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1. Performance of the 2016 ACR-EULAR myositis response criteria in juvenile dermatomyositis therapeutic trials and consensus profiles.

Author

Kim, Hanna, Saygin, Didem, Douglas, Christian, Wilkerson, Jesse, Erman, Brian, Pistorio, Angela, McGrath, John A, Reed, Ann M, Oddis, Chester V, Bracaglia, Claudia, Royen-Kerkhof, Annet van, Bica, Blanca, Dolezalova, Pavla, Ferriani, Virginia P L, Flato, Berit, Bernard-Medina, Ana G, Herlin, Troels, Miller, Frederick W, Vencovsky, Jiri, and Ruperto, Nicolino

Subjects
DRUG efficacy, RITUXIMAB, CONSENSUS (Social sciences), STATISTICS, DERMATOMYOSITIS, HEALTH outcome assessment, TREATMENT effectiveness, MUSCLE strength, RESEARCH funding, DATA analysis, SENSITIVITY & specificity (Statistics), EVALUATION, CHILDREN
Abstract

Objectives The 2016 ACR-EULAR Response Criteria for JDM was developed as a composite measure with differential weights of six core set measures (CSMs) to calculate a Total Improvement Score (TIS). We assessed the contribution of each CSM, representation of muscle-related and patient-reported CSMs towards improvement, and frequency of CSM worsening across myositis response criteria (MRC) categories in validation of MRC. Methods Data from JDM patients in the Rituximab in Myositis trial (n  = 48), PRINTO JDM trial (n  = 139), and consensus patient profiles (n  = 273) were included. Observed vs expected CSM contributions were compared using Sign test. Characteristics of MRC categories were compared by Wilcoxon tests with Bonferroni adjustment. Spearman correlation of changes in TIS and individual CSMs were examined. Agreement between physician-assessed change and MRC categories was evaluated by weighted Cohen's kappa. Results Of 457 JDM patients with IMACS CSMs and 380 with PRINTO CSMs, 9–13% had minimal, 19–23% had moderate and 41–50% had major improvement. The number of improved and absolute percentage change of CSMs increased by MRC improvement level. Patients with minimal improvement by MRC had a median of 0–1 CSM worsened, and those with moderate/major improvement had a median of zero worsening CSMs. Of patients improved by MRC, 94–95% had improvement in muscle strength and 93–95% had improvement in ≥ 1 patient-reported CSM. IMACS and PRINTO CSMs performed similarly. Physician-rated change and MRC improvement categories had moderate-to-substantial agreement (Kappa 0.5–0.7). Conclusion The ACR-EULAR MRC perform consistently across multiple studies, supporting its further use as an efficacy end point in JDM trials. [ABSTRACT FROM AUTHOR]

Published
2023
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2. European evidence-based recommendations for the diagnosis and treatment of childhood-onset lupus nephritis: the SHARE initiative

Author

Groot, Noortje, de Graeff, Nienke, Marks, Stephen D, Brogan, Paul, Avcin, Tadej, Bader-Meunier, Brigitte, Dolezalova, Pavla, Feldman, Brian M, Kone-Paut, Isabelle, Lahdenne, Pekka, McCann, Liza, Özen, Seza, Pilkington, Clarissa A, Ravelli, Angelo, Royen-Kerkhof, Annet van, Uziel, Yosef, Vastert, Bas J, Wulffraat, Nico M, Beresford, Michael W, and Kamphuis, Sylvia

Published
2017
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3. Siglec-1 expression on monocytes is associated with the interferon signature in juvenile dermatomyositis and can predict treatment response

Author

Lerkvaleekul, B., Veldkamp, S.R., Wal, Marlot van der, Schatorje, E.J., Kamphuis, S.S., Berg, J.Merlijn van den, Muller, P.C., Armbrust, W., Vastert, S.J., Wienke, J., Jansen, M.H., Royen-Kerkhof, A. Van, Wijk, F van, Lerkvaleekul, B., Veldkamp, S.R., Wal, Marlot van der, Schatorje, E.J., Kamphuis, S.S., Berg, J.Merlijn van den, Muller, P.C., Armbrust, W., Vastert, S.J., Wienke, J., Jansen, M.H., Royen-Kerkhof, A. Van, and Wijk, F van

Abstract

Contains fulltext : 283123.pdf (Publisher’s version ) (Open Access), OBJECTIVE: JDM is a rare chronic immune-mediated inflammatory disease with a predominant role for type I IFN responses. We aimed to determine the potential of Siglec-1 expression on monocytes as a novel IFN-inducible biomarker for disease activity monitoring and prediction of treatment response in patients with JDM. METHODS: Siglec-1 was measured by flow cytometry on circulating monocytes of 21 newly diagnosed JDM patients before start of treatment and, for 10 of these, also during follow-up. The expression levels of five type I IFN-stimulated genes, MX1, IFI44, IFI44L, LY6E and IFIT3, were measured by RT-qPCR to determine the IFN signature and calculate an IFN score. IFN-inducible plasma proteins CXCL10 and galectin-9 were measured by multiplex immunoassay. RESULTS: Siglec-1 and IFN score were increased in JDM patients compared with controls and correlated with clinical disease activity. Stratification of patients by Siglec-1 expression at diagnosis identified those with high Siglec-1 expression as having a higher risk of requiring treatment intensification within the first 3 months after diagnosis (55% vs 0% of patients, P = 0.01). Siglec-1 expression strongly correlated with plasma levels of previously validated biomarkers CXCL10 (rs = 0.81, P < 0.0001) and galectin-9 (rs = 0.83, P < 0.0001), and was superior to the IFN score in predicting treatment response (area under the curve 0.87 vs 0.53, P = 0.01). CONCLUSION: Siglec-1 on monocytes is a novel IFN-inducible biomarker in JDM that correlates with clinical disease activity and identifies patients at risk for a suboptimal treatment response. Further studies are required to validate these findings and their clinical potential.

Published
2022

4. European evidence-based recommendations for diagnosis and treatment of childhood-onset systemic lupus erythematosus: the SHARE initiative

Author

Groot, Noortje, de Graeff, Nienke, Avcin, Tadej, Bader-Meunier, Brigitte, Brogan, Paul, Dolezalova, Pavla, Feldman, Brian, Kone-Paut, Isabelle, Lahdenne, Pekka, Marks, Stephen D, McCann, Liza, Ozen, Seza, Pilkington, Clarissa, Ravelli, Angelo, Royen-Kerkhof, Annet van, Uziel, Yosef, Vastert, Bas, Wulffraat, Nico, Kamphuis, Sylvia, and Beresford, Michael W

Published
2017
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5. 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

6. Anti-Cytosolic 5'-Nucleotidase 1A Autoantibodies Are Absent in Juvenile Dermatomyositis

Author

Rietveld, A., Wienke, J., Visser, E.A., Vree Egberts, W.T.M., Schlumberger, W., Engelen, B.G.M. van, Royen-Kerkhof, A. Van, Lu, H., Wedderburn, L., Saris, C.G.J., Tansley, S., Pruijn, G., Rietveld, A., Wienke, J., Visser, E.A., Vree Egberts, W.T.M., Schlumberger, W., Engelen, B.G.M. van, Royen-Kerkhof, A. Van, Lu, H., Wedderburn, L., Saris, C.G.J., Tansley, S., and Pruijn, G.

Abstract

Contains fulltext : 238504.pdf (Publisher’s version ) (Open Access), OBJECTIVE: To assess anti-cytosolic 5'-nucleotidase 1A (anti-cN-1A) autoantibodies in children with juvenile dermatomyositis (DM) and healthy controls, using 3 different methods of antibody detection, as well as verification of the results in an independent cohort. METHODS: Anti-cN-1A reactivity was assessed in 34 Dutch juvenile DM patients and 20 healthy juvenile controls using the following methods: a commercially available full-length cN-1A enzyme-linked immunosorbent assay (ELISA), a synthetic peptide ELISA, and immunoblotting with a lysate from cN-1A-expressing HEK 293 cells. Sera from juvenile DM patients with active disease and those with disease in remission were analyzed. An independent British cohort of 110 juvenile DM patients and 43 healthy juvenile controls was assessed using an in-house full-length cN-1A ELISA. RESULTS: Anti-cN-1A reactivity was not present in sera from juvenile DM patients or healthy controls when tested with the commercially available full-length cN-1A ELISA or by immunoblotting, in either active disease or disease in remission. Additionally, in the British juvenile DM cohort, anti-cN-1A reactivity was not detected. Three Dutch juvenile DM patients had weakly positive results for 1 of 3 synthetic cN-1A peptides measured by ELISA. CONCLUSION: Juvenile DM patients and young healthy individuals did not show anti-cN-1A reactivity as assessed by different antibody detection techniques.

Published
2021

7. 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

8. Siglec-1 expression on monocytes is associated with the interferon signature in juvenile dermatomyositis and can predict treatment response.

Author

Lerkvaleekul, Butsabong, Veldkamp, Saskia R, Wal, M Marlot van der, Schatorjé, Ellen J H, Kamphuis, Sylvia S M, Berg, J Merlijn van den, Muller, Petra C E Hissink, Armbrust, Wineke, Vastert, Sebastiaan J, Wienke, Judith, Jansen, Marc H A, Royen-Kerkhof, Annet van, and Wijk, Femke van

Subjects
BIOMARKERS, FLOW cytometry, REVERSE transcriptase polymerase chain reaction, DERMATOMYOSITIS, BLOOD proteins, PEDIATRICS, INTERFERONS, TREATMENT effectiveness, IMMUNOASSAY, COMPARATIVE studies, RISK assessment, GENE expression, DESCRIPTIVE statistics, MEMBRANE proteins, MONOCYTES, EVALUATION, CHILDREN, ADOLESCENCE
Abstract

Objective JDM is a rare chronic immune-mediated inflammatory disease with a predominant role for type I IFN responses. We aimed to determine the potential of Siglec-1 expression on monocytes as a novel IFN-inducible biomarker for disease activity monitoring and prediction of treatment response in patients with JDM. Methods Siglec-1 was measured by flow cytometry on circulating monocytes of 21 newly diagnosed JDM patients before start of treatment and, for 10 of these, also during follow-up. The expression levels of five type I IFN-stimulated genes, MX1, IFI44, IFI44L, LY6E and IFIT3, were measured by RT-qPCR to determine the IFN signature and calculate an IFN score. IFN-inducible plasma proteins CXCL10 and galectin-9 were measured by multiplex immunoassay. Results Siglec-1 and IFN score were increased in JDM patients compared with controls and correlated with clinical disease activity. Stratification of patients by Siglec-1 expression at diagnosis identified those with high Siglec-1 expression as having a higher risk of requiring treatment intensification within the first 3 months after diagnosis (55% vs 0% of patients, P  = 0.01). Siglec-1 expression strongly correlated with plasma levels of previously validated biomarkers CXCL10 (rs = 0.81, P  < 0.0001) and galectin-9 (rs = 0.83, P  < 0.0001), and was superior to the IFN score in predicting treatment response (area under the curve 0.87 vs 0.53, P  = 0.01). Conclusion Siglec-1 on monocytes is a novel IFN-inducible biomarker in JDM that correlates with clinical disease activity and identifies patients at risk for a suboptimal treatment response. Further studies are required to validate these findings and their clinical potential. [ABSTRACT FROM AUTHOR]

Published
2022
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9. The identification of CCL18 as biomarker of disease activity in localized scleroderma

Author

Mertens, J.S., Jong, E.M.G.J. de, Hoogen, L.L. van den, Wienke, J., Thurlings, R.M., Seyger, M.M.B., Hoppenreijs, E.P.A.H., Wijngaarde, C.A., Vlijmen-Willems, I.M.J.J. van, Bogaard, E.H.J. van den, Giovannone, B., Wijk, F. van, Royen-Kerkhof, A. van, Marut, W., Radstake, T.R.D.J., Mertens, J.S., Jong, E.M.G.J. de, Hoogen, L.L. van den, Wienke, J., Thurlings, R.M., Seyger, M.M.B., Hoppenreijs, E.P.A.H., Wijngaarde, C.A., Vlijmen-Willems, I.M.J.J. van, Bogaard, E.H.J. van den, Giovannone, B., Wijk, F. van, Royen-Kerkhof, A. van, Marut, W., and Radstake, T.R.D.J.

Abstract

Contains fulltext : 204252.pdf (publisher's version ) (Closed access), BACKGROUND: Localized Scleroderma (LoS) encompasses a group of idiopathic skin conditions characterized by (sub)cutaneous inflammation and subsequent development of fibrosis. Currently, lack of accurate tools enabling disease activity assessment leads to suboptimal treatment approaches. OBJECTIVE: To investigate serum concentrations of cytokines and chemokines implicated in inflammation and angiogenesis in LoS and explore their potential to be utilized as biomarker of disease activity. Additionally, to investigate the implication of potential biomarkers in disease pathogenesis. METHODS: A 39-plex Luminex immuno-assay was performed in serum samples of 74 LoS and 22 Healthy Controls. The relation between a validated clinical measure of disease activity (mLoSSI) and serum analytes was investigated. Additionally, gene and protein expression were investigated in circulating cells and skin biopsies. RESULTS: From the total of 39, 10 analytes (CCL18, CXCL9, CXCL10, CXCL13, TNFRII, Galectin-9, TIE-1, sVCAM, IL-18, CCL19) were elevated in LoS serum. Cluster analysis of serum samples revealed CCL18 as most important analyte to discriminate between active and inactive disease. At individual patient level, CCL18 serum levels correlated strongest with mLoSSI-scores (rs=0.4604, P<0.0001) and in longitudinal measures CCL18 concentrations normalised with declining disease activity upon treatment initiation. Additionally, CCL18 was elevated in LoS serum, and not in (juvenile) dermatomyositis or spinal muscular atrophy. Importantly, CCL18 gene and protein expression was increased at the inflammatory border of cutaneous LoS lesions, with normal expression in unaffected skin and circulating immune cells. CONCLUSION: CCL18 is specific for disease activity in LoS thereby providing relevance as a biomarker for this debilitating disease.

Published
2019

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

Author

Wienke, Judith, Enders, Felicitas Bellutti, Lim, Johan, Mertens, J.S., Hoogen, Luuk L. van den, Wijngaarde, Camiel A., Hoppenreijs, E.P.A.H., Berg, J.M. van den, Royen-Kerkhof, Annet Van, Wijk, Femke van, Wienke, Judith, Enders, Felicitas Bellutti, Lim, Johan, Mertens, J.S., Hoogen, Luuk L. van den, Wijngaarde, Camiel A., Hoppenreijs, E.P.A.H., Berg, J.M. van den, Royen-Kerkhof, Annet Van, and Wijk, Femke van

Abstract

Contains fulltext : 206145.pdf (publisher's version ) (Open Access)

Published
2019

11. [Juvenile idiopathic arthritis].

Author

Schatorje, E.J., Royen-Kerkhof, A. Van, Schatorje, E.J., and Royen-Kerkhof, A. Van

Abstract

Item does not contain fulltext

Published
2018

12. Regarding 'Transcriptional and Cytokine Profiles Identify CXCL9 as a Biomarker of Disease Activity in Morphea'

Author

Mertens, J.S., Jong, E.M.G.J. de, Pandit, A., Seyger, M.M.B., Hoppenreijs, E.P.A.H., Thurlings, R.M., Vonk, M.C., Wienke, J., Wijk, F. van, Royen-Kerkhof, A. Van, Marut, W., Radstake, T.R.D.J., Mertens, J.S., Jong, E.M.G.J. de, Pandit, A., Seyger, M.M.B., Hoppenreijs, E.P.A.H., Thurlings, R.M., Vonk, M.C., Wienke, J., Wijk, F. van, Royen-Kerkhof, A. Van, Marut, W., and Radstake, T.R.D.J.

Abstract

Contains fulltext : 193258.pdf (publisher's version ) (Closed access)

Published
2018

13. European evidence-based recommendations for diagnosis and treatment of childhood-onset systemic lupus erythematosus: The SHARE initiative

Author

UMC Utrecht, Immuno/reuma onderzoek 1 (Vastert), Child Health, Medical Humanities Onderzoek Team 2, JC onderzoeksprogramma Methodologie, Cluster B, Reumatologie patientenzorg, Infection & Immunity, Divisieleiding O&O, Infectiezieken, Regenerative Medicine and Stem Cells, BOO, Immunologie/Reumatologie, Reumatologie onderzoek 2, Groot, Noortje, De Graeff, Nienke, Avcin, Tadej, Bader-Meunier, Brigitte, Brogan, Paul A., Dolezalova, Pavla, Feldman, Brian M., Kone-Paut, Isabelle, Lahdenne, Pekka, Marks, Stephen D, McCann, Liza J., Ozen, Seza, Pilkington, Clarissa, Ravelli, Angelo, Royen-Kerkhof, Annet Van, Uziel, Yosef, Vastert, Bas, Wulffraat, Nico, Kamphuis, Sylvia, Beresford, Michael W., UMC Utrecht, Immuno/reuma onderzoek 1 (Vastert), Child Health, Medical Humanities Onderzoek Team 2, JC onderzoeksprogramma Methodologie, Cluster B, Reumatologie patientenzorg, Infection & Immunity, Divisieleiding O&O, Infectiezieken, Regenerative Medicine and Stem Cells, BOO, Immunologie/Reumatologie, Reumatologie onderzoek 2, Groot, Noortje, De Graeff, Nienke, Avcin, Tadej, Bader-Meunier, Brigitte, Brogan, Paul A., Dolezalova, Pavla, Feldman, Brian M., Kone-Paut, Isabelle, Lahdenne, Pekka, Marks, Stephen D, McCann, Liza J., Ozen, Seza, Pilkington, Clarissa, Ravelli, Angelo, Royen-Kerkhof, Annet Van, Uziel, Yosef, Vastert, Bas, Wulffraat, Nico, Kamphuis, Sylvia, and Beresford, Michael W.

Published
2017

14. Quantitative muscle ultrasonography in the follow-up of juvenile dermatomyositis

Author

Habers, G.E., Brussel, M. Van, Bhansing, K.J., Hoppenreijs, E.P.A.H., Janssen, A.J., Royen-Kerkhof, A. Van, and Pillen, S.

Subjects
Disorders of movement Donders Center for Medical Neuroscience [Radboudumc 3], Inflammatory diseases Radboud Institute for Molecular Life Sciences [Radboudumc 5]
Abstract

Item does not contain fulltext INTRODUCTION: We explored the use of quantitative muscle ultrasonography (QMUS) for follow-up of juvenile dermatomyositis (JDM). METHODS: Seven JDM patients were evaluated at diagnosis and 1, 3, 6, 12, and 24 months using the Childhood Myositis Assessment Scale (CMAS) and QMUS. Muscle thickness (MT) and quantitative muscle echo intensity (EI) were assessed with QMUS in 4 muscles. RESULTS: Six patients experienced a monocyclic course. At diagnosis EI was slightly increased, and MT was relatively normal. After start of treatment MT first decreased and EI increased, with normalization of EI within 6-12 months (n = 4). One patient had higher EIs at diagnosis and slower normalization, indicating fibrosis, despite early normalization of CMAS. One patient experienced a chronic course, with high EIs and atrophy during follow-up. CONCLUSIONS: QMUS can provide additional information for follow-up of JDM regarding disease severity and residual muscle damage, particularly after normalization of CMAS. Muscle Nerve 52: 540-546, 2015.

Published
2015

15. European consensus-based recommendations for the diagnosis and treatment of Kawasaki disease – the SHARE initiative.

Author

Graeff, Nienke de, Groot, Noortje, Ozen, Seza, Eleftheriou, Despina, Avcin, Tadej, Bader-Meunier, Brigitte, Dolezalova, Pavla, Feldman, Brian M, Kone-Paut, Isabelle, Lahdenne, Pekka, McCann, Liza, Pilkington, Clarissa, Ravelli, Angelo, Royen-Kerkhof, Annet van, Uziel, Yosef, Vastert, Bas, Wulffraat, Nico, Kamphuis, Sylvia, Brogan, Paul, and Beresford, Michael W

Subjects
MUCOCUTANEOUS lymph node syndrome diagnosis, MUCOCUTANEOUS lymph node syndrome, ADRENOCORTICAL hormones, ASPIRIN, IMMUNOGLOBULINS, INTRAVENOUS therapy, MEDICAL care, MEDICAL protocols, MEETINGS, PATIENTS, PEDIATRICS, SURVEYS, SYSTEMATIC reviews, THERAPEUTICS
Abstract

Objectives The European Single Hub and Access point for paediatric Rheumatology in Europe initiative aimed to optimize care for children with rheumatic diseases. Kawasaki disease (KD) is the most common cause of acquired heart disease in children and an important cause of long-term cardiac disease into adulthood. Prompt diagnosis and treatment of KD is difficult due to the heterogeneity of the disease but is crucial for improving outcome. To date, there are no European internationally agreed, evidence-based guidelines concerning the diagnosis and treatment of KD in children. Accordingly, treatment regimens differ widely. The aim of this study is to provide consensus-based, European-wide evidence-informed recommendations for diagnosis and treatment of children with KD. Methods Recommendations were developed using the EULAR's standard operating procedures. An extensive systematic literature search was performed, and evidence-based recommendations were extrapolated from the included papers. These were evaluated by a panel of international experts via online surveys and subsequently discussed in three consensus meetings, using nominal group technique. Recommendations were accepted when ⩾80% agreed. Results In total, 17 recommendations for diagnosis and 14 for treatment of KD in children were accepted. Diagnostic recommendations included laboratory and imaging workup for complete as well as incomplete KD. Treatment recommendations included the importance of early treatment in both complete and incomplete KD, use of intravenous immunoglobulin, aspirin, corticosteroids for high-risk cases, and other treatment options for those with resistant disease. Conclusion The Single Hub and Access point for paediatric Rheumatology in Europe initiative provides international evidence-based recommendations for diagnosing and treating KD in children, facilitating improvement and uniformity of care. [ABSTRACT FROM AUTHOR]

Published
2019
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16. Serum inflammatory mediators correlate with disease activity in electrical status epilepticus in sleep (ESES) syndrome

Author

Munckhof, B. van den, Vries, E.E. de, Braun, K.P., Boss, H.M., Willemsen, M.A., Royen-Kerkhof, A. Van, Jager, W. de, Jansen, F.E., Munckhof, B. van den, Vries, E.E. de, Braun, K.P., Boss, H.M., Willemsen, M.A., Royen-Kerkhof, A. Van, Jager, W. de, and Jansen, F.E.

Abstract

Item does not contain fulltext, We aimed to study serum cytokine levels in 11 electrical status epilepticus in sleep (ESES) patients and 20 healthy control children. Patients showed significantly higher levels of interleukin (IL)-1alpha, IL-6, IL-10, chemokine (C-C motif) ligand (CCL)2 and chemokine (C-X-C motif) ligand (CXCL)8/IL-8 than controls, while macrophage migration inhibitory factor (MIF) and CCL3 were significantly lower. Follow-up analyses in five patients revealed a significant decrease of IL-6 levels after immunomodulating treatment. IL-6 changes were accompanied by clear improvement of electroencephalography (EEG) patterns and neuropsychological evaluation. We hypothesize that IL-6 correlates with disease activity and immunomodulating treatment efficacy.

Published
2016

18. Increased Presence of FOXP3+ Regulatory T Cells in Inflamed Muscle of Patients with Active Juvenile Dermatomyositis Compared to Peripheral Blood

Author

Vercoulen, Y., Bellutti Enders, F., Meerding, J., Plantinga, M., Elst, E.F., Varsani, H., Schieveen, C. van, Bakker, M.H., Klein, M., Scholman, R.C., Spliet, W., Ricotti, V., Koenen, H.J., Weger, R.A. de, Wedderburn, L.R., Royen-Kerkhof, A. van, Prakken, B.J., Vercoulen, Y., Bellutti Enders, F., Meerding, J., Plantinga, M., Elst, E.F., Varsani, H., Schieveen, C. van, Bakker, M.H., Klein, M., Scholman, R.C., Spliet, W., Ricotti, V., Koenen, H.J., Weger, R.A. de, Wedderburn, L.R., Royen-Kerkhof, A. van, and Prakken, B.J.

Abstract

Contains fulltext : 138284.pdf (publisher's version ) (Open Access)

Published
2014

20. [The practice guideline 'Dermatomyositis, polymyositis and sporadic inclusion body myositis']

Author

Hoogendijk, J.E., Bijlsma, J.W.J., Engelen, B.G.M. van, Lindeman, E.J.M., Royen-Kerkhof, A. van, Rie, M.A. de, Visser, M. de, and Jennekens, F.G.I.

Subjects
Human Movement & Fatigue [NCEBP 10], Functional Neurogenomics [DCN 2], Neuromuscular development and genetic disorders [UMCN 3.1]
Abstract

Item does not contain fulltext This guideline presents recommendations for the diagnosis and treatment of dermatomyositis, polymyositis and sporadic inclusion body myositis (sIBM) according to the best available evidence. Characteristic skin abnormalities can be sufficient for the diagnosis of dermatomyositis. In case of doubt, a skin biopsy is advisable. A muscle biopsy is indicated when other examinations are inconclusive and the musculature is involved. The working group considers screening for cancer to be required in adults with dermatomyositis and presents recommendations for the way that this should be done. At least one-third of all patients with polymyositis has, or will develop, an associated inflammatory connective tissue disease. If a patient with a connective tissue disease develops symmetrical, proximal muscle weakness in the course of weeks or months, this may be assumed to be due to polymyositis. In the absence ofpre-existing connective tissue disease, demonstration of a mononuclear cell infiltrate in muscle tissue is a prerequisite for the diagnosis ofpolymyositis. The histopathology of muscle tissue is used as the gold standard for the diagnosis of sIBM. The practice guideline presents criteria for the concept 'activity' of myositis. Disease activity serves as a guideline for the treatment of polymyositis and dermatomyositis. The treatment of choice for dermatomyositis and polymyositis is high-dose prednisone. Physical activity does not have a negative effect on the course of these diseases. The long-term prognosis ofdermatomyositis and polymyositis is not well known. The clinical course of sIBM is slowly progressive.

Published
2005

21. Calcinosis in juvenile dermatomyositis: a possible role for the vitamin K-dependent protein matrix Gla protein.

Author

Summeren, M. J. H. van, Spliet, W. G. M., Royen-Kerkhof, A. van, Vermeer, C., Lilien, M., Kuis, W., and Schurgers, L. J.

Subjects
DERMATOMYOSITIS, CALCIFICATION, PROTEINS, BIOPSY, CUTANEOUS manifestations of general diseases, MYOSITIS
Abstract

Objectives. The aims of the present study were to investigate whether the calcification inhibitor matrix Gla protein (MGP) is expressed in muscle biopsies of patients with juvenile dermatomyositis (JDM), and whether different forms of MGP are differentially expressed in JDM patients with and without subcutaneous calcifications. [ABSTRACT FROM PUBLISHER]

Published
2008
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22. CD4+FOXP3+ regulatory T cells are abundantly present in inflamed muscle of patients with juvenile dermatomyositis.

Author

Vercoulen, Yvonne, Elst, Elisabeth F., Meerding, Jenny, Plantinga, Maud, Varsani, Hemlata, Schieveen, Christa van, Bakker, Mette H., Klein, Mark, Spliet, Wim, Weger, R. de, Wedderburn, Lucy, Royen-Kerkhof, Annet van, and Prakken, Berent J.

Subjects
DERMATOMYOSITIS
Abstract

An abstract of the conference paper "CD4+FOXP3+ regulatory T cells are abundantly present in inflamed muscle of patients with juvenile dermatomyositis," by Yvonne Vercoulen and colleagues is presented.

Published
2011
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26. Exercise in action for JUVENILE DERMATOMYOSITIS : Steps towards understanding

Author

Habers, G.E.A., Prakken, A.B.J., Stegeman, D.F., Brussel, M. van, Royen-Kerkhof, A. van, and University Utrecht

Subjects
Near-infrared spectroscopy, Physical training, Immunology, Juvenile dermatomyositis, Exercise physiology, Quantitative muscle ultrasonography
Abstract

This thesis describes studies in the field of juvenile dermatomyositis (JDM), a chronic inflammatory disease in which the microvasculature is attacked by the immune system. Clinical characteristics include symptoms of muscles (e.g. weakness) and skin (e.g. Gottron’s papules over the extensor joint surfaces, heliotrope rash over the eyelids, and photosensitivity). The objectives of the studies in this rare disease were: A) to gain a better understanding of the exercise intolerance and fatigue; B) to contribute to an improvement in the prognostics and monitoring of disease course; and C) to examine the feasibility, safety, and efficacy of exercise training. In a cross-sectional study, the oxygenation and hemodynamics during exercise and recovery were assessed in children with JDM with near-infrared spectroscopy and compared with results from children in juvenile idiopathic arthritis as well as healthy controls. Patients with JDM showed lower values of total hemoglobin in the vastus medialis muscle during the whole exercise test compared to healthy children, whereas the results in patients with juvenile idiopathic arthritis were similar to those in healthy controls. In collaboration with groups in North-America and Canada, we examined the association of factors available at disease onset for their association with disease course in a large cohort (n=365) of patients with juvenile idiopathic inflammatory myopathies (JIIM), including JDM. These factors included demographics, clinical features present prior to diagnosis, the highest recorded serum muscle enzyme levels, myositis autoantibodies, environmental factors at illness onset and at diagnosis, and immunogenetic polymorphisms. By univariable and multivariable approaches, we determined myositis-specific and -associated autoantibodies, specifically the anti-p155/140 and anti-Ro autoantibodies, and a number of clinical features, and environmental exposures present at disease onset or diagnosis that were associated with a chronic disease course in patients with JIIM. In a small cohort of patients with JDM, the opportunities of non-invasive, quantitative muscle ultrasonography in the longitudinal follow-up were explored together with clinical variables. The findings of this study indicate that ultrasonography provides additional information in the follow-up of patients with JDM regarding disease activity, fibrosis, and atrophy, particularly after normalisation of the Childhood Myositis Assessment Scale. In a systematic review, the efficacy and safety of exercise training in juvenile and adult idiopathic inflammatory myopathies (including JDM) were examined. Exercise training appeared to be safe and effective in adult patients with idiopathic inflammatory myopathies in active as well as inactive disease, although most of the included studies had a high selection and/or allocation bias. Recently, two studies on exercise training in children with JDM were published. These studies indicate positive effects of exercise training in this group. However, both studies had a small sample size and were uncontrolled. In a randomized controlled trial, the feasibility, safety, and efficacy of a home-based exercise training program in patients with JDM were evaluated. The individually tailored 12-week home-based exercise training program was feasible in most patients, safe in all patients, and effective for aerobic fitness, muscle function, and functional ability. Therefore, exercise training is of additional value in the clinical management of patients with JDM.

Published
2015

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