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1. Idiopathic pulmonary fibrosis: current treatment options and critical appraisal of nintedanib

Author

Bonella F, Stowasser S, and Wollin L

Subjects
Tyrosine kinase, disease progression, treatment outcome, usual interstitial pneumonia, therapeutics, Therapeutics. Pharmacology, RM1-950
Abstract

Francesco Bonella,1 Susanne Stowasser,2 Lutz Wollin3 1Interstitial and Rare Lung Disease Unit, Ruhrlandklinik, University Hospital, University of Duisburg-Essen, Essen, 2Boehringer Ingelheim Pharma GmbH & Co. KG, Ingelheim am Rhein, 3Boehringer Ingelheim Pharma GmbH & Co. KG, Biberach an der Riss, Germany Abstract: Idiopathic pulmonary fibrosis (IPF) is the most common type of idiopathic interstitial pneumonia and is characterized by a poor prognosis, with an estimated 5-year survival of approximately 20%. Progressive and irreversible lung functional impairment leads to chronic respiratory insufficiency with a severely impaired quality of life. In the last 2 decades, novel treatments for IPF have been developed as a consequence of an increasing understanding of disease pathogenesis and pathobiology. In IPF, injured dysfunctional alveolar epithelial cells promote fibroblast recruitment and proliferation, resulting in scarring of the lung tissue. Recently, pirfenidone and nintedanib have been approved for the treatment of IPF, having shown efficacy to slow functional decline and disease progression. This article focuses on the pharmacologic characteristics and clinical evidence supporting the use of nintedanib, a potent small-molecule tyrosine kinase inhibitor, as therapy for IPF. After introducing the mechanism of action and pharmacokinetics, an overview of the safety and efficacy results from the most recent clinical trials of nintedanib in IPF is presented. Keywords: tyrosine kinase, disease progression, treatment outcome, usual interstitial pneumonia, therapeutics

Published
2015

2. Update on therapeutic management of idiopathic pulmonary fibrosis

Author

Tzouvelekis A, Bonella F, and Spagnolo P

Subjects
Therapeutics. Pharmacology, RM1-950
Abstract

Argyris Tzouvelekis,1 Francesco Bonella,2 Paolo Spagnolo31Department of Internal Medicine, Section of Pulmonary, Critical Care and Sleep Medicine, Yale School of Medicine, New Haven, CT, USA; 2Interstitial and Rare Lung Disease Unit, Ruhrlandklinik, University Hospital, University of Duisburg-Essen, Germany; 3Medical University Clinic, Canton Hospital Baselland and University of Basel, Liestal, SwitzerlandAbstract: Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive diffuse parenchymal lung disease of unknown origin, with a mortality rate exceeding that of many cancers. The diagnostic process is complex and relies on the clinician integrating clinical, laboratory, radiological, and histological data. In the last decade, major advances in our understanding of the pathogenesis of IPF have shifted the paradigm from a primarily inflammatory process evolving to fibrosis to a condition driven by aberrant wound healing following alveolar epithelial cell injury that results in scarring of the lung, architectural distortion, and irreversible loss of function. Improved understanding of disease pathogenesis has led to the identification of several therapeutic targets and the design of high-quality clinical trials evaluating novel compounds. However, the results of these studies have been mostly disappointing, probably due to the plethora of mediators, growth factors, and signaling pathways involved in the fibrotic process. Most recently, pirfenidone and nintedanib, two compounds with pleiotropic anti-fibrotic properties, have been proven effective in reducing functional decline and disease progression in IPF. This is a major breakthrough. Nevertheless, we still have 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 management of concomitant conditions and physical debility, as well as timely referral for lung transplantation, remains essential. Several agents with a high potential are currently being tested, and many more are ready for clinical trials. Their completion is critical for achieving the ultimate goal of curing patients with IPF.Keywords: pulmonary fibrosis, therapy, nintedanib, pirfenidone, treatment

Published
2015

3. Comparison of a 22G Crown-Cut Needle with a Conventional 22G Needle with EBUS Guidance in Diagnosis of Sarcoidosis

Author

Wälscher, J., Büscher, E., Bonella, F., Karpf-Wissel, R., Costabel, U., Theegarten, D., and Rawitzer, J.

Subjects
Medical tests -- Evaluation, Sarcoidosis -- Diagnosis -- Care and treatment, Health
Abstract

Introduction Endobronchial ultrasound transbronchial needle aspiration (EBUS-TBNA) is a standard procedure in cases of enlarged mediastinal lymph nodes. Recently, new tools were developed aiming to improve the diagnostic yield. A novel crown-cut needle is considered to obtain tissue cores which can be beneficial for the evaluation by the pathologist. This study aimed to compare the novel 22G crown-cut needle with a conventional 22G needle with EBUS guidance in the diagnosis of sarcoidosis. Methods We designed a single-center prospective randomized clinical trial between March 2020 and January 2021 with 30 patients with mediastinal lymphadenopathy and suspected sarcoidosis. Results 24 patients (mean age 49.5 vs 54.1, mean FVC 73.7% vs 86.7%, mean DLCO 72.4% vs 72.5% for crown-cut needle vs conventional needle, respectively) were diagnosed with sarcoidosis. In the remaining six patients, sarcoidosis was reasonably excluded. The diagnostic yield for sarcoidosis was 77% with the crown-cut needle vs. 82% with the conventional needle (p > 0.05). In patients with histopathologic hallmarks typical of sarcoidosis (n = 19), the crown-cut needle was superior in detecting granulomas (8.3 vs 3.8 per cytoblock, p < 0.05) and histiocytes (502 vs 186 per cytoblock, p < 0.05). Four of seven bronchoscopists experienced difficulties passing through the bronchial wall with the crown-cut needle and one episode of bleeding occurred in this group which made interventions necessary. Conclusions Despite equivalence in diagnostic accuracy, the crown-cut needle was superior to the conventional needle in detecting granulomas and histiocytes. This indicates greater potential for obtaining higher quality sample material with the crown-cut needle in cases of granulomatous inflammation., Author(s): J. Wälscher [sup.1] , E. Büscher [sup.2] , F. Bonella [sup.1] , R. Karpf-Wissel [sup.2] , U. Costabel [sup.1] , D. Theegarten [sup.3] , J. Rawitzer [sup.3] , J. [...]

Published
2022
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4. Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia (DIPNECH): An International Case Series

Author

Penugonda, M., primary, O'Callaghan, M., additional, Diesler, R., additional, O'Brien, H., additional, Almeamar, H., additional, Samhouri, B.F., additional, Ryu, J.H., additional, Bendstrup, E., additional, Lund, T.K., additional, Schultz, H.H.L., additional, Veltkamp, M., additional, Manali, E.D., additional, Lazaratou, A., additional, Papiris, S.A., additional, Bonella, F., additional, Carr, L., additional, Cottin, V., additional, Joubert, P., additional, Taillé, C., additional, Mccormack, F.X., additional, Gupta, N., additional, Strosberg, J., additional, Lococo, F.M., additional, Spagnolo, P., additional, Franciosi, A.N., additional, O' Toole, D., additional, Murphy, D.J., additional, Crowley, R., additional, Fabre, A., additional, Keane, M.P., additional, Fournel, L., additional, and McCarthy, C., additional

Published
2024
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5. Evaluating the Performance of an Ensemble Neural Network Pipeline in the Classification of Chest CT Scans as Control, Diffuse Cystic Lung Disease and Emphysema

Author

Noonan, K.J., primary, Hearne, R.T., additional, Murphy, D.J., additional, Gaffney, B., additional, Sheehy, B., additional, McVeigh, N., additional, Franciosi, A.N., additional, Keane, M.P., additional, Ataya, A., additional, Gupta, N., additional, Mccormack, F.X., additional, Bonella, F., additional, Borie, R., additional, Lynch, D.A., additional, Currran, K., additional, and Mccarthy, C., additional

Published
2024
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6. 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

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., Crestani, B., Criner, G., Dahlqvist, C., Danoff, S., Dematte D'Amico, J., Dilling, D., Elias, P., Ettinger, N., Falk, J., Fernández Pérez, E.R., Gamez-Dubuis, A., Giessel, G., Gifford, A., Glassberg, M., Glazer, C., Golden, J., Gómez Carrera, L., Guiot, J., Hallowell, R., Hayashi, H., Hetzel, J., Hirani, N., Homik, L., Hope-Gill, B., Hotchkin, D., Ichikado, K., Ilkovich, M., Inoue, Y., Izumi, S., Jassem, E., Jones, L., Jouneau, S., Kaner, R., Kang, J., Kawamura, T., Kessler, R., Kim, Y., Kishi, K., Kitamura, H., Kolb, M., Kondoh, Y., Kono, C., Koschel, D., Kreuter, M., Kulkarni, T., Kus, J., Lebargy, F., León Jiménez, A., Luo, Q., Mageto, Y., Maher, T.M., Makino, S., Marchand-Adam, S., Marquette, C., Martinez, R., Martínez, M., Maturana Rozas, R., Miyazaki, Y., Moiseev, S., Molina-Molina, M., Morrison, L., Morrow, L., Moua, T., Nambiar, A., Nishioka, Y., Nunes, H., Okamoto, M., Oldham, J., Otaola, M., Padilla, M., Park, J.S., Patel, N., Pesci, A., Piotrowski, W., Pitts, L., Poonyagariyagorn, H., Prasse, A., Quadrelli, S., Randerath, W., Refini, R., Reynaud-Gaubert, M., Riviere, F., Rodríguez Portal, J.A., Rosas, I., Rossman, M., Safdar, Z., Saito, T., Sakamoto, N., Salinas Fénero, 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, F., Veeraraghavan, S., Villar, A., Weigt, S., Wemeau, L., Wuyts, W., Xu, Z., Yakusevich, V., Yamada, Y., Yamauchi, H., Ziora, D., Wells, Athol U, Flaherty, Kevin R, Brown, Kevin K, Inoue, Yoshikazu, Devaraj, Anand, Richeldi, Luca, Moua, Teng, Crestani, Bruno, Wuyts, Wim A, Stowasser, Susanne, Quaresma, Manuel, Goeldner, Rainer-Georg, Schlenker-Herceg, Rozsa, and Kolb, Martin

Published
2020
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9. 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., and Costabel, U.

Subjects
Sarcoidosis -- Risk factors -- Prognosis, Perception (Psychology) -- Analysis, Quality of life -- Analysis, Health
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., Author(s): M. Voortman [sup.1] [sup.2] [sup.3] , C. M. R. Hendriks [sup.1] [sup.3] [sup.4] , M. D. P. Elfferich [sup.3] , F. Bonella [sup.5] , J. Møller [sup.6] , J. [...]

Published
2019
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10. 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

15. AI Techniques to Differentiate Between Sub-Groups of Diffuse Cystic Lung Diseases Using Phenotypic Characteristics

Author

Hearne, R.T., primary, Noonan, K., additional, Murphy, D.J., additional, Gaffney, B., additional, Sheehy, B., additional, McVeigh, N., additional, Ataya, A., additional, Gupta, N., additional, Mccormack, F.X., additional, Bonella, F., additional, Nensa, F., additional, Keane, M.P., additional, Borie, R., additional, Mccarthy, C., additional, and Curran, K.M., additional

Published
2023
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26. 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

27. Therapie von Komplikationen und nichtpharmakologisches Management der idiopathischen pulmonalen Fibrose

Author

Gläser, S., Glöckl, R., and Bonella, F.

Abstract

Komplikationen der idiopathischen pulmonalen Fibrose (IPF) sind für einen relevanten Mortalitätsanteil verantwortlich. Als wichtigstes Beispiel ist diesbezüglich die akute Exazerbation anzuführen, deren Krankenhausletalität über 50 % beträgt bei einem mittleren Überleben von nur wenigen Monaten. Somit kommt der Betrachtung von Komplikationen eine große Bedeutung für Krankheitsverständnis sowie Therapieplanung zu. Des Weiteren ist in den letzten Jahren die Evidenz für pneumologische Rehabilitation bei IPF deutlich gestiegen und wird von der Amerikanischen und Europäischen Gesellschaft für Pneumologie (American Thoracic Society [ATS]/European Respiratory Society [ERS]) zur Verbesserung der körperlichen Leistungsfähigkeit, Lebensqualität und der Symptome empfohlen.

Published
2024
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29. COVID-19 in patients with Pulmonary Alveolar Proteinosis A European multicenter study

Author

Papiris, S, primary, Campo, I, additional, Mariani, F, additional, Kallieri, M, additional, Kolilekas, L, additional, Papaioannou, A I, additional, Chousein, E G, additional, Cetinkaya, E, additional, Bonella, F, additional, Borie, R, additional, Kokosi, M, additional, Pickworth, T, additional, Molina-Molina, M, additional, Gasa, M, additional, Radzikowska, E, additional, Fijolek, J, additional, Jouneau, S, additional, Gomez, E, additional, Mccarthy, C, additional, Bendstrup, E, additional, Piotrowski, W J, additional, Pabary, R, additional, Hadchouel, A, additional, Coolen-Allou, N, additional, Alfaro, T, additional, Cordeiro, C R, additional, Antonogiannaki, E, additional, Tomos, I P, additional, Papakosta, D, additional, Kontakiotis, T, additional, Panagiotou, P, additional, Douros, K, additional, Schams, A, additional, Lettieri, S, additional, Papaevangelou, V, additional, Kanaka-Gantenbein, C, additional, Karakatsani, A, additional, Loukides, S, additional, Costabel, U, additional, Crestani, B, additional, Morgan, C, additional, Tazawa, R, additional, Bush, A, additional, Griese, M, additional, and Manali, E D, additional

Published
2022
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30. Association of mUC5B rs35705950 minor allele with age and survival in European patients with Idiopathic Pulmonary Fibrosis

Author

van der Vis, J, primary, Prasse, A, additional, Renzoni, E, additional, Stock, C, additional, Caliskan, C, additional, Maher, T, additional, Bonella, F, additional, Borie, R, additional, Crestani, B, additional, Petrek, M, additional, Wuyts, W, additional, Wind, A, additional, Molyneaux, P, additional, Grutters, J, additional, and Van Moorsel, C, additional

Published
2022
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36. 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., 3rd International Summit for ILD (ISILD), Erice collaborators listed below: Carlo Albera, Goksel, Altinisik, Kjetil, Ask, Elisabetta, Balestro, Elena, Bargagli, Elisabeth, Bendstrup, Marialuisa, Bocchino, Francesco, Bonella, Martina, Bonifazi, Giulia, Cacopardo, Maria, Calvello, Diego, M Castillo, Nazia, Chaudhuri, Ulrich, Costabel, Vincent, Cottin, Bruno, Crestani, Manuela, Funke-Chambour, Jack, Gauldie, Peter, M George, Johannes, C Grutters, Sergio, Harari, Richard, G Jenkins, Kerri, A Johannson, Mark, G Jones, Meena, Kalluri, Michael, P Keane, Maria, A Kokosi, Michael, Kreuter, Donato, Lacedonia, Brett, Ley, Alessandro, Libra, Fabrizio, Luppi, Toby, M Maher, George, A Margaritopoulos, Fernando, J Martinez, Jelle, Miedema, Nesrin, Mogulkoc, Maria, Molina-Molina, Philip, L Molyneaux, Julie, Morisset, Stefano, Palmucci, Mauro, Pavone, Ganesh, R Raghu, Elisabetta, A Renzoni, Luca, Richeldi, Gianluca, Sambataro, Alfredo, Sebastiani, Paolo, Spagnolo, Giulia Maria Stella, Martina, Sterclova, Irina, Strambu, Tomassetti, Sara, Sebastiano, Torrisi, Jacopo, Simonetti, Haluk, Turktas, Argyrios, Tzouvelekis, Claudia, Valenzuela, Ada, Vancheri, Carlo, Vancheri, Francesco, Varone, Patrizio, Vitulo, Athol, U Wells, Marlies, S Wijsenbeek, Wim, A Wuyts, 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, 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., and Bocchino, M.

Subjects
Pulmonary and Respiratory Medicine, medicine.medical_specialty, Concordance, Health Status, interstitial lung dieseases, MEDLINE, Medizin, Prom, 03 medical and health sciences, 0302 clinical medicine, Medicine, Humans, 030212 general & internal medicine, Patient Reported Outcome Measures, Intensive care medicine, business.industry, Minimal clinically important difference, Interstitial lung disease, respiratory system, medicine.disease, Idiopathic Pulmonary Fibrosis, female genital diseases and pregnancy complications, respiratory tract diseases, Clinical trial, patient reported outcomes, patient reported outcome measures, idiopathic pulmonary fibrosi, 030228 respiratory system, Patient-reported outcome, Computerized adaptive testing, Patient Participation, business, Lung Diseases, Interstitial
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

40. 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

48. Sustained Improvement of Interstitial Lung Disease Following Enzyme Replacement Therapy with Olipudase Alfa in Children and Adults with Chronic Acid Sphingomyelinase Deficiency

Author

Berger, K.I., primary, Scarpa, M., additional, Diaz, G.A., additional, Giugliani, R., additional, Lachmann, R., additional, Wasserstein, M.P., additional, Bonella, F., additional, Walsh, S.L.F., additional, Wuyts, W.A., additional, Jessel, A., additional, Rawlings, A.M., additional, and Kumar, M., additional

Published
2022
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49. Cellular activities of factor XII in lung fibrosis

Author

Schramm, F, additional, Hadzic, S, additional, Jablonska, E, additional, Birnhuber, A, additional, Rubio, K, additional, Schulte, L, additional, Kreuter, M, additional, Bonella, F, additional, Barreto, G, additional, Weissmann, N, additional, Kwapiszewska, G, additional, Renne, T, additional, Markart, P, additional, Preissner, K T, additional, Prasse, A, additional, and Wygrecka, M, additional

Published
2022
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