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13. French National Diagnostic and Care Protocol for antiphospholipid syndrome in adults and children

Author

Chauveau, D., Clouscard, J., Frere, C., Hachulla, E., Kone-Paut, I., Lasne, D., Lecompte, T., Le Guern, V., Ni Zard, J., Papo, T., Riviere, M., Schleinitz, N., Tossier, B., Amoura, Z., Bader-Meunier, B., BAL dit Sollier, C., Belot, A., Benhamou, Y., Bezanahary, H., Cohen, F., Costedoat-Chalumeau, N., Darnige, L., Drouet, L., Elefant, E., Harroche, A., Lambert, M., Martin, T., Martin-Toutain, I., Mathian, A., Mekinian, A., Pineton De Chambrun, M., de Pontual, L., Wahl, D., Yelnik, C., and Zuily, S.

Published
2023
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15. POS1185 IRON DEFICIENCY IN FAMILIAL MEDITERRANEAN FEVER: A STUDY ON 211 ADULT PATIENTS FROM THE JIR COHORT

Author

DI Cola, I., primary, Savey, L., additional, Delplanque, M., additional, Bourguiba, R., additional, Bartoli, A., additional, Aknouche, Z., additional, Bensalek, F., additional, Koné-Paut, I., additional, Rossi-Semerano, L., additional, Melki, I., additional, Bader-Meunier, B., additional, Neven, B., additional, Quartier, P., additional, Boursier, G., additional, Giurgea, I., additional, Cuisset, L., additional, Grateau, G., additional, Hentgen, V., additional, and Georgin-Lavialle, S., additional

Published
2024
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16. POS0378 HETEROZYGOUS LOSS-OF-FUNCTION RELA MUTATIONS LEAD TO SYSTEMIC LUPUS ERYTHEMATOSUS OR AUTOINFLAMMATORY SYNDROME

Author

Riller, Q., primary, Barnabei, L., additional, Rodrigo-Riestra, M., additional, Stolzenberg, M. C., additional, Pelle, O., additional, Delage, L., additional, Becquard, T., additional, Courteille, C., additional, Marchais, M., additional, De Cevins, C., additional, Brunaud, C., additional, Magerus, A., additional, Luka, M., additional, Sorin, B., additional, Boussard, C., additional, Salomon, R., additional, Ménager, M., additional, Hié, M., additional, Neven, B., additional, Baud, V., additional, Skokowa, J., additional, Sogkas, G., additional, Jamilloux, Y., additional, Merlin, E., additional, Belot, A., additional, Picard, C., additional, Boursier, G., additional, Riviere, E., additional, Georgin-Lavialle, S., additional, Quartier, P., additional, Bader-Meunier, B., additional, Fischer, A., additional, Miner, J. J., additional, and Rieux-Laucat, F., additional

Published
2024
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17. POS0221 CLUSTER ANALYSIS IDENTIFIES THREE CLINICAL PATTERNS OF PATIENTS WITH ANTI-KU ANTIBODIES: A MULTICENTRE STUDY OF 154 PATIENTS.

Author

Robert, M., primary, Nguyen, Y., additional, Allenbach, Y., additional, Sacre, K., additional, Terrier, B., additional, Borie, R., additional, Uzunhan, Y., additional, Amoura, Z., additional, Comparon, C., additional, Dieudé, P., additional, Le Guern, V., additional, Morélot-Panzini, C., additional, Humbert, M., additional, Sitbon, O., additional, Goujard, C., additional, Bader-Meunier, B., additional, Fautrel, B., additional, Chrétien, P., additional, Roland-Nicaise, P., additional, Goulvestre, C., additional, Charuel, J. L., additional, Benveniste, O., additional, Mouthon, L., additional, De Lastours, V., additional, Dusser, P., additional, Zaidan, M., additional, Aslangul, E., additional, Saillour, M., additional, Dingulu, G., additional, Chasset, F., additional, Nocturne, G., additional, Mariette, X., additional, Bitoun, S., additional, and Seror, R., additional

Published
2024
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19. Continuer les anti-TNF alpha pendant la grossesse est associé à un moindre risque infectieux maternel, sans sur-risque d’autres issues de grossesse défavorables : résultats de l’essai émulé BioGRIC

Author

Molto, A., primary, Ajrouche, A., additional, Tran, M.H.T.D., additional, Costedoat-Chalumeau, N., additional, Elefant, E., additional, Tsatsaris, V., additional, Fresson, J., additional, Bader-Meunier, B., additional, Fautrel, B., additional, and Tubach, F., additional

Published
2023
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20. Phénotypes cliniques et pronostiques associés aux anticorps anti-Ku déterminés par une analyse en clusters : une étude multicentrique de 154 patients

Author

Robert, M., primary, Nguyen, Y., additional, Allenbach, Y., additional, Sacre, K., additional, Terrier, B., additional, Borie, R., additional, Uzunhan, Y., additional, Amoura, Z., additional, Dieudé, P., additional, Le Guern, V., additional, Morelot-Panzini, C., additional, Humbert, M., additional, Sitbon, O., additional, Goujard, C., additional, Bader-Meunier, B., additional, Fautrel, B., additional, Chrétien, P., additional, Roland-Nicaise, P., additional, Goulvestre, C., additional, Charuel, J.L., additional, Benveniste, O., additional, Mouthon, L., additional, De Lastours, V., additional, Koné-Paut, I., additional, Zaidan, M., additional, Alsanglul, E., additional, Saillour, M., additional, Dingulu, G., additional, Nocturne, G., additional, Mariette, X., additional, Bitoun, S., additional, and Seror, R., additional

Published
2023
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22. French National Diagnostic and Care Protocol for antiphospholipid syndrome in adults and children

Author

Amoura, Z., primary, Bader-Meunier, B., additional, BAL dit Sollier, C., additional, Belot, A., additional, Benhamou, Y., additional, Bezanahary, H., additional, Cohen, F., additional, Costedoat-Chalumeau, N., additional, Darnige, L., additional, Drouet, L., additional, Elefant, E., additional, Harroche, A., additional, Lambert, M., additional, Martin, T., additional, Martin-Toutain, I., additional, Mathian, A., additional, Mekinian, A., additional, Pineton De Chambrun, M., additional, de Pontual, L., additional, Wahl, D., additional, Yelnik, C., additional, Zuily, S., additional, Chauveau, D., additional, Clouscard, J., additional, Frere, C., additional, Hachulla, E., additional, Kone-Paut, I., additional, Lasne, D., additional, Lecompte, T., additional, Le Guern, V., additional, Ni Zard, J., additional, Papo, T., additional, Riviere, M., additional, Schleinitz, N., additional, and Tossier, B., additional

Published
2023
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24. POS0305 CONTINUING TNFI AFTER PREGNANCY DIAGNOSIS IN WOMEN WITH CHRONIC RHEUMATIC INFLAMMATORY DISEASES IS NOT ASSOCIATED WITH WORSE OBSTETRICAL OR INFECTIOUS OUTCOMES: THE RESULTS OF AN EMULATED TARGET TRIAL

Author

Moltó, A., primary, Ajrouche, A., additional, Tran, D., additional, De-Rycke, Y., additional, Costedoat-Chalumeau, N., additional, Elefant, E., additional, Tsatsaris, V., additional, Fresson, J., additional, Bader-Meunier, B., additional, Fautrel, B., additional, and Tubach, F., additional

Published
2023
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25. OP0254 INSIGHTS FROM A NOVEL MONOGENIC AUTOIMMUNE DISEASE: OVERVIEW OF A MULTICENTRIC EUROPEAN COHORT OF 27 PATIENTS WITH COPA SYNDROME

Author

David-Gabarre, C., primary, Bader-Meunier, B., additional, Belot, A., additional, Labouret, G., additional, Brennan, M., additional, Al-Abadi, E., additional, Arkwright, P., additional, Newman, W., additional, Gattorno, M., additional, Stefano, V., additional, Tommasini, A., additional, Manna, R., additional, Taddio, A., additional, Lopez-Montesinos, B., additional, Clavaguera Poch, T., additional, Mensa, A., additional, Boulisfane, S., additional, Thumerelle, C., additional, Cadranel, J., additional, Maurier, F., additional, Wislez, M., additional, Crow, Y., additional, Nathan, N., additional, and Frémond, M. L., additional

Published
2023
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26. POS0753 TIF1-GAMMA IGG2 ISOTYPE IS ASSOCIATED WITH ETHNICITY IN JDM PATIENTS

Author

Nguyen, H., primary, Jouen, F., additional, Déchelotte, B., additional, Cordel, N., additional, Gitiaux, C., additional, Bodemer, C., additional, Quartier, P., additional, Belot, A., additional, O’brien, K., additional, Melki, I., additional, Fabien, N., additional, Tansley, S., additional, Wedderburn, L., additional, Boyer, O., additional, and Bader-Meunier, B., additional

Published
2023
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27. French practical guidelines for the diagnosis and management of relapsing polychondritis

Author

Arnaud, L., primary, Costedoat-Chalumeau, N., additional, Mathian, A., additional, Sailler, L., additional, Belot, A., additional, Dion, J., additional, Morel, N., additional, Moulis, G., additional, Bader-Meunier, B., additional, Bodaghi, B., additional, Bura Riviere, A., additional, Casadevall, M., additional, Fain, O., additional, Frances, C., additional, Hachulla, E., additional, Hamidou, M., additional, Karakoglou, C., additional, Lambert, M., additional, Lerebours, F., additional, Leroux, G., additional, Mariette, X., additional, Marquette, C.H., additional, Martin, T., additional, Mekinian, A., additional, Papo, T., additional, Piette, J.-C., additional, Puechal, X., additional, Richez, C., additional, Saraux, A., additional, Seve, P., additional, Tankere, F., additional, Terriou, L., additional, and Varin, P., additional

Published
2023
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28. Curation and expansion of the Human Phenotype Ontology for systemic autoinflammatory diseases improves phenotype-driven disease-matching.

Author

Maassen, W., Legger, G., Kul Cinar, O., Daele, P. van, Gattorno, M., Bader-Meunier, B., Wouters, C., Briggs, T., Johansson, L., Velde, J. van der, Swertz, M., Omoyinmi, E., Hoppenreijs, E.P., Belot, A., Eleftheriou, D., Caorsi, R., Aeschlimann, F., Boursier, G., Brogan, P., Haimel, M., Gijn, M. van, Maassen, W., Legger, G., Kul Cinar, O., Daele, P. van, Gattorno, M., Bader-Meunier, B., Wouters, C., Briggs, T., Johansson, L., Velde, J. van der, Swertz, M., Omoyinmi, E., Hoppenreijs, E.P., Belot, A., Eleftheriou, D., Caorsi, R., Aeschlimann, F., Boursier, G., Brogan, P., Haimel, M., and Gijn, M. van

Abstract

Item does not contain fulltext, INTRODUCTION: Accurate and standardized phenotypic descriptions are essential in diagnosing rare diseases and discovering new diseases, and the Human Phenotype Ontology (HPO) system was developed to provide a rich collection of hierarchical phenotypic descriptions. However, although the HPO terms for inborn errors of immunity have been improved and curated, it has not been investigated whether this curation improves the diagnosis of systemic autoinflammatory disease (SAID) patients. Here, we aimed to study if improved HPO annotation for SAIDs enhanced SAID identification and to demonstrate the potential of phenotype-driven genome diagnostics using curated HPO terms for SAIDs. METHODS: We collected HPO terms from 98 genetically confirmed SAID patients across eight different European SAID expertise centers and used the LIRICAL (Likelihood Ratio Interpretation of Clinical Abnormalities) computational algorithm to estimate the effect of HPO curation on the prioritization of the correct SAID for each patient. RESULTS: Our results show that the percentage of correct diagnoses increased from 66% to 86% and that the number of diagnoses with the highest ranking increased from 38 to 45. In a further pilot study, curation also improved HPO-based whole-exome sequencing (WES) analysis, diagnosing 10/12 patients before and 12/12 after curation. In addition, the average number of candidate diseases that needed to be interpreted decreased from 35 to 2. DISCUSSION: This study demonstrates that curation of HPO terms can increase identification of the correct diagnosis, emphasizing the high potential of HPO-based genome diagnostics for SAIDs.

Published
2023

29. Manifestations cutanées atypiques lors de l’apparition de l’arthrite juvénile idiopathique de forme systémique et difficultés de traitement : une étude rétrospective multicentrique

Author

Eveillard, L.A., primary, Quartier, P., additional, Ouldali, N., additional, Bader-Meunier, B., additional, Bourrat, E., additional, Aeschlimann, F., additional, Ballot, C., additional, Bouric, P., additional, Desdoits, A., additional, Dumaine, C., additional, Galeotti, C., additional, Hentgen, V., additional, Lefevre-Utile, A., additional, Chausset, A., additional, Hubiche, T., additional, Kupfer-Bessaguet, I., additional, Leclerq-Mercier, S., additional, Mallet, S., additional, Melki, I., additional, Merlin, E., additional, Miquel, J., additional, Piram, M., additional, Talmud, D., additional, Garcelon, N., additional, Vinit, C., additional, Welfringer, A., additional, and Meinzer, U., additional

Published
2022
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30. Two pediatric cases of multisystem inflammatory-like syndrome following COVID-19 vaccination

Author

Collignon, C., primary, Frachette, C., additional, Callot, D., additional, Pinhas, Y., additional, Bataille, P., additional, Bader-Meunier, B., additional, Chouchana, L., additional, Frémond, M.-L., additional, Belhadjer, Z., additional, Oualha, M., additional, Moulin, F., additional, Javouhey, E., additional, Belot, A., additional, and Renolleau, S., additional

Published
2022
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35. Mutations de UNC93B1 : une nouvelle cause de lupus érythémateux systémique et de lupus-engelures monogéniques

Author

David, C., Arango-Franco, C., Badony, M., Fouchet, J., Rice, G., Didry-Barca, B., Maisonneuve, L., Seabra, L., Kechiche, R., Masson, C., Cobat, A., Abel, L., Talouarn, E., Beziat, V., Deswarte, C., Livingstone, K., Paul, C., Malik, G., Ross, A., Adam, J., Walsch, J., Kumar, S., Bonnet, D., Bodemer, C., Bader-Meunier, B., Marsch, J., Crow, Y., Casanova, J.L., Manoury, B., Frémond, M.L., Bohlen, J., and Lepelley, A.

Published
2024
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36. Curation and expansion of Human Phenotype Ontology for defined groups of inborn errors of immunity

Author

Haimel, M., Pazmandi, J., Heredia, R.J., Dmytrus, J., Bal, S.K., Zoghi, S., Daele, P. van, Briggs, T.A., Wouters, C., Bader-Meunier, B., Aeschlimann, F.A., Caorsi, R., Eleftheriou, D., Hoppenreijs, E.P., Salzer, E., Bakhtiar, S., Derfalvi, B., Saettini, F., Kusters, M.A.A., Elfeky, R., Trück, J., Rivière, J.G., Burg, M. van der, Gattorno, M., Seidel, M.G., Burns, S., Warnatz, K., Hauck, F., Brogan, P., Gilmour, K.C., Schuetz, C., Simon, A., Bock, C., Hambleton, S., Vries, E de, Robinson, P.N., Gijn, M. van, Boztug, K., Haimel, M., Pazmandi, J., Heredia, R.J., Dmytrus, J., Bal, S.K., Zoghi, S., Daele, P. van, Briggs, T.A., Wouters, C., Bader-Meunier, B., Aeschlimann, F.A., Caorsi, R., Eleftheriou, D., Hoppenreijs, E.P., Salzer, E., Bakhtiar, S., Derfalvi, B., Saettini, F., Kusters, M.A.A., Elfeky, R., Trück, J., Rivière, J.G., Burg, M. van der, Gattorno, M., Seidel, M.G., Burns, S., Warnatz, K., Hauck, F., Brogan, P., Gilmour, K.C., Schuetz, C., Simon, A., Bock, C., Hambleton, S., Vries, E de, Robinson, P.N., Gijn, M. van, and Boztug, K.

Abstract

Contains fulltext : 248208.pdf (Publisher’s version ) (Open Access), BACKGROUND: Accurate, detailed, and standardized phenotypic descriptions are essential to support diagnostic interpretation of genetic variants and to discover new diseases. The Human Phenotype Ontology (HPO), extensively used in rare disease research, provides a rich collection of vocabulary with standardized phenotypic descriptions in a hierarchical structure. However, to date, the use of HPO has not yet been widely implemented in the field of inborn errors of immunity (IEIs), mainly due to a lack of comprehensive IEI-related terms. OBJECTIVES: We sought to systematically review available terms in HPO for the depiction of IEIs, to expand HPO, yielding more comprehensive sets of terms, and to reannotate IEIs with HPO terms to provide accurate, standardized phenotypic descriptions. METHODS: We initiated a collaboration involving expert clinicians, geneticists, researchers working on IEIs, and bioinformaticians. Multiple branches of the HPO tree were restructured and extended on the basis of expert review. Our ontology-guided machine learning coupled with a 2-tier expert review was applied to reannotate defined subgroups of IEIs. RESULTS: We revised and expanded 4 main branches of the HPO tree. Here, we reannotated 73 diseases from 4 International Union of Immunological Societies-defined IEI disease subgroups with HPO terms. We achieved a 4.7-fold increase in the number of phenotypic terms per disease. Given the new HPO annotations, we demonstrated improved ability to computationally match selected IEI cases to their known diagnosis, and improved phenotype-driven disease classification. CONCLUSIONS: Our targeted expansion and reannotation presents enhanced precision of disease annotation, will enable superior HPO-based IEI characterization, and hence benefit both IEI diagnostic and research activities.

Published
2022

37. Long-term efficacy and safety of canakinumab in patients with mevalonate kinase deficiency: results from the randomised Phase 3 CLUSTER trial

Author

Jeyaratnam, J., Simon, A., Calvo, I., Constantin, T., Shcherbina, A., Hofer, M., Gattorno, M., Martini, A., Bader-Meunier, B., Vastert, B., Levy, J., Dekker, E., Benedetti, F. De, Frenkel, J., Jeyaratnam, J., Simon, A., Calvo, I., Constantin, T., Shcherbina, A., Hofer, M., Gattorno, M., Martini, A., Bader-Meunier, B., Vastert, B., Levy, J., Dekker, E., Benedetti, F. De, and Frenkel, J.

Abstract

Item does not contain fulltext, OBJECTIVES: To evaluate the long-term efficacy and safety of canakinumab in patients with mevalonate kinase deficiency during the open label extension (weeks 41-113) of the randomized controlled CLUSTER trial. METHODS: During a 72-week period, patients received open-label canakinumab 150 or 300 mg, every 4 or 8 weeks. The disease activity was evaluated every 8 weeks using physician global assessment and counting the number of flares. Concentrations of CRP and serum amyloid A protein were measured. The safety was studied by determination and classification of observed adverse events. The safety and efficacy were analysed separately in three subgroups of patients receiving a cumulative dose of less than <35 mg/kg, ≥35 to <70 mg/kg or ≥70 mg/kg. RESULTS: Of the 74 patients who started the CLUSTER study, 66 entered Epoch 4 and 65 completed it. During the 72-week period, 42 (64%) patients experienced no flares, while 13 (20%) had one flare, as compared with a median of 12 flares per year reported at baseline. Low physician global assessment scores were seen at the end of the study for all groups with >90% reporting minimal disease activity or none at all. Median CRP concentrations were consistently equal or lower than 10 mg/l, while median serum amyloid A concentrations remained only slightly above the normal range of 10 mg/l. The study showed no new or unexpected adverse events. CONCLUSION: Canakinumab proved effective to control disease activity and prevent flares in mevalonate kinase deficiency during the 72-week study period. No new safety concerns were reported. TRIAL REGISTRATION: NCT02059291. https://clinicaltrials.gov.

Published
2022

38. A descriptive study of IgG4-related disease in children and young adults

Author

de Sainte Marie, B., Ebbo, M., Grados, A., Rebours, V., Reumaux, H., Briantais, A., Urbina, D., Cury, J., Morel, N., Lhote, F., Rohmer, B., Lazaro, E., Agbo-Kpati, K.P., Deroux, A., Domont, F., Delacroix, I., Lavigne, C., Perlat, A., Kahn, J.E., Godeau, B., Hamidou, M., Launay, D., Bader-Meunier, B., and Schleinitz, N.

Published
2022
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39. POS1295 SYSTEMIC AND AUTO-INFLAMMATORY DISEASES OF PEDIATRIC ONSET: THE EXPERIENCE OF THE FRENCH OVERSEAS DEPARTMENTS OF AMERICA

Author

Felix, A., primary, Delion, F., additional, Coignard, E., additional, Martin, E., additional, Mohamed Sahnoun, M., additional, Hospice, C., additional, Cuadro Alvarez, E., additional, Elenga, N., additional, Dramé, M., additional, Bader-Meunier, B., additional, Deligny, C., additional, and Hatchuel, Y., additional

Published
2022
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40. POS1405 LESS THAN 50% FEMALES WITH CHRONIC RHEUMATIC INFLAMMATORY DISEASES CONTINUE A DMARD DURING PREGNANCY: A DESCRIPTIVE ANALYSIS OF THE NATIONAL FRENCH SOCIAL SECURITY DATABASE

Author

Moltó, A., primary, Ajrouche, A., additional, Tran, D., additional, Roux, B., additional, Costedoat-Chalumeau, N., additional, Elefant, E., additional, Tsatsaris, V., additional, Fresson, J., additional, Bader-Meunier, B., additional, Fautrel, B., additional, and Tubach, F., additional

Published
2022
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41. AB1391 19% PATIENTS WITH CHRONIC RHEUMATIC INFLAMMATORY DISEASES PRESENT AN UNFAVORABLE PREGNANCY OUTCOME: A DESCRIPTIVE ANALYSIS OF THE NATIONAL FRENCH SOCIAL SECURITY DATABASE

Author

Moltó, A., primary, Ajrouche, A., additional, Tran, D., additional, Roux, B., additional, Costedoat-Chalumeau, N., additional, Elefant, E., additional, Tsatsaris, V., additional, Fresson, J., additional, Bader-Meunier, B., additional, Fautrel, B., additional, and Tubach, F., additional

Published
2022
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42. OP0220 CRITERIA ASSOCIATED WITH TREATMENT DECISIONS IN JUVENILE IDIOPATHIC ARTHRITIS WITH A FOCUS ON ULTRASONOGRAPHY: RESULTS FROM THE JIRECHO COHORT.

Author

Mahmoud, S., primary, Jousse-Joulin, S., additional, Saraux, A., additional, Dusser, P., additional, Borocco, C., additional, Galeotti, C., additional, Von Scheven, A., additional, Hofer, M., additional, Bader-Meunier, B., additional, Aeschlimann, F., additional, Breton, S., additional, Sparsa, L., additional, Aurélia, C., additional, Mouterde, G., additional, Rossi-Semerano, L., additional, and Devauchelle-Pensec, V., additional

Published
2022
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44. 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

45. The association of Greig syndrome and mastocytosis reveals the involvement of the hedgehog pathway in advanced mastocytosis

Author

Polivka, L., primary, Parietti, V., additional, Bruneau, J., additional, Soucie, E., additional, Madrange, M., additional, Bayard, E., additional, Rignault, R., additional, Canioni, D., additional, Fraitag, S., additional, Lhermitte, L., additional, Feroul, M., additional, Tissandier, M., additional, Rossignol, J., additional, Frenzel, L., additional, Cagnard, N., additional, Meni, C., additional, Bouktit, H., additional, Collange, A.-F., additional, Gougoula, C., additional, Parisot, M., additional, Bader-Meunier, B., additional, Livideanu, C., additional, Laurent, C., additional, Arock, M., additional, Hadj-Rabia, S., additional, Rüther, U., additional, Dubreuil, P., additional, Bodemer, C., additional, Hermine, O., additional, and Maouche-Chrétien, L., additional

Published
2021
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46. L’échographie ostéo-articulaire est-elle associée au changement thérapeutique chez les patients atteints d’arthrite juvénile idiopathique : résultats de la cohorte JIRECHO

Author

Mahmoud, S., primary, Jousse Joulin, S., additional, Saraux, A., additional, Koné-Paut, I., additional, Hofer, M., additional, Von Scheven-Gète, A., additional, Breton, S., additional, Bader-Meunier, B., additional, Sparsa, L., additional, Carbasse, A., additional, Mouterde, G., additional, Cohen, J.D., additional, Rossi-Semerano, L., additional, and Devauchelle Pensec, V., additional

Published
2021
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47. Cohorte française du syndrome COPA

Author

David, C., primary, Bader-Meunier, B., additional, Belot, A., additional, El Khalifi-Boulisfane, S., additional, Thumerelle, C., additional, Wislez, M., additional, Crow, Y., additional, Nathan, N., additional, and Frémond, M.L., additional

Published
2021
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48. Caractéristiques cliniques de la Covid-19 chez les enfants et adolescents atteints de maladies rhumatismales et inflammatoires : données de la cohorte française RMD COVID-19 de 95 patients

Author

Mammou Mraghni, S., primary, Drumez, E., additional, Aeschlimann, F., additional, Pajot-Audouin, C., additional, Bader-Meunier, B., additional, Carbasse, A., additional, Clet, J., additional, Deslandre, C., additional, Duquesne, A., additional, Dusser, P., additional, Guillaume-Czitrom, S., additional, Hentgen, V., additional, Koné-Paut, I., additional, Melki, I., additional, Quartier, P., additional, Hachulla, E., additional, and Belot, A., additional

Published
2021
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49. Seulement 50 % des femmes atteintes de rhumatisme inflammatoire chronique continuent leur traitement de fond pendant la grossesse : une analyse descriptive du Système national de données de santé

Author

Molto, A., primary, Ajrouche, A., additional, Tran, M.H.T.D., additional, Roux, B., additional, Costedoat-Chalumeau, N., additional, Elefant, E., additional, Tsatsaris, V., additional, Fresson, J., additional, Bader-Meunier, B., additional, Fautrel, B., additional, and Tubach, F., additional

Published
2021
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