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1. Defining remission in childhood-onset lupus: PReS-endorsed consensus definitions by an international task force

Author

Goilav, B., Goss, N., Oni, L., Marks, S.D., Smith, E.M.D., Aggarwal, A., Ainsworth, J., Al-Abadi, E., Avcin, T., Bortey, L., Burnham, J., Ciurtin, C., Hedrich, C.M., Kamphuis, S., Lambert, L., Levy, D.M., Lewandowski, L., Maxwell, N., Morand, E., Özen, S., Pain, C.E., Ravelli, A., Saad Magalhaes, C., Pilkington, C., Schonenberg-Meinema, D., Scott, C., Tullus, K., and Beresford, M.W.

Published
2024
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2. PReS-endorsed international childhood lupus T2T task force definition of childhood lupus low disease activity state (cLLDAS)

Author

Goilav, B., Marks, S., Oni, L., Smith, E.M.D., Aggarwal, A., Ainsworth, J., Al-Abadi, E., Avcin, T., Bortey, L., Burnham, J., Ciurtin, C., Hedrich, C.M., Kamphuis, S., Lambert, L., Levy, D.M., Lewandowski, L., Maxwell, N., Morand, E., Ozen, S., Pain, C.E., Ravelli, A., Saad Magalhaes, C., Pilkington, C., Schonenberg-Meinema, D., Scott, C., Tullus, K., and Beresford, M.W.

Published
2023
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3. POS0875 DEFINING THE THRESHOLD OF NORMALITY IN MUSCULOSKELETAL ULTRASOUND; AN OMERACT ULTRASOUND STUDY

Author

Sahbudin, I., primary, Trickey, J., additional, Gouze, H., additional, Stoenoiu, M. S., additional, Filippou, G., additional, Sakellariou, G., additional, Maruseac, M., additional, Wittoek, R., additional, Carron, P., additional, Tinazzi, I., additional, Iagnocco, A., additional, Serban, T., additional, Azzolin, I., additional, Terslev, L., additional, Ammitzbøll-Danielsen, M., additional, Nyhuus Bendix Rasch, M., additional, Hauge, E. M., additional, Hammer, H. B., additional, Milchert, M., additional, Fliciński, J., additional, Mandl, P., additional, Borst, C., additional, Fodor, D., additional, Vreju, F. A., additional, Karalilova, R., additional, Naredo, E., additional, Sifuentes-Cantú, C., additional, La Paglia, G. M. C., additional, Pineda, C., additional, Gutierrez, M., additional, Leon, G., additional, Reátegui-Sokolova, C., additional, Mortada, M. A., additional, Suzuki, T., additional, Ikeda, K., additional, Ciurtin, C., additional, Kortekaas, M., additional, Ohrndorf, S., additional, Keen, H., additional, Bruyn, G., additional, Filer, A., additional, and D’agostino, M. A., additional

Published
2024
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6. Defining remission in childhood-onset lupus: PReS-endorsed consensus definitions by an international task force

Author

Smith, E.M.D., primary, Aggarwal, A., additional, Ainsworth, J., additional, Al-Abadi, E., additional, Avcin, T., additional, Bortey, L., additional, Burnham, J., additional, Ciurtin, C., additional, Hedrich, C.M., additional, Kamphuis, S., additional, Lambert, L., additional, Levy, D.M., additional, Lewandowski, L., additional, Maxwell, N., additional, Morand, E., additional, Özen, S., additional, Pain, C.E., additional, Ravelli, A., additional, Saad Magalhaes, C., additional, Pilkington, C., additional, Schonenberg-Meinema, D., additional, Scott, C., additional, Tullus, K., additional, Beresford, M.W., additional, Goilav, B., additional, Goss, N., additional, Oni, L., additional, and Marks, S.D., additional

Published
2024
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7. Defining remission in childhood-onset lupus:PReS-endorsed consensus definitions by an international task force

Author

Smith, E. M.D., Aggarwal, A., Ainsworth, J., Al-Abadi, E., Avcin, T., Bortey, L., Burnham, J., Ciurtin, C., Hedrich, C. M., Kamphuis, S., Lambert, L., Levy, D. M., Lewandowski, L., Maxwell, N., Morand, E., Özen, S., Pain, C. E., Ravelli, A., Saad Magalhaes, C., Pilkington, C., Schonenberg-Meinema, D., Scott, C., Tullus, K., Beresford, M. W., Goilav, B., Goss, N., Oni, L., Marks, S. D., Smith, E. M.D., Aggarwal, A., Ainsworth, J., Al-Abadi, E., Avcin, T., Bortey, L., Burnham, J., Ciurtin, C., Hedrich, C. M., Kamphuis, S., Lambert, L., Levy, D. M., Lewandowski, L., Maxwell, N., Morand, E., Özen, S., Pain, C. E., Ravelli, A., Saad Magalhaes, C., Pilkington, C., Schonenberg-Meinema, D., Scott, C., Tullus, K., Beresford, M. W., Goilav, B., Goss, N., Oni, L., and Marks, S. D.

Abstract

Objective: To derive childhood-onset SLE (cSLE) specific remission definitions for future treat-to-target (T2T) trials, observational studies, and clinical practice. Methods: The cSLE International T2T Task Force conducted Delphi surveys exploring paediatric perspectives on adult-onset SLE remission targets. A modified nominal group technique was used to discuss, refine, and agree on the cSLE remission target criteria.Results: The Task Force proposed two definitions of remission: ‘cSLE clinical remission on steroids (cCR)’ and ‘cSLE clinical remission off steroids (cCR-0)’. The common criteria are: (1) Clinical-SLEDAI-2 K = 0; (2) PGA score < 0.5 (0–3 scale); (4) stable antimalarials, immunosuppressive, and biologic therapy (changes due to side-effects, adherence, weight, or when building up to target dose allowed). Criterion (3) in cCR is the prednisolone dose ≤0.1 mg/kg/day (maximum 5 mg/day), whereas in cCR-0 it is zero. Conclusions: cSLE definitions of remission have been proposed, maintaining sufficient alignment with the adult-SLE definition to facilitate life-course research.

Published
2024

11. LB0004 Paediatric Systemic Lupus Erythematosus (pSLE) patient stratification and high-performance serum biomarkers identified responders to statin and patients with high cardiovascular risk in the APPLE trial (Atorvastatin for Atherosclerosis Progression in Paediatric Systemic Lupus Erythematosus)

Author

Ciurtin, C., primary, Peng, J., additional, Donnes, P., additional, Lewandowski, L., additional, Ardoin, S., additional, Schanberg, L., additional, Robinson, G., additional, and Jury, E., additional

Published
2023
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12. OP0130 ABATACEPT IN INDIVIDUALS AT RISK OF DEVELOPING RHEUMATOID ARTHRITIS: RESULTS FROM THE ARTHRITIS PREVENTION IN THE PRE-CLINICAL PHASE OF RA WITH ABATACEPT (APIPPRA) TRIAL

Author

Cope, A., primary, Jasenecova, M., additional, Vasconcelos, J., additional, Filer, A., additional, Raza, K., additional, Qureshi, S., additional, D’agostino, M. A., additional, Mcinnes, I., additional, Isaacs, J., additional, Pratt, A., additional, Fisher, B., additional, Buckley, C. D., additional, Emery, P., additional, Ho, P., additional, Buch, M. H., additional, Ciurtin, C., additional, Huizinga, T., additional, Van Schaardenburg, D., additional, Murphy, C., additional, and Prevost, T., additional

Published
2023
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13. PReS-endorsed international childhood lupus T2T task force definition of childhood lupus low disease activity state (cLLDAS)

Author

Smith, E.M.D., primary, Aggarwal, A., additional, Ainsworth, J., additional, Al-Abadi, E., additional, Avcin, T., additional, Bortey, L., additional, Burnham, J., additional, Ciurtin, C., additional, Hedrich, C.M., additional, Kamphuis, S., additional, Lambert, L., additional, Levy, D.M., additional, Lewandowski, L., additional, Maxwell, N., additional, Morand, E., additional, Ozen, S., additional, Pain, C.E., additional, Ravelli, A., additional, Saad Magalhaes, C., additional, Pilkington, C., additional, Schonenberg-Meinema, D., additional, Scott, C., additional, Tullus, K., additional, Beresford, M.W., additional, Goilav, B., additional, Oni, L., additional, and Marks, S., additional

Published
2023
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14. Executive summary: The 2022 British Society for Rheumatology guideline for the treatment of psoriatic arthritis with biologic and targeted synthetic DMARDs

Author

Tucker, L, Allen, A, Chandler, D, Ciurtin, C, Dick, A, Foulkes, A, Gullick, N, Helliwell, P, Jadon, D, Jones, G, Kyle, S, Madhok, V, McHugh, N, Parkinson, A, Raine, T, Siebert, S, Smith, C, Tillett, W, and Coates, LC

Subjects
dactylitis, treatment, enthesitis, psoriasis, targeted synthetic DMARDs, PsA, Rheumatology, recommendations, biologics, Pharmacology (medical), guideline, management
Published
2022
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16. Development and external validation of a model predicting new-onset chronic uveitis at different disease durations in juvenile idiopathic arthritis

Author

van Straalen, Joeri W., Kearsley-Fleet, Lianne, Klotsche, Jens, de Roock, Sytze, Minden, Kirsten, Heiligenhaus, Arnd, Hyrich, Kimme L., de Boer, Joke H., Lamot, Lovro, Olivieri, Alma N., Gallizzi, Romina, Smolewska, Elzbieta, Faugier, Enrique, Pastore, Serena, Hashkes, Philip J., Herrera, Cristina N., Emminger, Wolfgang, Consolini, Rita, Wulffraat, Nico M., Ruperto, Nicolino, Swart, Joost F., Cleary, G., Baildam, E., Wedderburn, L., Davidson, J., Chieng, A., McErlane, F., Foster, H., Ciurtin, C., Ioannou, Y., Thomson, W., Kallinich, Tilmann, Dressler, Frank, Deschner, Jasmin Kümmerle, Weller-Heinemann, Frank, Horneff, Gerd, Hospach, Anton, Mönkemöller, Kirsten, Haas, Johannes Peter, Windschall, Daniel, Foeldvari, Ivan, and Foell, Dirk

Subjects
Rheumatology, Immunology, Medizin, Uveitis, Juvenile Idiopathic Arthritis, JIA, Immunology and Allergy
Abstract

Objective: To develop and externally validate a prediction model for new-onset chronic uveitis in children with juvenile idiopathic arthritis (JIA) for clinical application. Methods: Data from the international Pharmachild registry were used to develop a multivariable Cox proportional hazards model. Predictors were selected by backward selection, and missing values were handled by multiple imputation. The model was subsequently validated and recalibrated in 2 inception cohorts: the UK Childhood Arthritis Prospective Study (CAPS) study and the German Inception Cohort of Newly diagnosed patients with juvenile idiopathic arthritis (ICON) study. Model performance was evaluated by calibration plots and C statistics for the 2-, 4-, and 7-year risk of uveitis. A diagram and digital risk calculator were created for use in clinical practice. Results: A total of 5, 393 patients were included for model development, and predictor variables were age at JIA onset (hazard ratio [HR] 0.83 [95% confidence interval (95% CI) 0.77-0.89]), ANA positivity (HR 1.59 [95% CI 1.06-2.38]), and International League of Associations for Rheumatology category of JIA (HR for oligoarthritis, psoriatic arthritis, and undifferentiated arthritis versus rheumatoid factor-negative polyarthritis 1.40 [95% CI 0.91- 2.16]). Performance of the recalibrated prediction model in the validation cohorts was acceptable ; calibration plots indicated good calibration and C statistics for the 7-year risk of uveitis (0.75 [95% CI 0.72-0.79] for the ICON cohort and 0.70 [95% CI 0.64-0.76] for the CAPS cohort). Conclusion: We present for the first time a validated prognostic tool for easily predicting chronic uveitis risk for individual JIA patients using common clinical parameters. This model could be used by clinicians to inform patients/parents and provide guidance in choice of uveitis screening frequency and arthritis drug therapy.

Published
2022
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18. PO.6.126 Anti-rituximab antibodies demonstrate neutralising capacity, associate with lower circulating drug levels and early relapse in patients undergoing treatment for systemic lupus erythematosus

Author

Wincup, C, primary, Dunn, N, additional, Ruetsch-Chelli, C, additional, Manouchehrinia, A, additional, Kharlamova, N, additional, Naja, M, additional, Seitz-Polski, B, additional, Isenberg, D, additional, Fogdell-Hahn, A, additional, Ciurtin, C, additional, and Jury, E, additional

Published
2022
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19. International Consensus for the Dosing of Corticosteroids in Childhood-Onset Systemic Lupus Erythematosus With Proliferative Lupus Nephritis

Author

Chalhoub, N.E. Wenderfer, S.E. Levy, D.M. Rouster-Stevens, K. Aggarwal, A. Savani, S.I. Ruth, N.M. Arkachaisri, T. Qiu, T. Merritt, A. Onel, K. Goilav, B. Khubchandani, R.P. Deng, J. Fonseca, A.R. Ardoin, S.P. Ciurtin, C. Kasapcopur, O. Jelusic, M. Huber, A.M. Ozen, S. Klein-Gitelman, M.S. Appenzeller, S. Cavalcanti, A. Fotis, L. Lim, S.C. Silva, R.M. Miramontes, J.R. Rosenwasser, N.L. Saad-Magalhaes, C. Schonenberg-Meinema, D. Scott, C. Silva, C.A. Enciso, S. Terreri, M.T. Torres-Jimenez, A.-R. Trachana, M. Al-Mayouf, S.M. Devarajan, P. Huang, B. Brunner, H.I. Abulaban, K. Aguiar, C. Ahn, S.-Y. Akoghlanian, S. Al-Abrawi, S. Aljaberi, N. Alperin, R. Angeles-Han, S. Ardalan, K. Bader-Meunier, B. Balboni, I. Barbar-Smiley, F. Baxter, S. Beary, J. Boneparth, A. Brakeman, P. Bridges, J. Burgos-Vargas, R. Cabral, D.A. Cameto, J. Carter, C. Chang, J. Chédeville, G. Chhakchhuak, C. Chiraseveenuprapund, P. Cifuentes Alvarado, M. Concannon, A. Cooper, J. Cron, R. De Carvalho, L.M. De Quattro, K. De Ranieri, D. Dizon, B. Donnelly Wrigley, C. Duong, M.D. Eberhard, A. Ede, K. Edelheit, B. Edens, C. Espada, G. Farhey, Y. Flores, F. Fritz, D. Ganguli, S. Gilbert, M. Gittar, P. Greenbaum, L. Grom, A. Gulati, G. Harry, O. Hayward, K. Henrickson, M. Hersh, A. Hiraki, L. Hiskey, M. Hoffmann, S. Hollander, M. Hom, C. Houk, L. Houk, J.B. Hsieh, E.W.Y. Hsu, J. Jensen, P. Joos, R. Jurado, R. Jusan Fiorot, F. Kallash, M. Kamphuis, S. Keltsev, V., (deceased) Khanna, S. Kim, S. Kimseng, K.J. Knight, A. Kunder, R. Lai, J. Laskin, B. Lewandowski, L. Lim, L. Linda, W.-W. Lo, M. Lovell, D. Luggen, M. Madison, J. Mansuri, A. Martin, L. Mason, S. Miller, M. Mina, R. Mohammed, A. Moncrieffe, H. Moorthy, L. Morgan, E. Mosquera, A. Muntel, E. Muscal, E. Myones, B. Nocton, J. Ogbu, E. Okamura, D. Olson, J. Orrock, J. Paim-Marques, L. Pain, C. Park, C. Patel, P. Pereira, M. Prado, R.D. Radhakrishna, S. Rheault, M. Ridgway, W. Riskalla, M. Ronis, T. Sadun, R. Sagcal-Gironella, A.C. Santos, M.C. Schikle and Chalhoub, N.E. Wenderfer, S.E. Levy, D.M. Rouster-Stevens, K. Aggarwal, A. Savani, S.I. Ruth, N.M. Arkachaisri, T. Qiu, T. Merritt, A. Onel, K. Goilav, B. Khubchandani, R.P. Deng, J. Fonseca, A.R. Ardoin, S.P. Ciurtin, C. Kasapcopur, O. Jelusic, M. Huber, A.M. Ozen, S. Klein-Gitelman, M.S. Appenzeller, S. Cavalcanti, A. Fotis, L. Lim, S.C. Silva, R.M. Miramontes, J.R. Rosenwasser, N.L. Saad-Magalhaes, C. Schonenberg-Meinema, D. Scott, C. Silva, C.A. Enciso, S. Terreri, M.T. Torres-Jimenez, A.-R. Trachana, M. Al-Mayouf, S.M. Devarajan, P. Huang, B. Brunner, H.I. Abulaban, K. Aguiar, C. Ahn, S.-Y. Akoghlanian, S. Al-Abrawi, S. Aljaberi, N. Alperin, R. Angeles-Han, S. Ardalan, K. Bader-Meunier, B. Balboni, I. Barbar-Smiley, F. Baxter, S. Beary, J. Boneparth, A. Brakeman, P. Bridges, J. Burgos-Vargas, R. Cabral, D.A. Cameto, J. Carter, C. Chang, J. Chédeville, G. Chhakchhuak, C. Chiraseveenuprapund, P. Cifuentes Alvarado, M. Concannon, A. Cooper, J. Cron, R. De Carvalho, L.M. De Quattro, K. De Ranieri, D. Dizon, B. Donnelly Wrigley, C. Duong, M.D. Eberhard, A. Ede, K. Edelheit, B. Edens, C. Espada, G. Farhey, Y. Flores, F. Fritz, D. Ganguli, S. Gilbert, M. Gittar, P. Greenbaum, L. Grom, A. Gulati, G. Harry, O. Hayward, K. Henrickson, M. Hersh, A. Hiraki, L. Hiskey, M. Hoffmann, S. Hollander, M. Hom, C. Houk, L. Houk, J.B. Hsieh, E.W.Y. Hsu, J. Jensen, P. Joos, R. Jurado, R. Jusan Fiorot, F. Kallash, M. Kamphuis, S. Keltsev, V., (deceased) Khanna, S. Kim, S. Kimseng, K.J. Knight, A. Kunder, R. Lai, J. Laskin, B. Lewandowski, L. Lim, L. Linda, W.-W. Lo, M. Lovell, D. Luggen, M. Madison, J. Mansuri, A. Martin, L. Mason, S. Miller, M. Mina, R. Mohammed, A. Moncrieffe, H. Moorthy, L. Morgan, E. Mosquera, A. Muntel, E. Muscal, E. Myones, B. Nocton, J. Ogbu, E. Okamura, D. Olson, J. Orrock, J. Paim-Marques, L. Pain, C. Park, C. Patel, P. Pereira, M. Prado, R.D. Radhakrishna, S. Rheault, M. Ridgway, W. Riskalla, M. Ronis, T. Sadun, R. Sagcal-Gironella, A.C. Santos, M.C. Schikle

Abstract

Objective: To develop a standardized steroid dosing regimen (SSR) for physicians treating childhood-onset systemic lupus erythematosus (SLE) complicated by lupus nephritis (LN), using consensus formation methodology. Methods: Parameters influencing corticosteroid (CS) dosing were identified (step 1). Data from children with proliferative LN were used to generate patient profiles (step 2). Physicians rated changes in renal and extrarenal childhood-onset SLE activity between 2 consecutive visits and proposed CS dosing (step 3). The SSR was developed using patient profile ratings (step 4), with refinements achieved in a physician focus group (step 5). A second type of patient profile describing the course of childhood-onset SLE for ≥4 months since kidney biopsy was rated to validate the SSR-recommended oral and intravenous (IV) CS dosages (step 6). Patient profile adjudication was based on majority ratings for both renal and extrarenal disease courses, and consensus level was set at 80%. Results: Degree of proteinuria, estimated glomerular filtration rate, changes in renal and extrarenal disease activity, and time since kidney biopsy influenced CS dosing (steps 1 and 2). Considering these parameters in 5,056 patient profile ratings from 103 raters, and renal and extrarenal course definitions, CS dosing rules of the SSR were developed (steps 3–5). Validation of the SSR for up to 6 months post–kidney biopsy was achieved with 1,838 patient profile ratings from 60 raters who achieved consensus for oral and IV CS dosage in accordance with the SSR (step 6). Conclusion: The SSR represents an international consensus on CS dosing for use in patients with childhood-onset SLE and proliferative LN. The SSR is anticipated to be used for clinical care and to standardize CS dosage during clinical trials. © 2021, American College of Rheumatology

Published
2022

20. AB0433 ANTI-RITUXIMAB ANTIBODIES DEMONSTRATE NEUTRALISING CAPACITY, ASSOCIATE WITH LOWER CIRCULATING DRUG LEVELS AND EARLY RELAPSE IN PATIENTS UNDERGOING TREATMENT FOR SYSTEMIC LUPUS ERYTHEMATOSUS

Author

Wincup, C., primary, Dunn, N., additional, Ruetsch-Chelli, C., additional, Manouchehrinia, A., additional, Kharlamova, N., additional, Naja, M., additional, Seitz-Polski, B., additional, Isenberg, D., additional, Fogdell-Hahn, A., additional, Ciurtin, C., additional, and Jury, E., additional

Published
2022
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23. AB0571 DERIVATION AND INDEPENDENT VALIDATION OF THE LUPUS ARTHRITIS AND MUSCULOSKELETAL DISEASE ACTIVITY SCORE (LAMDA): A MORE SENSITIVE, SPECIFIC AND RESPONSIVE TOOL FOR LUPUS ARTHRITIS

Author

Mahmoud, K., primary, Zayat, A., additional, MD Yusof, M. Y., additional, Teh, L. S., additional, Khan, S., additional, Yee, C. S., additional, D’cruz, D., additional, Ng, N., additional, Isenberg, D., additional, Ciurtin, C., additional, Conaghan, P. G., additional, Emery, P., additional, Edwards, C. J., additional, Hensor, E., additional, and Vital, E., additional

Published
2022
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24. International Consensus for the Dosing of Corticosteroids in Childhood-Onset Systemic Lupus Erythematosus With Proliferative Lupus Nephritis

Author

Chalhoub, N.E. Wenderfer, S.E. Levy, D.M. Rouster-Stevens, K. Aggarwal, A. Savani, S.I. Ruth, N.M. Arkachaisri, T. Qiu, T. Merritt, A. Onel, K. Goilav, B. Khubchandani, R.P. Deng, J. Fonseca, A.R. Ardoin, S.P. Ciurtin, C. Kasapcopur, O. Jelusic, M. Huber, A.M. Ozen, S. Klein-Gitelman, M.S. Appenzeller, S. Cavalcanti, A. Fotis, L. Lim, S.C. Silva, R.M. Miramontes, J.R. Rosenwasser, N.L. Saad-Magalhaes, C. Schonenberg-Meinema, D. Scott, C. Silva, C.A. Enciso, S. Terreri, M.T. Torres-Jimenez, A.-R. Trachana, M. Al-Mayouf, S.M. Devarajan, P. Huang, B. Brunner, H.I. Abulaban, K. Aguiar, C. Ahn, S.-Y. Akoghlanian, S. Al-Abrawi, S. Aljaberi, N. Alperin, R. Angeles-Han, S. Ardalan, K. Bader-Meunier, B. Balboni, I. Barbar-Smiley, F. Baxter, S. Beary, J. Boneparth, A. Brakeman, P. Bridges, J. Burgos-Vargas, R. Cabral, D.A. Cameto, J. Carter, C. Chang, J. Chédeville, G. Chhakchhuak, C. Chiraseveenuprapund, P. Cifuentes Alvarado, M. Concannon, A. Cooper, J. Cron, R. De Carvalho, L.M. De Quattro, K. De Ranieri, D. Dizon, B. Donnelly Wrigley, C. Duong, M.D. Eberhard, A. Ede, K. Edelheit, B. Edens, C. Espada, G. Farhey, Y. Flores, F. Fritz, D. Ganguli, S. Gilbert, M. Gittar, P. Greenbaum, L. Grom, A. Gulati, G. Harry, O. Hayward, K. Henrickson, M. Hersh, A. Hiraki, L. Hiskey, M. Hoffmann, S. Hollander, M. Hom, C. Houk, L. Houk, J.B. Hsieh, E.W.Y. Hsu, J. Jensen, P. Joos, R. Jurado, R. Jusan Fiorot, F. Kallash, M. Kamphuis, S. Keltsev, V., (deceased) Khanna, S. Kim, S. Kimseng, K.J. Knight, A. Kunder, R. Lai, J. Laskin, B. Lewandowski, L. Lim, L. Linda, W.-W. Lo, M. Lovell, D. Luggen, M. Madison, J. Mansuri, A. Martin, L. Mason, S. Miller, M. Mina, R. Mohammed, A. Moncrieffe, H. Moorthy, L. Morgan, E. Mosquera, A. Muntel, E. Muscal, E. Myones, B. Nocton, J. Ogbu, E. Okamura, D. Olson, J. Orrock, J. Paim-Marques, L. Pain, C. Park, C. Patel, P. Pereira, M. Prado, R.D. Radhakrishna, S. Rheault, M. Ridgway, W. Riskalla, M. Ronis, T. Sadun, R. Sagcal-Gironella, A.C. Santos, M.C. Schikler, K. AL Suwairi, W. Siddiqi, N. Silva, M.F. Singh-Grewal, D. Smitherman, E. Smolewska, E. Son, M.B. Srinivasalu, H. Sule, S. Susic, G. Syed, R. Thatayatikom, A. Ting, T. Toth, M. Turnier, J. Vashisht, P. Vega Fernandez, P. Velasquez, M. von Scheven, E. Wahezi, D. Ware, A. Wu, E. Yan, J. Yildirim-Toruner, C. Zamparo, C. Zhang, Y. Lawson, E. for the Childhood Arthritis Rheumatology Research Alliance Lupus Nephritis Work Group the Pediatric Rheumatology European Society Lupus Working Party

Abstract

Objective: To develop a standardized steroid dosing regimen (SSR) for physicians treating childhood-onset systemic lupus erythematosus (SLE) complicated by lupus nephritis (LN), using consensus formation methodology. Methods: Parameters influencing corticosteroid (CS) dosing were identified (step 1). Data from children with proliferative LN were used to generate patient profiles (step 2). Physicians rated changes in renal and extrarenal childhood-onset SLE activity between 2 consecutive visits and proposed CS dosing (step 3). The SSR was developed using patient profile ratings (step 4), with refinements achieved in a physician focus group (step 5). A second type of patient profile describing the course of childhood-onset SLE for ≥4 months since kidney biopsy was rated to validate the SSR-recommended oral and intravenous (IV) CS dosages (step 6). Patient profile adjudication was based on majority ratings for both renal and extrarenal disease courses, and consensus level was set at 80%. Results: Degree of proteinuria, estimated glomerular filtration rate, changes in renal and extrarenal disease activity, and time since kidney biopsy influenced CS dosing (steps 1 and 2). Considering these parameters in 5,056 patient profile ratings from 103 raters, and renal and extrarenal course definitions, CS dosing rules of the SSR were developed (steps 3–5). Validation of the SSR for up to 6 months post–kidney biopsy was achieved with 1,838 patient profile ratings from 60 raters who achieved consensus for oral and IV CS dosage in accordance with the SSR (step 6). Conclusion: The SSR represents an international consensus on CS dosing for use in patients with childhood-onset SLE and proliferative LN. The SSR is anticipated to be used for clinical care and to standardize CS dosage during clinical trials. © 2021, American College of Rheumatology

Published
2022

25. Clinical and laboratory characteristics in juvenile-onset systemic lupus erythematosus across age groups

Author

Massias, JS, Smith, EMD, Al-Abadi, E, Armon, K, Bailey, K, Ciurtin, C, Davidson, J, Gardner-Medwin, J, Haslam, K, Hawley, DP, Leahy, A, Leone, V, McErlane, F, Mewar, D, Modgil, G, Moots, R, Pilkington, C, Ramanan, AV, Rangaraj, S, Riley, P, Sridhar, A, Wilkinson, N, Beresford, MW, Hedrich, CM, and Grp, UKJSLE Study

Subjects
Paper, Male, 0301 basic medicine, medicine.medical_specialty, Adolescent, phenotype, SLE, Severity of Illness Index, Cohort Studies, 03 medical and health sciences, Sex Factors, 0302 clinical medicine, Rheumatology, Age groups, Humans, Lupus Erythematosus, Systemic, Medicine, Age of Onset, Child, childhood, 030203 arthritis & rheumatology, Systemic lupus erythematosus, Clinical Laboratory Techniques, business.industry, Age group, medicine.disease, Dermatology, juvenile-onset SLE, United Kingdom, 030104 developmental biology, Juvenile onset, Disease Progression, Female, business
Abstract

Background Systemic lupus erythematous (SLE) is a systemic autoimmune/inflammatory condition. Approximately 15–20% of patients develop symptoms before their 18th birthday and are diagnosed with juvenile-onset SLE (JSLE). Gender distribution, clinical presentation, disease courses and outcomes vary significantly between JSLE patients and individuals with adult-onset SLE. This study aimed to identify age-specific clinical and/or serological patterns in JSLE patients enrolled to the UK JSLE Cohort Study. Methods Patient records were accessed and grouped based on age at disease-onset: pre-pubertal (≤7 years), peri-pubertal (8–13 years) and adolescent (14–18 years). The presence of American College of Rheumatology (ACR) classification criteria, laboratory results, disease activity [British Isles Lupus Assessment Group (BILAG) and Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2 K) scores] and damage [Systemic Lupus International Collaborating Clinics (SLICC) damage index] were evaluated at diagnosis and last follow up. Results A total of 418 JSLE patients were included in this study: 43 (10.3%) with pre-pubertal disease onset; 240 (57.4%) with peri-pubertal onset and 135 (32.3%) were diagnosed during adolescence. At diagnosis, adolescent JSLE patients presented with a higher number of ACR criteria when compared with pre-pubertal and peri-pubertal patients [pBILAG2004 scores: 9(4–20] vs. 7(3–13] vs. 7(3–14], respectively, p = 0.015] with increased activity in the following BILAG domains: mucocutaneous ( p = 0.025), musculoskeletal ( p = 0.029), renal ( p = 0.027) and cardiorespiratory ( p = 0.001). Furthermore, adolescent JSLE patients were more frequently ANA-positive ( p = 0.034) and exhibited higher anti-dsDNA titres ( p = 0.001). Pre-pubertal individuals less frequently presented with leukopenia ( p = 0.002), thrombocytopenia ( p = 0.004) or low complement ( p = 0.002) when compared with other age groups. No differences were identified in disease activity (pBILAG2004 score), damage (SLICC damage index) and the number of ACR criteria fulfilled at last follow up. Conclusions Disease presentations and laboratory findings vary significantly between age groups within a national cohort of JSLE patients. Patients diagnosed during adolescence exhibit greater disease activity and “classic” autoantibody, immune cell and complement patterns when compared with younger patients. This supports the hypothesis that pathomechanisms may vary between patient age groups.

Published
2020
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26. POS0746 THE ARTHRITIS COMPONENT OF THE SLEDAI SHOULD ONLY BE SCORED IF THERE IS JOINT SWELLING

Author

Mahmoud, K., primary, Zayat, A., additional, MD Yusof, M. Y., additional, Dutton, K., additional, Teh, L. S., additional, Yee, C. S., additional, D’cruz, D., additional, Ng, N., additional, Isenberg, D., additional, Ciurtin, C., additional, Conaghan, P. G., additional, Emery, P., additional, Edwards, C. J., additional, Hensor, E., additional, and Vital, E., additional

Published
2021
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29. FRI0599 USEFUL II: DERIVATION OF THE LUPUS ARTHRITIS AND MUSCULOSKELETAL DISEASE ACTIVITY SCORE (LAMDA) USING DATA FROM A MULTICENTRE LONGITUDINAL STUDY

Author

Mahmoud, K., primary, Zayat, A., additional, MD Yusof, M. Y., additional, Ciurtin, C., additional, Yee, C. S., additional, Isenberg, D., additional, Teh, L. S., additional, Dutton, K., additional, D’cruz, D., additional, Ng, N., additional, Conaghan, P. G., additional, Emery, P., additional, Edwards, C., additional, Hensor, E., additional, and Vital, E., additional

Published
2020
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32. OP0179 USEFUL STUDY I: A MULTICENTRE LONGITUDINAL STUDY TO TEST WHETHER ULTRASOUND CAN IDENTIFY PATIENTS WITH MUSCULOSKELETAL SYMPTOMS OF LUPUS WITH BETTER RESPONSE TO THERAPY

Author

Mahmoud, K., primary, Zayat, A., additional, MD Yusof, M. Y., additional, Dutton, K., additional, Teh, L. S., additional, Yee, C. S., additional, D’cruz, D., additional, Ng, N., additional, Isenberg, D., additional, Ciurtin, C., additional, Conaghan, P. G., additional, Emery, P., additional, Edwards, C., additional, Hensor, E., additional, and Vital, E., additional

Published
2020
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34. FRI0599 USEFUL II: DERIVATION OF THE LUPUS ARTHRITIS AND MUSCULOSKELETAL DISEASE ACTIVITY SCORE (LAMDA) USING DATA FROM A MULTICENTRE LONGITUDINAL STUDY

Author

Mahmoud, K., Zayat, A., Yusof, M. Y. MD, Ciurtin, C., Yee, C. S., Isenberg, D., Teh, L.S., Mahmoud, K., Zayat, A., Yusof, M. Y. MD, Ciurtin, C., Yee, C. S., Isenberg, D., and Teh, L.S.

Abstract

Background: Musculoskeletal (MSK) disease is the commonest manifestation of SLE. We showed that the MSK components of the BILAG index and SLEDAI have limited sensitivity, specificity and responsiveness compared to ultrasound (US) synovitis. The USEFUL study evaluated response to glucocorticoids in SLE patients with inflammatory pain. Objectives: To develop a disease activity tool for lupus MSK manifestations that is continuous, responsive, sensitive, and correlates with US-synovitis Methods: 133 patients who received depomedrone 120mg IM were assessed at 0, 2 and 6 weeks for 66/68 swollen and tender joint counts, BILAG2004 index, SLEDAI-2K, physician global and MSK-VAS, inflammatory markers, patient pain and disease activity-VAS. Total US score (OMERACT-EULAR) in the hands and wrists was calculated blinded to patient and clinical assessor. Patients reported overall response using a Likert scale. The LAMDA was developed by modelling a core set of clinical variables against total US score using penalized (Lasso) regression. Responsiveness was compared between LAMDA and other variables at week 6 using effect sizes. Minimum clinically important difference (MCID) was explored using the SEM and minimal disease activity threshold using ROC. Results: The variables selected for the LAMDA score were swollen joint count, patient MSK pain VAS, physician MSK disease activity VAS and ESR. A continuous score was derived. This had a theoretical range from 0 to 26.5 based on maximum ESR of 100. The highest value observed in USEFUL was 15. LAMDA was significantly higher in patients with active US (mean (SD) 5.71 (2.67), n=78) compared to patients with normal US (3.27 (1.77), n=55; difference (95% CI) -2.45 (-3.26, -1.63), t=-5.93, p<0.001). This difference remained significant in patients with no swollen joints (difference (95% CI) -0.71 (-1.40, -0.02), t=-2.06, p=0.044). Effect size was greater for the LAMDA (0.37) than the BILAG-MSK (0.31), SLEDAI-MSK (0.27) and total US score (0.3

Published
2020

35. Novel methodology to discern predictors of remission and patterns of disease activity over time using rheumatoid arthritis clinical trials data

Author

Tom, BDM, Symmons, D, Brockbank, S, Carini, C, Cope, AP, Ehrenstein, MR, Fisher, BA, Goodyear, CS, Gozzard, N, Harris, R, Hicks, K, Hollis, S, Hughes-Morley, A, Isaacs, J, Kola, B, McInnes, IB, Mela, CM, Parker, G, Pedersen, AW, Ponchel, F, Sabin, T, Scott, DL, Scott, IC, Sleeman, MA, Taylor, PC, Tsuji, W, Zhong, Y, Hilkens, C, Anderson, A, Stocks, P, Lendrem, D, Tarn, J, Smith, G, Allen, B, Casement, J, Diboll, J, Harry, R, Simpson, G, Toward, R, Noble, H, Parke, A, Wu, W, Clarke, F, Galloway, J, Lempp, H, Ibrahim, F, Schwank, S, Molyneux, G, Lazarov, T, Geissmann, F, Donnelly, I, Gilmour, A, Virlan, AT, Porter, D, Emery, P, El-Jawhari, J, Parmar, R, McDermot, MF, Buch, M, Buckley, C, Young, SP, Jones, P, Raza, K, Filer, A, Pitzalis, C, Barnes, MR, Watson, DS, Tzanis, E, Thorborn, G, Fossati-Jimack, L, Kelly, S, Humby, F, Bombardieri, M, Rana, S, Jia, Z, Goldmann, K, Lewis, M, Altobelli, G, John, C, Martins, S, Nguyen, D, Ali, H, Ciurtin, C, Worthington, J, Bruce, IN, Sergeant, JC, Verstappen, SMM, Stirling, F, Farewell, V, Keidel, S, Cuff, C, Levesque, M, Long, A, Liu, Z, Lipsky, S, Harvey, B, Macoritto, M, Hong, F, Kaymakcalan, S, Ward, N, Talbot, S, Padhji, D, Sleeman, M, Finch, D, Herath, A, Lindholm, C, Jenkins, M, Ho, M, Marshall, C, Page, M, Edwards, H, Cuza, A, Rowe, A, Capdevila, FB, Loza, M, Curran, M, Verbeeck, D, Baker, D, Vranic, I, Mela, CT, Wright, S, Rowell, L, Vernon, E, Joseph, N, Payne, N, Rao, R, Binks, M, Belson, A, Ludbrook, V, Tipney, H, Ellis, J, Hasan, S, Didierlaurent, A, Burny, W, Haynes, A, Larminie, C, Dastros-Pitel, D, Jelinsky, S, Hodge, M, Maciejewski, M, Ziemek, D, Schulz-Knappe, P, Zucht, H-D, Budde, P, Coles, MC, Butler, JA, Read, S, and Consortium, RA-MAP

Subjects
musculoskeletal diseases, immune system diseases, skin and connective tissue diseases
Abstract

Objectives: To identify predictors of remission and disease activity patterns in patients with rheumatoid arthritis (RA) using individual participant data (IPD) from clinical trials. Methods: Phase II and III clinical trials completed between 2002 and 2012 were identified by systematic literature review and contact with UK market authorisation holders. Anonymised baseline and follow-up IPD from non-biological arms were amalgamated. Multiple imputation was used to handle missing outcome and covariate information. Random effects logistic regression was used to identify predictors of remission, measured by the Disease Activity Score 28 (DAS28) at 6 months. Novel latent class mixed models characterised DAS28 over time. Results: IPD of 3290 participants from 18 trials were included. Of these participants, 92% received methotrexate (MTX). Remission rates were estimated at 8.4%(95%CI 7.4%to9.5%) overall, 17%(95%CI 14.8%to19.4%) for MTX-naïve patients with early RA and 3.2% (95% CI 2.4% to 4.3%) for those with prior MTX exposure at entry. In prior MTX-exposed patients, lower baseline DAS28 and MTX reinitiation were associated with remission. In MTX-naïve patients, being young, white, male, with better functional and mental health, lower baseline DAS28 and receiving concomitant glucocorticoids were associated with remission. Three DAS28 trajectory subpopulations were identified in MTX-naïve and MTX-exposed patients. A number of variables were associated with subpopulation membership and DAS28 levels within subpopulations. Conclusions: Predictors of remission differed between MTX-naïve and prior MTX-exposed patients at entry. Latent class mixed models supported differential non-biological therapy response, with three distinct trajectories observed in both MTX-naïve and MTX-exposed patients. Findings should be useful when designing future RA trials and interpreting results of biomarker studies.

Published
2018

36. Diet and lupus: what do the patients think?

Author

Robinson, G A, primary, Mcdonnell, T, additional, Wincup, C, additional, Martin-Gutierrez, L, additional, Wilton, J, additional, Kalea, A Z, additional, Ciurtin, C, additional, Pineda-Torra, I, additional, and Jury, E C, additional

Published
2019
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37. Arthritis prevention in the pre-clinical phase of RA with abatacept (the APIPPRA study): A multi-centre, randomised, double-blind, parallel-group, placebo-controlled clinical trial protocol

Author

Al-Laith, M., Jasenecova, M., Abraham, S., Bosworth, A., Bruce, I. N., Buckley, C. D., Ciurtin, C., D'Agostino, Maria Antonietta, Emery, P., Gaston, H., Isaacs, J. D., Filer, A., Fisher, B. A., Huizinga, T. W. J., Ho, P., Jacklin, C., Lempp, H., Mcinnes, I. B., Pratt, A. G., Ostor, A., Raza, K., Taylor, P. C., Van Schaardenburg, D., Shivapatham, D., Wright, A. J., Vasconcelos, J. C., Kelly, J., Murphy, C., Prevost, A. T., Cope, A. P., D'Agostino M. A. (ORCID:0000-0002-5347-0060), Al-Laith, M., Jasenecova, M., Abraham, S., Bosworth, A., Bruce, I. N., Buckley, C. D., Ciurtin, C., D'Agostino, Maria Antonietta, Emery, P., Gaston, H., Isaacs, J. D., Filer, A., Fisher, B. A., Huizinga, T. W. J., Ho, P., Jacklin, C., Lempp, H., Mcinnes, I. B., Pratt, A. G., Ostor, A., Raza, K., Taylor, P. C., Van Schaardenburg, D., Shivapatham, D., Wright, A. J., Vasconcelos, J. C., Kelly, J., Murphy, C., Prevost, A. T., Cope, A. P., and D'Agostino M. A. (ORCID:0000-0002-5347-0060)

Abstract

Trial design: We present a study protocol for a multi-centre, randomised, double-blind, parallel-group, placebo-controlled trial that seeks to test the feasibility, acceptability and effectiveness of a 52-week period of treatment with the first-in-class co-stimulatory blocker abatacept for preventing or delaying the onset of inflammatory arthritis. Methods: The study aimed to recruit 206 male or female subjects from the secondary care hospital setting across the UK and the Netherlands. Participants who were at least 18 years old, who reported inflammatory sounding joint pain (clinically suspicious arthralgia) and who were found to be positive for serum autoantibodies associated with rheumatoid arthritis (RA) were eligible for enrolment. All study subjects were randomly assigned to receive weekly injections of investigational medicinal product, either abatacept or placebo treatment over the course of a 52-week period. Participants were followed up for a further 52 weeks. The primary endpoint was defined as the time to development of at least three swollen joints or to the fulfilment of the 2010 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) classification criteria for RA using swollen but not tender joints, whichever endpoint was met first. In either case, swollen joints were confirmed by ultrasonography. Participants, care givers, and those assessing the outcomes were all blinded to group assignment. Clinical assessors and ultrasonographers were also blinded to each other's assessments for the duration of the study. Conclusions: There is limited experience of the design and implementation of trials for the prevention of inflammatory joint diseases. We discuss the rationale behind choice and duration of treatment and the challenges associated with defining the "at risk" state and offer pragmatic solutions in the protocol to enrolling subjects at risk of RA. Trial registration: Current Controlled Trials, ID: ISRCTN46017566. Registered

Published
2019

38. Outcomes following mycophenolate mofetil versus cyclophosphamide induction treatment for proliferative juvenile-onset lupus nephritis

Author

Smith, EMD, primary, Al-Abadi, E, additional, Armon, K, additional, Bailey, K, additional, Ciurtin, C, additional, Davidson, J, additional, Gardner-Medwin, J, additional, Haslam, K, additional, Hawley, D, additional, Leahy, A, additional, Leone, V, additional, McErlane, F, additional, Mewar, D, additional, Modgil, G, additional, Moots, R, additional, Pilkington, C, additional, Ramanan, A, additional, Rangaraj, S, additional, Riley, P, additional, Sridhar, A, additional, Wilkinson, N, additional, Beresford, M W, additional, and Hedrich, C M, additional

Published
2019
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43. THU0023 Complex immunophenotyping stratifies patients with primary sjÖgren’s syndrome, systemic lupus erythematosus and secondary sjÖgren’s syndrome associated with systemic lupus erythematosus into distinct clinically relevant groups with potential therapeutic implications

Author

Thompson, N., primary, Gandhi, A., additional, Radmmore, R., additional, Gupta, V., additional, Robinson, G., additional, Martin-Gutierrez, L., additional, Isenberg, D., additional, Jury, E., additional, and Ciurtin, C., additional

Published
2018
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46. HEPATITIS VIRUS C INFECTION, ADIPOKINES AND HEPATIC STEATO–FIBROSIS

Author

Ciurtin, C and Stoica, V

Subjects
Fatty Liver, Liver Cirrhosis, Adipokines, Homeostasis, Humans, General Article, Hepatitis C, Chronic, Antiviral Agents
Abstract

Hepatitis C viral infection is accompanied by various serum alterations that could explain its molecular impact on hepatic structure and metabolic homeostasis. Recently it has been shown that adipocytokines play a pivotal role in development of hepatic steato-fibrosis, different studies giving a support of the hypothesis that the balance of adipocytokine expression is a key regulator for the progression of hepatic steatosis and fibrosis. The association between insulin resistance and hepatitis C virus genotypes and liver fibrosis stage foreshadowed that virus-induced insulin resistance may be a mechanism for fibrogenesis in chronic hepatitis C virus infection. The main importance of adipocytokine profile detection consists in the prediction of steatosis induction that has clinical relevance, being associated with advanced fibrosis and hyporesponsiveness to antiviral therapy.

Published
2008

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