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2. OP0198 COMPARATIVE IMMUNOMICS OF CHILDHOOD AND ADULT-ONSET SYSTEMIC LUPUS ERYTHEMATOSUS REVEALS DISTINCT MECHANISTIC DIFFERENCES WITH THERAGNOSTIC APPLICATIONS

Author

Yeo, J. G., primary, Nay Yaung, K., additional, Law Hui Nee, A., additional, Wasser, M., additional, Poh, S. L., additional, Teh, K. L., additional, Das, L., additional, Book, Y. X., additional, Huang, C., additional, Arkachaisri, T., additional, Thumboo, J., additional, Low, A., additional, and Albani, S., additional

Published
2024
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3. POS0156 CD4+HLADR+ T CELLS WITH AUTOIMMUNE REACTIVITY THAT RESIST REGULATORY CONTROL AND PERPETUATE DISEASE INFLAMMATION IN JUVENILE IDIOPATHIC ARTHRITIS

Author

Leong, J. Y., primary, Kumar, P., additional, Tay, S. H., additional, Mijnheer, G., additional, Yeo, J. G., additional, Hazirah, S., additional, Lim, A. J. M., additional, Chua, C., additional, Chen, P., additional, Consolaro, A., additional, Gattorno, M., additional, Arkachaisri, T., additional, Van Wijk, F., additional, Martini, A., additional, and Albani, S., additional

Published
2024
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4. OP0239 DECODING THE COMPLEXITY OF CHILDHOOD-ONSET SLE (cSLE) USING A HIGH-DIMENSIONAL APPROACH: ENHANCING THERAGNOSIS WITH AN IMMUNOLOGICAL-BASED DIAGNOSTIC MODEL REVEALING MECHANISTIC INSIGHTS

Author

Nay Yaung, K., primary, Yeo, J. G., additional, Wasser, M., additional, Poh, S. L., additional, Teh, K. L., additional, Das, L., additional, Book, Y. X., additional, Khoo, N. K. H., additional, Tay, Z. M. Y., additional, Cheong, R., additional, Wong, J. J. M., additional, Arkachaisri, T., additional, and Albani, S., additional

Published
2024
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5. AB1116 AGE-ASSOCIATED INCREASE IN CYTOTOXIC T LYMPHOCYTE-ASSOCIATED PROTEIN 4 (CTLA-4) EXPRESSION ON EFFECTOR T CELLS AND ITS ROLE IN SYSTEMIC LUPUS ERYTHEMATOSUS

Author

Cheong, R., primary, Wasser, M., additional, Nay Yaung, K., additional, Law Hui Nee, A., additional, Khoo, N. K. H., additional, Kumar, P., additional, Arkachaisri, T., additional, Poh, S. L., additional, Leong, J. Y., additional, Thumboo, J., additional, Low, A., additional, Albani, S., additional, and Yeo, J. G., additional

Published
2024
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6. AB1461 ASSOCIATION OF FLARES OF CONNECTIVE TISSUE DISEASE AND SYSTEMIC LUPUS ERYTHEMATOSUS WITH DRUG USE FOLLOWING COVID-19 MRNA VACCINATION: RESULTS FROM THE CORONAVIRUS NATIONAL VACCINE REGISTRY FOR IMMUNE DISEASES SINGAPORE (CONVIN-SING)

Author

Dhanasekaran, P., primary, Mak, A., additional, MA, M., additional, Chan, Y. H., additional, Santosa, A., additional, Cheung, P., additional, Fong, W., additional, Angkodjojo, S., additional, Xu, C., additional, Kong, K. O., additional, Arkachaisri, T., additional, Phang, K. F., additional, Tan, T. C., additional, Sriranganathan, M., additional, Tay, S. H., additional, and Lahiri, M., additional

Published
2024
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9. AB0007 DYNAMIC INTERPLAY OF FUNCTIONALLY DISCORDANT CD4+HLA-DR+ SUBSETS WITH A COMMON PATHOLOGICAL ONTOGENIC ORIGIN IN HUMAN ARTHRITIS

Author

Leong, J. Y., primary, Kumar, P., additional, Lim, A. J. M., additional, Tay, S. H., additional, Hazirah, S., additional, Yeo, J. G., additional, Chua, C., additional, Chen, P., additional, Lajam, A. M., additional, Consolaro, A., additional, Gattorno, M., additional, Ng, C. T., additional, Arkachaisri, T., additional, Martini, A., additional, and Albani, S., additional

Published
2023
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16. POS0200 POST-mRNA VACCINE FLARES IN AUTOIMMUNE INFLAMMATORY RHEUMATIC DISEASES: INTERIM RESULTS FROM THE CORONAVIRUS NATIONAL VACCINE REGISTRY FOR IMMUNE DISEASES SINGAPORE (CONVIN-SING)

Author

Ma, M., primary, Santosa, A., additional, Kong, K. O., additional, Xu, C., additional, Xiang, J. T. G., additional, Teng, G. G., additional, Mak, A., additional, Tay, S. H., additional, Ng, V. W. W., additional, Koh, J. Z. E., additional, Fong, W., additional, Chew, L. C., additional, Low, A., additional, Law, A., additional, Poh, Y. J., additional, Yeo, S. I., additional, Leung, Y. Y., additional, Goh, W. R., additional, Yu, C. T., additional, Roslan, N. E., additional, Angkodjojo, S., additional, Phang, K. F., additional, Arkachaisri, T., additional, Sriranganathan, M., additional, Tan, T. C., additional, Cheung, P., additional, and Lahiri, M., additional

Published
2022
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18. Prevalence of autoimmune diseases in parents of children with juvenile idiopathic arthritis: results from the international pharmachild register

Author

van Straalen, J.W., de Roock, S., Stanevicha, V., Lamot, Lovro, Estmann Christensen, A., Rumba- Rozenfelde, I., Lazar, C., Uziel, Y., Arkachaisri, T., Wulffraat, N.M., Ruperto, N., Swart, J.F., and on behalf of Pediatric Rheumatology INternational Trials Organisation (PRINTO)

Subjects
autoimmune disease, juvenile idiopathic arthritis, pediatrics
Abstract

Introduction: Autoimmune diseases (ADs) tend to cluster within families. However, little is known about the disposition to AD among children diagnosed with juvenile idiopathic arthritis (JIA). Objectives: To determine the prevalence of and factors associated with ADs in parents of children with JIA. Methods: Data were collected from the international Pharmachild register. Characteristics were compared between patients with and without a family history of AD. Prevalence rates of familial ADs were calculated and compared with general population prevalence rates as reported in the literature. Results: The most common ADs in parents of the included JIA patients (n = 8, 673) are listed in Table 1. For several ADs, prevalence was higher compared to reported numbers in the general population. Clinical Juvenile Arthritis Disease Activity Scores (cJADAS) at study entry and last follow-up were not significantly different between patients with (n = 1, 231) and without a family history of AD (n = 7, 442) (P = 0.61 and P = 0.82, respectively). Factors associated with a family history of AD were older age at JIA onset (P < 0.01), Scandinavian residence (P < 0.01), enthesitis-related arthritis, psoriatic arthritis and undifferentiated arthritis (P < 0.01), ANA positivity (P = 0.03) and HLA-B27 positivity (P < 0.01). Conclusion: Several ADs have an increased prevalence in parents of children with JIA. Familial AD therefore proves to be a risk factor for JIA development and certain diseases should not be overlooked during family health history at the diagnosis stage. A family history of AD is associated with the JIA subtype but does not influence the severity or disease course

Published
2022

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. 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

20. Opportunistic infections in immunosuppressed patients with juvenile idiopathic arthritis: analysis by the Pharmachild Safety Adjudication Committee

Author

Giancane G1, Swart JF2, Castagnola E3, Groll AH4, Horneff G5, 6, Huppertz HI7, Lovell DJ8, Wolfs T2, Herlin T9, Dolezalova P10, Sanner H11, Susic G13, Sztajnbok F14, Maritsi D15, Constantin T16, Vargova V17, Sawhney S18, Rygg M19, K Oliveira S21, Cattalini M22, Bovis F1, Bagnasco F1, Pistorio A23, Martini A24, Wulffraat N2, Ruperto N25, Paediatric Rheumatology International Trials Organisation (PRINTO). Cuttica R, Garay SM, Brunner J, Emminger W, Appenzeller S, Len C, Saad Magalhaes C, Telcharova-Mihaylovska A, Harjacek M, Jelusic M, Estmann A, Nielsen S, Herrera Mora C, Gervais E, Koné-Paut I, Quartier P, Foeldvari I, Horneff G, Lutz T, Minden K, Tzaribachev N, Trachana M, Tsitsami E, Vougiouka O, Orban I, Harel L, Hashkes P, Uziel Y, Cimaz R, Civino A, Consolini R, D'Angelo G, De Benedetti F, Filocamo G, Fueri E, Gallizzi R, Maggio MC, Magnolia MG, Miniaci A, Montin D, Olivieri A. N., Pastore S, Rigante D, Zulian F, Rumba-Rozenfelde I, Stanevicha V, Panaviene V, Rodriguez Lozano AL, Rubio-Perez N, Vega Cornejo G, Hoppenreijs E, Kamphuis S, Flato B, Nordal EB, Abdwani R, Miraval T, Paz Gastanaga ME, Smolewska E, Ailioaie C, Cochino AV, Laday M, Lazar C, Alexeeva E, Chasnyk V, Keltsev V, Suwairi WMS, Vijatov-Djuric G, Vojinovic J, Arkachaisri T, Koskova E, Avcin T, Ally M, Van Rensburg CJ, Louw I, Lopez JA, Boteanu AL, Calvo Penades I, De Inocencio J, Mesa-Del-Castillo P, Moreno E, Remesal A, Hofer M, Gok F, Ozen S, Ramanan A, Pallotti C, Villa L., Giancane, G1, Swart, Jf2, Castagnola, E3, Groll, Ah4, Horneff, G5, Huppertz, Hi7, Lovell, Dj8, Wolfs, T2, Herlin, T9, Dolezalova, P10, Sanner, H11, Susic, G13, Sztajnbok, F14, Maritsi, D15, Constantin, T16, Vargova, V17, Sawhney, S18, Rygg, M19, K Oliveira, S21, Cattalini, M22, Bovis, F1, Bagnasco, F1, Pistorio, A23, Martini, A24, Wulffraat, N2, Ruperto, N25, Paediatric Rheumatology International Trials Organisation (PRINTO)., Cuttica R, Garay, Sm, Brunner, J, Emminger, W, Appenzeller, S, Len, C, Saad Magalhaes, C, Telcharova-Mihaylovska, A, Harjacek, M, Jelusic, M, Estmann, A, Nielsen, S, Herrera Mora, C, Gervais, E, Koné-Paut, I, Quartier, P, Foeldvari, I, Horneff, G, Lutz, T, Minden, K, Tzaribachev, N, Trachana, M, Tsitsami, E, Vougiouka, O, Orban, I, Harel, L, Hashkes, P, Uziel, Y, Cimaz, R, Civino, A, Consolini, R, D'Angelo, G, De Benedetti, F, Filocamo, G, Fueri, E, Gallizzi, R, Maggio, Mc, Magnolia, Mg, Miniaci, A, Montin, D, Olivieri, A. N., Pastore, S, Rigante, D, Zulian, F, Rumba-Rozenfelde, I, Stanevicha, V, Panaviene, V, Rodriguez Lozano, Al, Rubio-Perez, N, Vega Cornejo, G, Hoppenreijs, E, Kamphuis, S, Flato, B, Nordal, Eb, Abdwani, R, Miraval, T, Paz Gastanaga, Me, Smolewska, E, Ailioaie, C, Cochino, Av, Laday, M, Lazar, C, Alexeeva, E, Chasnyk, V, Keltsev, V, Suwairi, Wm, Vijatov-Djuric, G, Vojinovic, J, Arkachaisri, T, Koskova, E, Avcin, T, Ally, M, Van Rensburg, Cj, Louw, I, Lopez, Ja, Boteanu, Al, Calvo Penades, I, De Inocencio, J, Mesa-Del-Castillo, P, Moreno, E, Remesal, A, Hofer, M, Gok, F, Ozen, S, Ramanan, A, Pallotti, C, Villa, L., Giancane, Gabriella, Swart, Joost F, Castagnola, Elio, Groll, Andreas H, Horneff, Gerd, Huppertz, Hans-Iko, Lovell, Daniel J, Wolfs, Tom, Herlin, Troel, Dolezalova, Pavla, Sanner, Helga, Susic, Gordana, Sztajnbok, Flavio, Maritsi, Despoina, Constantin, Tama, Vargova, Veronika, Sawhney, Sujata, Rygg, Marite, K Oliveira, Sheila, Cattalini, Marco, Bovis, Francesca, Bagnasco, Francesca, Pistorio, Angela, Martini, Alberto, Wulffraat, Nico, Ruperto, Nicolino, Cuttica, Ruben, Garay, Stella Mari, Brunner, Jurgen, Emminger, Wolfgang, Appenzeller, Simone, Len, Claudio, Saad Magalhaes, Claudia, Telcharova-Mihaylovska, Albena, Harjacek, Miroslav, Jelusic, Marija, Estmann, Anne, Nielsen, Susan, Herrera Mora, Cristina, Gervais, Elisabeth, Koné-Paut, Isabelle, Quartier, Pierre, Foeldvari, Ivan, Lutz, Thoma, Minden, Kirsten, Tzaribachev, Nikolay, Trachana, Maria, Tsitsami, Elena, Vougiouka, Olga, Orban, Ilonka, Harel, Liora, Hashkes, Philip, Uziel, Yosef, Cimaz, Rolando, Civino, Adele, Consolini, Rita, D'Angelo, Gianfranco, De Benedetti, Fabrizio, Filocamo, Giovanni, Fueri, Elena, Gallizzi, Romina, Maggio, Maria Cristina, Magnolia, Maria Greca, Miniaci, Angela, Montin, Davide, Olivieri, Alma Nunzia, Pastore, Serena, Rigante, Donato, Zulian, Francesco, Rumba-Rozenfelde, Ingrida, Stanevicha, Valda, Panaviene, Violeta, Rodriguez Lozano, Ana Luisa, Rubio-Perez, Nadina, Vega Cornejo, Gabriel, Hoppenreijs, Esther, Kamphuis, Sylvia, Flato, Berit, Nordal, Ellen Berit, Abdwani, Reem, Miraval, Tatiana, Paz Gastanaga, Maria Eliana, Smolewska, Elzbieta, Ailioaie, Constantin, Cochino, Alexis-Virgil, Laday, Matilda, Lazar, Calin, Alexeeva, Ekaterina, Chasnyk, Vyacheslav, Keltsev, Vladimir, Suwairi, Wafaa Mohammed Saad, Vijatov-Djuric, Gordana, Vojinovic, Jelena, Arkachaisri, Thaschawee, Koskova, Elena, Avcin, Tadej, Ally, Mahmood, Van Rensburg, Christa Janse, Louw, Ingrid, Lopez, Jordi Anton, Boteanu, Alina Lucica, Calvo Penades, Inmaculada, De Inocencio, Jaime, Mesa-Del-Castillo, Pablo, Moreno, Estefania, Remesal, Agustin, Hofer, Michael, Gok, Faysal, Ozen, Seza, Ramanan, Athimalaipet, Pallotti, Chiara, Villa, Luca, and Pediatrics

Subjects
Male, lcsh:Diseases of the musculoskeletal system, Biologic, Paediatrics: 760 [VDP], Artritis infecciosa, MedDRA, Infants malalts, Arthritis, Severity of Illness Index, Hospital patients, Cohort Studies, Pharmacovigilance, 0302 clinical medicine, 030212 general & internal medicine, Registries, Child, Biologics, Immunosuppressive therapy, Infections, Juvenile idiopathic arthritis, Opportunistic, biologics, immunosuppressive therapy, infections, juvenile idiopathic arthritis, opportunistic, Barneleddgikt, 3. Good health, Settore MED/38 - PEDIATRIA GENERALE E SPECIALISTICA, Antirheumatic Agents, Child, Preschool, Cohort, Pediatric Infectious Disease, Female, Infection, Research Article, medicine.medical_specialty, Tuberculosis, juvenil idiopathic arthritis, Opportunistic Infections, Herpes Zoster, 03 medical and health sciences, Immunocompromised Host, Juvenile idiopathic arthriti, Internal medicine, medicine, Humans, book, 030203 arthritis & rheumatology, Malalts hospitalitzats, Immunosupressió, business.industry, Sick children, medicine.disease, Rheumatology, Arthritis, Juvenile, Infectious arthritis, Pediatri: 760 [VDP], Orthopedic surgery, Opportunistiske infeksjoner, book.journal, lcsh:RC925-935, business, Infeccions oportunistes, Immunosuppression
Abstract

Background: To derive a list of opportunistic infections (OI) through the analysis of the juvenile idiopathic arthritis (JIA) patients in the Pharmachild registry by an independent Safety Adjudication Committee (SAC). Methods: The SAC (3 pediatric rheumatologists and 2 pediatric infectious disease specialists) elaborated and approved by consensus a provisional list of OI for use in JIA. Through a 5 step-procedure, all the severe and serious infections, classified as per MedDRA dictionary and retrieved in the Pharmachild registry, were evaluated by the SAC by answering six questions and adjudicated with the agreement of 3/5 specialists. A final evidence-based list of OI resulted by matching the adjudicated infections with the provisional list of OI. Results: A total of 772 infectious events in 572 eligible patients, of which 335 serious/severe/very severe non-OI and 437 OI (any intensity/severity), according to the provisional list, were retrieved. Six hundred eighty-two of 772 (88.3%) were adjudicated as infections, of them 603/682 (88.4%) as common and 119/682 (17.4%) as OI by the SAC. Matching these 119 opportunistic events with the provisional list, 106 were confirmed by the SAC as OI, and among them infections by herpes viruses were the most frequent (68%), followed by tuberculosis (27.4%). The remaining events were divided in the groups of non-OI and possible/patient and/or pathogen-related OI. Conclusions: We found a significant number of OI in JIA patients on immunosuppressive therapy. The proposed list of OI, created by consensus and validated in the Pharmachild cohort, could facilitate comparison among future pharmacovigilance studies. © The Author(s). 2020 Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.

Published
2020

22. A clinical prediction model for estimating the risk of developing uveitis in patients with juvenile idiopathic arthritis

Author

Straalen, J.W. van, Giancane, G., Amazrhar, Y., Tzaribachev, N., Lazar, C., Uziel, Y., Telcharova-Mihaylovska, A., Len, C.A., Miniaci, A., Boteanu, A.L., Filocamo, G., Mastri, M.V., Arkachaisri, T., Magnolia, M.G., Hoppenreijs, E.P., Roock, S. de, Wulffraat, N.M., Ruperto, N., Swart, J.F., Straalen, J.W. van, Giancane, G., Amazrhar, Y., Tzaribachev, N., Lazar, C., Uziel, Y., Telcharova-Mihaylovska, A., Len, C.A., Miniaci, A., Boteanu, A.L., Filocamo, G., Mastri, M.V., Arkachaisri, T., Magnolia, M.G., Hoppenreijs, E.P., Roock, S. de, Wulffraat, N.M., Ruperto, N., and Swart, J.F.

Abstract

Item does not contain fulltext, OBJECTIVE: To build a prediction model for uveitis in children with JIA for use in current clinical practice. METHODS: Data from the international observational Pharmachild registry were used. Adjusted risk factors as well as predictors for JIA-associated uveitis (JIA-U) were determined using multivariable logistic regression models. The prediction model was selected based on the Akaike information criterion. Bootstrap resampling was used to adjust the final prediction model for optimism. RESULTS: JIA-U occurred in 1102 of 5529 JIA patients (19.9%). The majority of patients that developed JIA-U were female (74.1%), ANA positive (66.0%) and had oligoarthritis (59.9%). JIA-U was rarely seen in patients with systemic arthritis (0.5%) and RF positive polyarthritis (0.2%). Independent risk factors for JIA-U were ANA positivity [odds ratio (OR): 1.88 (95% CI: 1.54, 2.30)] and HLA-B27 positivity [OR: 1.48 (95% CI: 1.12, 1.95)] while older age at JIA onset was an independent protective factor [OR: 0.84 (9%% CI: 0.81, 0.87)]. On multivariable analysis, the combination of age at JIA onset [OR: 0.84 (95% CI: 0.82, 0.86)], JIA category and ANA positivity [OR: 2.02 (95% CI: 1.73, 2.36)] had the highest discriminative power among the prediction models considered (optimism-adjusted area under the receiver operating characteristic curve = 0.75). CONCLUSION: We developed an easy to read model for individual patients with JIA to inform patients/parents on the probability of developing uveitis.

Published
2021

23. Biomarker profiles of endothelial activation and dysfunction in rare systemic autoimmune diseases: implications for cardiovascular risk

Author

Wienke, J., Mertens, J.S., Garcia, S., Lim, J., Wijngaarde, Camiel A., Yeo, J.G., Meyer, A, Hoogen, L.L. van den, Tekstra, J., Hoogendijk, J.E., Otten, H.G., Fritsch-Stork, R.D.E., Jager, W. de, Seyger, M.M.B., Thurlings, R.M., Jong, E.M.G.J. de, Kooi, A.J. van der, Pol, W.Ludo van der, Arkachaisri, T., Radstake, T.R., Royen-Kerkhof, A. Van, Wijk, F van, Wienke, J., Mertens, J.S., Garcia, S., Lim, J., Wijngaarde, Camiel A., Yeo, J.G., Meyer, A, Hoogen, L.L. van den, Tekstra, J., Hoogendijk, J.E., Otten, H.G., Fritsch-Stork, R.D.E., Jager, W. de, Seyger, M.M.B., Thurlings, R.M., Jong, E.M.G.J. de, Kooi, A.J. van der, Pol, W.Ludo van der, Arkachaisri, T., Radstake, T.R., Royen-Kerkhof, A. Van, and Wijk, F van

Abstract

Item does not contain fulltext, OBJECTIVES: Vasculopathy is an important hallmark of systemic chronic inflammatory connective tissue diseases (CICTD) and is associated with increased cardiovascular risk. We investigated disease-specific biomarker profiles associated with endothelial dysfunction, angiogenic homeostasis and (tissue) inflammation, and their relation to disease activity in rare CICTD. METHODS: A total of 38 serum proteins associated with endothelial (dys)function and inflammation were measured by multiplex-immunoassay in treatment-naive patients with localized scleroderma (LoS, 30), eosinophilic fasciitis (EF, 8) or (juvenile) dermatomyositis (34), 119 (follow-up) samples during treatment, and 65 controls. Data were analysed by unsupervised clustering, Spearman correlations, non-parametric t test and ANOVA. RESULTS: The systemic CICTD, EF and dermatomyositis, had distinct biomarker profiles, with 'signature' markers galectin-9 (dermatomyositis) and CCL4, CCL18, CXCL9, fetuin, fibronectin, galectin-1 and TSP-1 (EF). In LoS, CCL18, CXCL9 and CXCL10 were subtly increased. Furthermore, dermatomyositis and EF shared upregulation of markers related to interferon (CCL2, CXCL10), endothelial activation (VCAM-1), inhibition of angiogenesis (angiopoietin-2, sVEGFR-1) and inflammation/leucocyte chemo-attraction (CCL19, CXCL13, IL-18, YKL-40), as well as disturbance of the Angiopoietin-Tie receptor system and VEGF-VEGFR system. These profiles were related to disease activity, and largely normalized during treatment. However, a subgroup of CICTD patients showed continued elevation of CXCL10, CXCL13, galectin-9, IL-18, TNFR2, VCAM-1, and/or YKL-40 during clinically inactive disease, possibly indicating subclinical interferon-driven inflammation and/or endothelial dysfunction. CONCLUSION: CICTD-specific biomarker profiles revealed an anti-angiogenic, interferon-driven environment during active disease, with incomplete normalization under treatment. This warrants further investigation into monitoring

Published
2021

27. Endothelial and Inflammation Biomarker Profiles at Diagnosis Reflecting Clinical Heterogeneity and Serving as a Prognostic Tool For Treatment Response in Two Independent Cohorts of Patients With Juvenile Dermatomyositis

Author

Wienke, J. (Judith), Pachman, L.M. (Lauren M.), Morgan, G.A. (Gabrielle A.), Yeo, J.G. (Joo Guan), Amoruso, M.C. (Maria C.), Hans, V. (Victoria), Kamphuis, S.S.M. (Sylvia), Hoppenreijs, E.P.A.H. (Esther), Armbrust, W. (Wineke), Berg, J.M. (Merlijn) van den, Hissink Muller, P.C.E. (Petra), Gelderman, K.A. (Kyra), Arkachaisri, T. (Thaschawee), Wijk, F. (Femke) van, van Royen-Kerkhof, A. (Annet), Wienke, J. (Judith), Pachman, L.M. (Lauren M.), Morgan, G.A. (Gabrielle A.), Yeo, J.G. (Joo Guan), Amoruso, M.C. (Maria C.), Hans, V. (Victoria), Kamphuis, S.S.M. (Sylvia), Hoppenreijs, E.P.A.H. (Esther), Armbrust, W. (Wineke), Berg, J.M. (Merlijn) van den, Hissink Muller, P.C.E. (Petra), Gelderman, K.A. (Kyra), Arkachaisri, T. (Thaschawee), Wijk, F. (Femke) van, and van Royen-Kerkhof, A. (Annet)

Abstract

Objective: Juvenile dermatomyositis (DM) is a heterogeneous systemic immune-mediated vasculopathy. This study was undertaken to 1) identify inflammation/endothelial dysfunction–related biomarker profiles reflecting disease severity at diagnosis, and 2) establish whether such biomarker profiles could be used for predicting the response to treatment in patients with juvenile DM. Methods: In total, 39 biomarkers related to activation of endothelial c

Published
2020
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28. Endothelial and Inflammation Biomarker Profiles at Diagnosis Reflecting Clinical Heterogeneity and Serving as a Prognostic Tool for Treatment Response in Two Independent Cohorts of Patients With Juvenile Dermatomyositis

Author

Wienke, J., Pachman, L.M., Morgan, Gabrielle A., Yeo, J.G., Amoruso, M.C., Hans, V., Kamphuis, S.S., Hoppenreijs, E.P.A.H., Armbrust, W., Berg, J.M. ten, Muller, P.C., Gelderman, K.A., Arkachaisri, T., Wijk, F. van, Royen-Kerkhof, A. Van, Wienke, J., Pachman, L.M., Morgan, Gabrielle A., Yeo, J.G., Amoruso, M.C., Hans, V., Kamphuis, S.S., Hoppenreijs, E.P.A.H., Armbrust, W., Berg, J.M. ten, Muller, P.C., Gelderman, K.A., Arkachaisri, T., Wijk, F. van, and Royen-Kerkhof, A. Van

Abstract

Contains fulltext : 220769.pdf (Publisher’s version ) (Open Access), OBJECTIVE: Juvenile dermatomyositis (DM) is a heterogeneous systemic immune-mediated vasculopathy. This study was undertaken to 1) identify inflammation/endothelial dysfunction-related biomarker profiles reflecting disease severity at diagnosis, and 2) establish whether such biomarker profiles could be used for predicting the response to treatment in patients with juvenile DM. METHODS: In total, 39 biomarkers related to activation of endothelial cells, endothelial dysfunction, and inflammation were measured using multiplex technology in serum samples from treatment-naive patients with juvenile DM from 2 independent cohorts (n = 30 and n = 29). Data were analyzed by unsupervised hierarchical clustering, nonparametric tests with correction for multiple comparisons, and Kaplan-Meier tests with Cox proportional hazards models for analysis of treatment duration. Myositis-specific antibodies (MSAs) were measured in the patients' serum using line blot assays. RESULTS: Severe vasculopathy in patients with juvenile DM was associated with low serum levels of intercellular adhesion molecule 1 (Spearman's rho [rs ] = 0.465, P = 0.0111) and high serum levels of endoglin (rs = -0.67, P < 0.0001). In the discovery cohort, unsupervised hierarchical clustering analysis of the biomarker profiles yielded 2 distinct patient clusters, of which the smaller cluster (cluster 1; n = 8) exhibited high serum levels of CXCL13, CCL19, galectin-9, CXCL10, tumor necrosis factor receptor type II (TNFRII), and galectin-1 (false discovery rate <0.0001), and this cluster had greater severity of muscle disease and global disease activity (each P < 0.05 versus cluster 2). In the validation cohort, correlations between the serum levels of galectin-9, CXCL10, TNFRII, and galectin-1 and the severity of global disease activity were confirmed (rs = 0.40-0.52, P < 0.05). Stratification of patients according to the 4 confirmed biomarkers identified a cluster of patients with severe symptoms (comprising 64.7%

Published
2020

29. Endothelial and Inflammation Biomarker Profiles at Diagnosis Reflecting Clinical Heterogeneity and Serving as a Prognostic Tool For Treatment Response in Two Independent Cohorts of Patients With Juvenile Dermatomyositis

Author

Wienke, J, Pachman, LM, Morgan, GA, Yeo, JG, Amoruso, MC, Hans, V, Kamphuis, Sylvia, Hoppenreijs, E, Armbrust, W, Berg, JM, Muller, P, Gelderman, KA, Arkachaisri, T, van Wijk, F, van Royen-kerkhof, A, Wienke, J, Pachman, LM, Morgan, GA, Yeo, JG, Amoruso, MC, Hans, V, Kamphuis, Sylvia, Hoppenreijs, E, Armbrust, W, Berg, JM, Muller, P, Gelderman, KA, Arkachaisri, T, van Wijk, F, and van Royen-kerkhof, A

Published
2020

30. Opportunistic infections in immunosuppressed patients with juvenile idiopathic arthritis: analysis by the Pharmachild Safety Adjudication Committee

Author

Giancane, G, Swart, Jf, Castagnola, E, Groll, Ah, Horneff, G, Huppertz, Hi, Lovell, Dj, Wolfs, T, Herlin, T, Dolezalova, P, Sanner, H, Susic, G, Sztajnbok, F, Maritsi, D, Constantin, T, Vargova, V, Sawhney, S, Rygg, M, K Oliveira, S, Cattalini, M, Bovis, F, Bagnasco, F, Pistorio, A, Martini, A, Wulffraat, N, Ruperto, N, Cuttica, R, Garay, Sm, Brunner, J, Emminger, W, Appenzeller, S, Len, C, Saad Magalhaes, C, Telcharova-Mihaylovska, A, Harjacek, M, Jelusic, M, Estmann, A, Nielsen, S, Herrera Mora, C, Gervais, E, Koné-Paut, I, Quartier, P, Foeldvari, I, Lutz, T, Minden, K, Tzaribachev, N, Trachana, M, Tsitsami, E, Vougiouka, O, Orban, I, Harel, L, Hashkes, P, Uziel, Y, Cimaz, R, Civino, A, Consolini, R, D'Angelo, G, De Benedetti, F, Filocamo, G, Fueri, E, Gallizzi, R, Maggio, Mc, Magnolia, Mg, Miniaci, A, Montin, D, Olivieri, An, Pastore, S, Rigante, Donato, Zulian, F, Rumba-Rozenfelde, I, Stanevicha, V, Panaviene, V, Rodriguez Lozano, Al, Rubio-Perez, N, Vega Cornejo, G, Hoppenreijs, E, Kamphuis, S, Flato, B, Nordal, Eb, Abdwani, R, Miraval, T, Paz Gastanaga, Me, Smolewska, E, Ailioaie, C, Cochino, Av, Laday, M, Lazar, C, Alexeeva, E, Chasnyk, V, Keltsev, V, Suwairi, Wm, Vijatov-Djuric, G, Vojinovic, J, Arkachaisri, T, Koskova, E, Avcin, T, Ally, M, Van Rensburg, Cj, Louw, I, Lopez, Ja, Boteanu, Al, Calvo Penades, I, De Inocencio, J, Mesa-Del-Castillo, P, Moreno, E, Remesal, A, Hofer, M, Gok, F, Ozen, S, Ramanan, A, Pallotti, C, Villa, L &amp, Paediatric Rheumatology International Trials Organisation, (PRINTO), Rigante D (ORCID:0000-0001-7032-7779), Giancane, G, Swart, Jf, Castagnola, E, Groll, Ah, Horneff, G, Huppertz, Hi, Lovell, Dj, Wolfs, T, Herlin, T, Dolezalova, P, Sanner, H, Susic, G, Sztajnbok, F, Maritsi, D, Constantin, T, Vargova, V, Sawhney, S, Rygg, M, K Oliveira, S, Cattalini, M, Bovis, F, Bagnasco, F, Pistorio, A, Martini, A, Wulffraat, N, Ruperto, N, Cuttica, R, Garay, Sm, Brunner, J, Emminger, W, Appenzeller, S, Len, C, Saad Magalhaes, C, Telcharova-Mihaylovska, A, Harjacek, M, Jelusic, M, Estmann, A, Nielsen, S, Herrera Mora, C, Gervais, E, Koné-Paut, I, Quartier, P, Foeldvari, I, Lutz, T, Minden, K, Tzaribachev, N, Trachana, M, Tsitsami, E, Vougiouka, O, Orban, I, Harel, L, Hashkes, P, Uziel, Y, Cimaz, R, Civino, A, Consolini, R, D'Angelo, G, De Benedetti, F, Filocamo, G, Fueri, E, Gallizzi, R, Maggio, Mc, Magnolia, Mg, Miniaci, A, Montin, D, Olivieri, An, Pastore, S, Rigante, Donato, Zulian, F, Rumba-Rozenfelde, I, Stanevicha, V, Panaviene, V, Rodriguez Lozano, Al, Rubio-Perez, N, Vega Cornejo, G, Hoppenreijs, E, Kamphuis, S, Flato, B, Nordal, Eb, Abdwani, R, Miraval, T, Paz Gastanaga, Me, Smolewska, E, Ailioaie, C, Cochino, Av, Laday, M, Lazar, C, Alexeeva, E, Chasnyk, V, Keltsev, V, Suwairi, Wm, Vijatov-Djuric, G, Vojinovic, J, Arkachaisri, T, Koskova, E, Avcin, T, Ally, M, Van Rensburg, Cj, Louw, I, Lopez, Ja, Boteanu, Al, Calvo Penades, I, De Inocencio, J, Mesa-Del-Castillo, P, Moreno, E, Remesal, A, Hofer, M, Gok, F, Ozen, S, Ramanan, A, Pallotti, C, Villa, L &amp, Paediatric Rheumatology International Trials Organisation, (PRINTO), and Rigante D (ORCID:0000-0001-7032-7779)

Abstract

BACKGROUND: To derive a list of opportunistic infections (OI) through the analysis of the juvenile idiopathic arthritis (JIA) patients in the Pharmachild registry by an independent Safety Adjudication Committee (SAC). METHODS: The SAC (3 pediatric rheumatologists and 2 pediatric infectious disease specialists) elaborated and approved by consensus a provisional list of OI for use in JIA. Through a 5 step-procedure, all the severe and serious infections, classified as per MedDRA dictionary and retrieved in the Pharmachild registry, were evaluated by the SAC by answering six questions and adjudicated with the agreement of 3/5 specialists. A final evidence-based list of OI resulted by matching the adjudicated infections with the provisional list of OI. RESULTS: A total of 772 infectious events in 572 eligible patients, of which 335 serious/severe/very severe non-OI and 437 OI (any intensity/severity), according to the provisional list, were retrieved. Six hundred eighty-two of 772 (88.3%) were adjudicated as infections, of them 603/682 (88.4%) as common and 119/682 (17.4%) as OI by the SAC. Matching these 119 opportunistic events with the provisional list, 106 were confirmed by the SAC as OI, and among them infections by herpes viruses were the most frequent (68%), followed by tuberculosis (27.4%). The remaining events were divided in the groups of non-OI and possible/patient and/or pathogen-related OI. CONCLUSIONS: We found a significant number of OI in JIA patients on immunosuppressive therapy. The proposed list of OI, created by consensus and validated in the Pharmachild cohort, could facilitate comparison among future pharmacovigilance studies.

Published
2020

34. AB0050 EXTENDED POLYDIMENSIONAL IMMUNOME CHARACTERISATION (EPIC) PLATFORM AS A TOOL FOR TRANSLATIONAL RESEARCH

Author

Yeo, J. G., primary, Wasser, M., additional, Kumar, P., additional, Pan, L., additional, Poh, S. L., additional, Ally, F., additional, Arkachaisri, T., additional, Lim, A. J. M., additional, Leong, J. Y., additional, Yeo, K. T., additional, Lai, L., additional, Lee, E. S. C., additional, Chua, C., additional, Paleja, B., additional, Tang, S. P., additional, Ng, S. K., additional, Tan, A. Y. J., additional, Lee, S. Y., additional, Ginhoux, F., additional, Ng, T. P., additional, Larbi, A., additional, and Albani, S., additional

Published
2020
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36. THU0047 THE SYNOVIUM REWIRES AN IMMUNOLOGICAL RHEOSTAT THAT DEFINES TWO FUNCTIONALLY DISPARATE PATHOGENIC CD4+HLA-DR+ SUBSETS IN HUMAN ARTHRITIS

Author

Leong, J. Y., primary, Kumar, P., additional, Mijnheer, G., additional, Chen, P., additional, Yeo, J. G., additional, Tay, S. H., additional, Chua, C., additional, Hazirah, S. N., additional, Lai, L., additional, Consolaro, A., additional, Gattorno, M., additional, Arkachaisri, T., additional, Martini, A., additional, Van Wijk, F., additional, and Albani, S., additional

Published
2020
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38. Galectin-9 and CXCL10 as Biomarkers for Disease Activity in Juvenile Dermatomyositis: A Longitudinal Cohort Study and Multicohort Validation

Author

Wienke, J., Enders, F.B., Lim, J, Mertens, J.S., van den Hoogen, L. L., Wijngaarde, C.A., Yeo, J.G., Meyer, A. (Andreas), Otten, H.G. (Henderikus), Fritsch-Stork, R. D. E., Kamphuis, S.S.M. (Sylvia), Hoppenreijs, E., Armbrust, W. (Wineke), van den Berg, J.M., Muller, P., Tekstra, J., Hoogendijk, J.E., Deakin, C.T., Jager, W. (Wilco) de, Roon, J.A.G. (J. A G) van, Pol, W.-L. (W-Ludo) van der, Nistala, K., Pilkington, C., Visser, M. (Mirjam), Arkachaisri, T., Radstake, T, Kooj, A.J. (Anneke), Nierkens, S., Wedderburn, L.R. (Lucy), van Royen-kerkhof, A., van Wijk, F., Wienke, J., Enders, F.B., Lim, J, Mertens, J.S., van den Hoogen, L. L., Wijngaarde, C.A., Yeo, J.G., Meyer, A. (Andreas), Otten, H.G. (Henderikus), Fritsch-Stork, R. D. E., Kamphuis, S.S.M. (Sylvia), Hoppenreijs, E., Armbrust, W. (Wineke), van den Berg, J.M., Muller, P., Tekstra, J., Hoogendijk, J.E., Deakin, C.T., Jager, W. (Wilco) de, Roon, J.A.G. (J. A G) van, Pol, W.-L. (W-Ludo) van der, Nistala, K., Pilkington, C., Visser, M. (Mirjam), Arkachaisri, T., Radstake, T, Kooj, A.J. (Anneke), Nierkens, S., Wedderburn, L.R. (Lucy), van Royen-kerkhof, A., and van Wijk, F.

Abstract

Objective. Objective evaluation of disease activity is challenging in patients with juvenile dermatomyositis (DM) due to a lack of reliable biomarkers, but it is crucial to avoid both under- and overtreatment of patients. Recently, we identified 2 proteins, galectin-9 and CXCL10, whose levels are highly correlated with the extent of juvenile DM disease activity. This study was undertaken to validate galectin-9 and CXCL10 as biomarkers for disease activity in juvenile DM, and to assess their disease specificity and potency in predicting the occurrence of flares. Methods. Levels of galectin-9 and CXCL10 were measured by multiplex immunoassay in serum samples from 125 unique patients with juvenile DM in 3 international cross-sectional cohorts and a local longitudinal cohort. The disease specificity of both proteins was examined in 50 adult patients with DM or nonspecific myositis (NSM) and 61 patients with other systemic autoimmune diseases. Results. Both cross-sectionally and longitudinally, galectin-9 and CXCL10 outperformed the currently used laboratory marker, creatine kinase (CK), in distinguishing between juvenile DM patients with active disease and those in remission (area under the receiver operating characteristic curve [AUC] 0.86–0.90 for galectin-9 and CXCL10; AUC 0.66–0.68 for CK). The sensitivity and specificity for active disease in juvenile DM was 0.84 and 0.92, respectively, for galectin-9 and 0.87 and 1.00, respectively, for CXCL10. In 10 patients with juvenile DM who experienced a flare and were prospectively followed up, continuously elevated or rising biomarker levels suggested an imminent flare up to several months before the onset of symptoms, even in the absence of elevated CK levels. Galectin-9 and CXCL10 distinguished between active disease and remission in adult patients with DM or NSM (P = 0.0126 for galectin-9 and P < 0.0001 for CXCL10) and were suited for measurement in minimally invasive dried blood spots (healthy controls versus juvenile

Published
2019
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39. Galectin-9 and CXCL10 as Biomarkers for Disease Activity in Juvenile Dermatomyositis: A Longitudinal Cohort Study and Multicohort Validation

Author

Wienke, J, Enders, FB, Lim, J, Mertens, JS, van den Hoogen, L L, Wijngaarde, CA, Yeo, JG, Meyer, A, Otten, HG, Fritsch-Stork, R D E, Kamphuis, Sylvia, Hoppenreijs, E, Armbrust, W, Berg, JM, Muller, P, Tekstra, J, Hoogendijk, JE, Deakin, CT, de Jager, W, van Roon, JAG, van der Pol, WL, Nistala, K, Pilkington, C, Visser, M, Arkachaisri, T, Radstake, T, van der Kooi, AJ, Nierkens, S, Wedderburn, LR, van Royen-kerkhof, A, van Wijk, F, Wienke, J, Enders, FB, Lim, J, Mertens, JS, van den Hoogen, L L, Wijngaarde, CA, Yeo, JG, Meyer, A, Otten, HG, Fritsch-Stork, R D E, Kamphuis, Sylvia, Hoppenreijs, E, Armbrust, W, Berg, JM, Muller, P, Tekstra, J, Hoogendijk, JE, Deakin, CT, de Jager, W, van Roon, JAG, van der Pol, WL, Nistala, K, Pilkington, C, Visser, M, Arkachaisri, T, Radstake, T, van der Kooi, AJ, Nierkens, S, Wedderburn, LR, van Royen-kerkhof, A, and van Wijk, F

Published
2019

40. THU0030 Molecular mechanisms of autophagic memory in pathogenic t cells in human rheumatoid arthritis

Author

Kumar, P., primary, Leong, J.Y., additional, Saidin, S., additional, Paleja, B., additional, Loosdregt, J.V., additional, Chua, C., additional, Arkachaisri, T., additional, Consolaro, A., additional, Gattorno, M., additional, Martini, A., additional, Williams, G.W., additional, Pischel, K.D, additional, Lotz, M., additional, and Albani, S., additional

Published
2018
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48. Impact of Hiatal Hernia on Pediatric Gastroesophageal Reflux Disease

Author

Au Yeung Kj, Gillespie S, Karnsakul W, Arkachaisri T, and Cannon Ml

Subjects
medicine.medical_specialty, business.industry, medicine.medical_treatment, Reflux, Disease, Nissen fundoplication, Omics, medicine.disease, Gastroenterology, humanities, digestive system diseases, Hiatal hernia, Internal medicine, medicine, GERD, business, Prospective cohort study, Esophagitis
Abstract

Objectives: To identify the frequency of hiatal hernia (HH) and the impact of HH size in children with gastroesophageal reflux disease (GERD). Methods: Outpatient records were retrospectively reviewed in children ages 0-17 years old, diagnosed clinically with GERD. HH was diagnosed based on an upper gastrointestinal (UGI) series and HH body was characterized as either small ( 5yr) with a large HH. Children with large HH were more frequently found to fail medications and require Nissen fundoplication than those with a small HH (p=0.032). Conclusions: Children with GERD had high frequency of HH based on UGI. Children with large HH required significantly more anti-reflux surgery than those with small HH. A larger scale longitudinal prospective study is needed to further investigate the impact of HH on GERD complications and the effects of therapy.

Published
2015

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