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6. Association between age at disease onset of anti-neutrophil cytoplasmic antibody-associated vasculitis and clinical presentation and short-term outcomes

Author

Monti, S., Craven, A., Klersy, C., Montecucco, C., Caporali, R., Watts, R., Merkel, P. A., Luqmani, R., Achilleos, K., Adler, M., Alba, M. A., Albert, D. A., Alibaz-Oner, F., Allcoat, P., Amano, K., Amarasuriya, M., Amudala, N. A., Andrews, J., Archer, A. M., Arimura, Y., Atukorala, I., Azevedo, E., Bajad, S., Baldwin, C., Barra, L. J., Baslund, B., Basu, N., Baykal, M., Berger, C., Berglin, E., Besada, E., Bhardwaj, M., Bischof, A., Blockmans, D., Blood, J., Draibe, J. B., Brand, S., Brandao, M., Bruce, I. N., Butler, A., Calabrese, L. H., Ferrer, D. C., Carette, S., Carmona, D., Ceunen, H., Chakravarty, K., Chapman, P. T., Chocova, Z., Chung, S. A., Ci, W., Cid, M. C., Clark, T. M., Clarkson, M. R., De Jesus Contreras-Rodriguez, F., Conway, R., Cooke, K., Viros, X. C., Cordeiro, A., Costa, A., Culfear, K., Daikeler, T., Danda, D., Das, S. K., Dasgupta, B., De Castro, A. M., Dehghan, N., Devassy, R., Dhindsa, N., Diamantopoulos, A. P., Direskeneli, H., Dobashi, H., Juan, D., Durrani, M., Edelsten, C., Eifert, J., Elhayek, S., Elsideeg, S., Endo, T., Erden, A., Erer, B., Eriksson, P., Erturk, Z., Espigol-Frigole, G., Felicetti, M., Ferraro, A., Ferro, J. M., Fifi-Mah, A., Flores-Suarez, L. F., Flossmann, O., Flynn, D., Fonseca, J. E., Foot, J., Foote, M., Forbess, L., Fujimoto, S., Fukuoka, K., Furtado, C., Furuta, S., Gaffo, A. L., Gallagher, P., Gao, N., Gatenby, P., Gendi, N., Geraldes, R., Gerits, A., Gioffredi, A., Gomples, L., Goncalves, M. J., Gondo, P., Graham, A., Grainger, R., Gray, D. T., Grayson, P. C., Griffiths, L., Guo, Y., Gupta, R., Gylling, M., Hajj-Ali, R. A., Hammam, N., Harigai, M., Hartley, L., Haslett, J., Hassan, A., Hatemi, G., Hellmich, B., Henckaerts, L., Henes, J. C., Hepburn, J., Herd, V., Hess, C., Hill, C., Hinojosa-Azaola, A., Hirahashi, J., Hirano, F., Hocevar, A., Holle, J., Hollinger, N., Homma, S., Howard, T., Hoyles, R. K., Hruskova, Z., Hutcheon, G., Ignacak, M., Igney-Oertel, A., Ikeda, K., Ikegaya, N., Jagadeesh, S., Jaquith, J., Jayne, D. R. W., Jewell, T., Jones, C., Joshi, A., Kalyoncu, U., Kamall, S., Kamath, S., Lai, K. S., Kaname, S., Kanchinadham, S., Karadag, O., Karube, M., Kaszuba, M., Kaur, R., Kawakami, T., Kawashima, S., Khalidi, N., Khan, A., Kikuchi, M., Kilic, L., Kimura, M., King, M. J., Klapa, S., Klocke, R., Kobayashi, T., Kobayashi, S., Komagata, Y., Kronbichler, A., Kuczia, P., Kumar, M. S., Kurosawa, M., Lamprecht, P., Langford, C. A., Lanyon, P., Laversuch, C., Lee, S. J., Leoni, S., Li, J., Liang, K., Liang, P., Liao, H., Lee, L. A., Luqmani, R. A., Lyle, A., Macdonald, M., Mackie, S. L., Madden, L., Magliano, M., Makino, H., Makol, A., Malaiya, R., Malaviya, A., Manthri, R., Maritati, F., Da Silva, A. M., Mason, J. C., Matara, C., Matsui, K., Matteson, E. L., Mcbride, D., Mccullough, K., Mcgeoch, L., Mclaren, J., Mcmillian, C., Mendiratta, N., Menon, A., Merinopoulos, D., Merkel, P., Messier, S., Micheletti, R. G., Mills, K., Milman, N., Minoda, M., Minz, R. W., Mock, C., Mohammad, A. J., Moiseev, S., Moitinho, M., Molloy, E., Monach, P. A., Montgomery, M., Moosig, F., Moradizadeh, M., Morgan, M., Morgan, A. W., Morgan, A. -M., Muir, A., Mukhtyar, C., Muller, A., Muratore, F., Muso, E., Nada, R., Nakajima, H., Nakajima, T., Nakano, H., Nandagudi, A., Neumann, T., Y. F., Ng, K. H., Ng, Nogueira, E. L., Nolkha, N., Nordstrom, D., Novikov, P., Nugaliyadde, A., O'Donnell, J. L., O'Donoghue, J., O'Neill, L., O'Riordan, E., Oatley, M., Okubo, K., Oliva, E., Oshikawa, H., Ota, Y., Padoan, R., Pagnoux, C., Pan, L., Panaritis, K., Park, J. K., Patel, S., Patil, P., Pazzola, G., Peall, A., Pearce, F., Pehlevan, S., Pereira, L., Pettersson, T., Pineau, C. A., Pirila, L., Poglodek, B., Ponte, C., Prieto-Gonzalez, S., Priya, S. R., Purewal, B., Purschke, S., Putaala, J., Quickert, S., Quincey, V., Raghuvanshi, S., Rajasekhar, L., Ranganathan, D., Rathi, M., Rees, D., Rees, F., Renken, U., Restuccia, G., Rhee, R. L., Rice, B., Robins, D., Robson, J., Rodrigues, M., Romao, V. C., Rotar, C., Ruediger, C., Rutgers, A., A. C., Sa, Saavedra, M. J., Sada, K. -E., Sahbudin, I., Salvarani, C., Sandhu, N., Santos, E., Sato, Y., Schafer, V. S., Schiavon, F., Schmidt, W. A., Segelmark, M., Shahin, A., Sharma, A., Shotton, J., Silva, C., Singer, O. G., Sivasuthan, G., Smolen, S., Solanich-Moreno, X., Boixader, L. S., Song, Y. W., Springer, J., Sreih, A. G., Srivastava, R., Stamp, L. K., Stevens, R., Strbian, D., Sugino, K., Sunderkotter, C., Suppiah, R., Suzuki, K., Szekanecz, Z., Sznajd, J., Taimen, K., Tak, P. P., Takeuchi, T., Takizawa, N., Tames, L., Tan, B. E., Tanaka, M., Tang, M. W., Tatlisumak, T., Tesar, V., Thomas, A., Tian, X., Tokunaga, K., Tombetti, E., Tomsic, M., Toz, B., Tsukamoto, T., Uchida, S., Unal, A. U., Urban, M. L., Usui, J., Vaglio, A., Venkatachalam, S., Vermaak, E., Viswanath, V., Wada, T., Wagh, S., Wallace, D. J., Walters, G., Walz, B., Wan, J., Wang, T., Wang, G., Warrington, K. J., Watts, R. A., Wawrzycka-Adamczyk, K., Weeratunga, P., Weisman, M. H., Wickramasinghe, S., Williams, M., Wojcik, K., Woodruff, L., Xenitidis, T., Yamada, H., Yamagata, K., Yee, C. -S., Yoon, M., Yoshida, K., Yoshifuji, H., Ytterberg, S. R., Yumura, W., Zayed, H., Zeng, X., Zhao, M. -H., Zugaj, A., Zuk, J., İç Hastalıkları, Clinical Haematology, and Translational Immunology Groningen (TRIGR)

Subjects
Male, Outcome, Antineutrophil Cytoplasmic, 030232 urology & nephrology, 0302 clinical medicine, Risk Factors, 80 and over, Pharmacology (medical), Age of Onset, Young adult, Aged, 80 and over, education.field_of_study, age, anti-neutrophil cytoplasmic antibody-associated vasculitis, outcome, Adolescent, Adult, Aged, Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis, Antibodies, Antineutrophil Cytoplasmic, Female, Humans, Middle Aged, Morbidity, Prognosis, Retrospective Studies, Risk Assessment, Survival Rate, United Kingdom, Young Adult, Vasculitis, Systemic vasculitis, medicine.medical_specialty, Population, anti-neutrophil cytoplasmic antibody–associated vasculitis, Antibodies, 03 medical and health sciences, Rheumatology, Internal medicine, medicine, education, Anti-neutrophil cytoplasmic antibody–associated vasculitis, Survival rate, Anti-neutrophil cytoplasmic antibody, 030203 arthritis & rheumatology, business.industry, Retrospective cohort study, medicine.disease, Age of onset, business
Abstract

Objectives ANCA-associated vasculitis (AAV) can affect all age groups. We aimed to show that differences in disease presentation and 6 month outcome between younger- and older-onset patients are still incompletely understood. Methods We included patients enrolled in the Diagnostic and Classification Criteria for Primary Systemic Vasculitis (DCVAS) study between October 2010 and January 2017 with a diagnosis of AAV. We divided the population according to age at diagnosis: Results A total of 1338 patients with AAV were included: 66% had disease onset at Conclusion Within 6 months of diagnosis of AAV, patients >65 years of age display a different pattern of organ involvement and an increased risk of significant damage and mortality compared with younger patients.

Published
2021

7. Factors Prognostic of Greater Decline in Forced Vital Capacity in Patients with Systemic Sclerosis-Associated Interstitial Lung Disease (SSc-ILD): Data from the Placebo Group of the SENSCIS Trial*

Author

Prasse, A, additional, Kuwana, M, additional, Assassi, S, additional, Avouac, J, additional, Hoyles, R K, additional, Pope, J, additional, Smith, V, additional, Miede, C, additional, Clerisme-Beaty, E, additional, Alves, M, additional, and Distler, O, additional

Published
2022
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9. 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis With Polyangiitis

Author

Grayson, P. C., Ponte, C., Suppiah, R., Robson, J. C., Craven, A., Judge, A., Khalid, S., Hutchings, A., Luqmani, R. A., Watts, R. A., Merkel, P. A., Gatenby, P., Hill, C., Ranganathan, D., Kronbichler, A., Blockmans, D., Barra, L., Carette, S., Pagnoux, C., Dhindsa, N., Fifi-Mah, A., Khalidi, N., Liang, P., Milman, N., Pineau, C., Tian, X., Wang, G., Wang, T., Zhao, M. -H., Tesar, V., Baslund, B., Hammam, N., Shahin, A., Pirila, L., Putaala, J., Hellmich, B., Henes, J., Lamprecht, P., Neumann, T., Schmidt, W., Sunderkoetter, C., Szekanecz, Z., Danda, D., Das, S., Gupta, R., Rajasekhar, L., Sharma, A., Wagh, S., Clarkson, M., Molloy, E., Salvarani, C., Schiavon, F., Tombetti, E., Vaglio, A., Amano, K., Arimura, Y., Dobashi, H., Fujimoto, S., Harigai, M., Hirano, F., Hirahashi, J., Honma, S., Kawakami, T., Kobayashi, S., Kono, H., Makino, H., Matsui, K., Muso, E., Suzuki, K., Ikeda, K., Takeuchi, T., Tsukamoto, T., Uchida, S., Wada, T., Yamada, H., Yamagata, K., Yumura, W., Lai, K. S., Flores-Suarez, L. F., Hinojosa, A., Rutgers, B., Tak, P. -P., Grainger, R., Quincey, V., Stamp, L., Besada, E., Diamantopoulos, A., Sznajd, J., Azevedo, E., Geraldes, R., Rodrigues, M., Santos, E., Song, Y. -W., Moiseev, S., Hocevar, A., Cid, M. C., Moreno, X. S., Atukorala, I., Berglin, E., Mohammed, A., Segelmark, M., Daikeler, T., Direskeneli, H., Hatemi, G., Kamali, S., Karadag, O., Pehlevan, S., Adler, M., Basu, N., Bruce, I., Chakravarty, K., Dasgupta, B., Flossmann, O., Gendi, N., Hassan, A., Hoyles, R., Jayne, D., Jones, C., Klocke, R., Lanyon, P., Laversuch, C., Luqmani, R., Robson, J., Magliano, M., Mason, J., Maw, W. W., Mcinnes, I., Mclaren, J., Morgan, M., Morgan, A., Mukhtyar, C., O'Riordan, E., Patel, S., Peall, A., Venkatachalam, S., Vermaak, E., Menon, A., Watts, R., Yee, C. -S., Albert, D., Calabrese, L., Chung, S., Forbess, L., Gaffo, A., Gewurz-Singer, O., Grayson, P., Liang, K., Matteson, E., Springer, J., Sreih, A., and Translational Immunology Groningen (TRIGR)

Subjects
Adult, Male, Vasculitis, Myeloblastin, Immunology, Churg-Strauss Syndrome, Sensitivity and Specificity, General Biochemistry, Genetics and Molecular Biology, Antibodies, Antineutrophil Cytoplasmic, Diagnosis, Differential, Rheumatology, Risk Factors, Humans, Immunology and Allergy, anti-neutrophil cytoplasm antibody, Prospective Studies, Aged, Granulomatosis with Polyangiitis, Reproducibility of Results, Middle Aged, United States, Female, eosinophilic granulomatosis with polyangiitis, Eosinophilic Granuloma, Europe, classification, Societies
Abstract

ObjectiveTo develop and validate revised classification criteria for eosinophilic granulomatosis with polyangiitis (EGPA).MethodsPatients with vasculitis or comparator diseases were recruited into an international cohort. The study proceeded in five phases: (1) identification of candidate criteria items using consensus methodology, (2) prospective collection of candidate items present at the time of diagnosis, (3) data-driven reduction of the number of candidate items, (4) expert panel review of cases to define the reference diagnosis and (5) derivation of a points-based risk score for disease classification in a development set using least absolute shrinkage and selection operator logistic regression, with subsequent validation of performance characteristics in an independent set of cases and comparators.ResultsThe development set for EGPA consisted of 107 cases of EGPA and 450 comparators. The validation set consisted of an additional 119 cases of EGPA and 437 comparators. From 91 candidate items, regression analysis identified 11 items for EPGA, 7 of which were retained. The final criteria and their weights were as follows: maximum eosinophil count ≥1×109/L (+5), obstructive airway disease (+3), nasal polyps (+3), cytoplasmic antineutrophil cytoplasmic antibody (ANCA) or anti-proteinase 3–ANCA positivity (−3), extravascular eosinophilic predominant inflammation (+2), mononeuritis multiplex/motor neuropathy not due to radiculopathy (+1) and haematuria (−1). After excluding mimics of vasculitis, a patient with a diagnosis of small- or medium-vessel vasculitis could be classified as having EGPA if the cumulative score was ≥6 points. When these criteria were tested in the validation data set, the sensitivity was 85% (95% CI 77% to 91%) and the specificity was 99% (95% CI 98% to 100%).ConclusionThe 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis with Polyangiitis demonstrate strong performance characteristics and are validated for use in research.

Published
2022

12. Identifying causation in hypersensitivity pneumonitis: a British perspective

Author

Barber, CM, Burge, PS, Feary, JR, Parfrey, H, Renzoni, EA, Spencer, LG, Walters, GI, Wiggans, RE, Adamali, H, Babu, S, Barrat, S, Basran, A, Beirne, P, Bianchi, S, Chalmers, G, Chaudhuri, N, Davies, S, Dempsey, O, Eccles, S, Fiddler, C, Foley, N, Forrest, I, Fletcher, S, George, P, Ghani, S, Gibbons, M, Greenstone, M, Hart, S, Hirani, N, Hoyle, J, Hoyles, R, Hutchinson, J, Jenkins, G, Judge, E, Kamath, A, Kokosi, M, Lee, C, Maher, T, Marshall, B, McAndrew, N, Molyneux, P, Morrison, D, O'Hickey, S, Porter, J, Renshaw, S, Sharp, C, Simler, N, Spears, M, Spiers, A, Spinks, K, Spiteri, M, Stenton, C, Sturney, S, Warburton, C, Wiscombe, S, and Woodhead, F

Subjects
Respiratory System, PULMONARY, Bronchoalveolar Lavage, 0302 clinical medicine, Surveys and Questionnaires, Epidemiology, Medicine, EPIDEMIOLOGY, FIBROSIS, 030212 general & internal medicine, Occupational lung disease, Causation, Pulmonologists, Occupational Lung Disease, Interstitial lung disease, Questionnaire, Interstitial Fibrosis, England, GUIDELINE, SURVIVAL, Bronchoalveolar Lavage Fluid, Life Sciences & Biomedicine, Hypersensitivity pneumonitis, Alveolitis, Extrinsic Allergic, Pulmonary and Respiratory Medicine, medicine.medical_specialty, Consensus, ANTIGEN, GB HP Survey Participants, DIAGNOSIS, Interstitial Lung Disease, 03 medical and health sciences, INTERSTITIAL LUNG-DISEASES, Humans, Wales, Science & Technology, IDENTIFICATION, business.industry, Guideline, Allergens, medicine.disease, Pulmonary Alveoli, Scotland, 030228 respiratory system, Family medicine, business, Allergic Alveolitis
Abstract

BackgroundEstablishing whether patients are exposed to a ‘known cause’ is a key element in both the diagnostic assessment and the subsequent management of hypersensitivity pneumonitis (HP).ObjectiveThis study surveyed British interstitial lung disease (ILD) specialists to document current practice and opinion in relation to establishing causation in HP.MethodsBritish ILD consultants (pulmonologists) were invited by email to take part in a structured questionnaire survey, to provide estimates of demographic data relating to their service and to rate their level of agreement with a series of statements. A priori ‘consensus agreement’ was defined as at least 70% of participants replying that they ‘Strongly agree’ or ‘Tend to agree’.Results54 consultants took part in the survey from 27 ILD multidisciplinary teams. Participants estimated that 20% of the patients in their ILD service have HP, and of these, a cause is identifiable in 32% of cases. For patients with confirmed HP, an estimated 40% have had a bronchoalveolar lavage for differential cell counts, and 10% a surgical biopsy. Consensus agreement was reached for 25 of 33 statements relating to causation and either the assessment of unexplained ILD or management of confirmed HP.ConclusionsThis survey has demonstrated that although there is a degree of variation in the diagnostic approach for patients with suspected HP in Britain, there is consensus opinion for some key areas of practice. There are several factors in clinical practice that currently act as potential barriers to identifying the cause for British HP patients.

Published
2019

14. Diagnostic accuracy of a clinical diagnosis of idiopathic pulmonary fibrosis: an international case-cohort study

Author

Walsh S. L. F., Maher T. M., Kolb M., Poletti V., Nusser R., Richeldi L., Vancheri C., Wilsher M. L., Antoniou K. M., Behr J., Bendstrup E., Brown K., Calandriello L., Corte T. J., Cottin V., Crestani B., Flaherty K., Glaspole I., Grutters J., Inoue Y., Kokosi M., Kondoh Y., Kouranos V., Kreuter M., Johannson K., Judge E., Ley B., Margaritopoulos G., Martinez F. J., Molina-Molina M., Morais A., Nunes H., Raghu G., Ryerson C. J., Selman M., Spagnolo P., Taniguchi H., Tomassetti S., Valeyre D., Wijsenbeek M., Wuyts W., Hansell D., Wells A., Zhu P. S., Yuan Y., Yoshito Fukuda C., Yoshimatsu Y., Xaubet A., Wong A. M., White P., Westney G., West A., Wessendorf T., Waseda Y., Wang C., Vienna J. M., Videnovic Ivanov J., Vicens Zygmunt V., Venero Caceres M. C., Velasquez Pinto G., Veitch E., Vasakova M., Varone F., Varela B. E., Van Hal P., Van De Ven M., Van Der Lee I., Van Den Toorn L., Urrutia Gajate A., Urban J., Ugarte Fornell L. G., Tzouvelekis A., Twohig K., Turner A., Trujillo S., Triani A., Traila D., Torres V., Tomioka H., Tomii K., Tomic R., Toma C., Tokgoz Akyil F., Tobino K., Tobar R., Tiwari A., Tibana R., Tian X., Thillai M., Tham W., Teo F., Tekavec Trkanjec J., Teixeira P., Tarpey D., Tapias L., Tanizawa K., Tanino Y., Takada T., Tabaj G., Szolnoki E., Swarnakar R., Strambu I., Sterclova M., Spinks K., Soo C. I., Soltani A., Solanki S., Sobh E., Soares M. R., Smith J., Smith B., Slocum P., Slabbynck H., Sivokozov I., Shifren A., Shen S. M., Sharp C., Shanmuganathan A., Sebastiani A., Scarlata S., Savas R., Sasaki S., Santeliz J., Santana ANC., Sanchez R., Salinas M., Saito S., Ryan F., Royo Prats J. A., Rosi E., Rokadia H., Robles Perez A., Rivera Ortega P., Rio Ramirez M., Righetti S., Reichner C., Ravaglia C., Ratanawatkul P., Ramalingam V., Rajasekaran A., Radzikowska E., Ra S. W., Quadrelli S., Precerutti J., Prasad J., Popa D., Pizzalato S., Piotrowski W., Pineiro A., Piloni D., Peros Golubicic T., Perez R., Pereira C., Pereira B., Perch M., Patel N., Patel D., Papanikolaou I., Papakosta D., Panselinas E., Pang Y. K., Pandya P., Padrao E., Ozdemir Kumbasar O., Overbeek M. J., Otto Minasian A., O'Riordan D., Ora J., Oldham J., Okutan O., Ohshimo S., Oguzulgen I. K., Ogura T., O'Donnell T., O'Dochartaigh C., O'Beirne S., Novikova L., Novelli L., Noth I., Nogueira Mendes Neto N., Niroumand M., Nieto A., Neves A., Nambiar A., Nair S., Nadama R., Murtagh E., Mura M., Muller Quernheim J., Mukhopadhyay A., Mukherjee S., Morisset J., Moran O., Mooney J., Moller J., Mogulkoc N., Miyamoto A., Milenkovic B., Mette S., Mejia M., Mei F., Mazzei M., Matsuda T., Mason C., Martinez Frances M., Mannarino S., Mancuzo E., Malli F., Malhotra P., Maillo M., Maia J., Mahdavian M., Madsen F., Luckhardt T., Lucht W., Low S. Y., Lopez Miguel C. P., Lipchik R., Levy S., Levin K., Lee K. L., Lederer D., Lammi M. R., Kwan H. Y., Kukreja S., Kruavit A., Kotecki M., Kolilekas L., Knoop H., Kiyan E., Kishaba T., King Biggs M., Khor Y. H., Khan A., Khalil N., Kedia R., Kebba N., Kawano Dourado L., Kapitan K., Kan C. D., Kalyoncu A. F., Kalluri M., Kabasakal Y., Jyothula S., Juretschke M. A., Jovanovic D., Jonkers R., Jo H., Izumi S., Ishii H., Ikeda S., Ibrahim A., Hyldgaard C., Hunninghake G., Huie T., Hufton A., Hu X., Hseih W. C., Hoyos R., Hoyles R., Holguin Rodriguez O., Hogan M. P., Hodgson U., Hilkin Sogoloff H., Herrera E., Henry B. M., Hellemons M., Hecimovic A., Hayashi R., Hart S., Harari S., Haney S., Hambly N., Hakkim R., Gutierrez M., Gripaldo R., Gomez A., Goh N., Godoy R., Gilbert C., Giannarakis I., Gasparini S., Garcha P., Furtado S., Fois A., Flood Page P., Fletcher S., Fiss E., Figueroa Casas J., Figueroa Casas M., Fiddler C. A., Ferrara G., Fernandez Casares M., Felton C., Faverio P., Fabro A. T., Estrada A., Errhalt P., Enomoto N., Enghelmayer J. I., El Kersh K., Eiger G., Dubaniewicz A., Drakopanagiotakis F., Disayabutr S., Dijkstra A., Diaz Patino J. C., Diaz Castanon J. J., Dhooria S., Dhasmana D. J., De Rosa M., De Luca S., Delobbe A., Delgado D., Delgado C., De La Fuente I., De Kruif M., De Gier M., De Andrade J., Davidsen J. R., Daoud B., Dalhoff K., Cotera Solano J. V., Costa A. N., Coronel S., Confalonieri M., Conemans L., Comellas A., Colella S., Clemente S., Clark J., Ciuffreda M., Chung C. L., Chong S. G., Chirita D., Chen P. L., Chaudhuri N., Chambers D., Chalmers G., Chairman D., Chai G. T., Chacon Chaves R., Cetinsu V., Ceruti M., Ceballos Zuniga C. O., Castillo D., Carbone R. G., Caminati A., Callejas Gonzalez F. J., Butler M., Bustos C., Bukowczan M., Buendia I., Brunetti G., Brockway B., Bresser P., Breseghello J., Bouros D., Botero Zaccour J. A., Borzone G., Borie R., Blum H. C., Blank J., Biswas A., Bennett D., Benjamin M., Belaconi I. N., Beirne P., Beckert L., Bastiampillai S., Bascom R., Bartholmai B., Barros M., Ban AYL., Balestro E., Baldi B., Baddini Martinez J., Baburao A., Babu S., Averyanov A., Avdeev S., Athanazio R., Atahan E., Asuquo B., Assayag D., Antuni J., Antillon S., Anderson K. C., Anderson A., Alwani F., Altinisik G., Alsouofi N., Allam J. S., Al Jahdali H., Al Farttoosi A., Alfaro T., Al Busaidi N., Alavi Foumani A., Agreda Vedia M. G., Agarwal A., Afridi F., Adeyeye O. O., Adegunsoye A., Adamali H., Abedini A., Walsh, S. L. F., Maher, T. M., Kolb, M., Poletti, V., Nusser, R., Richeldi, L., Vancheri, C., Wilsher, M. L., Antoniou, K. M., Behr, J., Bendstrup, E., Brown, K., Calandriello, L., Corte, T. J., Cottin, V., Crestani, B., Flaherty, K., Glaspole, I., Grutters, J., Inoue, Y., Kokosi, M., Kondoh, Y., Kouranos, V., Kreuter, M., Johannson, K., Judge, E., Ley, B., Margaritopoulos, G., Martinez, F. J., Molina-Molina, M., Morais, A., Nunes, H., Raghu, G., Ryerson, C. J., Selman, M., Spagnolo, P., Taniguchi, H., Tomassetti, S., Valeyre, D., Wijsenbeek, M., Wuyts, W., Hansell, D., Wells, A., Zhu, P. S., Yuan, Y., Yoshito Fukuda, C., Yoshimatsu, Y., Xaubet, A., Wong, A. M., White, P., Westney, G., West, A., Wessendorf, T., Waseda, Y., Wang, C., Vienna, J. M., Videnovic Ivanov, J., Vicens Zygmunt, V., Venero Caceres, M. C., Velasquez Pinto, G., Veitch, E., Vasakova, M., Varone, F., Varela, B. E., Van Hal, P., Van De Ven, M., Van Der Lee, I., Van Den Toorn, L., Urrutia Gajate, A., Urban, J., Ugarte Fornell, L. G., Tzouvelekis, A., Twohig, K., Turner, A., Trujillo, S., Triani, A., Traila, D., Torres, V., Tomioka, H., Tomii, K., Tomic, R., Toma, C., Tokgoz Akyil, F., Tobino, K., Tobar, R., Tiwari, A., Tibana, R., Tian, X., Thillai, M., Tham, W., Teo, F., Tekavec Trkanjec, J., Teixeira, P., Tarpey, D., Tapias, L., Tanizawa, K., Tanino, Y., Takada, T., Tabaj, G., Szolnoki, E., Swarnakar, R., Strambu, I., Sterclova, M., Spinks, K., Soo, C. I., Soltani, A., Solanki, S., Sobh, E., Soares, M. R., Smith, J., Smith, B., Slocum, P., Slabbynck, H., Sivokozov, I., Shifren, A., Shen, S. M., Sharp, C., Shanmuganathan, A., Sebastiani, A., Scarlata, S., Savas, R., Sasaki, S., Santeliz, J., Santana, Anc., Sanchez, R., Salinas, M., Saito, S., Ryan, F., Royo Prats, J. A., Rosi, E., Rokadia, H., Robles Perez, A., Rivera Ortega, P., Rio Ramirez, M., Righetti, S., Reichner, C., Ravaglia, C., Ratanawatkul, P., Ramalingam, V., Rajasekaran, A., Radzikowska, E., Ra, S. W., Quadrelli, S., Precerutti, J., Prasad, J., Popa, D., Pizzalato, S., Piotrowski, W., Pineiro, A., Piloni, D., Peros Golubicic, T., Perez, R., Pereira, C., Pereira, B., Perch, M., Patel, N., Patel, D., Papanikolaou, I., Papakosta, D., Panselinas, E., Pang, Y. K., Pandya, P., Padrao, E., Ozdemir Kumbasar, O., Overbeek, M. J., Otto Minasian, A., O'Riordan, D., Ora, J., Oldham, J., Okutan, O., Ohshimo, S., Oguzulgen, I. K., Ogura, T., O'Donnell, T., O'Dochartaigh, C., O'Beirne, S., Novikova, L., Novelli, L., Noth, I., Nogueira Mendes Neto, N., Niroumand, M., Nieto, A., Neves, A., Nambiar, A., Nair, S., Nadama, R., Murtagh, E., Mura, M., Muller Quernheim, J., Mukhopadhyay, A., Mukherjee, S., Morisset, J., Moran, O., Mooney, J., Moller, J., Mogulkoc, N., Miyamoto, A., Milenkovic, B., Mette, S., Mejia, M., Mei, F., Mazzei, M., Matsuda, T., Mason, C., Martinez Frances, M., Mannarino, S., Mancuzo, E., Malli, F., Malhotra, P., Maillo, M., Maia, J., Mahdavian, M., Madsen, F., Luckhardt, T., Lucht, W., Low, S. Y., Lopez Miguel, C. P., Lipchik, R., Levy, S., Levin, K., Lee, K. L., Lederer, D., Lammi, M. R., Kwan, H. Y., Kukreja, S., Kruavit, A., Kotecki, M., Kolilekas, L., Knoop, H., Kiyan, E., Kishaba, T., King Biggs, M., Khor, Y. H., Khan, A., Khalil, N., Kedia, R., Kebba, N., Kawano Dourado, L., Kapitan, K., Kan, C. D., Kalyoncu, A. F., Kalluri, M., Kabasakal, Y., Jyothula, S., Juretschke, M. A., Jovanovic, D., Jonkers, R., Jo, H., Izumi, S., Ishii, H., Ikeda, S., Ibrahim, A., Hyldgaard, C., Hunninghake, G., Huie, T., Hufton, A., Hu, X., Hseih, W. C., Hoyos, R., Hoyles, R., Holguin Rodriguez, O., Hogan, M. P., Hodgson, U., Hilkin Sogoloff, H., Herrera, E., Henry, B. M., Hellemons, M., Hecimovic, A., Hayashi, R., Hart, S., Harari, S., Haney, S., Hambly, N., Hakkim, R., Gutierrez, M., Gripaldo, R., Gomez, A., Goh, N., Godoy, R., Gilbert, C., Giannarakis, I., Gasparini, S., Garcha, P., Furtado, S., Fois, A., Flood Page, P., Fletcher, S., Fiss, E., Figueroa Casas, J., Figueroa Casas, M., Fiddler, C. A., Ferrara, G., Fernandez Casares, M., Felton, C., Faverio, P., Fabro, A. T., Estrada, A., Errhalt, P., Enomoto, N., Enghelmayer, J. I., El Kersh, K., Eiger, G., Dubaniewicz, A., Drakopanagiotakis, F., Disayabutr, S., Dijkstra, A., Diaz Patino, J. C., Diaz Castanon, J. J., Dhooria, S., Dhasmana, D. J., De Rosa, M., De Luca, S., Delobbe, A., Delgado, D., Delgado, C., De La Fuente, I., De Kruif, M., De Gier, M., De Andrade, J., Davidsen, J. R., Daoud, B., Dalhoff, K., Cotera Solano, J. V., Costa, A. N., Coronel, S., Confalonieri, M., Conemans, L., Comellas, A., Colella, S., Clemente, S., Clark, J., Ciuffreda, M., Chung, C. L., Chong, S. G., Chirita, D., Chen, P. L., Chaudhuri, N., Chambers, D., Chalmers, G., Chairman, D., Chai, G. T., Chacon Chaves, R., Cetinsu, V., Ceruti, M., Ceballos Zuniga, C. O., Castillo, D., Carbone, R. G., Caminati, A., Callejas Gonzalez, F. J., Butler, M., Bustos, C., Bukowczan, M., Buendia, I., Brunetti, G., Brockway, B., Bresser, P., Breseghello, J., Bouros, D., Botero Zaccour, J. A., Borzone, G., Borie, R., Blum, H. C., Blank, J., Biswas, A., Bennett, D., Benjamin, M., Belaconi, I. N., Beirne, P., Beckert, L., Bastiampillai, S., Bascom, R., Bartholmai, B., Barros, M., Ban, Ayl., Balestro, E., Baldi, B., Baddini Martinez, J., Baburao, A., Babu, S., Averyanov, A., Avdeev, S., Athanazio, R., Atahan, E., Asuquo, B., Assayag, D., Antuni, J., Antillon, S., Anderson, K. C., Anderson, A., Alwani, F., Altinisik, G., Alsouofi, N., Allam, J. S., Al Jahdali, H., Al Farttoosi, A., Alfaro, T., Al Busaidi, N., Alavi Foumani, A., Agreda Vedia, M. G., Agarwal, A., Afridi, F., Adeyeye, O. O., Adegunsoye, A., Adamali, H., Abedini, A., National Institute for Health Research, British Lung Foundation, Walsh, S, Maher, T, Kolb, M, Poletti, V, Nusser, R, Richeldi, L, Vancheri, C, Wilsher, M, Antoniou, K, Behr, J, Bendstrup, E, Brown, K, Calandriello, L, Corte, T, Cottin, V, Crestani, B, Flaherty, K, Glaspole, I, Grutters, J, Inoue, Y, Kokosi, M, Kondoh, Y, Kouranos, V, Kreuter, M, Johannson, K, Judge, E, Ley, B, Margaritopoulos, G, Martinez, F, Molina-Molina, M, Morais, A, Nunes, H, Raghu, G, Ryerson, C, Selman, M, Spagnolo, P, Taniguchi, H, Tomassetti, S, Valeyre, D, Wijsenbeek, M, Wuyts, W, Hansell, D, Wells, A, Zhu, P, Yuan, Y, Yoshito Fukuda, C, Yoshimatsu, Y, Xaubet, A, Wong, A, White, P, Westney, G, West, A, Wessendorf, T, Waseda, Y, Wang, C, Vienna, J, Videnovic Ivanov, J, Vicens Zygmunt, V, Venero Caceres, M, Velasquez Pinto, G, Veitch, E, Vasakova, M, Varone, F, Varela, B, Van Hal, P, Van De Ven, M, Van Der Lee, I, Van Den Toorn, L, Urrutia Gajate, A, Urban, J, Ugarte Fornell, L, Tzouvelekis, A, Twohig, K, Turner, A, Trujillo, S, Triani, A, Traila, D, Torres, V, Tomioka, H, Tomii, K, Tomic, R, Toma, C, Tokgoz Akyil, F, Tobino, K, Tobar, R, Tiwari, A, Tibana, R, Tian, X, Thillai, M, Tham, W, Teo, F, Tekavec Trkanjec, J, Teixeira, P, Tarpey, D, Tapias, L, Tanizawa, K, Tanino, Y, Takada, T, Tabaj, G, Szolnoki, E, Swarnakar, R, Strambu, I, Sterclova, M, Spinks, K, Soo, C, Soltani, A, Solanki, S, Sobh, E, Soares, M, Smith, J, Smith, B, Slocum, P, Slabbynck, H, Sivokozov, I, Shifren, A, Shen, S, Sharp, C, Shanmuganathan, A, Sebastiani, A, Scarlata, S, Savas, R, Sasaki, S, Santeliz, J, Santana, A, Sanchez, R, Salinas, M, Saito, S, Ryan, F, Royo Prats, J, Rosi, E, Rokadia, H, Robles Perez, A, Rivera Ortega, P, Rio Ramirez, M, Righetti, S, Reichner, C, Ravaglia, C, Ratanawatkul, P, Ramalingam, V, Rajasekaran, A, Radzikowska, E, Ra, S, Quadrelli, S, Precerutti, J, Prasad, J, Popa, D, Pizzalato, S, Piotrowski, W, Pineiro, A, Piloni, D, Peros Golubicic, T, Perez, R, Pereira, C, Pereira, B, Perch, M, Patel, N, Patel, D, Papanikolaou, I, Papakosta, D, Panselinas, E, Pang, Y, Pandya, P, Padrao, E, Ozdemir Kumbasar, O, Overbeek, M, Otto Minasian, A, O'Riordan, D, Ora, J, Oldham, J, Okutan, O, Ohshimo, S, Oguzulgen, I, Ogura, T, O'Donnell, T, O'Dochartaigh, C, O'Beirne, S, Novikova, L, Novelli, L, Noth, I, Nogueira Mendes Neto, N, Niroumand, M, Nieto, A, Neves, A, Nambiar, A, Nair, S, Nadama, R, Murtagh, E, Mura, M, Muller Quernheim, J, Mukhopadhyay, A, Mukherjee, S, Morisset, J, Moran, O, Mooney, J, Moller, J, Mogulkoc, N, Miyamoto, A, Milenkovic, B, Mette, S, Mejia, M, Mei, F, Mazzei, M, Matsuda, T, Mason, C, Martinez Frances, M, Mannarino, S, Mancuzo, E, Malli, F, Malhotra, P, Maillo, M, Maia, J, Mahdavian, M, Madsen, F, Luckhardt, T, Lucht, W, Low, S, Lopez Miguel, C, Lipchik, R, Levy, S, Levin, K, Lee, K, Lederer, D, Lammi, M, Kwan, H, Kukreja, S, Kruavit, A, Kotecki, M, Kolilekas, L, Knoop, H, Kiyan, E, Kishaba, T, King Biggs, M, Khor, Y, Khan, A, Khalil, N, Kedia, R, Kebba, N, Kawano Dourado, L, Kapitan, K, Kan, C, Kalyoncu, A, Kalluri, M, Kabasakal, Y, Jyothula, S, Juretschke, M, Jovanovic, D, Jonkers, R, Jo, H, Izumi, S, Ishii, H, Ikeda, S, Ibrahim, A, Hyldgaard, C, Hunninghake, G, Huie, T, Hufton, A, Hu, X, Hseih, W, Hoyos, R, Hoyles, R, Holguin Rodriguez, O, Hogan, M, Hodgson, U, Hilkin Sogoloff, H, Herrera, E, Henry, B, Hellemons, M, Hecimovic, A, Hayashi, R, Hart, S, Harari, S, Haney, S, Hambly, N, Hakkim, R, Gutierrez, M, Gripaldo, R, Gomez, A, Goh, N, Godoy, R, Gilbert, C, Giannarakis, I, Gasparini, S, Garcha, P, Furtado, S, Fois, A, Flood Page, P, Fletcher, S, Fiss, E, Figueroa Casas, J, Figueroa Casas, M, Fiddler, C, Ferrara, G, Fernandez Casares, M, Felton, C, Faverio, P, Fabro, A, Estrada, A, Errhalt, P, Enomoto, N, Enghelmayer, J, El Kersh, K, Eiger, G, Dubaniewicz, A, Drakopanagiotakis, F, Disayabutr, S, Dijkstra, A, Diaz Patino, J, Diaz Castanon, J, Dhooria, S, Dhasmana, D, De Rosa, M, De Luca, S, Delobbe, A, Delgado, D, Delgado, C, De La Fuente, I, De Kruif, M, De Gier, M, De Andrade, J, Davidsen, J, Daoud, B, Dalhoff, K, Cotera Solano, J, Costa, A, Coronel, S, Confalonieri, M, Conemans, L, Comellas, A, Colella, S, Clemente, S, Clark, J, Ciuffreda, M, Chung, C, Chong, S, Chirita, D, Chen, P, Chaudhuri, N, Chambers, D, Chalmers, G, Chairman, D, Chai, G, Chacon Chaves, R, Cetinsu, V, Ceruti, M, Ceballos Zuniga, C, Castillo, D, Carbone, R, Caminati, A, Callejas Gonzalez, F, Butler, M, Bustos, C, Bukowczan, M, Buendia, I, Brunetti, G, Brockway, B, Bresser, P, Breseghello, J, Bouros, D, Botero Zaccour, J, Borzone, G, Borie, R, Blum, H, Blank, J, Biswas, A, Bennett, D, Benjamin, M, Belaconi, I, Beirne, P, Beckert, L, Bastiampillai, S, Bascom, R, Bartholmai, B, Barros, M, Ban, A, Balestro, E, Baldi, B, Baddini Martinez, J, Baburao, A, Babu, S, Averyanov, A, Avdeev, S, Athanazio, R, Atahan, E, Asuquo, B, Assayag, D, Antuni, J, Antillon, S, Anderson, K, Anderson, A, Alwani, F, Altinisik, G, Alsouofi, N, Allam, J, Al Jahdali, H, Al Farttoosi, A, Alfaro, T, Al Busaidi, N, Alavi Foumani, A, Agreda Vedia, M, Agarwal, A, Afridi, F, Adeyeye, O, Adegunsoye, A, Adamali, H, Abedini, A, and Pulmonary Medicine

Subjects
Male, Pediatrics, International Cooperation, Respiratory System, Hospitals, University, Idiopathic pulmonary fibrosis, 0302 clinical medicine, Cohen's kappa, Diagnosis, UK, 030212 general & internal medicine, Medical diagnosis, Referral and Consultation, Pulmonologists, Idiopathic Pulmonary Fibrosi, Interstitial lung disease, 11 Medical And Health Sciences, Middle Aged, respiratory system, Prognosis, Hospitals, humanities, Dimensional Measurement Accuracy, Clinical Competence, Diagnosis, Differential, Diagnostic Techniques, Respiratory System, Female, Humans, Idiopathic Pulmonary Fibrosis, Quality of Health Care, Reproducibility of Results, Human, Cohort study, Pulmonary and Respiratory Medicine, medicine.medical_specialty, Prognosi, education, MEDLINE, Reproducibility of Result, INTERSTITIAL PNEUMONIA, Interstitial Lung Diseases, 03 medical and health sciences, Internal medicine, PARENCHYMAL LUNG-DISEASE, MANAGEMENT, medicine, Idiopathic pulmonary fibrosis, diagnosis, Pulmonologist, University, business.industry, MORTALITY, Original Articles, medicine.disease, respiratory tract diseases, Diagnostic Techniques, IPF Project Consortium, 030228 respiratory system, Differential, INTEROBSERVER AGREEMENT, UPDATE, COOPERAÇÃO INTERNACIONAL, Differential diagnosis, business
Abstract

We conducted an international study of idiopathic pulmonary fibrosis (IPF) diagnosis among a large group of physicians and compared their diagnostic performance to a panel of IPF experts. A total of 1141 respiratory physicians and 34 IPF experts participated. Participants evaluated 60 cases of interstitial lung disease (ILD) without interdisciplinary consultation. Diagnostic agreement was measured using the weighted kappa coefficient (κw). Prognostic discrimination between IPF and other ILDs was used to validate diagnostic accuracy for first-choice diagnoses of IPF and were compared using the C-index. A total of 404 physicians completed the study. Agreement for IPF diagnosis was higher among expert physicians (κw=0.65, IQR 0.53–0.72, p20 years of experience (C-index=0.72, IQR 0.0–0.73, p=0.229) and non-university hospital physicians with more than 20 years of experience, attending weekly MDT meetings (C-index=0.72, IQR 0.70–0.72, p=0.052), did not differ significantly (p=0.229 and p=0.052 respectively) from the expert panel (C-index=0.74 IQR 0.72–0.75). Experienced respiratory physicians at university-based institutions diagnose IPF with similar prognostic accuracy to IPF experts. Regular MDT meeting attendance improves the prognostic accuracy of experienced non-university practitioners to levels achieved by IPF experts., Academic status, access to MDT meetings and clinician experience predict accuracy of a clinical diagnosis of IPF http://ow.ly/k43W30cTMg1

Published
2017

17. S143 Serum CYFRA 21–1 as a prognostic marker in scleroderma-associated interstitial lung disease

Author

Stock, CJW, primary, Hoyles, R, additional, D’accord, C, additional, Kokosi, M, additional, Alfieri, V, additional, Campochiaro, C, additional, Donovan, J, additional, Mori, L, additional, Maher, TM, additional, Kouranos, V, additional, Margaritopoulos, G, additional, George, PM, additional, Molyneaux, PL, additional, Chua, F, additional, Abraham, DJ, additional, Ong, V, additional, Denton, CP, additional, Wells, AU, additional, and Renzoni, EA, additional

Published
2018
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22. P9 Nintedanib for the treatment of Idiopathic Pulmonary Fibrosis – initial clinical experience in a UK cohort

Author

Fletcher, SV, primary, Jones, MG, additional, Renzoni, E, additional, Parfrey, H, additional, Hoyles, R, additional, Spinks, K, additional, Kokosi, M, additional, Kwok, A, additional, Warburton, C, additional, Titmuss, V, additional, Maher, T, additional, Chua, F, additional, Wells, A, additional, Richeldi, L, additional, and Spencer, LG, additional

Published
2015
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23. M272 Estimated Cost And Payment By Results (pbr) Tariff Reimbursement For Idiopathic Pulmonary Fibrosis Services Across 14 Specialist Providers In England

Author

Hill, C., primary, Nasr, R., additional, Fisher, M., additional, Maher, T., additional, Spiteri, M., additional, Allen, M., additional, Birring, S., additional, Parfrey, H., additional, Hoyles, R., additional, Gibbons, M., additional, Burge, G., additional, Scullion, J., additional, Adams, E., additional, and Wickremasinghe, M., additional

Published
2014
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27. Basic Science * 208. Stem Cell Factor Expression is Increased in the Skin of Patients with Systemic Sclerosis and Promotes Proliferation and Migration of Fibroblasts in vitro

Author

Karrar, S., primary, Shiwen, X., additional, Nikotorowicz-Buniak, J., additional, Abraham, D. J., additional, Denton, C., additional, Stratton, R., additional, Bayley, R., additional, Kite, K. A., additional, Clay, E., additional, Smith, J. P., additional, Kitas, G. D., additional, Buckley, C., additional, Young, S. P., additional, Ye, L., additional, Zhang, L., additional, Goodall, J., additional, Gaston, H., additional, Xu, H., additional, Lutalo, P. M., additional, Zhao, Y., additional, Meng Choong, L., additional, Sangle, S., additional, Spencer, J., additional, D'Cruz, D., additional, Rysnik, O. J., additional, McHugh, K., additional, Bowness, P., additional, Rump-Goodrich, L., additional, Mattey, D., additional, Kehoe, O., additional, Middleton, J., additional, Cartwright, A., additional, Schmutz, C., additional, Askari, A., additional, Gardner, D. H., additional, Jeffery, L. E., additional, Raza, K., additional, Sansom, D. M., additional, Fitzpatrick, M., additional, Wallace, G., additional, Young, S., additional, Shaw, J., additional, Hatano, H., additional, Cauli, A., additional, Giles, J. L., additional, Mathieu, A., additional, Kollnberger, S., additional, Webster, S., additional, Ellis, L., additional, O'Brien, L. M., additional, Fitzmaurice, T. J., additional, Nazeer Moideen, A., additional, Evans, L., additional, Osgood, L., additional, Williams, A., additional, Jones, S., additional, Thomas, C., additional, O'Donnell, V., additional, Nowell, M., additional, Ouboussad, L., additional, Savic, S., additional, Dickie, L. J., additional, Hintze, J., additional, Wong, C. H., additional, Cook, G. P., additional, Buch, M., additional, Emery, P., additional, McDermott, M. F., additional, Hardcastle, S. A., additional, Gregson, C. L., additional, Deere, K., additional, Davey Smith, G., additional, Dieppe, P., additional, Tobias, J. H., additional, Dennison, E., additional, Edwards, M., additional, Bennett, J., additional, Coggon, D., additional, Palmer, K., additional, Cooper, C., additional, McWilliams, D., additional, Young, A., additional, Kiely, P. D., additional, Walsh, D., additional, Taylor, H. J., additional, Harding, I., additional, Hutchinson, J., additional, Nelson, I., additional, Blom, A., additional, Tobias, J., additional, Clark, E., additional, Parker, J., additional, Bukhari, M., additional, Jayakumar, K., additional, Kiely, P., additional, Diffin, J., additional, Lunt, M., additional, Marshall, T., additional, Chipping, J., additional, Symmons, D., additional, Verstappen, S., additional, Bluett, J., additional, Bowes, J., additional, Ho, P., additional, McHugh, N., additional, Buden, D., additional, Fitzgerald, O., additional, Barton, A., additional, Glossop, J. R., additional, Nixon, N. B., additional, Emes, R. D., additional, Dawes, P. T., additional, Farrell, W. E., additional, Mattey, D. L., additional, Scott, I. C., additional, Steer, S., additional, Seegobin, S., additional, Hinks, A. M., additional, Eyre, S., additional, Morgan, A., additional, Wilson, A. G., additional, Hocking, L., additional, Wordsworth, P., additional, Worthington, J., additional, Cope, A., additional, Lewis, C. M., additional, Guerra, S., additional, Ahmed, B. A., additional, Abraham, D., additional, Fonseca, C., additional, Robinson, J., additional, Taylor, J., additional, Haroon Rashid, L., additional, Flynn, E., additional, Isaacs, J., additional, Barrett, J. H., additional, Kingston, B., additional, Ahmed, M., additional, Kirwan, J. R., additional, Marshall, R., additional, Chapman, K., additional, Pearson, R., additional, Heycock, C., additional, Kelly, C., additional, Rynne, M., additional, Saravanan, V., additional, Hamilton, J., additional, Saeed, A., additional, Coughlan, R., additional, Carey, J. J., additional, Farah, Z., additional, Matthews, W., additional, Bell, C., additional, Petford, S., additional, Tibbetts, L.-M., additional, Douglas, K. M. J., additional, Holden, W., additional, Ledingham, J., additional, Fletcher, M., additional, Winfield, R., additional, Price, Z., additional, Mackay, K., additional, Dixon, C., additional, Oppong, R., additional, Jowett, S., additional, Nicholls, E., additional, Whitehurst, D., additional, Hill, S., additional, Hammond, A., additional, Hay, E., additional, Dziedzic, K., additional, Righetti, C., additional, Lebmeier, M., additional, Manning, V. L., additional, Hurley, M., additional, Scott, D. L., additional, Choy, E., additional, Bearne, L., additional, Nikiphorou, E., additional, Morris, S., additional, James, D., additional, Wong, E. C., additional, Long, J., additional, Fletcher, A., additional, Holmes, S., additional, Hockey, P., additional, Abbas, M., additional, Chattopadhyay, C., additional, Flint, J., additional, Gayed, M., additional, Schreiber, K., additional, Arthanari, S., additional, Nisar, M., additional, Khamashta, M., additional, Gordon, C., additional, Giles, I., additional, Robson, J., additional, Kiran, A., additional, Maskell, J., additional, Arden, N., additional, Hutchings, A., additional, Emin, A., additional, Culliford, D., additional, Dasgupta, B., additional, Hamilton, W., additional, Luqmani, R., additional, Jethwa, H., additional, Rowczenio, D., additional, Trojer, H., additional, Russell, T., additional, Loeffler, J., additional, Hawkins, P., additional, Lachmann, H., additional, Verma, I., additional, Syngle, A., additional, Krishan, P., additional, Garg, N., additional, McGowan, S. P., additional, Gerrard, D. T., additional, Chinoy, H., additional, Ollier, W. E., additional, Cooper, R. G., additional, Lamb, J. A., additional, Taborda, L., additional, Correia Azevedo, P., additional, Isenberg, D., additional, Leyland, K. M., additional, Judge, A., additional, Hunter, D., additional, Hart, D., additional, Javaid, M. K., additional, Edwards, M. H., additional, Litwic, A. E., additional, Jameson, K. A., additional, Deeg, D., additional, Cushnaghan, J., additional, Aihie Sayer, A., additional, Jagannath, D., additional, Parsons, C., additional, Stoppiello, L., additional, Mapp, P., additional, Ashraf, S., additional, Wilson, D., additional, Hill, R., additional, Scammell, B., additional, Wenham, C., additional, Shore, P., additional, Hodgson, R., additional, Grainger, A., additional, Aaron, J., additional, Hordon, L., additional, Conaghan, P., additional, Bar-Ziv, Y., additional, Beer, Y., additional, Ran, Y., additional, Benedict, S., additional, Halperin, N., additional, Drexler, M., additional, Mor, A., additional, Segal, G., additional, Lahad, A., additional, Haim, A., additional, Rath, U., additional, Morgensteren, D. M., additional, Salai, M., additional, Elbaz, A., additional, Vasishta, V. G., additional, Derrett-Smith, E., additional, Hoyles, R., additional, Khan, K., additional, Ezeonyeji, A., additional, Takhar, G., additional, Ong, V., additional, Loughrey, L., additional, Bissell, L.-A., additional, Hensor, E., additional, Abignano, G., additional, Redmond, A., additional, Del Galdo, F., additional, Hall, F. C., additional, Malaviya, A., additional, Baker, S., additional, Furlong, A., additional, Mitchell, A., additional, Godfrey, A. L., additional, Ruddlesden, M., additional, Hadjinicolaou, A., additional, Hughes, M., additional, Moore, T., additional, O'Leary, N., additional, Tracey, A., additional, Ennis, H., additional, Dinsdale, G., additional, Roberts, C., additional, Herrick, A., additional, Denton, C. P., additional, Guillevin, L., additional, Hunsche, E., additional, Rosenberg, D., additional, Schwierin, B., additional, Scott, M., additional, Krieg, T., additional, Anderson, M., additional, Matucci-Cerinic, M., additional, Alade, R., additional, Xu, S., additional, Nihtyanova, S., additional, Clark, K. E., additional, Tam, F. W. K., additional, Unwin, R., additional, Stratton, R. J., additional, Schreiber, B., additional, Seng Edwin Lim, C., additional, Corsiero, E., additional, Sutcliffe, N., additional, Wardemann, H., additional, Pitzalis, C., additional, Bombardieri, M., additional, Tahir, H., additional, Donnelly, S., additional, Greenwood, M., additional, Smith, T. O., additional, Easton, V., additional, Bacon, H., additional, Jerman, E., additional, Armon, K., additional, Poland, F., additional, Macgregor, A., additional, van der Heijde, D., additional, Sieper, J., additional, Elewaut, D., additional, Pangan, A. L., additional, Nguyen, D., additional, Badenhorst, C., additional, Kirby, S., additional, White, D., additional, Harrison, A., additional, Garcia, J. A., additional, Stebbings, S., additional, MacKay, J. W., additional, Aboelmagd, S., additional, Gaffney, K., additional, Deodhar, A., additional, Braun, J., additional, Mack, M., additional, Hsu, B., additional, Gathany, T., additional, Han, C., additional, Inman, R. D., additional, Cooper-Moss, N., additional, Packham, J., additional, Strauss, V., additional, Freeston, J. E., additional, Coates, L., additional, Nam, J., additional, Moverley, A. R., additional, Helliwell, P., additional, Wakefield, R., additional, Mease, P., additional, Fleischmann, R., additional, Wollenhaupt, J., additional, Kielar, D., additional, Woltering, F., additional, Stach, C., additional, Hoepken, B., additional, Arledge, T., additional, Gladman, D., additional, Coteur, G., additional, Kavanaugh, A., additional, Purcaru, O., additional, McInnes, I., additional, Gottlieb, A. B., additional, Puig, L., additional, Rahman, P., additional, Ritchlin, C., additional, Li, S., additional, Wang, Y., additional, Mendelsohn, A., additional, Doyle, M., additional, Tillett, W., additional, Jadon, D., additional, Shaddick, G., additional, Cavill, C., additional, Robinson, G., additional, Sengupta, R., additional, Korendowych, E., additional, de Vries, C., additional, Thomas, R. C., additional, Shuto, T., additional, Busquets-Perez, N., additional, Marzo-Ortega, H., additional, McGonagle, D., additional, Richards, G., additional, Bingham, S., additional, John Hamlin, P., additional, Adshead, R., additional, Cambridge, S., additional, Suppiah, P., additional, Cullinan, M., additional, Nolan, A., additional, Thompson, W. M., additional, Mathieson, H. R., additional, Mackie, S. L., additional, Bryer, D., additional, Krutikov, M., additional, Gray, L., additional, Bruce, E., additional, Keat, A., additional, Innes, W., additional, Pandit, R., additional, Kay, L., additional, Lapshina, S., additional, Myasoutova, L., additional, Erdes, S., additional, Wallis, D., additional, Waldron, N., additional, Thorne, I., additional, Harris, C., additional, Vohra, K., additional, Khinchi, D., additional, Kaur, L., additional, Jones, A., additional, Harrison, N., additional, Harris, D., additional, Jones, T., additional, Rees, J., additional, Bennett, A., additional, Fazal, S., additional, Tugnet, N., additional, Barkham, N., additional, Basu, N., additional, McClean, A., additional, Harper, L., additional, Amft, E. N., additional, Dhaun, N., additional, Luqmani, R. A., additional, Little, M. A., additional, Jayne, D. R., additional, Flossmann, O., additional, McLaren, J., additional, Kumar, V., additional, Reid, D. M., additional, Macfarlane, G. J., additional, Jones, G., additional, Yates, M., additional, Watts, R. A., additional, Igali, L., additional, Mukhtyar, C., additional, Doll, H., additional, Yew, S., additional, Suppiah, R., additional, Hoglund, P., additional, Jayne, D., additional, Westman, K., additional, Win Maw, W., additional, Patil, P., additional, Williams, M., additional, Adizie, T., additional, Christidis, D., additional, Borg, F., additional, Robertson, A., additional, Croft, A. P., additional, Smith, S., additional, Carr, S., additional, Youssouf, S., additional, Salama, A., additional, Pusey, C., additional, and Morgan, M., additional

Published
2013
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29. Scleroderma and related disorders: 223. Long Term Outcome in a Contemporary Systemic Sclerosis Cohort

Author

Nihtyanova, S., primary, Ong, V., additional, Black, C., additional, Denton, C., additional, Lutalo, P., additional, Shattles, W., additional, Jones, H., additional, Nouri, R., additional, Hepburn, A., additional, Chard, M., additional, Horwood, N., additional, Lynn, M., additional, Duke, O., additional, Kiely, P., additional, Zouita, L., additional, Davies, U., additional, Hughes, R., additional, Lloyd, M., additional, Nikitorowicz Buniak, J., additional, Shiwen, X., additional, Abraham, D., additional, Stratton, R., additional, Hugle, T., additional, Schuetz, P., additional, Daikeler, T., additional, Tyndall, A., additional, Matucci-Cerinic, M., additional, Walker, U. A., additional, van Laar, J. M., additional, Pauling, J. D., additional, Flower, V., additional, McHugh, N., additional, Liu, S., additional, Leask, A., additional, Aden, N., additional, Khan, K., additional, Hoyles, R., additional, Bhagat, S., additional, Drummond, T., additional, Goh, C., additional, Busch, R., additional, Hall, F., additional, Meyer, P., additional, Moinzadeh, P., additional, Krieg, T., additional, Hellmich, M., additional, Brinckmann, J., additional, Neumann, E., additional, Mueller-Ladner, U., additional, Kreuter, A., additional, Dumitresco, D., additional, Rosenkranz, S., additional, Hunzelmann, N., additional, Binai, N., additional, Huegle, T., additional, van Laar, J., additional, Sonnylal, S., additional, Tam, A., additional, Norman, J., additional, de Crombrugghe, B., additional, Chighizola, C. B., additional, Luigi Meroni, P., additional, Coghlan, G., additional, Newton, F., additional, Derrett-Smith, E. C., additional, Dooley, A., additional, Baliga, R., additional, Hobbs, A., additional, MacAllister, R., additional, Futema, M., additional, Pantelidis, P., additional, Renzoni, E., additional, Schreiber, B. E., additional, Coghlan, G. J., additional, Wells, A. U., additional, Welsh, K., additional, Fonseca, C., additional, Ponticos, M., additional, Wells, A., additional, Guillevin, L., additional, Schwierin, B., additional, Rosenberg, D., additional, Silkey, M., additional, Parapuram, S., additional, Shi-wen, X., additional, Nihtyanova, S., additional, Ahmed Abdi, B., additional, and Xu, S., additional

Published
2011
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31. Scleroderma and Related Disorders [202-212]: 202. Multi-Centre Audit of Treatment of Interstitial Lung Disease in Systemic Sclerosis with IV Cyclophosphamide

Author

Yazdani, R., primary, Abhishek, A., additional, Fiona, P., additional, Lim, K., additional, Regan, M., additional, Lanyon, P., additional, Khan, K., additional, Hoyles, R. K., additional, Shiwen, X., additional, Derrett-Smith, E., additional, Abraham, D., additional, Denton, C. P., additional, Ottewell, L., additional, Walker, K., additional, Griffiths, B., additional, Ali Nazarinia, M., additional, Abbasi, N., additional, Karimi, A., additional, Amiri, A., additional, Derrett-Smith, E. C., additional, Baliga, R., additional, Dooley, A., additional, Shi-Wen, X., additional, Stretton, K., additional, Shukla, S., additional, Hall, F., additional, Nandagudi, A., additional, Kingsley, G., additional, Scott, D., additional, Stratton, R., additional, Leask, A., additional, Guillevin, L., additional, Krieg, T., additional, Schwierin, B., additional, Rosenberg, D., additional, Silkey, M., additional, Matucci-Cerinic, M., additional, Jones, H., additional, Bou-Gharios, G., additional, So, P., additional, Renzoni, E., additional, Denton, C., additional, and Wells, A., additional

Published
2010
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34. Nintedanib for non-IPF progressive pulmonary fibrosis: 12-month outcome data from a real-world multicentre observational study.

Author

Raman L, Stewart I, Barratt SL, Chua F, Chaudhuri N, Crawshaw A, Gibbons M, Hogben C, Hoyles R, Kouranos V, Martinovic J, Mulholland S, Myall KJ, Naqvi M, Renzoni EA, Saunders P, Steward M, Suresh D, Thillai M, Wells AU, West A, Mitchell JA, and George PM

Abstract

Background: Nintedanib slows lung function decline for patients with non-idiopathic pulmonary fibrosis progressive pulmonary fibrosis (PPF) in clinical trials, but the real-world safety and efficacy are not known., Methods: In this retrospective cohort study, standardised data were collected from patients in whom nintedanib was initiated for PPF between 2019 and 2020 through an early-access programme across eight centres in the United Kingdom. Rate of lung function change in the 12   months pre- and post-nintedanib initiation was the primary analysis. Symptoms, drug safety, tolerability and stratification by interstitial lung disease subtype and computed tomography pattern were secondary analyses., Results: 126 patients were included; 67 (53%) females; mean±sd age 60±13   years. At initiation of nintedanib, mean forced vital capacity (FVC) was 1.87   L (58% predicted) and diffusing capacity of the lung for carbon monoxide ( D LCO ) was 32.7% predicted. 68% of patients were prescribed prednisolone (median dose 10   mg) and 69% were prescribed a steroid-sparing agent. In the 12   months after nintedanib initiation, lung function decline was significantly lower than in the preceding 12   months: FVC -88.8 mL versus -239.9 mL (p=0.004), and absolute decline in D LCO -2.1% versus -6.1% (p=0.004). Response to nintedanib was consistent in sensitivity and secondary analyses. 89 (71%) out of 126 patients reported side-effects, but 86 (80%) of the surviving 108 patients were still taking nintedanib at 12   months with patients reporting a reduced perception of symptom decline. There were no serious adverse events., Conclusion: In PPF, the real-world efficacy of nintedanib replicated that of clinical trials, significantly attenuating lung function decline. Despite the severity of disease, nintedanib was safe and well tolerated in this real-world multicentre study., Competing Interests: Conflict of interest: I. Stewart receives funding from the Rayne Foundation. F. Chua reports personal fees from Boehringer Ingelheim. N. Chaudhuri reports personal fees from Boehringer Ingelheim, Redex, Novartis, Liminal Biosciences, Vicor Pharma, Bridge Biotherapeutics and Roche. M. Gibbons reports personal fees for advisory boards and support for attending conferences from Boehringer Ingelheim. C. Hogben has received personal fees from Boehringer Ingelheim. V. Kouranos reports personal fees from Boehringer Ingelheim and Novartis. S. Mulholland reports personal fees from Boehringer Ingelheim. M. Naqvi reports honoraria from Boehringer Ingelheim, Astra Zeneca and Roche, and grant support paid to their institution from an NHSX digital award. E.A. Renzoni reports fees paid to their institution from Boehringer Ingelheim, Novartis and Roche, and research grants paid to their institution from Boehringer Ingelheim. M. Thillai reports personal fees from Boehringer Ingelheim, research grants paid to their institution from Boehringer Ingelheim and support for conference attendance from Boehringer Ingelheim. A.U. Wells is president elect of WASOG, and reports personal fees from Boehringer Ingelheim and Roche, and support for conference from Boehringer Ingelheim. P.M. George reports personal fees from Boehringer Ingelheim, Roche, Teva, Cipla and Brainomix, research grants paid to their institution from Boehringer Ingelheim, and support for conference attendance from Boehringer Ingelheim and Roche. No other authors report any conflicts of interest., (Copyright ©The authors 2023.)

Published
2023
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35. Increased monocyte level is a risk factor for radiological progression in patients with early fibrotic interstitial lung abnormality.

Author

Achaiah A, Lyon P, Fraser E, Saunders P, Hoyles R, Benamore R, and Ho LP

Abstract

Background: Interstitial lung abnormalities (ILA) are specific spatial patterns on computed tomography (CT) scan potentially compatible with early interstitial lung disease. A proportion will progress; management involves risk stratification and surveillance. Elevated blood monocyte levels have been shown to associate with progression of idiopathic pulmonary fibrosis. The aims of the present study were: 1) to estimate the proportion of "early fibrotic" (EF)-ILAs (reticular±ground-glass opacities, excluding traction bronchiectasis and honeycombing) on CT scans of patients attending all-indications thoracic CTs, and proportion demonstrating radiological progression; and 2) to explore association between peripheral blood leukocyte levels and ILA progression., Methods: We analysed all thoracic CT reports in individuals aged 45-75 years performed between January 2015 and December 2020 in one large teaching hospital (Oxford, UK) to identify patient CT reports consistent with EF-ILA. CT-contemporaneous blood leukocyte counts were examined to explore contribution to progression and all-cause mortality, using multivariate Cox regression., Results: 40 711 patients underwent thoracic CT imaging during this period. 1259 (3.1%) demonstrated the EF-ILA pattern (mean±sd age 65.4±7.32 years; 735 (47.8%) male). EF-ILA was significantly associated with all-cause mortality (hazard ratio 1.87, 95% CI 1.25-2.78; p=0.002). 362 cases underwent at least one follow-on CT. Radiological progression was observed in 157 (43.4%) cases: increase in reticulation n=51, new traction bronchiectasis n=84, honeycombing n=22. Monocyte count, neutrophil count, monocyte:lymphocyte ratio, neutrophil:lymphocyte ratio and "systemic inflammatory response index" were significantly associated with radiological progression., Conclusion: 3.1% of subjects requiring thoracic CT during a 6-year period demonstrated EF-ILA. Monocyte levels and blood leukocyte-derived indexes were associated with radiological progression and could indicate which patients may require closer follow-up., Competing Interests: Conflict of interest: A. Achaiah has nothing to disclose. Conflict of interest: P. Lyon has nothing to disclose. Conflict of interest: E. Fraser has nothing to disclose. Conflict of interest: P. Saunders has received consultancy fees from Trevi Therapeutics and lecture fees from Boehringer Ingelheim, but no other conflict of interest. Conflict of interest: R. Hoyles has nothing to disclose. Conflict of interest: R. Benamore has nothing to disclose. Conflict of interest: L-P. Ho has nothing to disclose., (Copyright ©The authors 2022.)

Published
2022
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36. Multi-Modal Characterization of Monocytes in Idiopathic Pulmonary Fibrosis Reveals a Primed Type I Interferon Immune Phenotype.

Author

Fraser E, Denney L, Antanaviciute A, Blirando K, Vuppusetty C, Zheng Y, Repapi E, Iotchkova V, Taylor S, Ashley N, St Noble V, Benamore R, Hoyles R, Clelland C, Rastrick JMD, Hardman CS, Alham NK, Rigby RE, Simmons A, Rehwinkel J, and Ho LP

Subjects
Case-Control Studies, Cells, Cultured, Chemokine CCL2 blood, Flow Cytometry, Gene Expression Profiling, Humans, Idiopathic Pulmonary Fibrosis genetics, Idiopathic Pulmonary Fibrosis metabolism, Idiopathic Pulmonary Fibrosis pathology, Immunophenotyping, Interferon Type I genetics, Interleukin-6 blood, Lung metabolism, Lung pathology, Macrophage Colony-Stimulating Factor blood, Macrophages immunology, Macrophages metabolism, Monocytes metabolism, Phenotype, Receptors, IgG genetics, Receptors, IgG metabolism, Single-Cell Analysis, Idiopathic Pulmonary Fibrosis immunology, Interferon Type I metabolism, Lung immunology, Monocytes immunology
Abstract

Idiopathic pulmonary fibrosis (IPF) is the most severe form of chronic lung fibrosis. Circulating monocytes have been implicated in immune pathology in IPF but their phenotype is unknown. In this work, we determined the immune phenotype of monocytes in IPF using multi-colour flow cytometry, RNA sequencing and corresponding serum factors, and mapped the main findings to amount of lung fibrosis and single cell transcriptomic landscape of myeloid cells in IPF lungs. We show that monocytes from IPF patients displayed increased expression of CD64 (FcγR1) which correlated with amount of lung fibrosis, and an amplified type I IFN response ex vivo . These were accompanied by markedly raised CSF-1 levels, IL-6, and CCL-2 in serum of IPF patients. Interrogation of single cell transcriptomic data from human IPF lungs revealed increased proportion of CD64 hi monocytes and "transitional macrophages" with higher expression of CCL-2 and type I IFN genes. Our study shows that monocytes in IPF patients are phenotypically distinct from age-matched controls, with a primed type I IFN pathway that may contribute to driving chronic inflammation and fibrosis. These findings strengthen the potential role of monocytes in the pathogenesis of IPF., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Fraser, Denney, Antanaviciute, Blirando, Vuppusetty, Zheng, Repapi, Iotchkova, Taylor, Ashley, St Noble, Benamore, Hoyles, Clelland, Rastrick, Hardman, Alham, Rigby, Simmons, Rehwinkel and Ho.)

Published
2021
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37. Serum interleukin 6 is predictive of early functional decline and mortality in interstitial lung disease associated with systemic sclerosis.

Author

De Lauretis A, Sestini P, Pantelidis P, Hoyles R, Hansell DM, Goh NS, Zappala CJ, Visca D, Maher TM, Denton CP, Ong VH, Abraham DJ, Kelleher P, Hector L, Wells AU, and Renzoni EA

Subjects
Adult, Aged, Cytokines blood, Disease Progression, Female, Humans, Lung Diseases, Interstitial etiology, Lung Diseases, Interstitial mortality, Male, Middle Aged, Predictive Value of Tests, Prognosis, Prospective Studies, Respiratory Function Tests, Scleroderma, Systemic complications, Scleroderma, Systemic mortality, Vital Capacity, Interleukin-6 blood, Lung Diseases, Interstitial blood, Scleroderma, Systemic blood
Abstract

Objective: Biomarkers of progression of interstitial lung disease (ILD) are needed to allow early therapeutic intervention in patients with scleroderma-associated disease (SSc-ILD)., Methods: A panel of 8 serum cytokines [interleukin 6 (IL-6), IL-8, IL-10, CCL2, CXCL10, vascular endothelial growth factor, fibroblast growth factor 2, and CX3CL1] was assessed by Luminex bead technology in exploratory cohorts of 74 patients with SSc and 58 patients with idiopathic pulmonary fibrosis (IPF). Mortality and significant lung function decline [forced vital capacity (FVC) ≥ 10%; DLCO ≥ 15%] from date of serum collection were evaluated by proportional hazards analysis. Based on these findings, the prognostic value of serum IL-6, evaluated by ELISA, was assessed in a larger test cohort of 212 patients with SSc-ILD., Results: In the exploratory cohort, only serum IL-6 was an independent predictor of DLCO decline in both IPF and SSc-ILD. The IL-6 threshold level most predictive of DLCO decline within a year was 7.67 pg/ml. In the larger test cohort, serum IL-6 > 7.67 pg/ml was predictive of decline in FVC (HR 2.58 ± 0.98, p = 0.01) and in DLCO (HR 3.2 ± 1.7, p = 0.02) within the first year, and predictive of death within the first 30 months (HR 2.69 ± 0.96, p = 0.005). When stratified according to severity (FVC < 70%), serum IL-6 > 7.67 pg/ml was predictive of functional decline or death within the first year in patients with milder disease (OR 3.1, 95% CI 1.4-7.2, p = 0.007), but not in those with severe ILD., Conclusion: In SSc-ILD, serum IL-6 levels appear to be predictive of early disease progression in patients with mild ILD, and could be used to target treatment in this group, if confirmed by prospective studies.

Published
2013
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38. Increased epithelial permeability in pulmonary fibrosis in relation to disease progression.

Author

Goh NS, Desai SR, Anagnostopoulos C, Hansell DM, Hoyles RK, Sato H, Denton CP, Black CM, du Bois RM, and Wells AU

Subjects
Adult, Aged, Cohort Studies, Disease Progression, Female, Humans, Idiopathic Pulmonary Fibrosis pathology, Male, Middle Aged, Odds Ratio, Prognosis, Proportional Hazards Models, Radiopharmaceuticals pharmacology, Treatment Outcome, Epithelium pathology, Permeability, Pulmonary Fibrosis pathology, Technetium Tc 99m Pentetate pharmacology
Abstract

Epithelial injury contributes to pathogenesis in idiopathic pulmonary fibrosis (IPF) but its role in the interstitial lung disease (ILD) of systemic sclerosis (SSc) is uncertain. We quantified the prognostic significance of inhaled technetium-99m ((99m)Tc)-labelled diethylene triamine pentacetate (DTPA) pulmonary clearance, a marker of the extent of epithelial injury, in both diseases. Baseline (99m)Tc-DTPA pulmonary clearance was evaluated retrospectively in patients with SSc-ILD (n = 168) and IPF (n = 97) against mortality and disease progression. In SSc-ILD, the rapidity of total clearance (hazard ratio (HR) 1.02, 95% CI 1.01-1.03; p = 0.001) and the presence of abnormally rapid clearance (HR 2.10; 95% CI 1.25-3.53; p = 0.005) predicted a shorter time to forced vital capcity (FVC) decline, independent of disease severity. These associations were robust in both mild and severe disease. By contrast, in IPF, delayed clearance of the slow component, an expected consequence of honeycomb change, was an independent predictor of a shorter time to FVC decline (HR 1.01, 95% CI 1.00-1.02; p<0.01). Epithelial injury should be incorporated in pathogenetic models in SSc-ILD. By contrast, outcome is not linked to the overall extent of epithelial injury in IPF, apart from abnormalities ascribable to honeycombing, suggesting that core pathogenetic events may be more spatially focal in that disease.

Published
2011
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