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3. Evidence-Based Guideline for the diagnosis and management of eosinophilic granulomatosis with polyangiitis

Author

Emmi, G, Bettiol, A, Gelain, E, Bajema, I, Berti, A, Burns, S, Cid, M, Cohen Tervaert, J, Cottin, V, Durante, E, Holle, J, Mahr, A, Del Pero, M, Marvisi, C, Mills, J, Moiseev, S, Moosig, F, Mukhtyar, C, Neumann, T, Olivotto, I, Salvarani, C, Seeliger, B, Sinico, R, Taillé, C, Terrier, B, Venhoff, N, Bertsias, G, Guillevin, L, Jayne, D, Vaglio, A, Emmi G., Bettiol A., Gelain E., Bajema I. M., Berti A., Burns S., Cid M. C., Cohen Tervaert J. W., Cottin V., Durante E., Holle J. U., Mahr A. D., Del Pero M. M., Marvisi C., Mills J., Moiseev S., Moosig F., Mukhtyar C., Neumann T., Olivotto I., Salvarani C., Seeliger B., Sinico R. A., Taillé C., Terrier B., Venhoff N., Bertsias G., Guillevin L., Jayne D. R. W., Vaglio A., Emmi, G, Bettiol, A, Gelain, E, Bajema, I, Berti, A, Burns, S, Cid, M, Cohen Tervaert, J, Cottin, V, Durante, E, Holle, J, Mahr, A, Del Pero, M, Marvisi, C, Mills, J, Moiseev, S, Moosig, F, Mukhtyar, C, Neumann, T, Olivotto, I, Salvarani, C, Seeliger, B, Sinico, R, Taillé, C, Terrier, B, Venhoff, N, Bertsias, G, Guillevin, L, Jayne, D, Vaglio, A, Emmi G., Bettiol A., Gelain E., Bajema I. M., Berti A., Burns S., Cid M. C., Cohen Tervaert J. W., Cottin V., Durante E., Holle J. U., Mahr A. D., Del Pero M. M., Marvisi C., Mills J., Moiseev S., Moosig F., Mukhtyar C., Neumann T., Olivotto I., Salvarani C., Seeliger B., Sinico R. A., Taillé C., Terrier B., Venhoff N., Bertsias G., Guillevin L., Jayne D. R. W., and Vaglio A.

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, characterized by asthma, eosinophilia and granulomatous or vasculitic involvement of several organs. The diagnosis and management of EGPA are often challenging and require an integrated, multidisciplinary approach. Current practice relies on recommendations and guidelines addressing the management of ANCA-associated vasculitis and not specifically developed for EGPA. Here, we present evidence-based, cross-discipline guidelines for the diagnosis and management of EGPA that reflect the substantial advances that have been made in the past few years in understanding the pathogenesis, clinical subphenotypes and differential diagnosis of the disease, as well as the availability of new treatment options. Developed by a panel of European experts on the basis of literature reviews and, where appropriate, expert opinion, the 16 statements and five overarching principles cover the diagnosis and staging, treatment, outcome and follow-up of EGPA. These recommendations are primarily intended to be used by healthcare professionals, pharmaceutical industries and drug regulatory authorities, to guide clinical practice and decision-making in EGPA. These guidelines are not intended to limit access to medications by healthcare agencies, nor to impose a fixed order on medication use.

Published
2023

4. The provisional OMERACT ultrasonography score for giant cell arteritis

Author

Dejaco, C, Ponte, C, Monti, S, Rozza, D, Scire, C, Terslev, L, Bruyn, G, Boumans, D, Hartung, W, Hocevar, A, Milchert, M, Dohn, U, Mukhtyar, C, Aschwanden, M, Bosch, P, Camellino, D, Chrysidis, S, Ciancio, G, D'Agostino, M, Daikeler, T, Dasgupta, B, De Miguel, E, Diamantopoulos, A, Duftner, C, Agueda, A, Fredberg, U, Hanova, P, Hansen, I, Hauge, E, Iagnocco, A, Inanc, N, Juche, A, Karalilova, R, Kawamoto, T, Keller, K, Keen, H, Kermani, T, Kohler, M, Koster, M, Luqmani, R, Macchioni, P, Mackie, S, Naredo, E, Nielsen, B, Ogasawara, M, Pineda, C, Schafer, V, Seitz, L, Tomelleri, A, Torralba, K, Van Der Geest, K, Warrington, K, Schmidt, W, Dejaco C., Ponte C., Monti S., Rozza D., Scire C. A., Terslev L., Bruyn G. A. W., Boumans D., Hartung W., Hocevar A., Milchert M., Dohn U. Mo., Mukhtyar C. B., Aschwanden M., Bosch P., Camellino D., Chrysidis S., Ciancio G., D'Agostino M. A., Daikeler T., Dasgupta B., De Miguel E., Diamantopoulos A. P., Duftner C., Agueda A., Fredberg U., Hanova P., Hansen I. To., Hauge E. -M., Iagnocco A., Inanc N., Juche A., Karalilova R., Kawamoto T., Keller K. K., Keen H. I., Kermani T. A., Kohler M. J., Koster M., Luqmani R. A., Macchioni P., Mackie S. L., Naredo E., Nielsen B. D., Ogasawara M., Pineda C., Schafer V. S., Seitz L., Tomelleri A., Torralba K. D., Van Der Geest K. S. M., Warrington K. J., Schmidt W. A., Dejaco, C, Ponte, C, Monti, S, Rozza, D, Scire, C, Terslev, L, Bruyn, G, Boumans, D, Hartung, W, Hocevar, A, Milchert, M, Dohn, U, Mukhtyar, C, Aschwanden, M, Bosch, P, Camellino, D, Chrysidis, S, Ciancio, G, D'Agostino, M, Daikeler, T, Dasgupta, B, De Miguel, E, Diamantopoulos, A, Duftner, C, Agueda, A, Fredberg, U, Hanova, P, Hansen, I, Hauge, E, Iagnocco, A, Inanc, N, Juche, A, Karalilova, R, Kawamoto, T, Keller, K, Keen, H, Kermani, T, Kohler, M, Koster, M, Luqmani, R, Macchioni, P, Mackie, S, Naredo, E, Nielsen, B, Ogasawara, M, Pineda, C, Schafer, V, Seitz, L, Tomelleri, A, Torralba, K, Van Der Geest, K, Warrington, K, Schmidt, W, Dejaco C., Ponte C., Monti S., Rozza D., Scire C. A., Terslev L., Bruyn G. A. W., Boumans D., Hartung W., Hocevar A., Milchert M., Dohn U. Mo., Mukhtyar C. B., Aschwanden M., Bosch P., Camellino D., Chrysidis S., Ciancio G., D'Agostino M. A., Daikeler T., Dasgupta B., De Miguel E., Diamantopoulos A. P., Duftner C., Agueda A., Fredberg U., Hanova P., Hansen I. To., Hauge E. -M., Iagnocco A., Inanc N., Juche A., Karalilova R., Kawamoto T., Keller K. K., Keen H. I., Kermani T. A., Kohler M. J., Koster M., Luqmani R. A., Macchioni P., Mackie S. L., Naredo E., Nielsen B. D., Ogasawara M., Pineda C., Schafer V. S., Seitz L., Tomelleri A., Torralba K. D., Van Der Geest K. S. M., Warrington K. J., and Schmidt W. A.

Abstract

Objectives To develop an Outcome Measures in Rheumatology (OMERACT) ultrasonography score for monitoring disease activity in giant cell arteritis (GCA) and evaluate its metric properties. Methods The OMERACT Instrument Selection Algorithm was followed. Forty-nine members of the OMERACT ultrasonography large vessel vasculitis working group were invited to seven Delphi rounds. An online reliability exercise was conducted using images of bilateral common temporal arteries, parietal and frontal branches as well as axillary arteries from 16 patients with GCA and 7 controls. Sensitivity to change and convergent construct validity were tested using data from a prospective cohort of patients with new GCA in which ultrasound-based intima-media thickness (IMT) measurements were conducted at weeks 1, 3, 6, 12 and 24. Results Agreement was obtained (92.7%) for the OMERACT GCA Ultrasonography Score (OGUS), calculated as follows: sum of IMT measured in every segment divided by the rounded cut-off values of IMTs in each segment. The resulting value is then divided by the number of segments available. Thirty-five members conducted the reliability exercise, the interrater intraclass correlation coefficient (ICC) for the OGUS was 0.72-0.84 and the median intrareader ICC was 0.91. The prospective cohort consisted of 52 patients. Sensitivity to change between baseline and each follow-up visit up to week 24 yielded standardised mean differences from -1.19 to -2.16, corresponding to large and very large magnitudes of change, respectively. OGUS correlated moderately with erythrocyte sedimentation rate, C reactive protein and Birmingham Vasculitis Activity Score (corr coeff 0.37-0.48). Conclusion We developed a provisional OGUS for potential use in clinical trials.

Published
2023

8. OMERACT definition and reliability assessment of chronic ultrasound lesions of the axillary artery in giant cell arteritis

Author

Schafer, V, Chrysidis, S, Schmidt, W, Duftner, C, Iagnocco, A, Bruyn, G, Carrara, G, De Miguel, E, Diamantopoulos, A, Nielsen, B, Fredberg, U, Hartung, W, Hanova, P, Hansen, I, Hocevar, A, Juche, A, Kermani, T, Lorenzen, T, Macchioni, P, Milchert, M, Dohn, U, Mukhtyar, C, Monti, S, Ponte, C, Seitz, L, Scire, C, Terslev, L, Dasgupta, B, Keen, H, Pineda, C, Dejaco, C, Schafer V. S., Chrysidis S., Schmidt W. A., Duftner C., Iagnocco A., Bruyn G. A., Carrara G., De Miguel E., Diamantopoulos A. P., Nielsen B. D., Fredberg U., Hartung W., Hanova P., Hansen I. T., Hocevar A., Juche A., Kermani T. A., Lorenzen T., Macchioni P., Milchert M., Dohn U. M., Mukhtyar C., Monti S., Ponte C., Seitz L., Scire C. A., Terslev L., Dasgupta B., Keen H. I., Pineda C., Dejaco C., Schafer, V, Chrysidis, S, Schmidt, W, Duftner, C, Iagnocco, A, Bruyn, G, Carrara, G, De Miguel, E, Diamantopoulos, A, Nielsen, B, Fredberg, U, Hartung, W, Hanova, P, Hansen, I, Hocevar, A, Juche, A, Kermani, T, Lorenzen, T, Macchioni, P, Milchert, M, Dohn, U, Mukhtyar, C, Monti, S, Ponte, C, Seitz, L, Scire, C, Terslev, L, Dasgupta, B, Keen, H, Pineda, C, Dejaco, C, Schafer V. S., Chrysidis S., Schmidt W. A., Duftner C., Iagnocco A., Bruyn G. A., Carrara G., De Miguel E., Diamantopoulos A. P., Nielsen B. D., Fredberg U., Hartung W., Hanova P., Hansen I. T., Hocevar A., Juche A., Kermani T. A., Lorenzen T., Macchioni P., Milchert M., Dohn U. M., Mukhtyar C., Monti S., Ponte C., Seitz L., Scire C. A., Terslev L., Dasgupta B., Keen H. I., Pineda C., and Dejaco C.

Abstract

Objectives: To define chronic ultrasound lesions of the axillary artery (AA) in long-standing giant cell arteritis (GCA) and to evaluate the reliability of the new ultrasound definition in a web-based exercise. Methods: A structured Delphi, involving an expert panel of the Large Vessel Vasculitis subgroup of the Outcome Measures in Rheumatology (OMERACT) Ultrasound Working Group was carried out. The reliability of the new definition was tested in a 2-round web-based exercise involving 23 experts and using 50 still images each from AA of long-standing and acute GCA patients, as well as 50 images from healthy individuals. Results: The final OMERACT ultrasound definition of chronic changes was based on measurement and appearance of the intima-media complex. The overall reliability of the new definition for chronic ultrasound changes in longstanding GCA of the AA was good to excellent with Light's kappa values of 0.79-0.80 for inter-reader reliability and mean Light's-kappa of 0.88 for intra-reader reliability. The mean inter-rater and intra-rater agreements were 86-87% and 92%, respectively. Good reliabilities were observed comparing the vessels with longstanding versus acute GCA with a mean agreement and kappa values of 81% and 0.63, respectively. Conclusion: The new OMERACT ultrasound definition for chronic vasculitis of the AA in GCA revealed a good to excellent inter- and intra-reader reliability in a web-based exercise of experts.

Published
2021

9. 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

15. 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

16. Genome-wide association study of eosinophilic granulomatosis with polyangiitis reveals genomic loci stratified by ANCA status

Author

Lyons, P. A., Peters, J. E., Alberici, F., Liley, J., Coulson, R. M. R., Astle, W., Baldini, C., Bonatti, F., Cid, María Cinta, Elding, H., Emmi, G., Epplen, J., Guillevin, L., Jayne, D. R. W., Jiang, T., Gunnarsson, I., Lamprecht, P., Leslie, S., Little, M. A., Martorana, D., Moosig, F., Neumann, T., Ohlsson, S., Quickert, S., Ramirez, G. A., Rewerska, B., Schett, George, Sinico, R. A., Szczeklik, W., Tesar, Vladimir, Vukcevic, D., Akil, M., Barratt, J., Basu, N., Butterworth, A. S., Bruce, I., Clarkson, M., Conlon, N., DasGupta, B., Doulton, T. W. R., Espígol-Frigolé, Georgina, Flossmann, O., Gabrielli, A., Gasior, J., Gregorini, G., Guida, G., Hernández Rodríguez, José, Hruskova, Z., Hudson, A., Knight, A., Lanyon, P., Luqmani, R., Magliano, M., Manfredi, A. A., Marguerie, C., Maritati, F., Marvisi, C., McHugh, N. J., Molloy, E., Motyer, A., Mukhtyar, C., Padyukov, Leonid, Pesci, Alberto, Prieto-Gonzalez, S., Ramentol-Sintas, Marc, Reis, P., Roccatello, D., Rovere-Querini, P., Salvarani, C., Santarsia, F., Solans, Roser, Soranzo, N., Taylor, J., Wessels, J., Zwerina, J., Terrier, B., Watts, R. A., Vaglio, A., Holle, J. U., Wallace, C., Smith, K. G. C., Universitat Autònoma de Barcelona, Lyons, P. A., Peters, J. E., Alberici, F., Liley, J., Coulson, R. M. R., Astle, W., Baldini, C., Bonatti, F., Cid, M. C., Elding, H., Emmi, G., Epplen, J., Guillevin, L., Jayne, D. R. W., Jiang, T., Gunnarsson, I., Lamprecht, P., Leslie, S., Little, M. A., Martorana, D., Moosig, F., Neumann, T., Ohlsson, S., Quickert, S., Ramirez, G. A., Rewerska, B., Schett, G., Sinico, R. A., Szczeklik, W., Tesar, V., Vukcevic, D., Akil, M., Barratt, J., Basu, N., Butterworth, A. S., Bruce, I., Clarkson, M., Conlon, N., Dasgupta, B., Doulton, T. W. R., Espigol-Frigole, G., Flossmann, O., Gabrielli, A., Gasior, J., Gregorini, G., Guida, G., Hernandez-Rodriguez, J., Hruskova, Z., Hudson, A., Knight, A., Lanyon, P., Luqmani, R., Magliano, M., Manfredi, A. A., Marguerie, C., Maritati, F., Marvisi, C., Mchugh, N. J., Molloy, E., Motyer, A., Mukhtyar, C., Padyukov, L., Pesci, A., Prieto-Gonzalez, S., Ramentol-Sintas, M., Reis, P., Roccatello, D., Rovere-Querini, P., Salvarani, C., Santarsia, F., Solans-Laque, R., Soranzo, N., Taylor, J., Wessels, J., Zwerina, J., Terrier, B., Watts, R. A., Vaglio, A., Holle, J. U., Wallace, C., Smith, K. G. C., Lyons, P, Peters, J, Alberici, F, Liley, J, Coulson, R, Astle, W, Baldini, C, Bonatti, F, Cid, M, Elding, H, Emmi, G, Epplen, J, Guillevin, L, Jayne, D, Jiang, T, Gunnarsson, I, Lamprecht, P, Leslie, S, Little, M, Martorana, D, Moosig, F, Neumann, T, Ohlsson, S, Quickert, S, Ramierez, G, Rewerska, B, Schett, G, Sinico, R, Szczeklik, W, Tesar, V, Vukcevic, D, Akil, M, Barratt, J, Basu, N, Butterworth, A, Bruce, I, Clarkson, M, Conlon, N, Dasgupta, B, Doulton, T, Espigol-Frigole, G, Flossmann, O, Gabrielli, A, Gasior, J, Gregorini, G, Guida, G, Hernandez-Rodriguez, J, Hruskova, Z, Hudson, A, Knight, A, Lanyon, P, Luqmani, R, Magliano, M, Manfredi, A, Marguerie, C, Maritati, F, Marvisi, C, Mchugh, N, Molloy, E, Motyer, A, Mukhtyar, C, Padyukov, L, Pesci, A, Prieto-Gonzalez, S, Ramentol-Sintas, M, Reis, P, Roccatello, D, Rovere-Querini, P, Salvarani, C, Santarsia, F, Solans-Laque, R, Soranzo, N, Taylor, J, Wessels, J, Zwerina, J, Terrier, B, Watts, R, Vaglio, A, Holle, J, Wallace, C, Smith, K, and United Kingdom Research and Innovation

Subjects
0301 basic medicine, Candidate gene, Antineutrophil Cytoplasmic, General Physics and Astronomy, Genome-wide association study, Autoimmunity, Genome-wide association studies, 0302 clinical medicine, Rheumatic diseases, immune system diseases, Eosinophilic, Eosinophilia, lcsh:Science, education.field_of_study, Multidisciplinary, Genome-wide association, eosinophilic granulomatosis with polyangiitis, ANCA status, 3. Good health, medicine.symptom, Vasculitis, Granulomatosis with polyangiitis, Antibodies, Antineutrophil Cytoplasmic, Eosinophils, Genetic Association Studies, Granulomatosis with Polyangiitis, Humans, Mendelian Randomization Analysis, Genetic Loci, Genetic Predisposition to Disease, Genome-Wide Association Study, European Vasculitis Genetics Consortium, Science, Population, General Biochemistry, Genetics and Molecular Biology, Article, Antibodies, 03 medical and health sciences, medicine, Immunogenetics, education, Rheumatology and Autoimmunity, Anti-neutrophil cytoplasmic antibody, 030203 arthritis & rheumatology, Reumatologi och inflammation, business.industry, General Chemistry, medicine.disease, respiratory tract diseases, 030104 developmental biology, Immunology, EGPA, HLA, ANCA, genetics, lcsh:Q, business
Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare inflammatory disease of unknown cause. 30% of patients have anti-neutrophil cytoplasmic antibodies (ANCA) specific for myeloperoxidase (MPO). Here, we describe a genome-wide association study in 676 EGPA cases and 6809 controls, that identifies 4 EGPA-associated loci through conventional case-control analysis, and 4 additional associations through a conditional false discovery rate approach. Many variants are also associated with asthma and six are associated with eosinophil count in the general population. Through Mendelian randomisation, we show that a primary tendency to eosinophilia contributes to EGPA susceptibility. Stratification by ANCA reveals that EGPA comprises two genetically and clinically distinct syndromes. MPO+ ANCA EGPA is an eosinophilic autoimmune disease sharing certain clinical features and an HLA-DQ association with MPO+ ANCA-associated vasculitis, while ANCA-negative EGPA may instead have a mucosal/barrier dysfunction origin. Four candidate genes are targets of therapies in development, supporting their exploration in EGPA., Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare inflammatory disorder characterised by asthma, eosinophilia and vasculitis. Here, the authors describe a genome-wide association study of EGPA that reveals clinical and genetic differences between subgroups stratified by autoantibody status (ANCA).

Published
2019

17. Clinical Utility of Random Anti–Tumor Necrosis Factor Drug–Level Testing and Measurement of Antidrug Antibodies on the Long-Term Treatment Response in Rheumatoid Arthritis

Author

Jani, Meghna, Chinoy, Hector, Warren, Richard B., Griffiths, Christopher E. M., Plant, Darren, Fu, Bo, Morgan, Ann W., Wilson, Anthony G., Isaacs, John D., Hyrich, Kimme L., Barton, Anne, Prouse, P. J., Moitra, R. K., Shawe, D. J., Nisar, M., Fairburn, K., Nixon, J., Barnes, T., Hui, M., Coady, D., Wright, D., Morley, C., Raftery, G., Bracewell, C., Bridges, M., Armstrong, D., Chuck, A. J., Hailwood, S., Kumar, N., Ashok, D., Reece, R., OʼReilly, S. C., Ding, T., Badcock, L. J., Deighton, C. M., Raj, N., Regan, M. R., Summers, G. D., Williams, R. A., Lambert, J. R., Stevens, R., Wilkinson, C., Kelly, C. A., Hamilton, J., Heycock, C. R., Saravanan, V., Cope, A., Garrood, T., Ng, N., Kirkham, B., Green, M., Gough, A., Lawson, C., Das, D., Borbas, E., Wazir, T., Emery, P., Bingham, S., Bird, H. A., Conaghan, P. G., Pease, C. T., Wakefield, R. J., Buch, M., Bruce, I., Gorodkin, R., Ho, P., Parker, B., Smith, W., Jenkins, E., Mukhtyar, C., Gaffney, K., Macgregor, A. J., Marshall, T., Merry, P., DeSilva, C., Birrell, F. N., Crook, P. R., Szebenyi, B., Bates, D., James, D., Gillott, T., Alvi, A., Grey, C., Browning, J., McHale, J. F., Gaywood, I. C., Jones, A. C., Lanyon, P., Pande, I., Doherty, M., Gupta, A., Courtney, P. A., Srikanth, A., Abhishek, A., Das, L., Pattrick, M., Snowden, H. N., Bowden, A. P., Smith, E. E., Klimiuk, P., Speden, D. J., Naz, S., Ledingham, J. M., Hull, R. G., McCrae, F., Cooper, A., Young-Min, S. A., Wong, E., Shaban, R., Woolf, A. D., Davis, M., Hutchinson, D., Endean, A., Mewar, D., Tunn, E. J., Nelson, K., Kennedy, T. D., Dubois, C., Pauling, J., Korendowych, E., Jenkinson, T., Sengupta, R., Bhalla, A., McHugh, N., OʼNeil, T., Herrick, A. L., Jones, A. K., Cooper, R. G., Dixon, W. G., Harrison, B., Buckley, C. D., Carruthers, D. C., Elamanchi, R., Gordon, P. C., Grindulis, K. A., Khattak, F., Raza, K., Situnayake, K., Akil, M., Till, S., Dunkley, L., Tattersall, R., Kilding, R., Tait, T., Maxwell, J., Kuet, K.-P., Plant, M. J., Clarke, F., Fordham, J. N., Tuck, S., Pathare, S. K., Paul, A., Marguerie, C. P., Rigby, S. P., Dunn, N., Abbas, I., Filer, C., Abernethy, V. E., Clewes, A. R., Dawson, J. K., Kitas, G., Erb, N., Klocke, R., Whallett, A. J., Douglas, K., Pace, A., Sandhu, R., John, H., Shand, L., Lane, S., Foster, H., Griffiths, B., Griffiths, I., Kay, L., Ng, W.-F., Platt, P. N., Walker, D. J., Peterson, P., Lorenzi, A., Friswell, M., Thompson, B., Lee, M., Pratt, A., Hopkinson, N. D., Dunne, C. A., Quilty, B., Marks, J., Mukherjee, S., Mulherin, D., Chalam, S. V., Price, T., Sheeran, T., Venkatachalam, S., Baskar, S., Al-Allaf, W., McKenna, F., Shah, P., Filer, A., Bowman, S. J., Jobanputra, P., Rankin, E. C., Allen, M., Chaudhuri, K., Dubey, S., Price-Forbes, A., Ravindran, J., Samanta, A., Sheldon, P., Hassan, W., Francis, J., Kinder, A., Neame, R., Moorthy, A., Bukhari, M., Ottewell, L., Palkonyai, E., Hider, S., Hassell, A., Menon, A., Dowson, C., Kamath, S., Packham, J., Dutta, S., Price, S., Roddy, E., Paskins, Z., OʼReilly, D. T., Rajagopal, V., Bhagat, S., Chattopadhyay, C. B., Quinn, D., Isdale, A., Brown, A., Saleem, B., Foo, B., Al Saffar, Z., and Koduri, G.

Published
2015
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18. Testing the role of vitamin D in response to antitumour necrosis factor α therapy in a UK cohort: a Mendelian randomisation approach

Author

Yarwood, Annie, Viatte, Sebastien, Plant, Darren, Morgan, Ann W, Isaacs, John, Wilson, Anthony G, Hyrich, Kimme, Eyre, Steve, Barton, Anne, Gaston, H, Mulherin, D, Price, T, Sheeran, T, Chalam, V, Baskar, S, Emery, P, Morgan, A W, Buch, M, Bingham, S, O’Reilly, S, Badcock, L, Regan, M, Ding, T, Deighton, C, Summers, G, Raj, N, Stevens, R, Williams, N, Isaacs, J, Platt, P, Walker, D, Kay, L, Griffiths, B, Ng, W-F, Peterson, P, Lorenzi, A, Foster, H, Friswell, M, Thompson, B, Lee, M, Griffiths, I, Hassell, A, Dawes, P, Dowson, C, Kamath, S, Packham, J, Shadforth, M, Williams, R, Mukhtyar, C, Harrison, B, Snowden, N, Naz, S, Ledingham, J, Hull, R, McCrae, F, Thomas, A, Young Min, S, Shaban, R, Wong, E, Kelly, C, Heycock, C, Hamilton, J, Saravanan, V, Wilson, G, Bax, D, Dunkley, L, Akil, M, Tattersall, R, Kilding, R, Till, S, Boulton, J, Tait, T, Bukhari, M, Halsey, J, Ottewell, L, Buckley, C, Situnayake, D, Carruthers, D, Grindulis, K, Khatack, F, Elamanchi, S, Raza, K, Filer, A, Jubb, R, Abernathy, R, Plant, M, Pathare, S, Clarke, F, Tuck, S, Fordham, J, Paul, A, Bridges, M, Hakim, A, O’Reilly, D, Rajagopal, V, Bhagat, S, Edwards, C, Prouse, P, Moitra, R, Shawe, D, Bamji, A, Klimiuk, P, Bowden, A, Mitchell, W, Bruce, I, Barton, A, Gorodkin, R, Ho, P, Hyrich, K, Dixon, W, Rai, A, Kitas, G, Erb, N, Klocke, R, Douglas, K, Pace, A, Sandhu, R, Whallett, A, Birrell, F, Allen, M, Chaudhuri, K, Chattopadhyay, C, McHale, J, Jones, A, Gupta, A, Pande, I, Gaywood, I, Lanyon, P, Courtney, P, Doherty, M, Chinoy, H, O’Neill, T, Herrick, A, Jones, A, Cooper, R, Bucknall, R, Marguerie, C, Rigby, S, Dunn, N, Green, S, Al-Ansari, A, Webber, S, Hopkinson, N, Dunne, C, Quilty, B, Szebenyi, B, Green, M, Quinn, M, Isdale, A, Brown, A, Saleem, B, Samanta, A, Sheldon, P, Hassan, W, Francis, J, Kinder, A, Neame, R, Moorthy, A, Al-Allaf, W, Taggart, A, Fairburn, K, McKenna, F, Green, M, Gough, A, Lawson, C, Piper, M, Korendowych, E, Jenkinson, T, Sengupta, R, Bhalla, A, McHugh, N, Bond, Debbie, Luqmani, R, Bowness, B, Wordsworth, P, David, J, Smith, W, Mewar, D, Tunn, E, Nelson, K, Kennedy, T, Nixon, J, Woolf, A, Davis, M, Hutchinson, D, Endean, A, Coady, D, Wright, D, Morley, C, Raftery, G, Bracewell, C, Kidd, L, Abbas, I, Filer, C, and Kallarackal, G

Published
2014
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19. Definitions and reliability assessment of elementary ultrasound lesions in giant cell arteritis: A study from the OMERACT large vessel vasculitis ultrasound working group

Author

Chrysidis, S, Duftner, C, Dejaco, C, Schafer, V, Ramiro, S, Carrara, G, Scire, C, Hocevar, A, Diamantopoulos, A, Iagnocco, A, Mukhtyar, C, Ponte, C, Naredo, E, De Miguel, E, Bruyn, G, Warrington, K, Terslev, L, Milchert, M, D'Agostino, M, Koster, M, Rastalsky, N, Hanova, P, Macchioni, P, Kermani, T, Lorenzen, T, Dohn, U, Fredberg, U, Hartung, W, Dasgupta, B, Schmidt, W, Chrysidis S., Duftner C., Dejaco C., Schafer V. S., Ramiro S., Carrara G., Scire C. A., Hocevar A., Diamantopoulos A. P., Iagnocco A., Mukhtyar C., Ponte C., Naredo E., De Miguel E., Bruyn G. A., Warrington K. J., Terslev L., Milchert M., D'Agostino M. A., Koster M. J., Rastalsky N., Hanova P., Macchioni P., Kermani T. A., Lorenzen T., Dohn U. Mo., Fredberg U., Hartung W., Dasgupta B., Schmidt W. A., Chrysidis, S, Duftner, C, Dejaco, C, Schafer, V, Ramiro, S, Carrara, G, Scire, C, Hocevar, A, Diamantopoulos, A, Iagnocco, A, Mukhtyar, C, Ponte, C, Naredo, E, De Miguel, E, Bruyn, G, Warrington, K, Terslev, L, Milchert, M, D'Agostino, M, Koster, M, Rastalsky, N, Hanova, P, Macchioni, P, Kermani, T, Lorenzen, T, Dohn, U, Fredberg, U, Hartung, W, Dasgupta, B, Schmidt, W, Chrysidis S., Duftner C., Dejaco C., Schafer V. S., Ramiro S., Carrara G., Scire C. A., Hocevar A., Diamantopoulos A. P., Iagnocco A., Mukhtyar C., Ponte C., Naredo E., De Miguel E., Bruyn G. A., Warrington K. J., Terslev L., Milchert M., D'Agostino M. A., Koster M. J., Rastalsky N., Hanova P., Macchioni P., Kermani T. A., Lorenzen T., Dohn U. Mo., Fredberg U., Hartung W., Dasgupta B., and Schmidt W. A.

Abstract

Objectives To define the elementary ultrasound (US) lesions in giant cell arteritis (GCA) and to evaluate the reliability of the assessment of US lesions according to these definitions in a web-based reliability exercise. Methods Potential definitions of normal and abnormal US findings of temporal and extracranial large arteries were retrieved by a systematic literature review. As a subsequent step, a structured Delphi exercise was conducted involving an expert panel of the Outcome Measures in Rheumatology (OMERACT) US Large Vessel Vasculitis Group to agree definitions of normal US appearance and key elementary US lesions of vasculitis of temporal and extracranial large arteries. The reliability of these definitions on normal and abnormal blood vessels was tested on 150 still images and videos in a web-based reliability exercise. Results Twenty-four experts participated in both Delphi rounds. From originally 25 statements, nine definitions were obtained for normal appearance, vasculitis and arteriosclerosis of cranial and extracranial vessels. The 'halo' and 'compression' signs were the key US lesions in GCA. The reliability of the definitions for normal temporal and axillary arteries, the 'halo' sign and the 'compression' sign was excellent with inter-rater agreements of 91-99% and mean kappa values of 0.83-0.98 for both inter-rater and intra-rater reliabilities of all 25 experts. Conclusions The 'halo' and the 'compression' signs are regarded as the most important US abnormalities for GCA. The inter-rater and intra-rater agreement of the new OMERACT definitions for US lesions in GCA was excellent.

Published
2018

20. Assessing vasculitis in giant cell arteritis by ultrasound: Results of OMERACT patient-based reliability exercises

Author

Schafer, V, Chrysidis, S, Dejaco, C, Duftner, C, Iagnocco, A, Bruyn, G, Carrara, G, D'Agostino, M, De Miguel, E, Diamantopoulos, A, Fredberg, U, Hartung, W, Hocevar, A, Juche, A, Kermani, T, Koster, M, Lorenzen, T, Macchioni, P, Milchert, M, Dohn, U, Mukhtyar, C, Ponte, C, Ramiro, S, Scire, C, Terslev, L, Warrington, K, Dasgupta, B, Schmidt, W, Schafer V. S., Chrysidis S., Dejaco C., Duftner C., Iagnocco A., Bruyn G. A., Carrara G., D'Agostino M. A., De Miguel E., Diamantopoulos A. P., Fredberg U., Hartung W., Hocevar A., Juche A., Kermani T. A., Koster M. J., Lorenzen T., Macchioni P., Milchert M., Dohn U. M., Mukhtyar C., Ponte C., Ramiro S., Scire C. A., Terslev L., Warrington K. J., Dasgupta B., Schmidt W. A., Schafer, V, Chrysidis, S, Dejaco, C, Duftner, C, Iagnocco, A, Bruyn, G, Carrara, G, D'Agostino, M, De Miguel, E, Diamantopoulos, A, Fredberg, U, Hartung, W, Hocevar, A, Juche, A, Kermani, T, Koster, M, Lorenzen, T, Macchioni, P, Milchert, M, Dohn, U, Mukhtyar, C, Ponte, C, Ramiro, S, Scire, C, Terslev, L, Warrington, K, Dasgupta, B, Schmidt, W, Schafer V. S., Chrysidis S., Dejaco C., Duftner C., Iagnocco A., Bruyn G. A., Carrara G., D'Agostino M. A., De Miguel E., Diamantopoulos A. P., Fredberg U., Hartung W., Hocevar A., Juche A., Kermani T. A., Koster M. J., Lorenzen T., Macchioni P., Milchert M., Dohn U. M., Mukhtyar C., Ponte C., Ramiro S., Scire C. A., Terslev L., Warrington K. J., Dasgupta B., and Schmidt W. A.

Abstract

Objective: To test the reliability of Outcome Measures in Rheumatology Clinical Trials (OMERACT) consensus-based ultrasound definitions for normal and vasculitic temporal and axillary arteries in patients with giant cell arteritis (GCA) and in controls. Methods: A preliminary 1-day meeting and a full 3-day meeting fulfilling OMERACT Ultrasound Group guidelines were held. Temporal and axillary arteries were examined at 2 timepoints by 12 sonographers on 4 patients with GCA and 2 controls. The aim was to test inter- and intrareader reliability for normal findings, halo sign, and compression sign. In both meetings, patients had established GCA. Pathology was more recent in the full meeting, which was preceded by 6 h of training. Scanning time was 15-20 min instead of 10-13 min. Results: In the preliminary exercise, interreader reliabilities were fair to moderate for the overall diagnosis of GCA (Light k 0.29-0.51), and poor to fair for identifying vasculitis in the respective anatomical segments (Light k 0.02-0.46). Intrareader reliabilities were moderate (Cohen k 0.32-0.64). In the main exercise, interreader reliability was good to excellent (Light k 0.76-0.86) for the overall diagnosis of GCA, and moderate to good (Light k 0.46-0.71) for identifying vasculitis in the respective anatomical segments. Intrareader reliability was excellent for diagnosis of GCA (Cohen k 0.91) and good (Cohen k 0.71-0.80) for the anatomical segments. Conclusion: OMERACT-derived definitions of halo and compression signs of temporal and axillary arteries are reliable in recent-onset GCA if experienced sonographers (> 300 examinations) have 15-20 min for a standardized examination with prior training and apply > 15 MHz probes.

Published
2018

21. Feasibility and Face Validity of Outcome Measures for Use in Future Studies of Polymyalgia Rheumatica: An OMERACT Study

Author

Yates, M, Owen, CE, Muller, SN, Graham, K, Neill, L, Twohig, H, Boers, M, Pujades-Rodriguez, M, Goodman, S, Cheah, J, Dejaco, C, Mukhtyar, C, Nielsen, BD, Robson, J, Simon, LS, Shea, B, Mackie, SL, Hill, CL, OMERACT PMR Working Group, PMR, Epidemiology and Data Science, and APH - Methodology

Subjects
medicine.medical_specialty, Visual analogue scale, Immunology, Context (language use), Blood Sedimentation, POLYMYALGIA RHEUMATICA, Q1, Polymyalgia rheumatica, Rheumatology, GIANT-CELL ARTERITIS, Outcome Assessment, Health Care, medicine, Immunology and Allergy, Humans, Face validity, medicine.diagnostic_test, business.industry, Reproducibility of Results, OUTCOME MEASURES, medicine.disease, R1, PREVALENCE, Clinical trial, DESCRIPTIVE QUALITATIVE STUDY, TRIALS, FILTER, Health assessment, Polymyalgia Rheumatica, Erythrocyte sedimentation rate, Physical therapy, H1, Feasibility Studies, Thematic analysis, business
Abstract

Objective.To survey participants with polymyalgia rheumatica (PMR) to evaluate the face validity, acceptability, and domain match of proposed candidate outcome measures.Methods.A structured, online, anonymous survey was disseminated by patient support groups through their networks and online forums. The candidate outcome measures comprised (1) visual analog scale (VAS) and numerical rating score (NRS) to assess pain; (2) VAS, NRS, and duration to assess stiffness; (3) the modified Health Assessment Questionnaire and Health Assessment Questionnaire Disability Index to assess physical function; and (4) C-reactive protein and erythrocyte sedimentation rate to assess inflammation. Free-text answers were analyzed using descriptive thematic analysis to determine respondents’ views of the candidate instruments.Results.Seventy-eight people with PMR from 6 countries (UK, France, USA, Canada, Australia, and New Zealand) participated in the survey. Most respondents agreed candidate instruments were acceptable or “good to go.” Free-text analysis identified 5 themes that participants considered inadequately covered by the proposed instruments. These related to (1) the variability, context, and location of pain; (2) the variability of stiffness; (3) fatigue; (4) disability; and (5) the correlation of inflammatory marker levels and severity of symptoms, sometimes reflecting disease activity and other times not.Conclusion.Participants reported additional aspects of their experience that are not covered by the proposed instruments, particularly for the experience of stiffness and effect of fatigue. New patient-reported outcome measures are required to increase the relevance of results from clinical trials to patients with PMR.

Published
2020

22. BSR guideline on diagnosis and treatment of giant cell arteritis

Author

Mackie, SL, Dejaco, C, Appenzeller, S, Camellino, D, Duftner, C, Gonzalez-Chiappe, S, Mahr, A, Mukhtyar, C, Reynolds, G, Wagner S. de Souza, A, Brouwer, E, Bukhari, M, Buttgereit, F, Byrne, D, Cid, MC, Cimmino, M, Direskeneli, H, Gilbert, K, Kermani, TA, Khan, A, Lanyon, P, Luqmani, R, Mallen, C, Mason, J, Matteson, EL, Merkel, PA, Mollan, S, Neill, L, O‘Sullivan, E, Sandovici, M, Schmidt, WA, Watts, R, Whitlock, M, Yacyshyn, E, Ytterberg, S, Dasgupta, B, and Imperial College Healthcare NHS Trust- BRC Funding

Subjects
1107 Immunology, 1103 Clinical Sciences, 1117 Public Health and Health Services, Arthritis & Rheumatology
Published
2019

30. Genome-wide association study of eosinophilic granulomatosis with polyangiitis reveals genomic loci stratified by ANCA status

Author

Lyons, P.A., Peters, J.E., Alberici, F., Liley, J., Coulson, R.M.R., Astle, W., Baldini, C., Bonatti, F., Cid, M.C., Elding, H., Emmi, G., Epplen, J., Guillevin, L., Jayne, D.R.W., Jiang, T., Gunnarsson, I., Lamprecht, P., Leslie, S., Little, M.A., Martorana, D., Moosig, F., Neumann, T., Ohlsson, S., Quickert, S., Ramirez, G.A., Rewerska, B., Schett, G., Sinico, R.A., Szczeklik, W., Tesar, V., Vukcevic, D., Akil, M., Barratt, J., Basu, N., Butterworth, A.S., Bruce, I., Clarkson, M., Conlon, N., DasGupta, B., Doulton, T.W.R., Espígol-Frigolé, Georgina, Flossmann, O., Gabrielli, A., Gasior, J., Gregorini, G., Guida, G., Hernández Rodríguez, José, Hruskova, Z., Hudson, A., Knight, A., Lanyon, P., Luqmani, R., Magliano, M., Manfredi, A.A., Marguerie, C., Maritati, F., Marvisi, C., McHugh, N.J., Molloy, E., Motyer, A., Mukhtyar, C., Padyukov, L., Pesci, A., Prieto-Gonzalez, S., Ramentol-Sintas, Marc, Reis, P., Roccatello, D., Rovere-Querini, P., Salvarani, C., Santarsia, F., Solans-Laque, R., Soranzo, N., Taylor, J., Wessels, J., Zwerina, J., Terrier, B., Watts, R.A., Vaglio, A., Holle, J.U., Wallace, C., Smith, K.G.C., and Universitat Autònoma de Barcelona

Subjects
Eosinophils, Genetic Loci, Granulomatosis with Polyangiitis, Humans, Genetic Predisposition to Disease, Mendelian Randomization Analysis, Genetic Association Studies, Antibodies, Antineutrophil Cytoplasmic, Genome-Wide Association Study
Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare inflammatory disease of unknown cause. 30% of patients have anti-neutrophil cytoplasmic antibodies (ANCA) specific for myeloperoxidase (MPO). Here, we describe a genome-wide association study in 676 EGPA cases and 6809 controls, that identifies 4 EGPA-associated loci through conventional case-control analysis, and 4 additional associations through a conditional false discovery rate approach. Many variants are also associated with asthma and six are associated with eosinophil count in the general population. Through Mendelian randomisation, we show that a primary tendency to eosinophilia contributes to EGPA susceptibility. Stratification by ANCA reveals that EGPA comprises two genetically and clinically distinct syndromes. MPO+ ANCA EGPA is an eosinophilic autoimmune disease sharing certain clinical features and an HLA-DQ association with MPO+ ANCA-associated vasculitis, while ANCA-negative EGPA may instead have a mucosal/barrier dysfunction origin. Four candidate genes are targets of therapies in development, supporting their exploration in EGPA.

Published
2019

33. Genome-wide association study of eosinophilic granulomatosis with polyangiitis reveals genomic loci stratified by ANCA status

Author

Lyons, P, Peters, J, Alberici, F, Liley, J, Coulson, R, Astle, W, Baldini, C, Bonatti, F, Cid, M, Elding, H, Emmi, G, Epplen, J, Guillevin, L, Jayne, D, Jiang, T, Gunnarsson, I, Lamprecht, P, Leslie, S, Little, M, Martorana, D, Moosig, F, Neumann, T, Ohlsson, S, Quickert, S, Ramierez, G, Rewerska, B, Schett, G, Sinico, R, Szczeklik, W, Tesar, V, Vukcevic, D, Akil, M, Barratt, J, Basu, N, Butterworth, A, Bruce, I, Clarkson, M, Conlon, N, Dasgupta, B, Doulton, T, Espigol-Frigole, G, Flossmann, O, Gabrielli, A, Gasior, J, Gregorini, G, Guida, G, Hernandez-Rodriguez, J, Hruskova, Z, Hudson, A, Knight, A, Lanyon, P, Luqmani, R, Magliano, M, Manfredi, A, Marguerie, C, Maritati, F, Marvisi, C, Mchugh, N, Molloy, E, Motyer, A, Mukhtyar, C, Padyukov, L, Pesci, A, Prieto-Gonzalez, S, Ramentol-Sintas, M, Reis, P, Roccatello, D, Rovere-Querini, P, Salvarani, C, Santarsia, F, Solans-Laque, R, Soranzo, N, Taylor, J, Wessels, J, Zwerina, J, Terrier, B, Watts, R, Vaglio, A, Holle, J, Wallace, C, Smith, K, Lyons, PA, Peters, JE, Cid, MC, Little, MA, Ramierez, GA, Butterworth, AS, DasGupta, B, Doulton, TWR, Manfredi, AA, McHugh, NJ, Watts, RA, Holle, JU, Smith, KGC, Lyons, P, Peters, J, Alberici, F, Liley, J, Coulson, R, Astle, W, Baldini, C, Bonatti, F, Cid, M, Elding, H, Emmi, G, Epplen, J, Guillevin, L, Jayne, D, Jiang, T, Gunnarsson, I, Lamprecht, P, Leslie, S, Little, M, Martorana, D, Moosig, F, Neumann, T, Ohlsson, S, Quickert, S, Ramierez, G, Rewerska, B, Schett, G, Sinico, R, Szczeklik, W, Tesar, V, Vukcevic, D, Akil, M, Barratt, J, Basu, N, Butterworth, A, Bruce, I, Clarkson, M, Conlon, N, Dasgupta, B, Doulton, T, Espigol-Frigole, G, Flossmann, O, Gabrielli, A, Gasior, J, Gregorini, G, Guida, G, Hernandez-Rodriguez, J, Hruskova, Z, Hudson, A, Knight, A, Lanyon, P, Luqmani, R, Magliano, M, Manfredi, A, Marguerie, C, Maritati, F, Marvisi, C, Mchugh, N, Molloy, E, Motyer, A, Mukhtyar, C, Padyukov, L, Pesci, A, Prieto-Gonzalez, S, Ramentol-Sintas, M, Reis, P, Roccatello, D, Rovere-Querini, P, Salvarani, C, Santarsia, F, Solans-Laque, R, Soranzo, N, Taylor, J, Wessels, J, Zwerina, J, Terrier, B, Watts, R, Vaglio, A, Holle, J, Wallace, C, Smith, K, Lyons, PA, Peters, JE, Cid, MC, Little, MA, Ramierez, GA, Butterworth, AS, DasGupta, B, Doulton, TWR, Manfredi, AA, McHugh, NJ, Watts, RA, Holle, JU, and Smith, KGC

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare inflammatory disease of unknown cause. 30% of patients have anti-neutrophil cytoplasmic antibodies (ANCA) specific for myeloperoxidase (MPO). Here, we describe a genome-wide association study in 676 EGPA cases and 6809 controls, that identifies 4 EGPA-associated loci through conventional case-control analysis, and 4 additional associations through a conditional false discovery rate approach. Many variants are also associated with asthma and six are associated with eosinophil count in the general population. Through Mendelian randomisation, we show that a primary tendency to eosinophilia contributes to EGPA susceptibility. Stratification by ANCA reveals that EGPA comprises two genetically and clinically distinct syndromes. MPO+ ANCA EGPA is an eosinophilic autoimmune disease sharing certain clinical features and an HLA-DQ association with MPO+ ANCA-associated vasculitis, while ANCA-negative EGPA may instead have a mucosal/barrier dysfunction origin. Four candidate genes are targets of therapies in development, supporting their exploration in EGPA.

Published
2019

34. Genome-wide association study of eosinophilic granulomatosis with polyangiitis reveals genomic loci stratified by ANCA status

Author

Lyons, PA, Peters, JE, Alberici, F, Liley, J, Coulson, RMR, Astle, W, Baldini, C, Bonatti, F, Cid, MC, Elding, H, Emmi, G, Epplen, J, Guillevin, L, Jayne, DRW, Jiang, T, Gunnarsson, I, Lamprecht, P, Leslie, S, Little, MA, Martorana, D, Moosig, F, Neumann, T, Ohlsson, S, Quickert, S, Ramirez, GA, Rewerska, B, Schett, G, Sinico, RA, Szczeklik, W, Tesar, V, Vukcevic, D, Akil, M, Barratt, J, Basu, N, Butterworth, AS, Bruce, I, Clarkson, M, Conlon, N, DasGupta, B, Doulton, TWR, Espigol-Frigole, G, Flossmann, O, Gabrielli, A, Gasior, J, Gregorini, G, Guida, G, Hernandez-Rodriguez, J, Hruskova, Z, Hudson, A, Knight, A, Lanyon, P, Luqmani, R, Magliano, M, Manfredi, AA, Marguerie, C, Maritati, F, Marvisi, C, McHugh, NJ, Molloy, E, Motyer, A, Mukhtyar, C, Padyukov, L, Pesci, A, Prieto-Gonzalez, S, Ramentol-Sintas, M, Reis, P, Roccatello, D, Rovere-Querini, P, Salvarani, C, Santarsia, F, Solans-Laque, R, Soranzo, N, Taylor, J, Wessels, J, Zwerina, J, Terrier, B, Watts, RA, Vaglio, A, Holle, JU, Wallace, C, Smith, KGC, Lyons, PA, Peters, JE, Alberici, F, Liley, J, Coulson, RMR, Astle, W, Baldini, C, Bonatti, F, Cid, MC, Elding, H, Emmi, G, Epplen, J, Guillevin, L, Jayne, DRW, Jiang, T, Gunnarsson, I, Lamprecht, P, Leslie, S, Little, MA, Martorana, D, Moosig, F, Neumann, T, Ohlsson, S, Quickert, S, Ramirez, GA, Rewerska, B, Schett, G, Sinico, RA, Szczeklik, W, Tesar, V, Vukcevic, D, Akil, M, Barratt, J, Basu, N, Butterworth, AS, Bruce, I, Clarkson, M, Conlon, N, DasGupta, B, Doulton, TWR, Espigol-Frigole, G, Flossmann, O, Gabrielli, A, Gasior, J, Gregorini, G, Guida, G, Hernandez-Rodriguez, J, Hruskova, Z, Hudson, A, Knight, A, Lanyon, P, Luqmani, R, Magliano, M, Manfredi, AA, Marguerie, C, Maritati, F, Marvisi, C, McHugh, NJ, Molloy, E, Motyer, A, Mukhtyar, C, Padyukov, L, Pesci, A, Prieto-Gonzalez, S, Ramentol-Sintas, M, Reis, P, Roccatello, D, Rovere-Querini, P, Salvarani, C, Santarsia, F, Solans-Laque, R, Soranzo, N, Taylor, J, Wessels, J, Zwerina, J, Terrier, B, Watts, RA, Vaglio, A, Holle, JU, Wallace, C, and Smith, KGC

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare inflammatory disease of unknown cause. 30% of patients have anti-neutrophil cytoplasmic antibodies (ANCA) specific for myeloperoxidase (MPO). Here, we describe a genome-wide association study in 676 EGPA cases and 6809 controls, that identifies 4 EGPA-associated loci through conventional case-control analysis, and 4 additional associations through a conditional false discovery rate approach. Many variants are also associated with asthma and six are associated with eosinophil count in the general population. Through Mendelian randomisation, we show that a primary tendency to eosinophilia contributes to EGPA susceptibility. Stratification by ANCA reveals that EGPA comprises two genetically and clinically distinct syndromes. MPO+ ANCA EGPA is an eosinophilic autoimmune disease sharing certain clinical features and an HLA-DQ association with MPO+ ANCA-associated vasculitis, while ANCA-negative EGPA may instead have a mucosal/barrier dysfunction origin. Four candidate genes are targets of therapies in development, supporting their exploration in EGPA.

Published
2019

36. Corrigendum: Giant cell arteritis: new concepts, treatments and the unmet need that remains

Author

Coath, F, Gillbert, K, Griffiths, B, Hall, F, Kay, L, Lanyon, P, Luqmani, R, Mackie, SL, Mason, JC, Mills, J, Mollan, S, Morgan, AW, Mukhtyar, C, Quick, V, Watts, R, Dasgupta, B, and Imperial College Healthcare NHS Trust- BRC Funding

Subjects
1107 Immunology, 1103 Clinical Sciences, 1117 Public Health and Health Services, Arthritis & Rheumatology
Abstract

This is a correction to: Rheumatology, Volume 58, Issue 7, July 2019, Pages 1123–1125, https://doi.org/10.1093/rheumatology/key326

Published
2018

38. Definitions and reliability assessment of elementary ultrasound lesions in giant cell arteritis: A study from the OMERACT large vessel vasculitis ultrasound working group

Author

Chrysidis, S., Duftner, C., Dejaco, C., Schafer, V. S., Ramiro, S., Carrara, G., Scire, C. A., Hocevar, A., Diamantopoulos, A. P., Iagnocco, A., Mukhtyar, C., Ponte, C., Naredo, E., De Miguel, E., Bruyn, G. A., Warrington, K. J., Terslev, L., Milchert, M., D'Agostino, Maria Antonietta, Koster, M. J., Rastalsky, N., Hanova, P., Macchioni, P., Kermani, T. A., Lorenzen, T., Dohn, U. Mo., Fredberg, U., Hartung, W., Dasgupta, B., Schmidt, W. A., D'Agostino M. A. (ORCID:0000-0002-5347-0060), Chrysidis, S., Duftner, C., Dejaco, C., Schafer, V. S., Ramiro, S., Carrara, G., Scire, C. A., Hocevar, A., Diamantopoulos, A. P., Iagnocco, A., Mukhtyar, C., Ponte, C., Naredo, E., De Miguel, E., Bruyn, G. A., Warrington, K. J., Terslev, L., Milchert, M., D'Agostino, Maria Antonietta, Koster, M. J., Rastalsky, N., Hanova, P., Macchioni, P., Kermani, T. A., Lorenzen, T., Dohn, U. Mo., Fredberg, U., Hartung, W., Dasgupta, B., Schmidt, W. A., and D'Agostino M. A. (ORCID:0000-0002-5347-0060)

Abstract

Objectives To define the elementary ultrasound (US) lesions in giant cell arteritis (GCA) and to evaluate the reliability of the assessment of US lesions according to these definitions in a web-based reliability exercise. Methods Potential definitions of normal and abnormal US findings of temporal and extracranial large arteries were retrieved by a systematic literature review. As a subsequent step, a structured Delphi exercise was conducted involving an expert panel of the Outcome Measures in Rheumatology (OMERACT) US Large Vessel Vasculitis Group to agree definitions of normal US appearance and key elementary US lesions of vasculitis of temporal and extracranial large arteries. The reliability of these definitions on normal and abnormal blood vessels was tested on 150 still images and videos in a web-based reliability exercise. Results Twenty-four experts participated in both Delphi rounds. From originally 25 statements, nine definitions were obtained for normal appearance, vasculitis and arteriosclerosis of cranial and extracranial vessels. The 'halo' and 'compression' signs were the key US lesions in GCA. The reliability of the definitions for normal temporal and axillary arteries, the 'halo' sign and the 'compression' sign was excellent with inter-rater agreements of 91-99% and mean kappa values of 0.83-0.98 for both inter-rater and intra-rater reliabilities of all 25 experts. Conclusions The 'halo' and the 'compression' signs are regarded as the most important US abnormalities for GCA. The inter-rater and intra-rater agreement of the new OMERACT definitions for US lesions in GCA was excellent.

Published
2018

39. Assessing vasculitis in giant cell arteritis by ultrasound: Results of OMERACT patient-based reliability exercises

Author

Schafer, V. S., Chrysidis, S., Dejaco, C., Duftner, C., Iagnocco, A., Bruyn, G. A., Carrara, G., D'Agostino, Maria Antonietta, De Miguel, E., Diamantopoulos, A. P., Fredberg, U., Hartung, W., Hocevar, A., Juche, A., Kermani, T. A., Koster, M. J., Lorenzen, T., Macchioni, P., Milchert, M., Dohn, U. M., Mukhtyar, C., Ponte, C., Ramiro, S., Scire, C. A., Terslev, L., Warrington, K. J., Dasgupta, B., Schmidt, W. A., D'Agostino M. A. (ORCID:0000-0002-5347-0060), Schafer, V. S., Chrysidis, S., Dejaco, C., Duftner, C., Iagnocco, A., Bruyn, G. A., Carrara, G., D'Agostino, Maria Antonietta, De Miguel, E., Diamantopoulos, A. P., Fredberg, U., Hartung, W., Hocevar, A., Juche, A., Kermani, T. A., Koster, M. J., Lorenzen, T., Macchioni, P., Milchert, M., Dohn, U. M., Mukhtyar, C., Ponte, C., Ramiro, S., Scire, C. A., Terslev, L., Warrington, K. J., Dasgupta, B., Schmidt, W. A., and D'Agostino M. A. (ORCID:0000-0002-5347-0060)

Abstract

Objective: To test the reliability of Outcome Measures in Rheumatology Clinical Trials (OMERACT) consensus-based ultrasound definitions for normal and vasculitic temporal and axillary arteries in patients with giant cell arteritis (GCA) and in controls. Methods: A preliminary 1-day meeting and a full 3-day meeting fulfilling OMERACT Ultrasound Group guidelines were held. Temporal and axillary arteries were examined at 2 timepoints by 12 sonographers on 4 patients with GCA and 2 controls. The aim was to test inter- and intrareader reliability for normal findings, halo sign, and compression sign. In both meetings, patients had established GCA. Pathology was more recent in the full meeting, which was preceded by 6 h of training. Scanning time was 15-20 min instead of 10-13 min. Results: In the preliminary exercise, interreader reliabilities were fair to moderate for the overall diagnosis of GCA (Light k 0.29-0.51), and poor to fair for identifying vasculitis in the respective anatomical segments (Light k 0.02-0.46). Intrareader reliabilities were moderate (Cohen k 0.32-0.64). In the main exercise, interreader reliability was good to excellent (Light k 0.76-0.86) for the overall diagnosis of GCA, and moderate to good (Light k 0.46-0.71) for identifying vasculitis in the respective anatomical segments. Intrareader reliability was excellent for diagnosis of GCA (Cohen k 0.91) and good (Cohen k 0.71-0.80) for the anatomical segments. Conclusion: OMERACT-derived definitions of halo and compression signs of temporal and axillary arteries are reliable in recent-onset GCA if experienced sonographers (> 300 examinations) have 15-20 min for a standardized examination with prior training and apply > 15 MHz probes.

Published
2018

40. The OMERACT core domain set for outcome measures for clinical trials in polymyalgia rheumatica

Author

Mackie, SL, Twohig, H, Neill, LM, Harrison, E, Shea, B, Black, RJ, Kermani, TA, Merkel, PA, Mallen, CD, Buttgereit, F, Mukhtyar, C, Simon, LS, Hill, CL, and OMERACT PMR Working Group, .

Subjects
musculoskeletal diseases, medicine.medical_specialty, Delphi Technique, Immunology, Delphi method, Outcome (game theory), Article, Domain (software engineering), Polymyalgia rheumatica, 03 medical and health sciences, 0302 clinical medicine, Quality of life (healthcare), Rheumatology, RC925, Outcome Assessment, Health Care, medicine, Humans, Immunology and Allergy, 030212 general & internal medicine, Set (psychology), 030203 arthritis & rheumatology, Clinical Trials as Topic, business.industry, medicine.disease, Clinical trial, Polymyalgia Rheumatica, Research Design, Antirheumatic Agents, Quality of Life, Physical therapy, business, RA, Qualitative research
Abstract

Objective.To inform development of a core domain set for outcome measures for clinical trials in polymyalgia rheumatica (PMR), we conducted patient consultations, a systematic review, a Delphi study, and 2 qualitative studies.Methods.Domains identified by 70% or more of physicians and/or patients in the Delphi study were selected. The conceptual framework derived from the 2 qualitative research studies helped inform the meaning of each domain and its relationship to the others. The draft core domain set was refined by further discussion with patients and physicians who had participated in the Delphi study. At the Outcome Measures in Rheumatology (OMERACT) 2016, the domains were discussed and prioritized by 8 breakout groups. Formal voting took place at the end of the workshop and in the final plenary.Results.Ninety-three percent of voters in the final plenary agreed that the inner core of domains considered mandatory for clinical trials of PMR should consist the following: laboratory markers of systemic inflammation, pain, stiffness, and physical function. Patient’s global and fatigue were considered important but not mandatory (outer core). The research agenda included psychological impact, weakness, physical activity, participation, sleep, imaging, and health-related quality of life.Conclusion.This core domain set was considered sufficiently well-defined that the next step will be to apply the OMERACT Filter 2.0 Instrument Selection Algorithm to select candidate instruments for a subsequent “deeper dive” into the data. This will allow instruments to be mapped onto each of our core domains to derive a core outcome set for PMR.

Published
2017

41. Response to: 'Renal biopsies should be performed whenever treatment strategies depend on renal involvement'

Author

Yates, M, Jayne, DR, and Mukhtyar, C

Abstract

We thank Chemouny et al for their letter and concur with their conclusions. As we state (1): “A positive biopsy for AAV is helpful when considering an initial diagnosis or recurrent disease.” In our view, renal biopsy is important to establish diagnosis and may also provide an indication of prognostic trajectory and although existing classification systems need further validation, changes like glomerular sclerosis have obvious adverse prognostic value for patients with AAV (2-4). The Delphi process, for the scope of the current recommendations, identified the role of biopsy at both diagnosis and follow-up as an important item for update. Histopathological evidence of vasculitis, such as pauci-immune glomerulonephritis or necrotising vasculitis in any organ, remains the gold standard for diagnostic purposes. The likely diagnostic yield varies and is dependent on the organ targeted and in patients with GPA with renal involvement can be as high as 91.5% from renal biopsy (5). As Chemouny and colleagues have demonstrated, a renal biopsy was definitive in determining their management decisions. However during follow-up when relapses occur, it may be prudent to consider judicious use of further kidney biopsy during suspected renal relapse since the cause for acute kidney injury may be due to another cause other than AAV (6). Kind regards, M Yates, C Mukhtyar and DR Jayne on behalf of co-authors.

Published
2017

43. Validation of the Birmingham Vasculitis Activity Score (Version 3)

Author

Mukhtyar, C, Mukhtyar, Chetan, Luqmani, R, and Sabokbar, A

Subjects
Rheumatology, Medical sciences
Abstract

Background: The Birmingham Vasculitis Activity Score is a clinical tool to quantify disease activity in systemic vasculitis. Following its extensive use, a previous version of BVAS has been revised to improve the face validity and the feasibility. Changes were made and approved by an expert committee. Objective: To validate the third version of the Birmingham Vasculitis Activity Score (BVAS version 3) Methods: In a series of prospective, multi-centre studies, different aspects of the BVAS (version 3) were validated in patients with systemic vasculitis. The data collection was done using standardized data entry forms. The studies were approved by local ethics committees. The recruitment of controls was approved by the local clinical audit and effectiveness department. Results: The convergent validity was established by correlating the BVAS (version 3) to the physician’s treatment decision (Spearman's ρ 0.66, 95% CI 0.59-0.72), BVAS1 of version 2 (ρ 0.94, 95% CI 0.92-0.96), BVAS2 of version 2 in patients with persistent disease (ρ 0.60, 95% CI 0.21-0.83), C-reactive protein levels (�� 0.43, 95% CI 0.31-0.54), physician's global assessment (ρ 0.91, 95% CI 0.89-0.93), and vasculitis activity index (ρ 0.88, 95%CI 0.86-0.91). The BVAS (version 3) was reproducible and repeatable (intra-class correlation coefficients were 0.96 (95%CI 0.95-0.97) and 0.96 (95%CI 0.92-0.97) respectively). The BVAS (version 3) was sensitive to changing disease status with a fall of 16.9 (95% CI 14.8-18.9) units (P

Published
2016

48. Long-term patient survival in ANCA-associated vasculitis

Author

Flossmann, O., Berden, A., Groot, K. de, Hagen, C., Harper, L., Heijl, C., Hoglund, P., Jayne, D., Luqmani, R., Mahr, A., Mukhtyar, C., Pusey, C., Rasmussen, N., Stegeman, C., Walsh, M., Westman, K., European Vasculitis Study Grp, Faculteit Medische Wetenschappen/UMCG, Groningen Kidney Center (GKC), and Translational Immunology Groningen (TRIGR)

Subjects
Male, MICROSCOPIC POLYANGIITIS, PLASMA-EXCHANGE, Birmingham Vasculitis Activity Score, urologic and male genital diseases, immune system diseases, Immunology and Allergy, Medicine, skin and connective tissue diseases, education.field_of_study, INDUCTION, Remission Induction, Middle Aged, Prognosis, Treatment Outcome, Female, Vasculitis, Microscopic polyangiitis, ANTIBODY-ASSOCIATED VASCULITIS, Immunosuppressive Agents, Systemic vasculitis, Glomerular Filtration Rate, Adult, medicine.medical_specialty, Immunology, Population, Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis, SYSTEMIC VASCULITIS, General Biochemistry, Genetics and Molecular Biology, Age Distribution, WEGENERS-GRANULOMATOSIS, RENAL INVOLVEMENT, GLOMERULONEPHRITIS, Rheumatology, Internal medicine, Humans, cardiovascular diseases, education, Anti-neutrophil cytoplasmic antibody, Aged, business.industry, medicine.disease, RANDOMIZED-TRIAL, respiratory tract diseases, Surgery, Standardized mortality ratio, business, Epidemiologic Methods, FOLLOW-UP
Abstract

BackgroundWegener's granulomatosis and microscopic polyangiitis are antineutrophil cytoplasm antibodies (ANCA)-associated vasculitides with significant morbidity and mortality. The long-term survival of patients with ANCA associated vasculitis treated with current regimens is uncertain.ObjectiveTo describe the long-term patient survival and possible prognostic factors at presentation in an international, multicentre, prospectively recruited representative patient cohort who were treated according to strictly defined protocols at presentation and included the full spectrum of ANCA-associated vasculitis disease.MethodsOutcome data were collected for 535 patients who had been recruited at the time of diagnosis to four randomised controlled trials between 1995 and 2002. Trial eligibility was defined by disease severity and extent, covered the spectrum of severity of ANCA-associated vasculitis and used consistent diagnostic criteria. Demographic, clinical and laboratory parameters at trial entry were tested as potential prognostic factors in multivariable models.ResultsThe median duration of follow-up was 5.2 years and 133 (25%) deaths were recorded. Compared with an age- and sex-matched general population there was a mortality ratio of 2.6 (95% CI 2.2 to 3.1). Main causes of death within the first year were infection (48%) and active vasculitis (19%). After the first year the major causes of death were cardiovascular disease (26%), malignancy (22%) and infection (20%). Multivariable analysis showed an estimated glomerular filtration rate ConclusionPatients with ANCA-associated vasculitis treated with conventional regimens are at increased risk of death compared with an age- and sex-matched population.

Published
2016

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