Search

Your search keyword '"O’NEIL, KATHLEEN M."' showing total 12 results
Start Over Author "O’NEIL, KATHLEEN M." Publication Type Electronic Resources
12 results on '"O’NEIL, KATHLEEN M."'

1. Distinct interferon signatures and cytokine patterns define additional systemic autoinflammatory diseases

Author

de Jesus, Adriana A., Hou, Yangfeng, Brooks, Stephen, Malle, Louise, Biancotto, Angelique, Huang, Yan, Calvo, Katherine R., Marrero, Bernadette, Moir, Susan, Oler, Andrew J., Deng, Zuoming, Sanchez, Gina A. Montealegre, Ahmed, Amina, Allenspach, Eric, Arabshahi, Bita, Behrens, Edward, Benseler, Susanne, Bezrodnik, Liliana, Bout-Tabaku, Sharon, Brescia, AnneMarie C., Brown, Diane, Burnham, Jon M., Soledad Caldirola, Maria, Carrasco, Ruy, Chan, Alice Y., Cimaz, Rolando, Dancey, Paul, Dare, Jason, DeGuzman, Marietta, Dimitriades, Victoria, Ferguson, Ian, Ferguson, Polly, Finn, Laura, Gattorno, Marco, Grom, Alexei A., Hanson, Eric P., Hashkes, Philip J., Hedrich, Christian M., Herzog, Ronit, Horneff, Gerd, Jerath, Rita, Kessler, Elizabeth, Kim, Hanna, Kingsbury, Daniel J., Laxer, Ronald M., Lee, Pui Y., Lee-Kirsch, Min Ae, Lewandowski, Laura, Li, Suzanne, Lilleby, Vibke, Mammadova, Vafa, Moorthy, Lakshmi N., Nasrullayeva, Gulnara, O'Neil, Kathleen M., Onel, Karen, Ozen, Seza, Pan, Nancy, Pillet, Pascal, Piotto, Daniela G. P., Punaro, Marilynn G., Reiff, Andreas, Reinhardt, Adam, Rider, Lisa G., Rivas-Chacon, Rafael, Ronis, Tova, Roesen-Wolff, Angela, Roth, Johannes, Ruth, Natasha Mckerran, Rygg, Marite, Schmeling, Heinrike, Schulert, Grant, Scott, Christiaan, Seminario, Gisella, Shulman, Andrew, Sivaraman, Vidya, Son, Mary Beth, Stepanovskiy, Yuriy, Stringer, Elizabeth, Taber, Sara, Terreri, Maria Teresa, Tifft, Cynthia, Torgerson, Troy, Tosi, Laura, Van Royen-Kerkhof, Annet, Muskardin, Theresa Wampler, Canna, Scott W., Goldbach-Mansky, Raphaela, de Jesus, Adriana A., Hou, Yangfeng, Brooks, Stephen, Malle, Louise, Biancotto, Angelique, Huang, Yan, Calvo, Katherine R., Marrero, Bernadette, Moir, Susan, Oler, Andrew J., Deng, Zuoming, Sanchez, Gina A. Montealegre, Ahmed, Amina, Allenspach, Eric, Arabshahi, Bita, Behrens, Edward, Benseler, Susanne, Bezrodnik, Liliana, Bout-Tabaku, Sharon, Brescia, AnneMarie C., Brown, Diane, Burnham, Jon M., Soledad Caldirola, Maria, Carrasco, Ruy, Chan, Alice Y., Cimaz, Rolando, Dancey, Paul, Dare, Jason, DeGuzman, Marietta, Dimitriades, Victoria, Ferguson, Ian, Ferguson, Polly, Finn, Laura, Gattorno, Marco, Grom, Alexei A., Hanson, Eric P., Hashkes, Philip J., Hedrich, Christian M., Herzog, Ronit, Horneff, Gerd, Jerath, Rita, Kessler, Elizabeth, Kim, Hanna, Kingsbury, Daniel J., Laxer, Ronald M., Lee, Pui Y., Lee-Kirsch, Min Ae, Lewandowski, Laura, Li, Suzanne, Lilleby, Vibke, Mammadova, Vafa, Moorthy, Lakshmi N., Nasrullayeva, Gulnara, O'Neil, Kathleen M., Onel, Karen, Ozen, Seza, Pan, Nancy, Pillet, Pascal, Piotto, Daniela G. P., Punaro, Marilynn G., Reiff, Andreas, Reinhardt, Adam, Rider, Lisa G., Rivas-Chacon, Rafael, Ronis, Tova, Roesen-Wolff, Angela, Roth, Johannes, Ruth, Natasha Mckerran, Rygg, Marite, Schmeling, Heinrike, Schulert, Grant, Scott, Christiaan, Seminario, Gisella, Shulman, Andrew, Sivaraman, Vidya, Son, Mary Beth, Stepanovskiy, Yuriy, Stringer, Elizabeth, Taber, Sara, Terreri, Maria Teresa, Tifft, Cynthia, Torgerson, Troy, Tosi, Laura, Van Royen-Kerkhof, Annet, Muskardin, Theresa Wampler, Canna, Scott W., and Goldbach-Mansky, Raphaela

Abstract

BACKGROUND. Undifferentiated systemic autoinflammatory diseases (USAIDs) present diagnostic and therapeutic challenges. Chronic interferon (IFN) signaling and cytokine dysregulation may identify diseases with available targeted treatments. METHODS. Sixty-six consecutively referred USAID patients underwent underwent screening for the presence of an interferon signature using a standardized type-I IFN-response-gene score (IRG-S), cytokine profiling, and genetic evaluation by next-generation sequencing. RESULTS. Thirty-six USAID patients (55%) had elevated IRG-S. Neutrophilic panniculitis (40% vs. 0%), basal ganglia calcifications (46% vs. 0%), interstitial lung disease (47% vs. 5%), and myositis (60% vs. 10%) were more prevalent in patients with elevated IRG-S. Moderate IRG-S elevation and highly elevated serum IL-18 distinguished 8 patients with pulmonary alveolar proteinosis (PAP) and recurrent macrophage activation syndrome (MAS). Among patients with panniculitis and progressive cytopenias, 2 patients were compound heterozygous for potentially novel LRBA mutations, 4 patients harbored potentially novel splice variants in IKBKG (which encodes NF-kappa B essential modulator [NEMO)), and 6 patients had de novo frameshift mutations in SAMD9L. Of additional 12 patients with elevated IRG-S and CANDLE-, SAVI- or Aicardi-Goutieres syndrome-like (AGS-like) phenotypes, 5 patients carried mutations in either SAMHD1, TREX1, PSMB8, or PSMG2. Two patients had anti-MDA5 autoantibody-positive juvenile dermatomyositis, and 7 could not be classified. Patients with LRBA, IKBKG, and SAMD9L mutations showed a pattern of IRG elevation that suggests prominent NF-kappa B activation different from the canonical interferonopathies CANDLE, SAVI, and AGS. CONCLUSIONS. In patients with elevated IRG-S, we identified characteristic clinical features and 3 additional autoinflammatory diseases: IL-18-mediated PAP and recurrent MAS (IL-18PAP-MAS), NEMO deleted exon 5- autoinflammatory syndrome (NEM

Published
2020

2. Distinct interferon signatures and cytokine patterns define additional systemic autoinflammatory diseases

Author

de Jesus, Adriana A., Hou, Yangfeng, Brooks, Stephen, Malle, Louise, Biancotto, Angelique, Huang, Yan, Calvo, Katherine R., Marrero, Bernadette, Moir, Susan, Oler, Andrew J., Deng, Zuoming, Sanchez, Gina A. Montealegre, Ahmed, Amina, Allenspach, Eric, Arabshahi, Bita, Behrens, Edward, Benseler, Susanne, Bezrodnik, Liliana, Bout-Tabaku, Sharon, Brescia, AnneMarie C., Brown, Diane, Burnham, Jon M., Soledad Caldirola, Maria, Carrasco, Ruy, Chan, Alice Y., Cimaz, Rolando, Dancey, Paul, Dare, Jason, DeGuzman, Marietta, Dimitriades, Victoria, Ferguson, Ian, Ferguson, Polly, Finn, Laura, Gattorno, Marco, Grom, Alexei A., Hanson, Eric P., Hashkes, Philip J., Hedrich, Christian M., Herzog, Ronit, Horneff, Gerd, Jerath, Rita, Kessler, Elizabeth, Kim, Hanna, Kingsbury, Daniel J., Laxer, Ronald M., Lee, Pui Y., Lee-Kirsch, Min Ae, Lewandowski, Laura, Li, Suzanne, Lilleby, Vibke, Mammadova, Vafa, Moorthy, Lakshmi N., Nasrullayeva, Gulnara, O'Neil, Kathleen M., Onel, Karen, Ozen, Seza, Pan, Nancy, Pillet, Pascal, Piotto, Daniela G. P., Punaro, Marilynn G., Reiff, Andreas, Reinhardt, Adam, Rider, Lisa G., Rivas-Chacon, Rafael, Ronis, Tova, Roesen-Wolff, Angela, Roth, Johannes, Ruth, Natasha Mckerran, Rygg, Marite, Schmeling, Heinrike, Schulert, Grant, Scott, Christiaan, Seminario, Gisella, Shulman, Andrew, Sivaraman, Vidya, Son, Mary Beth, Stepanovskiy, Yuriy, Stringer, Elizabeth, Taber, Sara, Terreri, Maria Teresa, Tifft, Cynthia, Torgerson, Troy, Tosi, Laura, Van Royen-Kerkhof, Annet, Muskardin, Theresa Wampler, Canna, Scott W., Goldbach-Mansky, Raphaela, de Jesus, Adriana A., Hou, Yangfeng, Brooks, Stephen, Malle, Louise, Biancotto, Angelique, Huang, Yan, Calvo, Katherine R., Marrero, Bernadette, Moir, Susan, Oler, Andrew J., Deng, Zuoming, Sanchez, Gina A. Montealegre, Ahmed, Amina, Allenspach, Eric, Arabshahi, Bita, Behrens, Edward, Benseler, Susanne, Bezrodnik, Liliana, Bout-Tabaku, Sharon, Brescia, AnneMarie C., Brown, Diane, Burnham, Jon M., Soledad Caldirola, Maria, Carrasco, Ruy, Chan, Alice Y., Cimaz, Rolando, Dancey, Paul, Dare, Jason, DeGuzman, Marietta, Dimitriades, Victoria, Ferguson, Ian, Ferguson, Polly, Finn, Laura, Gattorno, Marco, Grom, Alexei A., Hanson, Eric P., Hashkes, Philip J., Hedrich, Christian M., Herzog, Ronit, Horneff, Gerd, Jerath, Rita, Kessler, Elizabeth, Kim, Hanna, Kingsbury, Daniel J., Laxer, Ronald M., Lee, Pui Y., Lee-Kirsch, Min Ae, Lewandowski, Laura, Li, Suzanne, Lilleby, Vibke, Mammadova, Vafa, Moorthy, Lakshmi N., Nasrullayeva, Gulnara, O'Neil, Kathleen M., Onel, Karen, Ozen, Seza, Pan, Nancy, Pillet, Pascal, Piotto, Daniela G. P., Punaro, Marilynn G., Reiff, Andreas, Reinhardt, Adam, Rider, Lisa G., Rivas-Chacon, Rafael, Ronis, Tova, Roesen-Wolff, Angela, Roth, Johannes, Ruth, Natasha Mckerran, Rygg, Marite, Schmeling, Heinrike, Schulert, Grant, Scott, Christiaan, Seminario, Gisella, Shulman, Andrew, Sivaraman, Vidya, Son, Mary Beth, Stepanovskiy, Yuriy, Stringer, Elizabeth, Taber, Sara, Terreri, Maria Teresa, Tifft, Cynthia, Torgerson, Troy, Tosi, Laura, Van Royen-Kerkhof, Annet, Muskardin, Theresa Wampler, Canna, Scott W., and Goldbach-Mansky, Raphaela

Abstract

BACKGROUND. Undifferentiated systemic autoinflammatory diseases (USAIDs) present diagnostic and therapeutic challenges. Chronic interferon (IFN) signaling and cytokine dysregulation may identify diseases with available targeted treatments. METHODS. Sixty-six consecutively referred USAID patients underwent underwent screening for the presence of an interferon signature using a standardized type-I IFN-response-gene score (IRG-S), cytokine profiling, and genetic evaluation by next-generation sequencing. RESULTS. Thirty-six USAID patients (55%) had elevated IRG-S. Neutrophilic panniculitis (40% vs. 0%), basal ganglia calcifications (46% vs. 0%), interstitial lung disease (47% vs. 5%), and myositis (60% vs. 10%) were more prevalent in patients with elevated IRG-S. Moderate IRG-S elevation and highly elevated serum IL-18 distinguished 8 patients with pulmonary alveolar proteinosis (PAP) and recurrent macrophage activation syndrome (MAS). Among patients with panniculitis and progressive cytopenias, 2 patients were compound heterozygous for potentially novel LRBA mutations, 4 patients harbored potentially novel splice variants in IKBKG (which encodes NF-kappa B essential modulator [NEMO)), and 6 patients had de novo frameshift mutations in SAMD9L. Of additional 12 patients with elevated IRG-S and CANDLE-, SAVI- or Aicardi-Goutieres syndrome-like (AGS-like) phenotypes, 5 patients carried mutations in either SAMHD1, TREX1, PSMB8, or PSMG2. Two patients had anti-MDA5 autoantibody-positive juvenile dermatomyositis, and 7 could not be classified. Patients with LRBA, IKBKG, and SAMD9L mutations showed a pattern of IRG elevation that suggests prominent NF-kappa B activation different from the canonical interferonopathies CANDLE, SAVI, and AGS. CONCLUSIONS. In patients with elevated IRG-S, we identified characteristic clinical features and 3 additional autoinflammatory diseases: IL-18-mediated PAP and recurrent MAS (IL-18PAP-MAS), NEMO deleted exon 5- autoinflammatory syndrome (NEM

Published
2020

3. Proceedings of the 2016 Childhood Arthritis and Rheumatology Research Alliance (CARRA) Scientific Meeting : Toronto, Canada. 14-17 April 2016.

Author

Fotis, Lampros, Fotis, Lampros, Shaikh, Nur, Baszis, Kevin, French, Anthony, Tarr, Phillip, Grevich, Sriharsha, Lee, Peggy, Ringold, Sarah, Leroux, Brian, Leahey, Hannah, Yuasa, Megan, Foster, Jessica, Sokolove, Jeremy, Lahey, Lauren, Robinson, William, Newsom, Joshua, Stevens, Anne, Karasawa, Rie, Tamaki, Mayumi, Tanaka, Megumi, Sato, Toshiko, Yudoh, Kazuo, Jarvis, James N, Moncrieffe, Halima, Bennett, Mark F, Tsoras, Monica, Luyrink, Lorie, Xu, Huan, Prahalad, Sampath, Morris, Paula, Dare, Jason, Nigrovic, Peter A, Rosenkranz, Margalit, Becker, Mara, O’Neil, Kathleen M, Griffin, Thomas, Lovell, Daniel J, Grom, Alexei A, Medvedovic, Mario, Thompson, Susan D, Zhu, Lisha, Jiang, Kaiyu, Wong, Laiping, Buck, Michael J, Chen, Yanmin, Brungs, Laura, Liu, Tao, Wang, Ting, Alsaeid, Khaled, Alfailakawi, Jasim, Alenezi, Hamid, Alsaeed, Hazim, Beukelman, Tim, Natter, Marc, Ilowite, Norm, Mieszkalski, Kelly, Burrell, Grendel, Best, Brian, Bristow, Helen, Carr, Shannon, Dennos, Anne, Kaufmann, Rachel, Kimura, Yukiko, Schanberg, Laura, Blier, Peter R, Boneparth, Alexis, Wenderfer, Scott E, Moorthy, L Nandini, Radhakrishna, Suhas M, Sagcal-Gironella, Anna Carmela P, von Scheven, Emily, Gedik, Kader Cetin, Siddique, Salma, Aguiar, Cassyanne L, Erkan, Doruk, Cohen, Ezra, Lee, Yvonne, Dossett, Michelle, Mehta, Darshan, Davis, Roger, Gilbert, Mileka, Goilav, Beatrice, Meidan, Esra, Hsu, Joyce, Chua, Anabelle, Ardoin, Stacy, Von Scheven, Emily, Ruth, Natasha M, Hui-Yuen, Joyce, Bermudez, Liza, Cook, Ashlea, Imundo, Lisa, Starr, Amy, Eichenfield, Andrew, Askanase, Anca, Fotis, Lampros, Fotis, Lampros, Shaikh, Nur, Baszis, Kevin, French, Anthony, Tarr, Phillip, Grevich, Sriharsha, Lee, Peggy, Ringold, Sarah, Leroux, Brian, Leahey, Hannah, Yuasa, Megan, Foster, Jessica, Sokolove, Jeremy, Lahey, Lauren, Robinson, William, Newsom, Joshua, Stevens, Anne, Karasawa, Rie, Tamaki, Mayumi, Tanaka, Megumi, Sato, Toshiko, Yudoh, Kazuo, Jarvis, James N, Moncrieffe, Halima, Bennett, Mark F, Tsoras, Monica, Luyrink, Lorie, Xu, Huan, Prahalad, Sampath, Morris, Paula, Dare, Jason, Nigrovic, Peter A, Rosenkranz, Margalit, Becker, Mara, O’Neil, Kathleen M, Griffin, Thomas, Lovell, Daniel J, Grom, Alexei A, Medvedovic, Mario, Thompson, Susan D, Zhu, Lisha, Jiang, Kaiyu, Wong, Laiping, Buck, Michael J, Chen, Yanmin, Brungs, Laura, Liu, Tao, Wang, Ting, Alsaeid, Khaled, Alfailakawi, Jasim, Alenezi, Hamid, Alsaeed, Hazim, Beukelman, Tim, Natter, Marc, Ilowite, Norm, Mieszkalski, Kelly, Burrell, Grendel, Best, Brian, Bristow, Helen, Carr, Shannon, Dennos, Anne, Kaufmann, Rachel, Kimura, Yukiko, Schanberg, Laura, Blier, Peter R, Boneparth, Alexis, Wenderfer, Scott E, Moorthy, L Nandini, Radhakrishna, Suhas M, Sagcal-Gironella, Anna Carmela P, von Scheven, Emily, Gedik, Kader Cetin, Siddique, Salma, Aguiar, Cassyanne L, Erkan, Doruk, Cohen, Ezra, Lee, Yvonne, Dossett, Michelle, Mehta, Darshan, Davis, Roger, Gilbert, Mileka, Goilav, Beatrice, Meidan, Esra, Hsu, Joyce, Chua, Anabelle, Ardoin, Stacy, Von Scheven, Emily, Ruth, Natasha M, Hui-Yuen, Joyce, Bermudez, Liza, Cook, Ashlea, Imundo, Lisa, Starr, Amy, Eichenfield, Andrew, and Askanase, Anca

Published
2016

4. Proceedings of the 2016 Childhood Arthritis and Rheumatology Research Alliance (CARRA) Scientific Meeting : Toronto, Canada. 14-17 April 2016.

Author

Fotis, Lampros, Fotis, Lampros, Shaikh, Nur, Baszis, Kevin, French, Anthony, Tarr, Phillip, Grevich, Sriharsha, Lee, Peggy, Ringold, Sarah, Leroux, Brian, Leahey, Hannah, Yuasa, Megan, Foster, Jessica, Sokolove, Jeremy, Lahey, Lauren, Robinson, William, Newsom, Joshua, Stevens, Anne, Karasawa, Rie, Tamaki, Mayumi, Tanaka, Megumi, Sato, Toshiko, Yudoh, Kazuo, Jarvis, James N, Moncrieffe, Halima, Bennett, Mark F, Tsoras, Monica, Luyrink, Lorie, Xu, Huan, Prahalad, Sampath, Morris, Paula, Dare, Jason, Nigrovic, Peter A, Rosenkranz, Margalit, Becker, Mara, O’Neil, Kathleen M, Griffin, Thomas, Lovell, Daniel J, Grom, Alexei A, Medvedovic, Mario, Thompson, Susan D, Zhu, Lisha, Jiang, Kaiyu, Wong, Laiping, Buck, Michael J, Chen, Yanmin, Brungs, Laura, Liu, Tao, Wang, Ting, Alsaeid, Khaled, Alfailakawi, Jasim, Alenezi, Hamid, Alsaeed, Hazim, Beukelman, Tim, Natter, Marc, Ilowite, Norm, Mieszkalski, Kelly, Burrell, Grendel, Best, Brian, Bristow, Helen, Carr, Shannon, Dennos, Anne, Kaufmann, Rachel, Kimura, Yukiko, Schanberg, Laura, Blier, Peter R, Boneparth, Alexis, Wenderfer, Scott E, Moorthy, L Nandini, Radhakrishna, Suhas M, Sagcal-Gironella, Anna Carmela P, von Scheven, Emily, Gedik, Kader Cetin, Siddique, Salma, Aguiar, Cassyanne L, Erkan, Doruk, Cohen, Ezra, Lee, Yvonne, Dossett, Michelle, Mehta, Darshan, Davis, Roger, Gilbert, Mileka, Goilav, Beatrice, Meidan, Esra, Hsu, Joyce, Chua, Anabelle, Ardoin, Stacy, Von Scheven, Emily, Ruth, Natasha M, Hui-Yuen, Joyce, Bermudez, Liza, Cook, Ashlea, Imundo, Lisa, Starr, Amy, Eichenfield, Andrew, Askanase, Anca, Fotis, Lampros, Fotis, Lampros, Shaikh, Nur, Baszis, Kevin, French, Anthony, Tarr, Phillip, Grevich, Sriharsha, Lee, Peggy, Ringold, Sarah, Leroux, Brian, Leahey, Hannah, Yuasa, Megan, Foster, Jessica, Sokolove, Jeremy, Lahey, Lauren, Robinson, William, Newsom, Joshua, Stevens, Anne, Karasawa, Rie, Tamaki, Mayumi, Tanaka, Megumi, Sato, Toshiko, Yudoh, Kazuo, Jarvis, James N, Moncrieffe, Halima, Bennett, Mark F, Tsoras, Monica, Luyrink, Lorie, Xu, Huan, Prahalad, Sampath, Morris, Paula, Dare, Jason, Nigrovic, Peter A, Rosenkranz, Margalit, Becker, Mara, O’Neil, Kathleen M, Griffin, Thomas, Lovell, Daniel J, Grom, Alexei A, Medvedovic, Mario, Thompson, Susan D, Zhu, Lisha, Jiang, Kaiyu, Wong, Laiping, Buck, Michael J, Chen, Yanmin, Brungs, Laura, Liu, Tao, Wang, Ting, Alsaeid, Khaled, Alfailakawi, Jasim, Alenezi, Hamid, Alsaeed, Hazim, Beukelman, Tim, Natter, Marc, Ilowite, Norm, Mieszkalski, Kelly, Burrell, Grendel, Best, Brian, Bristow, Helen, Carr, Shannon, Dennos, Anne, Kaufmann, Rachel, Kimura, Yukiko, Schanberg, Laura, Blier, Peter R, Boneparth, Alexis, Wenderfer, Scott E, Moorthy, L Nandini, Radhakrishna, Suhas M, Sagcal-Gironella, Anna Carmela P, von Scheven, Emily, Gedik, Kader Cetin, Siddique, Salma, Aguiar, Cassyanne L, Erkan, Doruk, Cohen, Ezra, Lee, Yvonne, Dossett, Michelle, Mehta, Darshan, Davis, Roger, Gilbert, Mileka, Goilav, Beatrice, Meidan, Esra, Hsu, Joyce, Chua, Anabelle, Ardoin, Stacy, Von Scheven, Emily, Ruth, Natasha M, Hui-Yuen, Joyce, Bermudez, Liza, Cook, Ashlea, Imundo, Lisa, Starr, Amy, Eichenfield, Andrew, and Askanase, Anca

Abstract

P1 Serologic evidence of gut-driven systemic inflammation in juvenile idiopathic arthritis Lampros Fotis, Nur Shaikh, Kevin Baszis, Anthony French, Phillip Tarr P2 Oral health and anti-citrullinated peptide antibodies (ACPA) in juvenile idiopathic arthritis Sriharsha Grevich, Peggy Lee, Sarah Ringold, Brian Leroux, Hannah Leahey, Megan Yuasa, Jessica Foster, Jeremy Sokolove, Lauren Lahey, William Robinson, Joshua Newsom, Anne Stevens P3 Novel autoantigens for endothelial cell antibodies in pediatric rheumatic diseases identified by proteomics Rie Karasawa, Mayumi Tamaki, Megumi Tanaka, Toshiko Sato, Kazuo Yudoh, James N. Jarvis P4 Transcriptional profiling reveals monocyte signature associated with JIA patient poor response to methotrexate Halima Moncrieffe, Mark F. Bennett, Monica Tsoras, Lorie Luyrink, Huan Xu, Sampath Prahalad, Paula Morris, Jason Dare, Peter A. Nigrovic, Margalit Rosenkranz, Mara Becker, Kathleen M. O’Neil, Thomas Griffin, Daniel J. Lovell, Alexei A. Grom, Mario Medvedovic, Susan D. Thompson P5 A multi-dimensional genomic map for polyarticular juvenile idiopathic arthritis Lisha Zhu, Kaiyu Jiang, Laiping Wong, Michael J Buck, Yanmin Chen, Halima Moncrieffe, Laura Brungs, Tao Liu, Ting Wang, James N Jarvis P6 Tocilizumab for treatment of children with refractory JIA Khaled Alsaeid, Jasim Alfailakawi, Hamid Alenezi, Hazim Alsaeed P7 Clinical characteristics of the initial patients enrolled in the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry Tim Beukelman, Marc Natter, Norm Ilowite, Kelly Mieszkalski, Grendel Burrell, Brian Best, Helen Bristow, Shannon Carr, Anne Dennos, Rachel Kaufmann, Yukiko Kimura, Laura Schanberg P8 Comparative performance of small and large clinical centers in a comprehensive pediatric rheumatology disease registry Peter R Blier P9 Clinical characteristics of children with membranous lupus nephritis: The Childhood Arthritis and Rheumatology Research Alliance Legacy Registry Alexis Boneparth, Scott E

Published
2016

5. Cancer risk in childhood-onset systemic lupus

Author

Bernatsky, Sasha, Bernatsky, Sasha, Clarke, Ann E, Labrecque, Jeremy, von Scheven, Emily, Schanberg, Laura E, Silverman, Earl D, Brunner, Hermine I, Haines, Kathleen A, Cron, Randy Q, O’Neil, Kathleen M, Oen, Kiem, Rosenberg, Alan M, Duffy, Ciarán M, Joseph, Lawrence, Lee, Jennifer L, Kale, Mruganka, Turnbull, Elizabeth M, Ramsey-Goldman, Rosalind, Bernatsky, Sasha, Bernatsky, Sasha, Clarke, Ann E, Labrecque, Jeremy, von Scheven, Emily, Schanberg, Laura E, Silverman, Earl D, Brunner, Hermine I, Haines, Kathleen A, Cron, Randy Q, O’Neil, Kathleen M, Oen, Kiem, Rosenberg, Alan M, Duffy, Ciarán M, Joseph, Lawrence, Lee, Jennifer L, Kale, Mruganka, Turnbull, Elizabeth M, and Ramsey-Goldman, Rosalind

Abstract

Introduction The aim of this study was to assess cancer incidence in childhood-onset systemic lupus erythematosus (SLE). Methods We ascertained cancers within SLE registries at 10 pediatric centers. Subjects were linked to cancer registries for the observational interval, spanning 1974 to 2009. The ratio of observed to expected cancers represents the standardized incidence ratio (SIR) or relative cancer risk in childhood-onset SLE, versus the general population. Results There were 1020 patients aged <18 at cohort entry. Most (82%) were female and Caucasian; mean age at cohort entry was 12.6 years (standard deviation (SD) = 3.6). Subjects were observed for a total of 7,986 (average 7.8) patient-years. Within this interval, only three invasive cancers were expected. However, 14 invasive cancers occurred with an SIR of 4.7, 95% confidence interval (CI) 2.6 to 7.8. Three hematologic cancers were found (two non-Hodgkin’s lymphoma, one leukemia), for an SIR of 5.2 (95% CI 1.1 to 15.2). The SIRs stratified by age group and sex, were similar across these strata. There was a trend for highest cancer occurrence 10 to 19 years after SLE diagnosis. Conclusions These results suggest an increased cancer risk in pediatric onset SLE versus the general population. In absolute terms, this represents relatively few events. Of note, risk may be highest only after patients have transferred to adult care.

Published
2013

6. Cancer risk in childhood-onset systemic lupus

Author

Bernatsky, Sasha, Bernatsky, Sasha, Clarke, Ann E, Labrecque, Jeremy, von Scheven, Emily, Schanberg, Laura E, Silverman, Earl D, Brunner, Hermine I, Haines, Kathleen A, Cron, Randy Q, O’Neil, Kathleen M, Oen, Kiem, Rosenberg, Alan M, Duffy, Ciarán M, Joseph, Lawrence, Lee, Jennifer L, Kale, Mruganka, Turnbull, Elizabeth M, Ramsey-Goldman, Rosalind, Bernatsky, Sasha, Bernatsky, Sasha, Clarke, Ann E, Labrecque, Jeremy, von Scheven, Emily, Schanberg, Laura E, Silverman, Earl D, Brunner, Hermine I, Haines, Kathleen A, Cron, Randy Q, O’Neil, Kathleen M, Oen, Kiem, Rosenberg, Alan M, Duffy, Ciarán M, Joseph, Lawrence, Lee, Jennifer L, Kale, Mruganka, Turnbull, Elizabeth M, and Ramsey-Goldman, Rosalind

Abstract

Introduction The aim of this study was to assess cancer incidence in childhood-onset systemic lupus erythematosus (SLE). Methods We ascertained cancers within SLE registries at 10 pediatric centers. Subjects were linked to cancer registries for the observational interval, spanning 1974 to 2009. The ratio of observed to expected cancers represents the standardized incidence ratio (SIR) or relative cancer risk in childhood-onset SLE, versus the general population. Results There were 1020 patients aged <18 at cohort entry. Most (82%) were female and Caucasian; mean age at cohort entry was 12.6 years (standard deviation (SD) = 3.6). Subjects were observed for a total of 7,986 (average 7.8) patient-years. Within this interval, only three invasive cancers were expected. However, 14 invasive cancers occurred with an SIR of 4.7, 95% confidence interval (CI) 2.6 to 7.8. Three hematologic cancers were found (two non-Hodgkin’s lymphoma, one leukemia), for an SIR of 5.2 (95% CI 1.1 to 15.2). The SIRs stratified by age group and sex, were similar across these strata. There was a trend for highest cancer occurrence 10 to 19 years after SLE diagnosis. Conclusions These results suggest an increased cancer risk in pediatric onset SLE versus the general population. In absolute terms, this represents relatively few events. Of note, risk may be highest only after patients have transferred to adult care.

Published
2013

7. Algorithm development for corticosteroid management in systemic juvenile idiopathic arthritis trial using consensus methodology

Author

Ilowite, Norman T, Ilowite, Norman T, Sandborg, Christy I, Feldman, Brian M, Grom, Alexi, Schanberg, Laura E, Giannini, Edward H, Wallace, Carol A, Schneider, Rayfel, Kenney, Kathleen, Gottlieb, Beth, Hashkes, Philip J, Imundo, Lisa, Kimura, Yukiko, Lang, Bianca, Miller, Michael, Milojevic, Diana, O’Neil, Kathleen M, Punaro, Marilynn, Ruth, Natasha, Singer, Nora G, Vehe, Richard K, Verbsky, James, Woodward, Amy, Zemel, Lawrence, Ilowite, Norman T, Ilowite, Norman T, Sandborg, Christy I, Feldman, Brian M, Grom, Alexi, Schanberg, Laura E, Giannini, Edward H, Wallace, Carol A, Schneider, Rayfel, Kenney, Kathleen, Gottlieb, Beth, Hashkes, Philip J, Imundo, Lisa, Kimura, Yukiko, Lang, Bianca, Miller, Michael, Milojevic, Diana, O’Neil, Kathleen M, Punaro, Marilynn, Ruth, Natasha, Singer, Nora G, Vehe, Richard K, Verbsky, James, Woodward, Amy, and Zemel, Lawrence

Abstract

Background The management of background corticosteroid therapy in rheumatology clinical trials poses a major challenge. We describe the consensus methodology used to design an algorithm to standardize changes in corticosteroid dosing during the Randomized Placebo Phase Study of Rilonacept in Systemic Juvenile Idiopathic Arthritis Trial (RAPPORT). Methods The 20 RAPPORT site principal investigators (PIs) and 4 topic specialists constituted an expert panel that participated in the consensus process. The panel used a modified Delphi Method consisting of an on-line questionnaire, followed by a one day face-to-face consensus conference. Consensus was defined as ≥ 75% agreement. For items deemed essential but when consensus on critical values was not achieved, simple majority vote drove the final decision. Results The panel identified criteria for initiating or increasing corticosteroids. These included the presence or development of anemia, myocarditis, pericarditis, pleuritis, peritonitis, and either complete or incomplete macrophage activation syndrome (MAS). The panel also identified criteria for tapering corticosteroids which included absence of fever for ≥ 3 days in the previous week, absence of poor physical functioning, and seven laboratory criteria. A tapering schedule was also defined. Conclusion The expert panel established consensus regarding corticosteroid management and an algorithm for steroid dosing that was well accepted and used by RAPPORT investigators. Developed specifically for the RAPPORT trial, further study of the algorithm is needed before recommendation for more general clinical use.

Published
2012

8. The effects of early aggressive therapy in JIA: results of the TREAT study

Author

Wallace, Carol A, Wallace, Carol A, Giannini, Edward H, Spalding, Steven J, Hashkes, Philip J, O’Neil, Kathleen M, Zeft, Andrew S, Szer, Ilona S, Ringold, Sarah M, Brunner, Hermine, Schanberg, Laura E, Sundel, Robert P, Milojevic, Diana, Punaro, Marilynn G, Chira, Peter, Gottlieb, Beth S, Higgins, Gloria C, Ilowite, Norman T, Kimura, Yukiko, Huang, Bin, Lovell, Daniel J, Wallace, Carol A, Wallace, Carol A, Giannini, Edward H, Spalding, Steven J, Hashkes, Philip J, O’Neil, Kathleen M, Zeft, Andrew S, Szer, Ilona S, Ringold, Sarah M, Brunner, Hermine, Schanberg, Laura E, Sundel, Robert P, Milojevic, Diana, Punaro, Marilynn G, Chira, Peter, Gottlieb, Beth S, Higgins, Gloria C, Ilowite, Norman T, Kimura, Yukiko, Huang, Bin, and Lovell, Daniel J

Published
2012

9. Algorithm development for corticosteroid management in systemic juvenile idiopathic arthritis trial using consensus methodology

Author

Ilowite, Norman T, Ilowite, Norman T, Sandborg, Christy I, Feldman, Brian M, Grom, Alexi, Schanberg, Laura E, Giannini, Edward H, Wallace, Carol A, Schneider, Rayfel, Kenney, Kathleen, Gottlieb, Beth, Hashkes, Philip J, Imundo, Lisa, Kimura, Yukiko, Lang, Bianca, Miller, Michael, Milojevic, Diana, O’Neil, Kathleen M, Punaro, Marilynn, Ruth, Natasha, Singer, Nora G, Vehe, Richard K, Verbsky, James, Woodward, Amy, Zemel, Lawrence, Ilowite, Norman T, Ilowite, Norman T, Sandborg, Christy I, Feldman, Brian M, Grom, Alexi, Schanberg, Laura E, Giannini, Edward H, Wallace, Carol A, Schneider, Rayfel, Kenney, Kathleen, Gottlieb, Beth, Hashkes, Philip J, Imundo, Lisa, Kimura, Yukiko, Lang, Bianca, Miller, Michael, Milojevic, Diana, O’Neil, Kathleen M, Punaro, Marilynn, Ruth, Natasha, Singer, Nora G, Vehe, Richard K, Verbsky, James, Woodward, Amy, and Zemel, Lawrence

Abstract

Background The management of background corticosteroid therapy in rheumatology clinical trials poses a major challenge. We describe the consensus methodology used to design an algorithm to standardize changes in corticosteroid dosing during the Randomized Placebo Phase Study of Rilonacept in Systemic Juvenile Idiopathic Arthritis Trial (RAPPORT). Methods The 20 RAPPORT site principal investigators (PIs) and 4 topic specialists constituted an expert panel that participated in the consensus process. The panel used a modified Delphi Method consisting of an on-line questionnaire, followed by a one day face-to-face consensus conference. Consensus was defined as ≥ 75% agreement. For items deemed essential but when consensus on critical values was not achieved, simple majority vote drove the final decision. Results The panel identified criteria for initiating or increasing corticosteroids. These included the presence or development of anemia, myocarditis, pericarditis, pleuritis, peritonitis, and either complete or incomplete macrophage activation syndrome (MAS). The panel also identified criteria for tapering corticosteroids which included absence of fever for ≥ 3 days in the previous week, absence of poor physical functioning, and seven laboratory criteria. A tapering schedule was also defined. Conclusion The expert panel established consensus regarding corticosteroid management and an algorithm for steroid dosing that was well accepted and used by RAPPORT investigators. Developed specifically for the RAPPORT trial, further study of the algorithm is needed before recommendation for more general clinical use.

Published
2012

10. The effects of early aggressive therapy in JIA: results of the TREAT study

Author

Wallace, Carol A, Wallace, Carol A, Giannini, Edward H, Spalding, Steven J, Hashkes, Philip J, O’Neil, Kathleen M, Zeft, Andrew S, Szer, Ilona S, Ringold, Sarah M, Brunner, Hermine, Schanberg, Laura E, Sundel, Robert P, Milojevic, Diana, Punaro, Marilynn G, Chira, Peter, Gottlieb, Beth S, Higgins, Gloria C, Ilowite, Norman T, Kimura, Yukiko, Huang, Bin, Lovell, Daniel J, Wallace, Carol A, Wallace, Carol A, Giannini, Edward H, Spalding, Steven J, Hashkes, Philip J, O’Neil, Kathleen M, Zeft, Andrew S, Szer, Ilona S, Ringold, Sarah M, Brunner, Hermine, Schanberg, Laura E, Sundel, Robert P, Milojevic, Diana, Punaro, Marilynn G, Chira, Peter, Gottlieb, Beth S, Higgins, Gloria C, Ilowite, Norman T, Kimura, Yukiko, Huang, Bin, and Lovell, Daniel J

Published
2012

11. EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part I: Overall methodology and clinical characterisation

Author

Ruperto, Nicolino, Ozen, Seza, Pistorio, Angela, Dolezalova, Pavla, Brogan, Paul, Cabral, David A, Cuttica, Ruben, Khubchandani, Raju, Lovell, Daniel J, O'Neil, Kathleen M, Quartier, Pierre, Ravelli, Angelo, Iusan, Silvia M, Filocamo, Giovanni, Magalhães, Claudia Saad, Unsal, Erbil, Oliveira, Sheila, Bracaglia, Claudia, Bagga, Arvind, Stanevicha, Valda, Manzoni, Silvia Magni, Pratsidou, Polyxeni, Lepore, Loredana, Espada, Graciela, Kone-Paut, Isabella, Paut, Isabelle Kone, Zulian, Francesco, Barone, Patrizia, Bircan, Zelal, Maldonado, Maria del Rocio, Russo, Ricardo, Vilca, Iris, Tullus, Kjell, Cimaz, Rolando, Horneff, Gerd, Anton, Jordi, Garay, Stella, Nielsen, Susan Mary, Barbano, Giancarlo, Martini, Alberto, Ruperto, Nicolino, Ozen, Seza, Pistorio, Angela, Dolezalova, Pavla, Brogan, Paul, Cabral, David A, Cuttica, Ruben, Khubchandani, Raju, Lovell, Daniel J, O'Neil, Kathleen M, Quartier, Pierre, Ravelli, Angelo, Iusan, Silvia M, Filocamo, Giovanni, Magalhães, Claudia Saad, Unsal, Erbil, Oliveira, Sheila, Bracaglia, Claudia, Bagga, Arvind, Stanevicha, Valda, Manzoni, Silvia Magni, Pratsidou, Polyxeni, Lepore, Loredana, Espada, Graciela, Kone-Paut, Isabella, Paut, Isabelle Kone, Zulian, Francesco, Barone, Patrizia, Bircan, Zelal, Maldonado, Maria del Rocio, Russo, Ricardo, Vilca, Iris, Tullus, Kjell, Cimaz, Rolando, Horneff, Gerd, Anton, Jordi, Garay, Stella, Nielsen, Susan Mary, Barbano, Giancarlo, and Martini, Alberto

Abstract

To report methodology and overall clinical, laboratory and radiographic characteristics for Henoch-Schönlein purpura (HSP), childhood polyarteritis nodosa (c-PAN), c-Wegener granulomatosis (c-WG) and c-Takayasu arteritis (c-TA) classification criteria.

Published
2010

12. EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part I: Overall methodology and clinical characterisation

Author

Ruperto, Nicolino, Ozen, Seza, Pistorio, Angela, Dolezalova, Pavla, Brogan, Paul, Cabral, David A, Cuttica, Ruben, Khubchandani, Raju, Lovell, Daniel J, O'Neil, Kathleen M, Quartier, Pierre, Ravelli, Angelo, Iusan, Silvia M, Filocamo, Giovanni, Magalhães, Claudia Saad, Unsal, Erbil, Oliveira, Sheila, Bracaglia, Claudia, Bagga, Arvind, Stanevicha, Valda, Manzoni, Silvia Magni, Pratsidou, Polyxeni, Lepore, Loredana, Espada, Graciela, Kone-Paut, Isabella, Paut, Isabelle Kone, Zulian, Francesco, Barone, Patrizia, Bircan, Zelal, Maldonado, Maria del Rocio, Russo, Ricardo, Vilca, Iris, Tullus, Kjell, Cimaz, Rolando, Horneff, Gerd, Anton, Jordi, Garay, Stella, Nielsen, Susan Mary, Barbano, Giancarlo, Martini, Alberto, Ruperto, Nicolino, Ozen, Seza, Pistorio, Angela, Dolezalova, Pavla, Brogan, Paul, Cabral, David A, Cuttica, Ruben, Khubchandani, Raju, Lovell, Daniel J, O'Neil, Kathleen M, Quartier, Pierre, Ravelli, Angelo, Iusan, Silvia M, Filocamo, Giovanni, Magalhães, Claudia Saad, Unsal, Erbil, Oliveira, Sheila, Bracaglia, Claudia, Bagga, Arvind, Stanevicha, Valda, Manzoni, Silvia Magni, Pratsidou, Polyxeni, Lepore, Loredana, Espada, Graciela, Kone-Paut, Isabella, Paut, Isabelle Kone, Zulian, Francesco, Barone, Patrizia, Bircan, Zelal, Maldonado, Maria del Rocio, Russo, Ricardo, Vilca, Iris, Tullus, Kjell, Cimaz, Rolando, Horneff, Gerd, Anton, Jordi, Garay, Stella, Nielsen, Susan Mary, Barbano, Giancarlo, and Martini, Alberto

Abstract

To report methodology and overall clinical, laboratory and radiographic characteristics for Henoch-Schönlein purpura (HSP), childhood polyarteritis nodosa (c-PAN), c-Wegener granulomatosis (c-WG) and c-Takayasu arteritis (c-TA) classification criteria.

Published
2010

Catalog

Books, media, physical & digital resources
See catalog results