, E, Bravi, E, Migliorini, P, Mathieu, A, Piga, M, Drott, U, Delbrueck, C, Bauhammer, J, Cagnotto, G, Vancheri, C, Sambataro, G, De Langhe, E, Sainaghi, P, Monti, C, Gigli Berzolari, F, Romano, M, Bonella, F, Specker, C, Schwarting, A, Villa Blanco, I, Selmi, C, Ceribelli, A, Nuno, L, Mera-Varela, A, Perez Gomez, N, Fusaro, E, Parisi, S, Sinigaglia, L, Del Papa, N, Benucci, M, Cimmino, M, Riccieri, V, Conti, F, Sebastiani, G, Iuliano, A, Emmi, G, Cammelli, D, Sebastiani, M, Manfredi, A, Bachiller-Corral, J, Sifuentes Giraldo, W, Paolazzi, G, Saketkoo, L, Giorgi, R, Salaffi, F, Cifrian, J, Caporali, R, Locatelli, F, Marchioni, E, Pesci, A, Dei, G, Pozzi, M, Claudia, L, Distler, J, Knitza, J, Schett, G, Iannone, F, Fornaro, M, Franceschini, F, Quartuccio, L, Gerli, R, Bartoloni, E, Bellando Randone, S, Zampogna, G, Gonzalez Perez, M, Mejia, M, Vicente, E, Triantafyllias, K, Lopez-Mejias, R, Matucci-Cerinic, M, Selva-O'Callaghan, A, Castañeda, S, Montecucco, C, Gonzalez-Gay, M, Cavagna, Lorenzo, Trallero-Araguás, Ernesto, Meloni, Federica, Cavazzana, Ilaria, Rojas-Serrano, Jorge, Feist, Eugen, Zanframundo, Giovanni, Morandi, Valentina, Meyer, Alain, Pereira da Silva, Jose Antonio, Matos Costa, Carlo Jorge, Molberg, Oyvind, Andersson, Helena, Codullo, Veronica, Mosca, Marta, Barsotti, Simone, Neri, Rossella, Scirè, Carlo, Govoni, Marcello, Furini, Federica, Lopez-Longo, Francisco Javier, Martinez-Barrio, Julia, Schneider, Udo, Lorenz, Hanns-Martin, Doria, Andrea, Ghirardello, Anna, Ortego-Centeno, Norberto, Confalonieri, Marco, Tomietto, Paola, Pipitone, Nicolò, Rodriguez Cambron, Ana Belen, Blázquez Cañamero, María Ángeles, Voll, Reinhard Edmund, Wendel, Sarah, Scarpato, Salvatore, Maurier, Francois, Limonta, Massimiliano, Colombelli, Paolo, Giannini, Margherita, Geny, Bernard, Arrigoni, Eugenio, Bravi, Elena, Migliorini, Paola, Mathieu, Alessandro, Piga, Matteo, Drott, Ulrich, Delbrueck, Christiane, Bauhammer, Jutta, Cagnotto, Giovanni, Vancheri, Carlo, Sambataro, Gianluca, De Langhe, Ellen, Sainaghi, Pier Paolo, Monti, Cristina, Gigli Berzolari, Francesca, Romano, Mariaeva, Bonella, Francesco, Specker, Christof, Schwarting, Andreas, Villa Blanco, Ignacio, Selmi, Carlo, Ceribelli, Angela, Nuno, Laura, Mera-Varela, Antonio, Perez Gomez, Nair, Fusaro, Enrico, Parisi, Simone, Sinigaglia, Luigi, Del Papa, Nicoletta, Benucci, Maurizio, Cimmino, Marco Amedeo, Riccieri, Valeria, Conti, Fabrizio, Sebastiani, Gian Domenico, Iuliano, Annamaria, Emmi, Giacomo, Cammelli, Daniele, Sebastiani, Marco, Manfredi, Andreina, Bachiller-Corral, Javier, Sifuentes Giraldo, Walter Alberto, Paolazzi, Giuseppe, Saketkoo, Lesley Ann, Giorgi, Roberto, Salaffi, Fausto, Cifrian, Jose, Caporali, Roberto, Locatelli, Francesco, Marchioni, Enrico, Pesci, Alberto, Dei, Giulia, Pozzi, Maria Rosa, Claudia, Lomater, Distler, Jorg, Knitza, Johannes, Schett, George, Iannone, Florenzo, Fornaro, Marco, Franceschini, Franco, Quartuccio, Luca, Gerli, Roberto, Bartoloni, Elena, Bellando Randone, Silvia, Zampogna, Giuseppe, Gonzalez Perez, Montserrat I, Mejia, Mayra, Vicente, Esther, Triantafyllias, Konstantinos, Lopez-Mejias, Raquel, Matucci-Cerinic, Marco, Selva-O'Callaghan, Albert, Castañeda, Santos, Montecucco, Carlomaurizio, Gonzalez-Gay, Miguel Angel, Cavagna, L, Trallero-Araguás, E, Meloni, F, Cavazzana, I, Rojas-Serrano, J, Feist, E, Zanframundo, G, Morandi, V, Meyer, A, Pereira da Silva, J, Matos Costa, C, Molberg, O, Andersson, H, Codullo, V, Mosca, M, Barsotti, S, Neri, R, Scirè, C, Govoni, M, Furini, F, Lopez-Longo, F, Martinez-Barrio, J, Schneider, U, Lorenz, H, Doria, A, Ghirardello, A, Ortego-Centeno, N, Confalonieri, M, Tomietto, P, Pipitone, N, Rodriguez Cambron, A, Blázquez Cañamero, M, Voll, R, Wendel, S, Scarpato, S, Maurier, F, Limonta, M, Colombelli, P, Giannini, M, Geny, B, Arrigoni, E, Bravi, E, Migliorini, P, Mathieu, A, Piga, M, Drott, U, Delbrueck, C, Bauhammer, J, Cagnotto, G, Vancheri, C, Sambataro, G, De Langhe, E, Sainaghi, P, Monti, C, Gigli Berzolari, F, Romano, M, Bonella, F, Specker, C, Schwarting, A, Villa Blanco, I, Selmi, C, Ceribelli, A, Nuno, L, Mera-Varela, A, Perez Gomez, N, Fusaro, E, Parisi, S, Sinigaglia, L, Del Papa, N, Benucci, M, Cimmino, M, Riccieri, V, Conti, F, Sebastiani, G, Iuliano, A, Emmi, G, Cammelli, D, Sebastiani, M, Manfredi, A, Bachiller-Corral, J, Sifuentes Giraldo, W, Paolazzi, G, Saketkoo, L, Giorgi, R, Salaffi, F, Cifrian, J, Caporali, R, Locatelli, F, Marchioni, E, Pesci, A, Dei, G, Pozzi, M, Claudia, L, Distler, J, Knitza, J, Schett, G, Iannone, F, Fornaro, M, Franceschini, F, Quartuccio, L, Gerli, R, Bartoloni, E, Bellando Randone, S, Zampogna, G, Gonzalez Perez, M, Mejia, M, Vicente, E, Triantafyllias, K, Lopez-Mejias, R, Matucci-Cerinic, M, Selva-O'Callaghan, A, Castañeda, S, Montecucco, C, Gonzalez-Gay, M, Cavagna, Lorenzo, Trallero-Araguás, Ernesto, Meloni, Federica, Cavazzana, Ilaria, Rojas-Serrano, Jorge, Feist, Eugen, Zanframundo, Giovanni, Morandi, Valentina, Meyer, Alain, Pereira da Silva, Jose Antonio, Matos Costa, Carlo Jorge, Molberg, Oyvind, Andersson, Helena, Codullo, Veronica, Mosca, Marta, Barsotti, Simone, Neri, Rossella, Scirè, Carlo, Govoni, Marcello, Furini, Federica, Lopez-Longo, Francisco Javier, Martinez-Barrio, Julia, Schneider, Udo, Lorenz, Hanns-Martin, Doria, Andrea, Ghirardello, Anna, Ortego-Centeno, Norberto, Confalonieri, Marco, Tomietto, Paola, Pipitone, Nicolò, Rodriguez Cambron, Ana Belen, Blázquez Cañamero, María Ángeles, Voll, Reinhard Edmund, Wendel, Sarah, Scarpato, Salvatore, Maurier, Francois, Limonta, Massimiliano, Colombelli, Paolo, Giannini, Margherita, Geny, Bernard, Arrigoni, Eugenio, Bravi, Elena, Migliorini, Paola, Mathieu, Alessandro, Piga, Matteo, Drott, Ulrich, Delbrueck, Christiane, Bauhammer, Jutta, Cagnotto, Giovanni, Vancheri, Carlo, Sambataro, Gianluca, De Langhe, Ellen, Sainaghi, Pier Paolo, Monti, Cristina, Gigli Berzolari, Francesca, Romano, Mariaeva, Bonella, Francesco, Specker, Christof, Schwarting, Andreas, Villa Blanco, Ignacio, Selmi, Carlo, Ceribelli, Angela, Nuno, Laura, Mera-Varela, Antonio, Perez Gomez, Nair, Fusaro, Enrico, Parisi, Simone, Sinigaglia, Luigi, Del Papa, Nicoletta, Benucci, Maurizio, Cimmino, Marco Amedeo, Riccieri, Valeria, Conti, Fabrizio, Sebastiani, Gian Domenico, Iuliano, Annamaria, Emmi, Giacomo, Cammelli, Daniele, Sebastiani, Marco, Manfredi, Andreina, Bachiller-Corral, Javier, Sifuentes Giraldo, Walter Alberto, Paolazzi, Giuseppe, Saketkoo, Lesley Ann, Giorgi, Roberto, Salaffi, Fausto, Cifrian, Jose, Caporali, Roberto, Locatelli, Francesco, Marchioni, Enrico, Pesci, Alberto, Dei, Giulia, Pozzi, Maria Rosa, Claudia, Lomater, Distler, Jorg, Knitza, Johannes, Schett, George, Iannone, Florenzo, Fornaro, Marco, Franceschini, Franco, Quartuccio, Luca, Gerli, Roberto, Bartoloni, Elena, Bellando Randone, Silvia, Zampogna, Giuseppe, Gonzalez Perez, Montserrat I, Mejia, Mayra, Vicente, Esther, Triantafyllias, Konstantinos, Lopez-Mejias, Raquel, Matucci-Cerinic, Marco, Selva-O'Callaghan, Albert, Castañeda, Santos, Montecucco, Carlomaurizio, and Gonzalez-Gay, Miguel Angel
Abstract: Antisynthetase syndrome (ASSD) is a rare clinical condition that is characterized by the occurrence of a classic clinical triad, encompassing myositis, arthritis, and interstitial lung disease (ILD), along with specific autoantibodies that are addressed to different aminoacyl tRNA synthetases (ARS). Until now, it has been unknown whether the presence of a different ARS might affect the clinical presentation, evolution, and outcome of ASSD. In this study, we retrospectively recorded the time of onset, characteristics, clustering of triad findings, and survival of 828 ASSD patients (593 anti-Jo1, 95 anti-PL7, 84 anti-PL12, 38 anti-EJ, and 18 anti-OJ), referring to AENEAS (American and European NEtwork of Antisynthetase Syndrome) collaborative group's cohort. Comparisons were performed first between all ARS cases and then, in the case of significance, while using anti-Jo1 positive patients as the reference group. The characteristics of triad findings were similar and the onset mainly began with a single triad finding in all groups despite some differences in overall prevalence. The "ex-novo" occurrence of triad findings was only reduced in the anti-PL12-positive cohort, however, it occurred in a clinically relevant percentage of patients (30%). Moreover, survival was not influenced by the underlying anti-aminoacyl tRNA synthetase antibodies' positivity, which confirmed that antisynthetase syndrome is a heterogeneous condition and that antibody specificity only partially influences the clinical presentation and evolution of this condition.
Published: 2019

34. Research highlights from the 2018 ERS International Congress: interstitial lung diseases

Author: Alfaro, T, Moor, Karen, Alfieri, V, Jeny, F, Kreuter, M, Wijsenbeek - Lourens, Marlies, Renzoni, E A, Bargagli, E, Nunes, H, Spagnolo, P, Bonella, F, Molina-Molina, M, Antoniou, KM, Poletti, V, Alfaro, T, Moor, Karen, Alfieri, V, Jeny, F, Kreuter, M, Wijsenbeek - Lourens, Marlies, Renzoni, E A, Bargagli, E, Nunes, H, Spagnolo, P, Bonella, F, Molina-Molina, M, Antoniou, KM, and Poletti, V
Published: 2019

35. Gaps in care of patients living with pulmonary fibrosis: a joint patient and expert statement on the results of a Europe-wide survey

Author: Moor, Karen, Wijsenbeek - Lourens, Marlies, Balestro, E, Biondini, D, Bondue, B, Cottin, V, Kreuter, M, Bonella, F, Moor, Karen, Wijsenbeek - Lourens, Marlies, Balestro, E, Biondini, D, Bondue, B, Cottin, V, Kreuter, M, and Bonella, F
Published: 2019

36. Cardiovascular safety of nintedanib in subgroups by cardiovascular risk at baseline in the TOMORROW and INPULSIS trials

Author: Noth, I, Wijsenbeek - Lourens, Marlies, Kolb, M, Bonella, F, Moros, L, Wachtlin, D, Corte, TJ, Noth, I, Wijsenbeek - Lourens, Marlies, Kolb, M, Bonella, F, Moros, L, Wachtlin, D, and Corte, TJ
Published: 2019

37. The burden of sarcoidosis symptoms from a patient perspective

Author: Voortman, M., Hendriks, C. M. R., Elfferich, M. D. P., Bonella, F., Møller, J., De Vries, J., Costabel, U., Drent, M., Voortman, M., Hendriks, C. M. R., Elfferich, M. D. P., Bonella, F., Møller, J., De Vries, J., Costabel, U., and Drent, M.
Abstract: Purpose The clinical manifestations of sarcoidosis vary widely, depending on the intensity of the inflammation and the organ systems affected. Hence, sarcoidosis patients may suffer from a great variety of symptoms. The aim of this study was to compare the self-reported burden of sarcoidosis patients in Denmark, Germany and the Netherlands, especially the prevalence of fatigue and small fiber neuropathy (SFN)-related symptoms, as well as differences in treatment strategies. Methods A cross-sectional web-based anonymous survey about complaints was conducted among sarcoidosis patients. Patients were invited to take part through the sarcoidosis patient societies as well as through outpatient sarcoidosis clinics in these countries. Results The questionnaire was completed by 1072 sarcoidosis patients (152 Danish, 532 German and 388 Dutch). Almost all patients reported having sarcoidosis-associated symptoms (organ-related as well as non-specific, non-organ related). Fatigue was reported by almost all respondents (90%), followed by pulmonary symptoms (72.4%). More than 50% of the respondents were being treated with prednisone, which was comparable in all three countries. In contrast, second- and third-line treatment differed substantially between Denmark, Germany and the Netherlands. Conclusion Sarcoidosis patients in Denmark, Germany and the Netherlands present with similar self-reported symptoms, organ-related as well as non-specific, non-organ related. Fatigue (90%) and symptoms associated with SFN (86%) were highly prevalent in all three countries.
Published: 2019

38. Correction to: The Burden of Sarcoidosis Symptoms from a Patient Perspective (Lung, (2019), 197, 2, (155-161), 10.1007/s00408-019-00206-7)

Author: Longziekten, Voortman, M., Hendriks, C. M.R., Elfferich, M. D.P., Bonella, F., Møller, J., De Vries, J., Costabel, U., Drent, M., Longziekten, Voortman, M., Hendriks, C. M.R., Elfferich, M. D.P., Bonella, F., Møller, J., De Vries, J., Costabel, U., and Drent, M.
Published: 2019

39. Research highlights from the 2018 ERS International Congress

Author: Alfaro, T.M. (Tiago), Moor, C.C. (Karen), Alfieri, V. (Veronica), Jeny, F. (Florence), Kreuter, M. (Michael), Wijsenbeek-Lourens, M.S. (Marlies), Renzoni, E. A. (Elisabetta), Bargagli, E. (Elena), Nunes, H. (Hilario), Spagnolo, P. (Paolo), Bonella, F. (Francesco), Molina-Molina, M. (Maria), Antoniou, K.M. (Katerina), Poletti, V. (Venerino), Alfaro, T.M. (Tiago), Moor, C.C. (Karen), Alfieri, V. (Veronica), Jeny, F. (Florence), Kreuter, M. (Michael), Wijsenbeek-Lourens, M.S. (Marlies), Renzoni, E. A. (Elisabetta), Bargagli, E. (Elena), Nunes, H. (Hilario), Spagnolo, P. (Paolo), Bonella, F. (Francesco), Molina-Molina, M. (Maria), Antoniou, K.M. (Katerina), and Poletti, V. (Venerino)
Abstract: This article reviews a selection of the scientific presentations on interstitial lung disease (ILD)/diffuse parenchymal lung disease (DPLD) that were made at the 2018 European Respiratory Society (ERS) International Congress in Paris. A number of advances in the epidemiology, pathogenesis, diagnosis and treatment of these disorders were presented and discussed by clinicians and researchers. The research topics span over all four groups of ERS Assembly 12: Interstitial Lung Diseases.
Published: 2018
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40. European Respiratory Society International Congress 2017: highlights from the Clinical Assembly

Author: Kahn, N, Mekov, E, Fregonese, L, Andrianopoulos, V, Franssen, F, Grgic, A, Coolen, J, Bonta, P, Gompelmann, D, Annema, J, Faverio, P, Bonella, F, Daines, L, Pinnock, H, Kocks, J, Herth, F, Kahn, Nicolas, Mekov, Evgeni, Fregonese, Laura, Andrianopoulos, Vasileios, Franssen, Frits M E, Grgic, Aleksandar, Coolen, Johan, Bonta, Peter I, Gompelmann, Daniela, Annema, Jouke T, Faverio, Paola, Bonella, Francesco, Daines, Luke, Pinnock, Hillary, Kocks, Janwillem, Herth, Felix, Kahn, N, Mekov, E, Fregonese, L, Andrianopoulos, V, Franssen, F, Grgic, A, Coolen, J, Bonta, P, Gompelmann, D, Annema, J, Faverio, P, Bonella, F, Daines, L, Pinnock, H, Kocks, J, Herth, F, Kahn, Nicolas, Mekov, Evgeni, Fregonese, Laura, Andrianopoulos, Vasileios, Franssen, Frits M E, Grgic, Aleksandar, Coolen, Johan, Bonta, Peter I, Gompelmann, Daniela, Annema, Jouke T, Faverio, Paola, Bonella, Francesco, Daines, Luke, Pinnock, Hillary, Kocks, Janwillem, and Herth, Felix
Abstract: This article contains highlights and a selection of the scientific advances from the European Respiratory Society's Clinical Assembly (Assembly 1 and its six respective groups) that were presented at the 2017 European Respiratory Society International Congress in Milan, Italy. The most relevant topics from each of the groups will be discussed, covering a wide range of areas including clinical problems, rehabilitation and chronic care, thoracic imaging, interventional pulmonology, diffuse and parenchymal lung diseases, and general practice and primary care. In this comprehensive review, the newest research and actual data as well as award-winning abstracts and highlight sessions will be discussed.
Published: 2018

41. European Respiratory Society International Congress 2017: highlights from the Clinical Assembly

Author: Kahn, N, Mekov, E, Fregonese, L, Andrianopoulos, V, Franssen, F, Grgic, A, Coolen, J, Bonta, P, Gompelmann, D, Annema, J, Faverio, P, Bonella, F, Daines, L, Pinnock, H, Kocks, J, Herth, F, Kahn, Nicolas, Mekov, Evgeni, Fregonese, Laura, Andrianopoulos, Vasileios, Franssen, Frits M E, Grgic, Aleksandar, Coolen, Johan, Bonta, Peter I, Gompelmann, Daniela, Annema, Jouke T, Faverio, Paola, Bonella, Francesco, Daines, Luke, Pinnock, Hillary, Kocks, Janwillem, Herth, Felix, Kahn, N, Mekov, E, Fregonese, L, Andrianopoulos, V, Franssen, F, Grgic, A, Coolen, J, Bonta, P, Gompelmann, D, Annema, J, Faverio, P, Bonella, F, Daines, L, Pinnock, H, Kocks, J, Herth, F, Kahn, Nicolas, Mekov, Evgeni, Fregonese, Laura, Andrianopoulos, Vasileios, Franssen, Frits M E, Grgic, Aleksandar, Coolen, Johan, Bonta, Peter I, Gompelmann, Daniela, Annema, Jouke T, Faverio, Paola, Bonella, Francesco, Daines, Luke, Pinnock, Hillary, Kocks, Janwillem, and Herth, Felix
Abstract: This article contains highlights and a selection of the scientific advances from the European Respiratory Society's Clinical Assembly (Assembly 1 and its six respective groups) that were presented at the 2017 European Respiratory Society International Congress in Milan, Italy. The most relevant topics from each of the groups will be discussed, covering a wide range of areas including clinical problems, rehabilitation and chronic care, thoracic imaging, interventional pulmonology, diffuse and parenchymal lung diseases, and general practice and primary care. In this comprehensive review, the newest research and actual data as well as award-winning abstracts and highlight sessions will be discussed.
Published: 2018

42. Timing of onset affects arthritis presentation pattern in antisyntethase syndrome

Author: González-Gay, M, Montecucco, C, Selva-O'Callaghan, A, Trallero-Araguas, E, Molberg, O, Andersson, H, Rojas-Serrano, J, Perez-Roman, D, Bauhammer, J, Fiehn, C, Neri, R, Barsotti, S, Lorenz, H, Doria, A, Ghirardello, A, Iannone, F, Giannini, M, Franceschini, F, Cavazzana, I, Triantafyllias, K, Benucci, M, Infantino, M, Manfredi, M, Conti, F, Schwarting, A, Sebastiani, G, Iuliano, A, Emmi, G, Silvestri, E, Govoni, M, Scirè, C, Furini, F, Lopez-Longo, F, Martínez-Barrio, J, Sebastiani, M, Manfredi, A, Bachiller-Corral, J, Sifuentes Giraldo, W, Cimmino, M, Cosso, C, Belotti Masserini, A, Cagnotto, G, Codullo, V, Romano, M, Paolazzi, G, Pellerito, R, Saketkoo, L, Ortego-Centeno, N, Quartuccio, L, Batticciotto, A, Bartoloni Bocci, E, Gerli, R, Specker, C, Bravi, E, Selmi, C, Parisi, S, Salaffi, F, Meloni, F, Marchioni, E, Pesci, A, Dei, G, Confalonieri, M, Tomietto, P, Nuno, L, Bonella, F, Pipitone, N, Mera-Valera, A, Perez-Gomez, N, Gerzeli, S, Lopez-Mejias, R, Matos-Costa, C, Pereira da Silva, J, Cifrian, J, Alpini, C, Olivieri, I, Blázquez Cañamero, M, Rodriguez Cambrón, A, Castañeda, S, Cavagna, L, González-Gay, Miguel A, Montecucco, Carlomaurizio, Selva-O'Callaghan, Albert, Trallero-Araguas, Ernesto, Molberg, Ovynd, Andersson, Helena, Rojas-Serrano, Jorge, Perez-Roman, Diana Isabel, Bauhammer, Jutta, Fiehn, Christoph, Neri, Rossella, Barsotti, Simone, Lorenz, Hannes M, Doria, Andrea, Ghirardello, Anna, Iannone, Florenzo, Giannini, Margherita, Franceschini, Franco, Cavazzana, Ilaria, Triantafyllias, Konstantinos, Benucci, Maurizio, Infantino, Maria, Manfredi, Mariangela, Conti, Fabrizio, Schwarting, Andreas, Sebastiani, Giandomenico, Iuliano, Annamaria, Emmi, Giacomo, Silvestri, Elena, Govoni, Marcello, Scirè, Carlo Alberto, Furini, Federica, Lopez-Longo, Francisco Javier, Martínez-Barrio, Julia, Sebastiani, Marco, Manfredi, Andreina, Bachiller-Corral, Javier, Sifuentes Giraldo, Walter Alberto, Cimmino, Marco A, Cosso, Claudio, Belotti Masserini, Alessandro, Cagnotto, Giovanni, Codullo, Veronica, Romano, Mariaeva, Paolazzi, Giuseppe, Pellerito, Raffaele, Saketkoo, Lesley Ann, Ortego-Centeno, Norberto, Quartuccio, Luca, Batticciotto, Alberto, Bartoloni Bocci, Elena, Gerli, Roberto, Specker, Christof, Bravi, Elena, Selmi, Carlo, Parisi, Simone, Salaffi, Fausto, Meloni, Federica, Marchioni, Enrico, Pesci, Alberto, Dei, Giulia, Confalonieri, Marco, Tomietto, Paola, Nuno, Laura, Bonella, Francesco, Pipitone, Nicolò, Mera-Valera, Antonio, Perez-Gomez, Nair, Gerzeli, Simone, Lopez-Mejias, Raquel, Matos-Costa, Carlo Jorge, Pereira da Silva, Jose Antonio, Cifrian, José, Alpini, Claudia, Olivieri, Ignazio, Blázquez Cañamero, María Ángeles, Rodriguez Cambrón, Ana Belén, Castañeda, Santos, Cavagna, Lorenzo, González-Gay, M, Montecucco, C, Selva-O'Callaghan, A, Trallero-Araguas, E, Molberg, O, Andersson, H, Rojas-Serrano, J, Perez-Roman, D, Bauhammer, J, Fiehn, C, Neri, R, Barsotti, S, Lorenz, H, Doria, A, Ghirardello, A, Iannone, F, Giannini, M, Franceschini, F, Cavazzana, I, Triantafyllias, K, Benucci, M, Infantino, M, Manfredi, M, Conti, F, Schwarting, A, Sebastiani, G, Iuliano, A, Emmi, G, Silvestri, E, Govoni, M, Scirè, C, Furini, F, Lopez-Longo, F, Martínez-Barrio, J, Sebastiani, M, Manfredi, A, Bachiller-Corral, J, Sifuentes Giraldo, W, Cimmino, M, Cosso, C, Belotti Masserini, A, Cagnotto, G, Codullo, V, Romano, M, Paolazzi, G, Pellerito, R, Saketkoo, L, Ortego-Centeno, N, Quartuccio, L, Batticciotto, A, Bartoloni Bocci, E, Gerli, R, Specker, C, Bravi, E, Selmi, C, Parisi, S, Salaffi, F, Meloni, F, Marchioni, E, Pesci, A, Dei, G, Confalonieri, M, Tomietto, P, Nuno, L, Bonella, F, Pipitone, N, Mera-Valera, A, Perez-Gomez, N, Gerzeli, S, Lopez-Mejias, R, Matos-Costa, C, Pereira da Silva, J, Cifrian, J, Alpini, C, Olivieri, I, Blázquez Cañamero, M, Rodriguez Cambrón, A, Castañeda, S, Cavagna, L, González-Gay, Miguel A, Montecucco, Carlomaurizio, Selva-O'Callaghan, Albert, Trallero-Araguas, Ernesto, Molberg, Ovynd, Andersson, Helena, Rojas-Serrano, Jorge, Perez-Roman, Diana Isabel, Bauhammer, Jutta, Fiehn, Christoph, Neri, Rossella, Barsotti, Simone, Lorenz, Hannes M, Doria, Andrea, Ghirardello, Anna, Iannone, Florenzo, Giannini, Margherita, Franceschini, Franco, Cavazzana, Ilaria, Triantafyllias, Konstantinos, Benucci, Maurizio, Infantino, Maria, Manfredi, Mariangela, Conti, Fabrizio, Schwarting, Andreas, Sebastiani, Giandomenico, Iuliano, Annamaria, Emmi, Giacomo, Silvestri, Elena, Govoni, Marcello, Scirè, Carlo Alberto, Furini, Federica, Lopez-Longo, Francisco Javier, Martínez-Barrio, Julia, Sebastiani, Marco, Manfredi, Andreina, Bachiller-Corral, Javier, Sifuentes Giraldo, Walter Alberto, Cimmino, Marco A, Cosso, Claudio, Belotti Masserini, Alessandro, Cagnotto, Giovanni, Codullo, Veronica, Romano, Mariaeva, Paolazzi, Giuseppe, Pellerito, Raffaele, Saketkoo, Lesley Ann, Ortego-Centeno, Norberto, Quartuccio, Luca, Batticciotto, Alberto, Bartoloni Bocci, Elena, Gerli, Roberto, Specker, Christof, Bravi, Elena, Selmi, Carlo, Parisi, Simone, Salaffi, Fausto, Meloni, Federica, Marchioni, Enrico, Pesci, Alberto, Dei, Giulia, Confalonieri, Marco, Tomietto, Paola, Nuno, Laura, Bonella, Francesco, Pipitone, Nicolò, Mera-Valera, Antonio, Perez-Gomez, Nair, Gerzeli, Simone, Lopez-Mejias, Raquel, Matos-Costa, Carlo Jorge, Pereira da Silva, Jose Antonio, Cifrian, José, Alpini, Claudia, Olivieri, Ignazio, Blázquez Cañamero, María Ángeles, Rodriguez Cambrón, Ana Belén, Castañeda, Santos, and Cavagna, Lorenzo
Abstract: Objective To evaluate if the timing of appearance with respect to disease onset may influence the arthritis presentation pattern in antisynthetase syndrome (ASSD). Methods The patients were selected from a retrospective large international cohort of ASSD patients regularly followed-up in centres referring to AENEAS collaborative group. Patients were eligible if they had an antisynthetase antibody testing positive in at least two determinations along with arthritis occurring either at ASSD onset (Group 1) or during the course of the disease (Group 2). Results 445 (70%; 334 females, 110 males, 1 transsexual) out of the 636 ASSD we collected had arthritis, in the majority of cases (367, 83%) from disease onset (Group 1). Patients belonging to Group 1 with respect to Group 2 had an arthritis more commonly polyarticular and symmetrical (p=0.015), IgM-Rheumatoid factor positive (p=0.035), erosions at hands and feet plain x-rays (p=0.036) and more commonly satisfying the 1987 revised classification criteria for rheumatoid arthritis (RA) (p=0.004). Features such as Raynaud's phenomenon, mechanic's hands and fever (e.g. accompanying findings) were more frequently reported in Group 2 (p=0.005). Conclusion In ASSD, the timing of appearance with respect to disease onset influences arthritis characteristics. In particular, RA features are more common when arthritis occurs from ASSD onset, suggesting an overlap between RA and ASSD in these patients. When arthritis appears during the follow-up, it is very close to a connective tissue disease-related arthritis. Also, the different prevalence of accompanying features between these two groups is in line with this possibility
Published: 2018

43. DIFFUSING CAPACITY AND CLINICAL CHARACTERISTICS OF PATIENTS WITH SYSTEMIC SCLEROSIS - DATA FROM THE GERMAN NETWORK FOR SYSTEMIC SCLEROSIS

Author: Moinzadeh, P., Blank, N., Siegert, E., Henes, J., Worm, M., Sunderkoetter, C., Schmalzing, M., Kreuter, A., Gunther, 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., Hunzelmann, N., Bonella, F., Kreuter, M., Moinzadeh, P., Blank, N., Siegert, E., Henes, J., Worm, M., Sunderkoetter, C., Schmalzing, M., Kreuter, A., Gunther, 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., Hunzelmann, N., Bonella, F., and Kreuter, M.
Published: 2017

44. Zeitlicher Verlauf klinischer Manifestationen im Vergleich zwischen PL-7-, PL-12- und EJ-positiven Patienten mit Antisynthetase Syndrom

Author: Triantafyllias, K, Cavagna, L, Bauhammer, J, Delbrück, C, Drott, U, Specker, C, Weinmann-Menke, J, Wendel, S, Voll, R, Bonella, F, Lorenz, HM, Lütgendorf, F, Castaneda, S, Gonzalez-Gay, MA, Selva-O' Callaghan, A, Schwarting, A, Triantafyllias, K, Cavagna, L, Bauhammer, J, Delbrück, C, Drott, U, Specker, C, Weinmann-Menke, J, Wendel, S, Voll, R, Bonella, F, Lorenz, HM, Lütgendorf, F, Castaneda, S, Gonzalez-Gay, MA, Selva-O' Callaghan, A, and Schwarting, A
Published: 2017

45. Zeitlicher Verlauf klinischer Manifestationen im Vergleich zwischen PL-7-, PL-12- und EJ-positiven Patienten mit Antisynthetase Syndrom

Author: Triantafyllias, K, Cavagna, L, Bauhammer, J, Delbrück, C, Drott, U, Specker, C, Weinmann-Menke, J, Wendel, S, Voll, R, Bonella, F, Lorenz, HM, Lütgendorf, F, Castaneda, S, Gonzalez-Gay, MA, Selva-O' Callaghan, A, Schwarting, A, Triantafyllias, K, Cavagna, L, Bauhammer, J, Delbrück, C, Drott, U, Specker, C, Weinmann-Menke, J, Wendel, S, Voll, R, Bonella, F, Lorenz, HM, Lütgendorf, F, Castaneda, S, Gonzalez-Gay, MA, Selva-O' Callaghan, A, and Schwarting, A
Published: 2017

46. DIFFUSING CAPACITY AND CLINICAL CHARACTERISTICS OF PATIENTS WITH SYSTEMIC SCLEROSIS - DATA FROM THE GERMAN NETWORK FOR SYSTEMIC SCLEROSIS

Author: Moinzadeh, P., Blank, N., Siegert, E., Henes, J., Worm, M., Sunderkoetter, C., Schmalzing, M., Kreuter, A., Gunther, 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., Hunzelmann, N., Bonella, F., Kreuter, M., Moinzadeh, P., Blank, N., Siegert, E., Henes, J., Worm, M., Sunderkoetter, C., Schmalzing, M., Kreuter, A., Gunther, 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., Hunzelmann, N., Bonella, F., and Kreuter, M.
Published: 2017

47. Whole lung lavage therapy for pulmonary alveolar proteinosis: a global survey of current practices and procedures

Author: University of Helsinki, Keuhkosairaudet (-2009), Campo, I., Luisetti, M., Griese, M., Trapnell, B.C., Bonella, F., Grutters, J., Nakata, K., Van Moorsel, C.H., Costabel, U., Cottin, V., Ichiwata, T., Inoue, Y., Braschi, A., Bonizzoni, G., Iotti, G.A., Tinelli, C., Rodi, G., Halme, Maija, University of Helsinki, Keuhkosairaudet (-2009), Campo, I., Luisetti, M., Griese, M., Trapnell, B.C., Bonella, F., Grutters, J., Nakata, K., Van Moorsel, C.H., Costabel, U., Cottin, V., Ichiwata, T., Inoue, Y., Braschi, A., Bonizzoni, G., Iotti, G.A., Tinelli, C., Rodi, G., and Halme, Maija
Published: 2016

48. Zeitlicher Verlauf klinischer Manifestationen bei Jo-1-negativen Antisynthetase-Syndrom Patienten

Author: Triantafyllias, K, Specker, C, Cavagna, L, Bonella, F, Menke, J, Flaig, W, Lütgendorf, F, Gonzalez-Gay, MA, Castaneda, S, Sire, C, Bauhammer, J, Fiehn, C, Schwarting, A, Triantafyllias, K, Specker, C, Cavagna, L, Bonella, F, Menke, J, Flaig, W, Lütgendorf, F, Gonzalez-Gay, MA, Castaneda, S, Sire, C, Bauhammer, J, Fiehn, C, and Schwarting, A
Published: 2016

49. Zeitlicher Verlauf klinischer Manifestationen bei Jo-1-negativen Antisynthetase-Syndrom Patienten

Author: Triantafyllias, K, Specker, C, Cavagna, L, Bonella, F, Menke, J, Flaig, W, Lütgendorf, F, Gonzalez-Gay, MA, Castaneda, S, Sire, C, Bauhammer, J, Fiehn, C, Schwarting, A, Triantafyllias, K, Specker, C, Cavagna, L, Bonella, F, Menke, J, Flaig, W, Lütgendorf, F, Gonzalez-Gay, MA, Castaneda, S, Sire, C, Bauhammer, J, Fiehn, C, and Schwarting, A
Published: 2016

50. Pharmacological Treatment of Idiopathic Pulmonary Fibrosis: Current Approaches, Unsolved Issues, and Future Perspectives

Author: Kreuter, M. (Michael), Bonella, F. (Francesco), Wijsenbeek-Lourens, M.S. (Marlies), Maher, T.M. (Toby M.), Spagnolo, P. (Paolo), Kreuter, M. (Michael), Bonella, F. (Francesco), Wijsenbeek-Lourens, M.S. (Marlies), Maher, T.M. (Toby M.), and Spagnolo, P. (Paolo)
Abstract: Idiopathic pulmonary fibrosis (IPF) is a devastating condition with a 5-year survival of approximately 20%. The disease primarily occurs in elderly patients. IPF is a highly heterogeneous disorder with a clinical course that varies from prolonged periods of stability to episodes of rapid deterioration. In the last decade, improved understanding of disease mechanisms along with a more precise disease definition has allowed the design and completion of a number of high-quality clinical trials. Yet, until recently, IPF was essentially an untreatable disease. Finally, pirfenidone and nintedanib, two compounds with antifibrotic properties, have consistently proven effective in reducing functional decline and disease progression in IPF. This is a major breakthrough for patients and physicians alike, but there is still a long way to go. In fact, neither pirfenidone nor nintedanib is a cure for IPF, and most patients continue to progress despite treatment. As such, comprehensive care of patients with IPF, including manage
Published: 2015
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