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2. Correction: Engaging patients and parents to improve mental health intervention for youth with rheumatological disease

Author

Fawole, Oluwatunmise A., Reed, Michelle V., Harris, Julia G., Hersh, Aimee, Rodriguez, Martha, Onel, Karen, Lawson, Erica, Rubinstein, Tamar, Ardalan, Kaveh, Morgan, Esi, Paul, Anne, Barlin, Judy, Daly, R. Paola, Dave, Mitali, Malloy, Shannon, Hume, Shari, Schrandt, Suzanne, Marrow, Laura, Chapson, Angela, Napoli, Donna, Napoli, Michael, Moyer, Miranda, Delgaizo, Vincent, Danguecan, Ashley, von Scheven, Emily, and Knight, Andrea

Published
2024
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3. 2022 American College of Rheumatology Guideline for Vaccinations in Patients With Rheumatic and Musculoskeletal Diseases

Author

Bass, Anne R, Chakravarty, Eliza, Akl, Elie A, Bingham, Clifton O, Calabrese, Leonard, Cappelli, Laura C, Johnson, Sindhu R, Imundo, Lisa F, Winthrop, Kevin L, Arasaratnam, Reuben J, Baden, Lindsey R, Berard, Roberta, Bridges, S Louis, Cheah, Jonathan TL, Curtis, Jeffrey R, Ferguson, Polly J, Hakkarinen, Ida, Onel, Karen B, Schultz, Grayson, Sivaraman, Vidya, Smith, Benjamin J, Sparks, Jeffrey A, Vogel, Tiphanie P, Williams, Eleanor Anderson, Calabrese, Cassandra, Cunha, Joanne S, Fontanarosa, Joann, Gillispie‐Taylor, Miriah C, Gkrouzman, Elena, Iyer, Priyanka, Lakin, Kimberly S, Legge, Alexandra, Lo, Mindy S, Lockwood, Megan M, Sadun, Rebecca E, Singh, Namrata, Sullivan, Nancy, Tam, Herman, Turgunbaev, Marat, Turner, Amy S, and Reston, James

Subjects
Biomedical and Clinical Sciences, Clinical Sciences, Autoimmune Disease, Immunization, Prevention, Vaccine Related, 3.4 Vaccines, Prevention of disease and conditions, and promotion of well-being, Good Health and Well Being, Child, Humans, United States, Rheumatology, Antirheumatic Agents, Musculoskeletal Diseases, Vaccination
Abstract

ObjectiveTo provide evidence-based recommendations on the use of vaccinations in children and adults with rheumatic and musculoskeletal diseases (RMDs).MethodsThis guideline follows American College of Rheumatology (ACR) policy guiding management of conflicts of interest and disclosures and the ACR guideline development process, which includes the Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology. It also adheres to the Appraisal of Guidelines for Research and Evaluation (AGREE) criteria. A core leadership team consisting of adult and pediatric rheumatologists and a guideline methodologist drafted clinical population, intervention, comparator, outcomes (PICO) questions. A review team performed a systematic literature review for the PICO questions, graded the quality of evidence, and produced an evidence report. An expert Voting Panel reviewed the evidence and formulated recommendations. The panel included adult and pediatric rheumatology providers, infectious diseases specialists, and patient representatives. Consensus required ≥70% agreement on both the direction and strength of each recommendation.ResultsThis guideline includes expanded indications for some vaccines in patients with RMDs, as well as guidance on whether to hold immunosuppressive medications or delay vaccination to maximize vaccine immunogenicity and efficacy. Safe approaches to the use of live attenuated vaccines in patients taking immunosuppressive medications are also addressed. Most recommendations are conditional and had low quality of supporting evidence.ConclusionApplication of these recommendations should consider patients' individual risk for vaccine-preventable illness and for disease flares, particularly if immunosuppressive medications are held for vaccination. Shared decision-making with patients is encouraged in clinical settings.

Published
2023

4. Corrigendum: Increase in pediatric recurrent fever evaluations during the first year of the COVID-19 pandemic in North America.

Author

Mansfield, Leanne, Lapidus, Sivia, Romero, Samira, Moorthy, Lakshmi, Adler-Shohet, Felice, Hollander, Matthew, Cherian, Julie, Twilt, Marinka, Mohan, Smriti, DeLaMora, Patricia, Durrant, Karen, Muskardin, Theresa, Correia Marques, Mariana, Onel, Karen, Dedeoglu, Fatma, Gutierrez, Maria, Schulert, Grant, and Lionetti, Geraldina

Subjects
CARRA, COVID-19, fevers, pediatric, recurrent, rheumatology
Abstract

[This corrects the article DOI: 10.3389/fped.2023.1240242.].

Published
2023

5. Increase in pediatric recurrent fever evaluations during the first year of the COVID-19 pandemic in North America.

Author

Mansfield, Leanne, Lapidus, Sivia, Romero, Samira, Moorthy, Lakshmi, Adler-Shohet, Felice, Hollander, Matthew, Cherian, Julie, Twilt, Marinka, Lionetti, Geraldina, Mohan, Smriti, DeLaMora, Patricia, Durrant, Karen, Muskardin, Theresa, Correia Marques, Mariana, Onel, Karen, Dedeoglu, Fatma, Guttierez, Maria, and Schulert, Grant

Subjects
CARRA, COVID-19, fevers, pediatric, recurrent, rheumatology
Abstract

The impact of the COVID-19 pandemic on new diagnoses of recurrent fevers and autoinflammatory diseases is largely unknown. The Childhood Arthritis and Rheumatology Research Alliance (CARRA) PFAPA/AID Working Group aimed to investigate the impact of the COVID-19 pandemic on the number of pediatric patients evaluated for recurrent fevers and autoinflammatory diseases in North America. The absolute number of new outpatient visits and the proportion of these visits attributed to recurrent fever diagnoses during the pre-pandemic period (1 March 2019-29 February 2020) and the first year of the COVID-19 pandemic (1 March 2020-28 February 2021) were examined. Data were collected from 27 sites in the United States and Canada. Our results showed an increase in the absolute number of new visits for recurrent fever evaluations in 21 of 27 sites during the COVID-19 pandemic compared to the pre-pandemic period. The increase was observed across different geographic regions in North America. Additionally, the proportion of new visits to these centers for recurrent fever in relation to all new patient evaluations was significantly higher during the first year of the pandemic, increasing from 7.8% before the pandemic to 10.9% during the pandemic year (p

Published
2023

6. Interleukin (IL)-1/IL-6-Inhibitor–Associated Drug Reaction With Eosinophilia and Systemic Symptoms (DReSS) in Systemic Inflammatory Illnesses

Author

Aamir, R., Abulaban, K., Adams, A., Lapsia, C. Aguiar, Akinsete, A., Akoghlanian, S., Al Manaa, M., AlBijadi, A., Allenspach, E., Almutairi, A., Alperin, R., Amarilyo, G., Ambler, W., Amoruso, M., Angeles-Han, S., Ardoin, S., Armendariz, S., Asfaw, L., Aviran Dagan, N., Bacha, C., Balboni, I., Balevic, S., Ballinger, S., Baluta, S., Barillas-Arias, L., Basiaga, M., Baszis, K., Baxter, S., Becker, M., Begezda, A., Behrens, E., Beil, E., Benseler, S., Bermudez-Santiago, L., Bernal, W., Bigley, T., Bingham, C., Binstadt, B., Black, C., Blackmon, B., Blakley, M., Bohnsack, J., Boneparth, A., Bradfield, H., Bridges, J., Brooks, E., Brothers, M., Brunner, H., Buckley, L., Buckley, M., Bukulmez, H., Bullock, D., Canna, S., Cannon, L., Canny, S., Cartwright, V., Cassidy, E., Castro, D., Chalom, E., Chang, J., Chang, M., Chang-Hoftman, A., Chen, A., Chiraseveenuprapund, P., Ciaglia, K., Co, D., Cohen, E., Collinge, J., Conlon, H., Connor, R., Cook, K., Cooper, A., Cooper, J., Corbin, K., Correll, C., Cron, R., Curry, M., Dalrymple, A., Datyner, E., Davis, T., De Ranieri, D., Dean, J., DeCoste, C., Dedeoglu, F., DeGuzman, M., Delnay, N., DeSantis, E., Devine, R., Dhalla, M., Dhanrajani, A., Dissanayake, D., Dizon, B., Drapeau, N., Drew, J., Driest, K., Du, Q., Duncan, E., Dunnock, K., Durkee, D., Dvergsten, J., Eberhard, A., Ede, K., Edelheit, B., Edens, C., El Tal, T., Elder, M., Elzaki, Y., Fadrhonc, S., Failing, C., Fair, D., Favier, L., Feldman, B., Fennell, J., Ferguson, P., Ferguson, I., Figueroa, C., Flanagan, E., Fogel, L., Fox, E., Fox, M., Franklin, L., Fuhlbrigge, R., Fuller, J., Furey, M., Futch-West, T., Gagne, S., Gennaro, V., Gerstbacher, D., Gilbert, M., Gironella, A., Glaser, D., Goh, I., Goldsmith, D., Gorry, S., Goswami, N., Gottlieb, B., Graham, T., Grevich, S., Griffin, T., Grim, A., Grom, A., Guevara, M., Hahn, T., Halyabar, O., Hamda Natur, M., Hammelev, E., Hammond, T., Harel, L., Harris, J., Harry, O., Hausmann, J., Hay, A., Hays, K., Hayward, K., Henderson, L., Henrickson, M., Hersh, A., Hickey, K., Hiraki, L., Hiskey, M., Hobday, P., Hoffart, C., Holland, M., Hollander, M., Hong, S., Horton, D., Horwitz, M., Hsu, J., Huber, A., Huberts, A., Huggins, J., Huie, L., Hui-Yuen, J., Ibarra, M., Imlay, A., Imundo, L., Inman, C., Jackson, A., James, K., Janow, G., Jared, S., Jiang, Y., Johnson, L., Johnson, N., Jones, J., Kafisheh, D., Kahn, P., Kaidar, K., Kasinathan, S., Kaur, R., Kessler, E., Kienzle, B., Kim, S., Kimura, Y., Kingsbury, D., Kitcharoensakkul, M., Klausmeier, T., Klein, K., Klein-Gitelman, M., Knight, A., Kovalick, L., Kramer, S., Kremer, C., Kudas, O., LaFlam, T., Lang, B., Lapidus, S., Lapin, B., Lasky, A., Lawler, C., Lawson, E., Laxer, R., Lee, P., Lee, T., Lee, A., Leisinger, E., Lentini, L., Lerman, M., Levinsky, Y., Levy, D., Li, S., Lieberman, S., Lim, L., Limenis, E., Lin, C., Ling, N., Lionetti, G., Livny, R., Lloyd, M., Lo, M., Long, A., Lopez-Peña, M., Lovell, D., Luca, N., Lvovich, S., Lytch, A., Ma, M., Machado, A., MacMahon, J., Madison, J., Mannion, M., Manos, C., Mansfield, L., Marston, B., Mason, T., Matchett, D., McAllister, L., McBrearty, K., McColl, J., McCurdy, D., McDaniels, K., McDonald, J., Meidan, E., Mellins, E., Mian, Z., Miettunen, P., Miller, M., Milojevic, D., Mitacek, R., Modica, R., Mohan, S., Moore, T., Moore, K., Moorthy, L., Moreno, J., Morgan, E., Moyer, A., Murante, B., Murphy, A., Muscal, E., Mwizerwa, O., Najafi, A., Nanda, K., Nasah, N., Nassi, L., Nativ, S., Natter, M., Nearanz, K., Neely, J., Newhall, L., Nguyen, A., Nigrovic, P., Nocton, J., Nolan, B., Nowicki, K., Oakes, R., Oberle, E., Ogbonnaya-Whittesley, S., Ogbu, E., Oliver, M., Olveda, R., Onel, K., Orandi, A., Padam, J., Paller, A., Pan, N., Pandya, J., Panupattanapong, S., Toledano, A. Pappo, Parsons, A., Patel, J., Patel, P., Patrick, A., Patrizi, S., Paul, S., Perfetto, J., Perron, M., Peskin, M., Ponder, L., Pooni, R., Prahalad, S., Puplava, B., Quinlan-Waters, M., Rabinovich, C., Rafko, J., Rahimi, H., Rampone, K., Ramsey, S., Randell, R., Ray, L., Reed, A., Reid, H., Reiff, D., Richins, S., Riebschleger, M., Rife, E., Riordan, M., Riskalla, M., Robinson, A., Robinson, L., Rodgers, L., Rodriquez, M., Rogers, D., Ronis, T., Rosado, A., Rosenkranz, M., Rosenwasser, N., Rothermel, H., Rothman, D., Rothschild, E., Roth-Wojcicki, E., Rouster-Stevens, K., Rubinstein, T., Rupp, J., Ruth, N., Sabbagh, S., Sadun, R., Santiago, L., Saper, V., Sarkissian, A., Scalzi, L., Schahn, J., Schikler, K., Schlefman, A., Schmeling, H., Schmitt, E., Schneider, R., Schulert, G., Schultz, K., Schutt, C., Seper, C., Sheets, R., Shehab, A., Shenoi, S., Sherman, M., Shirley, J., Shishov, M., Siegel, D., Singer, N., Sivaraman, V., Sloan, E., Smith, C., Smith, J., Smitherman, E., Soep, J., Son, Mary B., Sosna, D., Spencer, C., Spiegel, L., Spitznagle, J., Srinivasalu, H., Stapp, H., Steigerwald, K., Stephens, A., Sterba Rakovchik, Y., Stern, S., Stevens, B., Stevenson, R., Stewart, K., Stewart, W., Stingl, C., Stoll, M., Stringer, E., Sule, S., Sullivan, J., Sundel, R., Sutter, M., Swaffar, C., Swayne, N., Syed, R., Symington, T., Syverson, G., Szymanski, A., Taber, S., Tal, R., Tambralli, A., Taneja, A., Tanner, T., Tarvin, S., Tate, L., Taxter, A., Taylor, J., Tesher, M., Thakurdeen, T., Theisen, A., Thomas, B., Thomas, L., Thomas, N., Ting, T., Todd, C., Toib, D., Torok, K., Tory, H., Toth, M., Tse, S., Tsin, C., Twachtman-Bassett, J., Twilt, M., Valcarcel, T., Valdovinos, R., Vallee, A., Van Mater, H., Vandenbergen, S., Vannoy, L., Varghese, C., Vasquez, N., Vega-Fernandez, P., Velez, J., Verbsky, J., Verstegen, R., von Scheven, E., Vora, S., Wagner-Weiner, L., Wahezi, D., Waite, H., Walker, B., Walters, H., Waterfield, M., Waters, A., Weiser, P., Weiss, P., Weiss, J., Wershba, E., Westheuser, V., White, A., Widrick, K., Williams, C., Wong, S., Woolnough, L., Wright, T., Wu, E., Yalcindag, A., Yasin, S., Yeung, R., Yomogida, K., Zeft, A., Zhang, Y., Zhao, Y., Zhu, A., Saper, Vivian E., Tian, Lu, Verstegen, Ruud H.J., Conrad, Carol K., Cidon, Michal, Hopper, Rachel K., Kuo, Christin S., Osoegawa, Kazutoyo, Baszis, Kevin, Bingham, Catherine A., Ferguson, Ian, Hahn, Timothy, Horne, Annacarin, Isupova, Eugenia A., Jones, Jordan T., Kasapcopur, Özgür, Klein-Gitelman, Marisa S., Kostik, Mikhail M., Ozen, Seza, Phadke, Omkar, Prahalad, Sampath, Randell, Rachel L., Sener, Seher, Stingl, Cory, Abdul-Aziz, Rabheh, Akoghlanian, Shoghik, Al Julandani, Dalila, Alvarez, Marcela B., Bader-Meunier, Brigitte, Balay-Dustrude, Erin E., Balboni, Imelda, Baxter, Sarah K., Berard, Roberta A., Bhattad, Sagar, Bolaria, Roxana, Boneparth, Alexis, Cassidy, Elaine A., Co, Dominic O., Collins, Kathleen P., Dancey, Paul, Dickinson, Aileen M., Edelheit, Barbara S., Espada, Graciela, Flanagan, Elaine R., Imundo, Lisa F., Jindal, Ankur K., Kim, Hyoun-Ah, Klaus, Günter, Lake, Carol, Lapin, W. Blaine, Lawson, Erica F., Marmor, Itay, Mombourquette, Joy, Ogunjimi, Benson, Olveda, Rebecca, Ombrello, Michael J., Onel, Karen, Poholek, Catherine, Ramanan, Athimalaipet V., Ravelli, Angelo, Reinhardt, Adam, Robinson, Amanda D., Rouster-Stevens, Kelly, Saad, Nadine, Schneider, Rayfel, Selmanovic, Velma, Sefic Pasic, Irmina, Shenoi, Susan, Shilo, Natalie R., Soep, Jennifer B., Sura, Angeli, Taber, Sarah F., Tesher, Melissa, Tibaldi, Jessica, Torok, Kathryn S., Tsin, Cathy Mei, Vasquez-Canizares, Natalia, Villacis Nunez, Diana S., Way, Emily E., Whitehead, Benjamin, Zemel, Lawrence S., Sharma, Surbhi, Fernández-Viña, Marcelo A., and Mellins, Elizabeth D.

Published
2024
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7. Social determinants of health influence disease activity and functional disability in Polyarticular Juvenile Idiopathic Arthritis.

Author

Soulsby, William Daniel, Balmuri, Nayimisha, Cooley, Victoria, Gerber, Linda M, Lawson, Erica, Goodman, Susan, Onel, Karen, Mehta, Bella, and CARRA Registry Investigators

Subjects
CARRA Registry Investigators, Humans, Disability Evaluation, Retrospective Studies, Cohort Studies, Child, Child, Preschool, Female, Male, Arthritis, Juvenile, Social Determinants of Health, Correlation of Data, Disease activity, Health disparities, Polyarticular juvenile idiopathic arthritis, Social determinants of health, Arthritis, Clinical Research, Autoimmune Disease, Behavioral and Social Science, Inflammatory and immune system, Good Health and Well Being, Clinical Sciences, Paediatrics and Reproductive Medicine, Arthritis & Rheumatology
Abstract

BackgroundSocial determinants of health (SDH) greatly influence outcomes during the first year of treatment in rheumatoid arthritis, a disease similar to polyarticular juvenile idiopathic arthritis (pJIA). We investigated the correlation of community poverty level and other SDH with the persistence of moderate to severe disease activity and functional disability over the first year of treatment in pJIA patients enrolled in the Childhood Arthritis and Rheumatology Research Alliance Registry.MethodsIn this cohort study, unadjusted and adjusted generalized linear mixed effects models analyzed the effect of community poverty and other SDH on disease activity, using the clinical Juvenile Arthritis Disease Activity Score-10, and disability, using the Child Health Assessment Questionnaire, measured at baseline, 6, and 12 months.ResultsOne thousand six hundred eighty-four patients were identified. High community poverty (≥20% living below the federal poverty level) was associated with increased odds of functional disability (OR 1.82, 95% CI 1.28-2.60) but was not statistically significant after adjustment (aOR 1.23, 95% CI 0.81-1.86) and was not associated with increased disease activity. Non-white race/ethnicity was associated with higher disease activity (aOR 2.48, 95% CI: 1.41-4.36). Lower self-reported household income was associated with higher disease activity and persistent functional disability. Public insurance (aOR 1.56, 95% CI 1.06-2.29) and low family education (aOR 1.89, 95% CI 1.14-3.12) was associated with persistent functional disability.ConclusionHigh community poverty level was associated with persistent functional disability in unadjusted analysis but not with persistent moderate to high disease activity. Race/ethnicity and other SDH were associated with persistent disease activity and functional disability.

Published
2022

8. Community poverty level influences time to first pediatric rheumatology appointment in Polyarticular Juvenile Idiopathic Arthritis

Author

Balmuri, Nayimisha, Soulsby, William Daniel, Cooley, Victoria, Gerber, Linda, Lawson, Erica, Goodman, Susan, Onel, Karen, and Mehta, Bella

Subjects
Arthritis, Pediatric, Prevention, Autoimmune Disease, Rare Diseases, Inflammatory and immune system, Good Health and Well Being, Appointments and Schedules, Arthritis, Juvenile, Child, Child, Preschool, Cohort Studies, Female, Humans, Male, Pediatrics, Poverty, Rheumatology, CARRA Registry Investigators, Clinical Sciences, Paediatrics and Reproductive Medicine, Arthritis & Rheumatology
Abstract

BackgroundThe impact of social determinants of health on children with polyarticular juvenile idiopathic arthritis (pJIA) is poorly understood. Prompt initiation of treatment for pJIA is important to prevent disease morbidity; however, a potential barrier to early treatment of pJIAs is delayed presentation to a pediatric rheumatologist. We examined the impact of community poverty level, a key social determinant of health, on time from patient reported symptom onset to first pediatric rheumatology visit among pJIA patients enrolled in the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry.MethodsThis is a cohort study of pJIA patients in the CARRA registry who lived in the United States from July 2015-February 2020. The primary exposure was community poverty level derived by geocoding patient addresses. The primary outcome was time to first rheumatology appointment. Kaplan-Meier analysis was performed to analyze time to first rheumatologist visit, stratified by community poverty and family income. Log-rank tests were used to identify differences between groups. Adjusted cox proportional-hazards models were used to determine the relationship between community poverty level and time from onset of disease symptoms to date first seen by rheumatologist.ResultsA total of 1684 patients with pJIA meeting study inclusion and exclusion criteria were identified. Median age of onset of pJIA was 7 years (IQR 3, 11), 79% were female, 17.6% identified as minority race and/or ethnicity, and 19% were from communities with ≥20% community poverty level. Kaplan-Meier analysis by community poverty level (

Published
2021

9. Part 5: Allogeneic HSCT in refractory SJIA with lung disease; recent cases from centers in North America & Europe

Author

Grom, Alexei A., Canna, Scott W., Abu-Arja, Rolla F., Sinha, Rashmi, Peixoto, Luciana, Cannizzaro, Elvira, Chandrakasan, Shanmuganathan, Driest, Kyla, Marsh, Rebecca, Neven, Bénédicte, Onel, Karen, Prahalad, Sampath, Prockop, Susan, Quartier, Pierre, Roth, Johannes, Schulert, Grant, Silva, Juliana M.F., Wall, Donna, and Zeilhofer, Ulrike

Published
2023
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10. 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, Montealegre Sanchez, Gina A, Ahmed, Amina, Allenspach, Eric, Arabshahi, Bita, Behrens, Edward, Benseler, Susanne, Bezrodnik, Liliana, Bout-Tabaku, Sharon, Brescia, AnneMarie C, Brown, Diane, Burnham, Jon M, Caldirola, Maria Soledad, 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 Gp, Punaro, Marilynn G, Reiff, Andreas, Reinhardt, Adam, Rider, Lisa G, Rivas-Chacon, Rafael, Ronis, Tova, Rösen-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, Wampler Muskardin, Theresa, Canna, Scott W, and Goldbach-Mansky, Raphaela

Subjects
Genetics, Clinical Research, Rare Diseases, 2.1 Biological and endogenous factors, Aetiology, Autoimmune Diseases, Female, Humans, Interferon Type I, Interleukin-18, Macrophage Activation Syndrome, Male, Mutation, Panniculitis, Pulmonary Alveolar Proteinosis, Genetic diseases, Immunology, Inflammation, Innate immunity, Monogenic diseases, Medical and Health Sciences
Abstract

BACKGROUNDUndifferentiated systemic autoinflammatory diseases (USAIDs) present diagnostic and therapeutic challenges. Chronic interferon (IFN) signaling and cytokine dysregulation may identify diseases with available targeted treatments.METHODSSixty-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.RESULTSThirty-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-κB 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-Goutières 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-κB activation different from the canonical interferonopathies CANDLE, SAVI, and AGS.CONCLUSIONSIn 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 (NEMO-NDAS), and SAMD9L-associated autoinflammatory disease (SAMD9L-SAAD). The IRG-S expands the diagnostic armamentarium in evaluating USAIDs and points to different pathways regulating IRG expression.TRIAL REGISTRATIONClinicalTrials.gov NCT02974595.FUNDINGThe Intramural Research Program of the NIH, NIAID, NIAMS, and the Clinical Center.

Published
2020

11. The interferon-rich skin environment regulates Langerhans cell ADAM17 to promote photosensitivity in lupus

Author

Li, Thomas Morgan, primary, Zyulina, Victoria, additional, Seltzer, Ethan S, additional, Dacic, Marija, additional, Chinenov, Yurii, additional, Daamen, Andrea R, additional, Veiga, Keila R, additional, Schwartz, Noa, additional, Oliver, David J, additional, Cabahug-Zuckerman, Pamela, additional, Lora, Jose, additional, Liu, Yong, additional, Shipman, William D, additional, Ambler, William G, additional, Taber, Sarah F, additional, Onel, Karen B, additional, Zippin, Jonathan H, additional, Rashighi, Mehdi, additional, Krueger, James G, additional, Anandasabapathy, Niroshana, additional, Rogatsky, Inez, additional, Jabbari, Ali, additional, Blobel, Carl P, additional, Lipsky, Peter E, additional, and Lu, Theresa T, additional

Published
2024
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13. 2022 American College of Rheumatology Guideline for Vaccinations in Patients With Rheumatic and Musculoskeletal Diseases

Author

Bass, Anne R., Chakravarty, Eliza, Akl, Elie A., Bingham, Clifton O., Calabrese, Leonard, Cappelli, Laura C., Johnson, Sindhu R., Imundo, Lisa F., Winthrop, Kevin L., Arasaratnam, Reuben J., Baden, Lindsey R., Berard, Roberta, Bridges, S. Louis, Jr., Cheah, Jonathan T. L., Curtis, Jeffrey R., Ferguson, Polly J., Hakkarinen, Ida, Onel, Karen B., Schultz, Grayson, Sivaraman, Vidya, Smith, Benjamin J., Sparks, Jeffrey A., Vogel, Tiphanie P., Williams, Eleanor Anderson, Calabrese, Cassandra, Cunha, Joanne S., Fontanarosa, Joann, Gillispie‐Taylor, Miriah C., Gkrouzman, Elena, Iyer, Priyanka, Lakin, Kimberly S., Legge, Alexandra, Lo, Mindy S., Lockwood, Megan M., Sadun, Rebecca E., Singh, Namrata, Sullivan, Nancy, Tam, Herman, Turgunbaev, Marat, Turner, Amy S., and Reston, James

Published
2023
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14. Establishing core domain sets for Chronic Nonbacterial Osteomyelitis (CNO) and Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis (SAPHO): A report from the OMERACT 2020 special interest group

Author

Oliver, Melissa, Jayatilleke, Aruni, Wu, Eveline, Nuruzzaman, Farzana, Aguiar, Cassyanne, Lenert, Aleksander, Romano, Micol, Simonini, Gabriele, Shah, Samir, Hollander, Matthew, Alsaleem, Alhanouf, Fox, Emily, Theos, Alexander, Ramanan, Athimalaipet V, Schnabel, Anja, Lapidus, Sivia, Tucker, Lori, Onel, Karen, Ozen, Seza, Dedeoglu, Fatma, Ferguson, Polly J, Shea, Beverley, Akikusa, Jonathan, Hedrich, Christian, Mease, Philip, and Zhao, Yongdong

Published
2021
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17. Engaging patients and parents to improve mental health intervention for youth with rheumatological disease

Author

Fawole, Oluwatunmise A., Reed, Michelle V., Harris, Julia G., Hersh, Aimee, Rodriguez, Martha, Onel, Karen, Lawson, Erica, Rubinstein, Tamar, Ardalan, Kaveh, Morgan, Esi, Paul, Anne, Barlin, Judy, Daly, R. Paola, Dave, Mitali, Malloy, Shannon, Hume, Shari, Schrandt, Suzanne, Marrow, Laura, Chapson, Angela, Napoli, Donna, Napoli, Michael, Moyer, Miranda, Delgaizo, Vincent, Danguecan, Ashley, von Scheven, Emily, and Knight, Andrea

Published
2021
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20. Part 5: Allogeneic HSCT in refractory SJIA with lung disease; recent cases from centers in North America & Europe

Author

Grom, Alexei A., primary, Canna, Scott W., additional, Abu-Arja, Rolla F., additional, Sinha, Rashmi, additional, Peixoto, Luciana, additional, Cannizzaro, Elvira, additional, Chandrakasan, Shanmuganathan, additional, Driest, Kyla, additional, Marsh, Rebecca, additional, Neven, Bénédicte, additional, Onel, Karen, additional, Prahalad, Sampath, additional, Prockop, Susan, additional, Quartier, Pierre, additional, Roth, Johannes, additional, Schulert, Grant, additional, Silva, Juliana M.F., additional, Wall, Donna, additional, and Zeilhofer, Ulrike, additional

Published
2024
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21. Disease Course, Treatments, and Outcomes of Children With Systemic Juvenile Idiopathic Arthritis–Associated Lung Disease

Author

Huang, Yannan, primary, Sompii‐Montgomery, Laura, additional, Patti, Jessica, additional, Pickering, Alex, additional, Yasin, Shima, additional, Do, Thuy, additional, Baker, Elizabeth, additional, Gao, Denny, additional, Abdul‐Aziz, Rabheh, additional, Behrens, Edward M., additional, Canna, Scott, additional, Clark, Matthew, additional, Co, Dominic O., additional, Collins, Kathleen P., additional, Eberhard, Barbara, additional, Friedman, Monica, additional, Graham, Thomas B., additional, Hahn, Timothy, additional, Hersh, Aimee O., additional, Hobday, Patricia, additional, Holland, Michael J., additional, Huggins, Jennifer, additional, Lu, Pai‐Yue, additional, Mannion, Melissa L., additional, Manos, Cynthia K., additional, Neely, Jessica, additional, Onel, Karen, additional, Orandi, Amir B., additional, Ramirez, Andrea, additional, Reinhardt, Adam, additional, Riskalla, Mona, additional, Santiago, Laisa, additional, Stoll, Matthew L., additional, Ting, Tracy, additional, Grom, Alexei A., additional, Towe, Christopher, additional, and Schulert, Grant S., additional

Published
2023
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23. Race, Income, and Disease Outcomes in Juvenile Dermatomyositis

Author

Abramson, Leslie S., Anderson, Eleanor S., Becker, Mara L., Benham, Heather, Beukelman, Timothy, Blier, Peter R., Brunner, Hermine I., Dean, Joni, Dedeoglu, Fatma, Feldman, Brian M., Ferguson, Polly I., Goldsmith, Donald P., Gottlieb, Beth S., Graham, Thomas B., Griffin, Thomas A., Haftel, Hilary M., Higgins, Gloria C., Hollister, J.R., Hsu, Joyce J., Huttenlocher, Anna, Ilowite, Norman T., Imundo, Lisa F., Jerath, Rita S., Jung, Lawrence K., Kahn, Philip J., Kingsbury, Daniel J., Klein, Kristin E., Klein-Gitelman, Marisa S., Lapidus, Sivia K., Lehman, Thomas J.A., Lindsley, Carol B., Malloy, Michael A., McCurdy, Deborah K., Muscal, Eyal, Olson, Judyann C., O'Neil, Kathleen M., Onel, Karen, Prahalad, Sampath, Punaro, Marilynn G., Rabinovich, C. Egla, Reed, Ann M., Ringold, Sarah, Riordan, Mary Ellen, Robinson, Angela B., Rothman, Deborah, Ruth, Natasha M., Schikler, Kenneth N., Singer, Nora G., Spalding, Steven, Syed, Reema H., Torok, Kathryn S., Tress, Jenna, Vehe, Richard K., Von Scheven, Emily, Walters, Lydia M., Weiss, Jennifer E., Weiss, Pamela, White, Andrew J., Woo, Jennifer M., Yalcindag, Ali, Zemel, Lawrence S., Phillippi, Kathryn, Hoeltzel, Mark, Byun Robinson, Angela, and Kim, Susan

Published
2017
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28. Feasibility of Conducting Comparative Effectiveness Research and Validation of a Clinical Disease Activity Score for Chronic Nonbacterial Osteomyelitis

Author

Wu, Eveline Y., primary, Oliver, Melissa, additional, Scheck, Joshua, additional, Lapidus, Sivia, additional, Akca, Ummusen Kaya, additional, Yasin, Shima, additional, Stern, Sara M., additional, Insalaco, Antonella, additional, Pardeo, Manuela, additional, Simonini, Gabriele, additional, Marrani, Edoardo, additional, Wang, Xing, additional, Huang, Bin, additional, Kovalick, Leonard K., additional, Rosenwasser, Natalie, additional, Casselman, Gabriel, additional, Liau, Adriel, additional, Shao, Yurong, additional, Yang, Claire, additional, Mosa, Doaa Mosad, additional, Tucker, Lori, additional, Girschick, Hermann, additional, Laxer, Ronald M., additional, Akikusa, Jonathan D., additional, Hedrich, Christian M., additional, Onel, Karen, additional, Dedeoglu, Fatma, additional, Twilt, Marinka, additional, Ferguson, Polly J., additional, Ozen, Seza, additional, and Zhao, Yongdong, additional

Published
2023
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29. The interferon-rich skin environment regulates Langerhans cell ADAM17 to promote photosensitivity in lupus

Author

Zyulina, Victoria, primary, Li, Thomas M., additional, Seltzer, Ethan S., additional, Dacic, Marija, additional, Chinenov, Yurii, additional, Daamen, Andrea, additional, Veiga, Keila R., additional, Schwartz, Noa, additional, Oliver, David J., additional, Lora, Jose, additional, Jabbari, Ali, additional, Liu, Yong, additional, Munir, Hafsa, additional, Shipman, William D., additional, Sandoval, Marvin J., additional, Sollohub, Isabel F., additional, Ambler, William G., additional, Mishra, Bikash, additional, Taber, Sarah F., additional, Onel, Karen B., additional, Zippin, Jonathan, additional, Rashighi, Mehdi, additional, Krueger, James G., additional, Anandasabapathy, Niroshana, additional, Rogatsky, Inez, additional, Blobel, Carl P., additional, Lipsky, Peter E, additional, and Lu, Theresa, additional

Published
2023
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30. Disease Course, Treatments, and Outcomes of Children With Systemic Juvenile Idiopathic Arthritis–Associated Lung Disease

Author

Huang, Yannan, Sompii‐Montgomery, Laura, Patti, Jessica, Pickering, Alex, Yasin, Shima, Do, Thuy, Baker, Elizabeth, Gao, Denny, Abdul‐Aziz, Rabheh, Behrens, Edward M., Canna, Scott, Clark, Matthew, Co, Dominic O., Collins, Kathleen P., Eberhard, Barbara, Friedman, Monica, Graham, Thomas B., Hahn, Timothy, Hersh, Aimee O., Hobday, Patricia, Holland, Michael J., Huggins, Jennifer, Lu, Pai‐Yue, Mannion, Melissa L., Manos, Cynthia K., Neely, Jessica, Onel, Karen, Orandi, Amir B., Ramirez, Andrea, Reinhardt, Adam, Riskalla, Mona, Santiago, Laisa, Stoll, Matthew L., Ting, Tracy, Grom, Alexei A., Towe, Christopher, and Schulert, Grant S.

Abstract

Systemic juvenile idiopathic arthritis–associated lung disease (SJIA‐LD) is a life‐threatening disease complication. Key questions remain regarding clinical course and optimal treatment approaches. The objectives of the study were to detail management strategies after SJIA‐LD detection, characterize overall disease courses, and measure long‐term outcomes. This was a prospective cohort study. Clinical data were abstracted from the electronic medical record, including current clinical status and changes since diagnosis. Serum biomarkers were determined and correlated with presence of LD. We enrolled 41 patients with SJIA‐LD, 85% with at least one episode of macrophage activation syndrome and 41% with adverse reactions to a biologic. Although 93% of patients were alive at last follow‐up (median 2.9 years), 37% progressed to requiring chronic oxygen or other ventilator support, and 65% of patients had abnormal overnight oximetry studies, which changed over time. Eighty‐four percent of patients carried the HLA‐DRB1*15 haplotype, significantly more than patients without LD. Patients with SJIA‐LD also showed markedly elevated serum interleukin‐18 (IL‐18), variable C‐X‐C motif chemokine ligand 9 (CXCL9), and significantly elevated matrix metalloproteinase 7. Treatment strategies showed variable use of anti–IL‐1/6 biologics and addition of other immunomodulatory treatments and lung‐directed therapies. We found a broad range of current clinical status independent of time from diagnosis or continued biologic treatment. Multidomain measures of change showed imaging features were the least likely to improve with time. Patients with SJIA‐LD had highly varied courses, with lower mortality than previously reported but frequent hypoxia and requirement for respiratory support. Treatment strategies were highly varied, highlighting an urgent need for focused clinical trials.

Published
2024
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32. Physician practices for withdrawal of medications in inactive systemic juvenile arthritis, Childhood Arthritis and Rheumatology Research Alliance (CARRA) survey

Author

Shenoi, Susan, Nanda, Kabita, Schulert, Grant S., Bohnsack, John F., Cooper, Ashley M., Edghill, Bridget, Gillispie-Taylor, Miriah C., Goldberg, Baruch, Halyabar, Olha, Mason, Thomas G., Ronis, Tova, Schneider, Rayfel, Vehe, Richard K., Onel, Karen, and for the Childhood Arthritis and Rheumatology Research Alliance Systemic Juvenile Idiopathic Arthritis Workgroup

Published
2019
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33. Juvenile dermatomyositis disease activity is associated with the expansion of blood B and T‐cell memory subsets lacking follicular markers

Author

Gofshteyn, Jacqueline, primary, Mansfield, Leanne, additional, Spitznagle, Jacob, additional, Balasubramanian, Preetha, additional, Cardenas, Jacob, additional, Miller, Thomas, additional, Gu, Jinghua, additional, Wang, Xuan, additional, Punaro, Marilynn, additional, Fuller, Julie, additional, Nassi, Lorien, additional, Stewart, Katie, additional, Ohouo, Marina, additional, Stagnar, Cristy, additional, Baisch, Jeanine, additional, Walters, Lynnette, additional, Wang, Yuanyuan, additional, Yan, Helena, additional, Rinchai, Darawan, additional, Chaussabel, Damien, additional, Caielli, Simone, additional, Hong, Seunghee, additional, Onel, Karen, additional, Wright, Tracey, additional, and Pascual, Virginia, additional

Published
2023
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36. Feasibility of Conducting Comparative Effectiveness Research and Validation of a Clinical Disease Activity Score for Chronic Nonbacterial Osteomyelitis

Author

Wu, Eveline Y., primary, Oliver, Melissa, additional, Scheck, Joshua, additional, Lapidus, Sivia, additional, Akca, Ummusen Kaya, additional, Yasin, Shima, additional, Stern, Sara M., additional, Insalaco, Antonella, additional, Pardeo, Manuela, additional, Simonini, Gabriele, additional, Marrani, Edoardo, additional, Wang, Xing, additional, Huang, Bin, additional, Kovalick, Leonard K., additional, Rosenwasser, Natalie, additional, Casselman, Gabriel, additional, Liau, Adriel, additional, Shao, Yurong, additional, Yang, Claire, additional, Mosa, Doaa Mosad, additional, Tucker, Lori, additional, Girschick, Hermann, additional, Laxer, Ronald M., additional, Akikusa, Jonathan D., additional, Hedrich, Christian, additional, Onel, Karen, additional, Dedeoglu, Fatma, additional, Twilt, Marinka, additional, Ferguson, Polly J., additional, Ozen, Seza, additional, and Zhao, Yongdong, additional

Published
2022
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39. 2021 American College of Rheumatology Guideline for the Treatment of Juvenile Idiopathic Arthritis: Therapeutic Approaches for Oligoarthritis, Temporomandibular Joint Arthritis, and Systemic Juvenile Idiopathic Arthritis

Author

Onel, Karen B., primary, Horton, Daniel B., additional, Lovell, Daniel J., additional, Shenoi, Susan, additional, Cuello, Carlos A., additional, Angeles‐Han, Sheila T., additional, Becker, Mara L., additional, Cron, Randy Q., additional, Feldman, Brian M., additional, Ferguson, Polly J., additional, Gewanter, Harry, additional, Guzman, Jaime, additional, Kimura, Yukiko, additional, Lee, Tzielan, additional, Murphy, Katherine, additional, Nigrovic, Peter A., additional, Ombrello, Michael J., additional, Rabinovich, C. Egla, additional, Tesher, Melissa, additional, Twilt, Marinka, additional, Klein‐Gitelman, Marisa, additional, Barbar‐Smiley, Fatima, additional, Cooper, Ashley M., additional, Edelheit, Barbara, additional, Gillispie‐Taylor, Miriah, additional, Hays, Kimberly, additional, Mannion, Melissa L., additional, Peterson, Rosemary, additional, Flanagan, Elaine, additional, Saad, Nadine, additional, Sullivan, Nancy, additional, Szymanski, Ann Marie, additional, Trachtman, Rebecca, additional, Turgunbaev, Marat, additional, Veiga, Keila, additional, Turner, Amy S., additional, and Reston, James T., additional

Published
2022
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40. 2021 American College of Rheumatology Guideline for the Treatment of Juvenile Idiopathic Arthritis: Recommendations for Nonpharmacologic Therapies, Medication Monitoring, Immunizations, and Imaging

Author

Onel, Karen B., primary, Horton, Daniel B., additional, Lovell, Daniel J., additional, Shenoi, Susan, additional, Cuello, Carlos A., additional, Angeles‐Han, Sheila T., additional, Becker, Mara L., additional, Cron, Randy Q., additional, Feldman, Brian M., additional, Ferguson, Polly J., additional, Gewanter, Harry, additional, Guzman, Jaime, additional, Kimura, Yukiko, additional, Lee, Tzielan, additional, Murphy, Katherine, additional, Nigrovic, Peter A., additional, Ombrello, Michael J., additional, Rabinovich, C. Egla, additional, Tesher, Melissa, additional, Twilt, Marinka, additional, Klein‐Gitelman, Marisa, additional, Barbar‐Smiley, Fatima, additional, Cooper, Ashley M., additional, Edelheit, Barbara, additional, Gillispie‐Taylor, Miriah, additional, Hays, Kimberly, additional, Mannion, Melissa L., additional, Peterson, Rosemary, additional, Flanagan, Elaine, additional, Saad, Nadine, additional, Sullivan, Nancy, additional, Szymanski, Ann Marie, additional, Trachtman, Rebecca, additional, Turgunbaev, Marat, additional, Veiga, Keila, additional, Turner, Amy S., additional, and Reston, James T., additional

Published
2022
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41. Multicenter Study of Utility and Acceptability of Depression and Anxiety Screening in Adolescents and Young Adults With Childhood‐Onset Systemic Lupus.

Author

Rubinstein, Tamar B., Dionizovik‐Dimanovski, Marija, Davis, Alaina M., Smith, Chelsey, Faust, Lauren, Rutstein, Beth, Mitchell, Manda, Jones, Jordan T., Harris, Julia G., Rodriguez, Martha, Tesher, Melissa, Onel, Karen, Gabbay, Vilma, Stein, Ruth E. K., von Scheven, Emily, and Knight, Andrea M.

Subjects
MEDICAL screening, YOUNG adults, MENTAL health screening, SYSTEMIC lupus erythematosus, ANXIETY
Abstract

Objective: To determine the utility and acceptability for depression and anxiety screening of adolescents and young adults (AYA) with childhood‐onset systemic lupus erythematosus (cSLE) in the pediatric rheumatology setting. Methods: AYA with cSLE, ages 12–21 years, from 8 collaborating sites, were consecutively screened for depression and anxiety with the Patient Health Questionnaire‐9 (PHQ‐9) and Generalized Anxiety Disorder 7‐item scale (GAD‐7). Demographic and disease characteristics were collected, as well as patient‐reported outcome measures using the Patient Reported Outcomes Measurement Information System (PROMIS) pediatric profile‐25. Acceptability of screening was assessed with postscreening surveys completed by AYA and parents. Chi‐square and Wilcoxon rank sum tests examined the relationship between patient characteristics and history of previous screening. Spearman correlations examined relationships between screening scores, PROMIS domains, and other disease factors. Results: Among 106 AYA screened, 64 (60%) had been previously screened, 25 (24%) by general pediatricians. Thirty‐two (30%) AYA screened positive, including 24% for depression, 17% for anxiety, and 14% for suicidal ideation. Depression and anxiety symptom severity were highly correlated with increased PROMIS domain scores for fatigue and pain interference and moderately correlated with increased pain severity, decreased mobility, and decreased peer relationships. Eighty‐six percent of AYA and 95% of parents expressed comfort with screening in the pediatric rheumatology setting. Conclusion: Depression, anxiety, and suicidal ideation are common among AYA with cSLE, and symptoms are correlated with important patient‐reported outcomes. Mental health screening in the pediatric rheumatology setting was highly acceptable among AYA with cSLE and their parents. [ABSTRACT FROM AUTHOR]

Published
2023
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42. American College of Rheumatology Provisional Criteria for Clinically Relevant Improvement in Children and Adolescents With Childhood-Onset Systemic Lupus Erythematosus

Author

Brunner, Hermine I, Holland, Michael J, Beresford, Michael W, Ardoin, Stacy P, Appenzeller, Simone, Silva, Clovis A, Flores, Francisco, Goilav, Beatrice, Aydin, Pinar Ozge Avar, Wenderfer, Scott E, Levy, Deborah M, Ravelli, Angelo, Khubchandani, Raju, Avcin, Tadej, Klein-Gitelman, Marisa S, Ruperto, Nicolino, Feldman, Brian M, Ying, Jun, Battagliotti, Cristina, Brusco, Maria Isabel, Cuttica, Ruben, De Cunto, Carmen, Espada, Graciela, Farfan, Maximiliano, Garay, Stella, Marcantoni, Maria, Marcela, Alvarez, Meiorin, Silvia, Rama, Maria Elena, Russo, Ricardo, Walsh, Carolina Torre, Zamparo, Celso, Adib, Navid, Akikusa, Jonathan, Boros, Christina, Lee, Senq J, Mckay, Damien, Piper, Susan, Joos, Rik, Bica, Blanca, Campos, Leonardo, Cavalcanti, Andre, do Prado, Rogerio, Donner-Maliki, Amanda, Fernandes, Taciana, Fonseca, Adriana, de Almeida, Rozana Gasparello, Guariento, Andressa, Gusman, Catherine, Fiorot, Fernanda Jusan, Oliveira, Sheila Knupp, Len, Claudio, Campos, Lucia M Arruda, Machado, Sandra, Marques, Luciana, de Carvalho, Luciana Martins, Moulin, Rodrigo, Pedroso, Soraya, Pileggi, Gecilmara, Romanelli, Paulo Roberto S, Saad-Magalhaes, Claudia, Sakamoto, Ana, Santos, Maria Carolina, Silva, Marco Felipe, Spelling, Paulo, Sztajnbok, Flavio, Terreri, Maria, Cabral, David, Chedeville, Gaelle, Ellsworth, Janet, Huber, Adam, Tucker, Lori, Houghton, Kristin, Borzutzky, Arturo, Ladino, Mabel, Norambuena, Ximena, Eraso, Ruth, Mosquera, Angela, Velasquez, Monica, Harjacek, Miroslav, Jelusic, Marija, Hermosilla, Cecilia Coto, Dolezalova, Pavla, Nielsen, Susan, Tineo, Carmen, Alegria, Mauricio, Dressler, Frank, Foell, Dirk, Ganser, Gerd, Hinze, Claas, Hufnagel, Markus, Lutz, Thomas, Trauzeddel, Ralf, Boiu, Sorina, Trachana, Maria, Tsitsami, Elena, Cifuentes, Mayra, Orban, Ilonka, Aggarwal, Amita, Sawhney, Sujata, Aviel, Yonatan Butbul, Cimaz, Rolando, Maggio, Maria Cristina, Rumba-Rozenfelde, Ingrid, Hashad, Soad, Lim, Sern Chin, Abud, Carlos, Burgos-Vargas, Ruben, Carreno-Manjarrez, Roberto, Enciso Pelaez, Sandra, Hernandez-Huirache, Hayde, Maldonado Velazquez, Rocio, Orozco, Javier, Rodriguez-Lozano, Ana Luisa, Rojas Pacheco, Omar Ernesto, Suarez Larios, Luz Maria, Vega, Gabriel, Ramirez Miramontes, Julia Veronica, Ruiz Lopez, Ivon Karina, Kamphuis, Sylvia, Schonenberg-Meinema, Dieneke, Concannon, Anthony, Yan, Jaqueline, Jaime, Martha Jarquin, Al Abrawi, Safiya, Vega, Cynthia, Lopez-Benitez, Jorge, Estrella, Amparo Ibanez, Miraval, Tatiana, Dans, Leonia, Kimseng, Karen Joy, Opoka-Winiarska, Violetta, Rutkowska-Sak, Lidia, Smolewska, Elzbieta, Conde, Marta, Guedes, Margarida, del Valle, Enid, Quintero-Del Rio, Ana, Ailioaie, Constantin, Sparchez, Mihaela, Alekseeva, Ekaterina, Keltsev, Vladimir, Al-Mayouf, Sulaiman, Asiri, Abdurhman, Suwairi, Wafaa, Susic, Gordana, Vijatov-Djuric, Gordana, Ang, Elizabeth, Arkachaisri, Thaschawee, Boteanu, Alina-Lucica, Lopez-Robledillo, Juan Carlos, San Ildefonso, Marta Medrano, Modesto, Consuelo, Calvo, Inmaculada, Sotoca-Fernandez, Jorge, Bolt, Isabel, Vilaiyuk, Soamarat, Al-Abadi, Eslam, Baildam, Eileen, Fotis, Lampros, Pain, Clare, Pilkington, Clarissa, Abulaban, Khalid, Barbar-Smiley, Fatima, Binstadt, Bryce, Bohnsack, John, Boneparth, Alexis, Brown, Diane, Chira, Peter, Cron, Randy, Dedeoglu, Fatma, Eberhard, Anne, Gedalia, Abraham, Grom, Alexei, Henrickson, Michael, Hom, Christine, Huggins, Jennifer, Jerath, Rita, Jones, Jordan, Jung, Lawrence, Kingsbury, Daniel, Lai, Jamie, Lovell, Daniel, Nanda, Kabita, Nocton, James, Olson, Judyann, O'Neil, Kathleen, Onel, Karen, Punaro, Lynn, Reiff, Andreas, Rouster-Stevens, Kelly, Ruth, Natasha, Schikler, Ken, Schmidt, Kara Murphy, Schulert, Grant, Shaham, Bracha, Singer, Nora, Smith, Judith, Sundel, Robert, Syverson, Grant, Vega-Fernandez, Patricia, Vehe, Richard, Wagner-Weiner, Linda, Cameto, Juan, Jurado, Rosario, Maldonado, Irama, Org, Paediat Rheumatology Int Trial, Collaborative, Pediat Rheumatology, AII - Inflammatory diseases, Graduate School, Paediatric Infectious Diseases / Rheumatology / Immunology, Brunner, Hermine I., Holland, Michael J., Beresford, Michael W., Ardoin, Stacy P., Appenzeller, Simone, Silva, Clovis A., Flores, Francisco, Goilav, Beatrice, Avar Aydin, Pinar Ozge, Wenderfer, Scott E., Levy, Deborah M., Ravelli, Angelo, Khubchandani, Raju, Avcin, Tadej, Klein-Gitelman, Marisa S., Ruperto, Nicolino, Feldman, Brian M., Ying, Jun, Battagliotti, Cristina, Brusco, Maria Isabel, Cuttica, Rubén, De Cunto, Carmen, Espada, Graciela, Farfan, Maximiliano, Garay, Stella, Marcantoni, Maria, Marcela, Alvarez, Meiorin, Silvia, Rama, Maria Elena, Russo, Ricardo, Torre Walsh, Carolina, Zamparo, Celso, Adib, Navid, Akikusa, Jonathan, Boros, Christina, Lee, Senq J., Mckay, Damien, Piper, Susan, Joos, Rik, Bica, Blanca, Campos, Leonardo, Cavalcanti, André, do Prado, Rogerio, Donner-Maliki, Amanda, Fernandes, Taciana, Fonseca, Adriana, Gasparello de Almeida, Rozana, Guariento, Andressa, Gusman, Catherine, Jusan Fiorot, Fernanda, Knupp Oliveira, Sheila, Len, Claudio, Arruda Campos, Lucia M., Machado, Sandra, Marques, Luciana, Martins de Carvalho, Luciana, Moulin, Rodrigo, Pedroso, Soraya, Pileggi, Gecilmara, Romanelli, Paulo Roberto S., Saad-Magalhaes, Claudia, Sakamoto, Ana, Santos, Maria Carolina, Silva, Marco Felipe, Spelling, Paulo, Sztajnbok, Slavio, Terreri, Maria, Cabral, David, Chédeville, Gaëlle, Ellsworth, Janet, Huber, Adam, Tucker, Lori, Houghton, Kristin, Borzutzky, Arturo, Ladino, Mabel, Norambuena, Ximena, Eraso, Ruth, Mosquera, Angela, Velasquez, Monica, Harjacek, Miroslav, Jelusic, Marija, Coto Hermosilla, Cecilia, Dolezalova, Pavla, Nielsen, Susan, Tineo, Carmen, Alegria, Mauricio, Dressler, Frank, Foell, Dirk, Ganser, Gerd, Hinze, Claa, Hufnagel, Marku, Lutz, Thoma, Trauzeddel, Ralf, Boiu, Sorina, Trachana, Maria, Tsitsami, Elena, Cifuentes, Mayra, Orbán, Ilonka, Aggarwal, Amita, Sawhney, Sujata, Butbul Aviel, Yonatan, Cimaz, Rolando, Maggio, Maria Cristina, Rumba-Rozenfelde, Ingrid, Hashad, Soad, Lim, Sern Chin, Abud, Carlo, Burgos-Vargas, Ruben, Carreño-Manjarrez, Roberto, Enciso Pelaez, Sandra, Hernandez-Huirache, Hayde, Maldonado Velázquez, Rocio, Orozco, Javier, Rodriguez-Lozano, Ana Luisa, Rojas Pacheco, Omar Ernesto, Suárez Larios, Luz Maria, Vega, Gabriel, Ramírez Miramontes, Julia Verónica, Ruíz Lopez, Ivon Karina, Kamphuis, Sylvia, Schonenberg-Meinema, Dieneke, Concannon, Anthony, Yan, Jaqueline, Jarquin Jaime, Martha, Al Abrawi, Safiya, Vega, Cynthia, Lopez-Benitez, Jorge, Ibáñez Estrella, Amparo, Miraval, Tatiana, Dans, Leonia, Kimseng, Karen Joy, Opoka-Winiarska, Violetta, Rutkowska-Sak, Lidia, Smolewska, Elzbieta, Conde, Marta, Guedes, Margarida, del Valle, Enid, Quintero-Del Rio, Ana, Ailioaie, Constantin, Sparchez, Mihaela, Alekseeva, Ekaterina, Keltsev, Vladimir, Al-Mayouf, Sulaiman, Asiri, Abdurhman, Suwairi, Wafaa, Susic, Gordana, Vijatov-Djuric, Gordana, Ang, Elizabeth, Arkachaisri, Thaschawee, Boteanu, Alina-Lucica, Lopez-Robledillo, Juan Carlo, Medrano San Ildefonso, Marta, Modesto, Consuelo, Calvo, Inmaculada, Sotoca-Fernandez, Jorge, Bolt, Isabel, Vilaiyuk, Soamarat, Al-Abadi, Eslam, Baildam, Eileen, Fotis, Lampro, Pain, Clare, Pilkington, Clarissa, Abulaban, Khalid, Barbar-Smiley, Fatima, Binstadt, Bryce, Bohnsack, John, Boneparth, Alexi, Brown, Diane, Chira, Peter, Cron, Randy, Dedeoglu, Fatma, Eberhard, Anne, Gedalia, Abraham, Grom, Alexei, Henrickson, Michael, Hom, Christine, Huggins, Jennifer, Jerath, Rita, Jones, Jordan, Jung, Lawrence, Kingsbury, Daniel, Lai, Jamie, Lovell, Daniel, Nanda, Kabita, Nocton, Jame, Olson, Judyann, O’Neil, Kathleen, Onel, Karen, Punaro, Lynn, Reiff, Andrea, Rouster-Stevens, Kelly, Ruth, Natasha, Schikler, Ken, Murphy Schmidt, Kara, Schulert, Grant, Shaham, Bracha, Singer, Nora, Smith, Judith, Sundel, Robert, Syverson, Grant, Vega-Fernandez, Patricia, Vehe, Richard, Wagner-Weiner, Linda, Cameto, Juan, Jurado, Rosario, Maldonado, Irama, and Pediatrics

Subjects
medicine.medical_specialty, Outcome Assessment, Health Care/methods, Adolescent, Delphi Technique, Antirheumatic Agents/therapeutic use, Severity of Illness Index, Child health, Article, 03 medical and health sciences, 0302 clinical medicine, Rheumatology, Internal medicine, Severity of illness, Outcome Assessment, Health Care, medicine, Pediatric nephrology, Humans, Lupus Erythematosus, Systemic, skin and connective tissue diseases, Child, 030203 arthritis & rheumatology, Systemic lupus erythematosus, Lupus erythematosus, Lupus Erythematosus, Receiver operating characteristic, business.industry, Consensus conference, childhood-onset systemic lupus erythematosus, Outcome Assessment, Health Care/methods, medicine.disease, Rheumatology,Systemic lupus erythematosus,autoimmune inflammatory disease, Antirheumatic Agents, Lupus Erythematosus, Systemic/drug therapy, Systemic/drug therapy, business, Algorithms
Abstract

OBJECTIVE: To develop a Childhood Lupus Improvement Index (CHILI) as a tool to measure response to therapy in childhood-onset systemic lupus erythematosus (cSLE), with a focus on clinically relevant improvement (CRIc SLE ). METHODS: Pediatric nephrology and rheumatology subspecialists (n = 213) experienced in cSLE management were invited to define CRIc SLE and rate a total of 433 unique patient profiles for the presence/absence of CRIc SLE . Patient profiles included the following cSLE core response variables (CRVs): global assessment of patient well-being (patient-global), physician assessment of cSLE activity (MD-global), disease activity index score (here, we used the Systemic Lupus Erythematosus Disease Activity Index), urine protein-to-creatinine ratio, and Child Health Questionnaire physical summary score. Percentage and absolute changes in these cSLE- CRVs (baseline versus follow-up) were considered in order to develop candidate algorithms and validate their performance (sensitivity, specificity, area under the receiver operating characteristic curve [AUC] ; range 0-1). RESULTS: During an international consensus conference, unanimous agreement on a definition of CRIc SLE was achieved ; cSLE experts (n = 13) concurred (100%) that the preferred CHILI algorithm considers absolute changes in the cSLE- CRVs. After transformation to a range of 0-100, a CHILI score of ≥54 had outstanding accuracy for identifying CRIc SLE (AUC 0.93, sensitivity 81.1%, and specificity 84.2%). CHILI scores also reflect minor, moderate, and major improvement for values exceeding 15, 68, and 92, respectively (all AUC ≥0.92, sensitivity ≥93.1%, and specificity ≥73.4%). CONCLUSION: The CHILI is a new, seemingly highly accurate index for measuring CRI in cSLE over time. This index is useful to categorize the degree of response to therapy in children and adolescents with cSLE.

Published
2019

43. 1501 Genetics of age at systemic lupus erythematosus diagnosis

Author

Carlomagno, Raffaella, primary, Liao, Fangming, additional, Cao, Jingjing, additional, Dominguez, Daniela, additional, Gladman, Dafna D, additional, Ishimori, Mariko, additional, Jefferies, Caroline, additional, Kamen, Diane L, additional, Kamphuis, Sylvia, additional, Klein-Gitelman, Marisa S, additional, Knight, Andrea M, additional, Lee, Chia-Chi J, additional, Levy, Deborah M, additional, Onel, Karen B, additional, Paterson, Andrew D, additional, Peschken, Christine A, additional, Pope, Janet E, additional, Touma, Zahi, additional, Urowitz, Murray B, additional, Wallace, Daniel J, additional, Webber, Declan, additional, Wither, Joan E, additional, Silverman, Earl D, additional, and Hiraki, Linda T, additional

Published
2021
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44. 503 Developing a standardized steroid dosing regimen in pediatric proliferative lupus nephritis

Author

Brunner, Hermine I, primary, Rouster-Stevens, K, additional, Klein-Gitelman, Marisa S, additional, Onel, Karen, additional, Goilav, Beatrice, additional, Ruth, Natasha, additional, Qiu, Tingting, additional, Aljaberi, Najla, additional, Deng, Jianghong, additional, Laskin, Benjamin L, additional, Sagcal-Gironella, Anna Carmela P, additional, Ardoin, Stacy P, additional, Levy, Deborah M, additional, Wenderfer, Scott E, additional, and Huang, Bin, additional

Published
2021
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46. Efficacy and safety of tocilizumab in patients with polyarticular-course juvenile idiopathic arthritis: results from a phase 3, randomised, double-blind withdrawal trial

Author

Brunner, Hermine I, Ruperto, Nicolino, Zuber, Zbigniew, Keane, Caroline, Harari, Olivier, Kenwright, Andrew, Lu, Peng, Cuttica, Ruben, Keltsev, Vladimir, Xavier, Ricardo M, Calvo, Inmaculada, Nikishina, Irina, Rubio-Pérez, Nadina, Alexeeva, Ekaterina, Chasnyk, Vyacheslav, Horneff, Gerd, Opoka-Winiarska, Violetta, Quartier, Pierre, Silva, Clovis A, Silverman, Earl, Spindler, Alberto, Baildam, Eileen, Gámir, M Luz, Martin, Alan, Rietschel, Christoph, Siri, Daniel, Smolewska, Elzbieta, Lovell, Daniel, Martini, Alberto, De Benedetti, Fabrizio, Espada, Graciela, Allen, Roger, Chaitow, Jeffrey, Joos, Rik, Wouters, Carine, Knupp, Sheila, Sztajnbok, Flavio, Cabral, David, Houghton, Kristin, Roth, Johannes, Schmeling, Heinrike, Job-Deslandre, Chantal, Jorgensen, Christian, Paut, Isabelle Kone, Minden, Kirsten, Weller-Heinemann, Frank, Gerloni, Valeria, Zulian, Francesco, Burgos-Vargas, Ruben, Salazar, Carolina Duarte, Solis-Vallejo, Eunice, Calvo, Armando, Chavez, José, Zavaler, Manuel Ferrandiz, Gruenpeter, Anna, Kobusinska, Katarzyna, Sarychev, Alexey, Zholobova, Elena, Ramanan, Athimalaipet, Woo, Patricia, Goodman, Steven, Gedalia, Abraham, Kimura, Yukiko, Onel, Karen, and Schikler, Kenneth

Published
2015
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47. Proceedings from the 4th NextGen Therapies for SJIA and MAS virtual symposium held February 13–14, 2022.

Author

Sinha, Rashmi, De Benedetti, Fabrizio, Grom, Alexei A., on behalf of the participants listed below, Abu-Arja, Rolla F., Behrens, Edward, Brunner, Hermine, Canna, Scott W., Cannizzaro, Elvira, Chandrakasan, Shanmuganathan, Cron, Randy, Driest, Kyla, Kimura, Yukiko, Leptak, Christopher, Lovell, Daniel J., Marsh, Rebecca, Neven, Bénédicte, Nigrovic, Peter A., Nikolov, Nikolay, and Onel, Karen

Subjects
MACROPHAGE activation syndrome, PULMONARY alveolar proteinosis, JUVENILE idiopathic arthritis, DISEASE risk factors, JUVENILE diseases
Abstract

The article discusses the proceedings of the 4th NextGen Therapies for SJIA and MAS virtual symposium, which aimed to identify barriers to clinical research and develop new strategies for refractory Systemic Juvenile Idiopathic Arthritis (SJIA) and Macrophage Activation Syndrome (MAS). SJIA is a severe subtype of chronic childhood arthropathy characterized by systemic immune activation, while MAS is a potentially fatal episode of systemic hyperinflammation. The symposium focused on the development of new therapies for SJIA-associated lung disease (SJIA-LD) and MAS, as well as alternative designs for clinical trials and the use of biomarkers for treatment response. The article also highlights the need for improved outcome measures that capture the systemic component of SJIA, particularly the lung disease. [Extracted from the article]

Published
2023
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48. The interferon-rich skin environment regulates Langerhans cell ADAM17 to promote photosensitivity in lupus

Author

Li, Thomas M., primary, Zyulina, Victoria, additional, Seltzer, Ethan S., additional, Dacic, Marija, additional, Chinenov, Yurii, additional, Daamen, Andrea R., additional, Veiga, Keila R., additional, Schwartz, Noa, additional, Oliver, David J., additional, Lora, Jose, additional, Jabbari, Ali, additional, Liu, Yong, additional, Shipman, William D., additional, Ambler, William G., additional, Taber, Sarah F., additional, Onel, Karen B., additional, Zippin, Jonathan H., additional, Rashighi, Mehdi, additional, Krueger, James G., additional, Anandasabapathy, Niroshana, additional, Rogatsky, Inez, additional, Blobel, Carl P., additional, Lipsky, Peter E., additional, and Lu, Theresa T., additional

Published
2021
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49. International Consensus for the Dosing of Corticosteroids in Childhood-Onset Systemic Lupus Erythematosus With Proliferative Lupus Nephritis

Author

Chalhoub, Nathalie E., Wenderfer, Scott E., Levy, Deborah M., Rouster-Stevens, Kelly, Aggarwal, Amita, Savani, Sonia I., Ruth, Natasha M., Arkachaisri, Thaschawee, Qiu, Tingting, Merritt, Angela, Onel, Karen, Goilav, Beatrice, Khubchandani, Raju P., Deng, Jianghong, Fonseca, Adriana R., Ardoin, Stacy P., Ciurtin, Coziana, Kasapcopur, Ozgur, Jelusic, Marija, Huber, Adam M., Ozen, Seza, Klein-Gitelman, Marisa S., Appenzeller, Simone, Cavalcanti, André, Fotis, Lampros, Lim, Sern Chin, Silva, Rodrigo M., Miramontes, Julia Ramírez, Rosenwasser, Natalie L., Saad-Magalhaes, Claudia [UNESP], Schonenberg-Meinema, Dieneke, Scott, Christiaan, Silva, Clovis A., Enciso, Sandra, Terreri, Maria T., Torres-Jimenez, Alfonso-Ragnar, Trachana, Maria, Al-Mayouf, Sulaiman M., Devarajan, Prasad, Huang, Bin, Brunner, Hermine I., Abulaban, Khalid, Aguiar, Cassyanne, Ahn, Sun-Young, Akoghlanian, Shoghik, Al-Abrawi, Safiya, Aljaberi, Najla, Alperin, Risa, Angeles-Han, Sheila, Ardalan, Kaveh, Bader-Meunier, Brigitte, Balboni, Imelda, Barbar-Smiley, Fatima, Baxter, Sarah, Beary, John, Boneparth, Alexis, Brakeman, Paul, Bridges, John, Burgos-Vargas, Ruben, Cabral, David A., Cameto, Juan, Carter, Caitlin, Chang, Joyce, Chédeville, Gaëlle, Chhakchhuak, Christine, Chiraseveenuprapund, Peter, Cifuentes Alvarado, Mayra, Concannon, Anthony, Cooper, Jennifer, Cron, Randy, De Carvalho, Luciana Martins, De Quattro, Kimberly, De Ranieri, Deirdre, Dizon, Brian, Donnelly Wrigley, Catherine, Duong, Minh Dien, Eberhard, Anne, Ede, Kaleo, Edelheit, Barbara, Edens, Cuoghi, Espada, Graciela, Farhey, Yolanda, Flores, Francisco, Fritz, Deborah, Ganguli, Suhas, Gilbert, Mileka, Gittar, Patsy, Greenbaum, Larry, Grom, Alexei, Gulati, Gaurav, Harry, Onengiya, Hayward, Kristen, Henrickson, Michael, Hersh, Aimee, Hiraki, Linda, Hiskey, Megan, Hoffmann, Sarah, Hollander, Matthew, Hom, Christine, Houk, Lawrence, Houk, J. Brian, Hsieh, Elena W.Y., Hsu, Joyce, Jensen, Paul, Joos, Rik, Jurado, Rosario, Jusan Fiorot, Fernanda, Kallash, Mahmoud, Kamphuis, Sylvia, Keltsev, Vladimir, Khanna, Surabhi, Kim, Susan, Kimseng, Karen Joy, Knight, Andrea, Kunder, Rebecca, Lai, Jamie, Laskin, Benjamin, Lewandowski, Laura, Lim, Lily, Linda, Wagner-Weiner, Lo, Mindy, Lovell, Daniel, Luggen, Michael, Madison, Jacqueline, Mansuri, Asif, Martin, Lorena, Mason, Sherene, Miller, Michael, Mina, Rina, Mohammed, Abdul, Moncrieffe, Halima, Moorthy, Lakshmi, Morgan, Esi, Mosquera, Angela, Muntel, Emily, Muscal, Eyal, Myones, Barry, Nocton, James, Ogbu, Ekemini, Okamura, Daryl, Olson, Judyann, Orrock, Janet, Paim-Marques, Luciana, Pain, Clare, Park, Catherine, Patel, Pooja, Pereira, Maria, Prado, Rogerio do, Radhakrishna, Suhas, Rheault, Michelle, Ridgway, William, Riskalla, Mona, Ronis, Tova, Sadun, Rebecca, Sagcal-Gironella, Anna Carmela, Santos, Maria carolina, Schikler, Kenneth, AL Suwairi, Wafaa, Siddiqi, Nabeela, Silva, Marco Felipe, Singh-Grewal, Davinder, Smitherman, Emily, Smolewska, Elzbieta, Son, Mary Beth, Srinivasalu, Hemalatha, Sule, Sangeeta, Susic, Gordana, Syed, Reema, Thatayatikom, Akaluck, Ting, Tracy, Toth, Mary, Turnier, Jessica, Vashisht, Priyanka, Vega Fernandez, Patricia, Velasquez, Monica, von Scheven, Emily, Wahezi, Dawn, Ware, Avis, Wu, Eveline, Yan, Jacqueline, Yildirim-Toruner, Cagri, Zamparo, Celso, Zhang, Yujuan, Lawson, Erica, Graduate School, Paediatric Infectious Diseases / Rheumatology / Immunology, AII - Inflammatory diseases, University of Cincinnati College of Medicine, Baylor College of Medicine, The Hospital for Sick Children and The University of Toronto, Emory University and Children's Healthcare of Atlanta, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Medical University of South Carolina, KK Women's and Children's Hospital, Cincinnati Children's Hospital Medical Center, Hospital for Special Surgery, Albert Einstein College of Medicine, SRCC Children's Hospital, Capital Medical University and National Center for Children's Health, Universidade Federal do Rio de Janeiro (UFRJ), Nationwide Children's Hospital, University College London, Istanbul University-Cerrahpasa, University of Zagreb School of Medicine, IWK Health Centre and Dalhousie University, Hacettepe University, Northwestern University Feinberg School of Medicine and Ann & Robert H. Lurie Children's Hospital of Chicago, Universidade Estadual de Campinas (UNICAMP), Universidade Federal de Pernambuco (UFPE), National and Kapodistian University of Athens, University Teknologi MARA, Instituto Mexicano del Seguro Social, University of Washington and Seattle Children's Hospital, Universidade Estadual Paulista (UNESP), Amsterdam University Medical Center, Red Cross War Memorial Children's Hospital and University of Cape Town, Universidade de São Paulo (USP), Hospital de la Beneficencia Española, Universidade Federal de São Paulo (UNIFESP), National Medical Center La Raza, Aristotle University of Thessaloniki, King Faisal Specialist Hospital and Research Center and Alfaisal University, and University of Cincinnati College of Medicine and Cincinnati Children's Hospital Medical Center

Subjects
Male, medicine.medical_specialty, Adolescent, medicine.drug_class, Immunology, Lupus nephritis, Renal function, Article, Rheumatology, childhood-onset systemic lupus erythematosus, corticosteroids, lupus nephritis, treatment, Internal medicine, Biopsy, Immunology and Allergy, Medicine, Humans, Lupus Erythematosus, Systemic, Dosing, Age of Onset, Child, Glucocorticoids, Retrospective Studies, Kidney, Proteinuria, medicine.diagnostic_test, business.industry, food and beverages, medicine.disease, Lupus Nephritis, Clinical trial, medicine.anatomical_structure, Corticosteroid, Female, medicine.symptom, business
Abstract

Made available in DSpace on 2022-04-28T19:49:00Z (GMT). No. of bitstreams: 0 Previous issue date: 2022-02-01 Arthritis Foundation Institute of Clinical and Translational Sciences Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq) Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP) National Institute of Arthritis and Musculoskeletal and Skin Diseases National Institute of Diabetes and Digestive and Kidney Diseases National Center for Advancing Translational Sciences 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. University of Cincinnati College of Medicine Baylor College of Medicine The Hospital for Sick Children and The University of Toronto Emory University and Children's Healthcare of Atlanta Sanjay Gandhi Postgraduate Institute of Medical Sciences Medical University of South Carolina KK Women's and Children's Hospital Cincinnati Children's Hospital Medical Center Hospital for Special Surgery Albert Einstein College of Medicine SRCC Children's Hospital Capital Medical University and National Center for Children's Health Universidade Federal do Rio de Janeiro Nationwide Children's Hospital University College London Cerrahpasa Medical School Istanbul University-Cerrahpasa University of Zagreb School of Medicine IWK Health Centre and Dalhousie University Hacettepe University Northwestern University Feinberg School of Medicine and Ann & Robert H. Lurie Children's Hospital of Chicago University of Campinas Hospital das Clínicas da Universidade Federal de Pernambuco National and Kapodistian University of Athens University Teknologi MARA Instituto Mexicano del Seguro Social University of Washington and Seattle Children's Hospital São Paulo State University Amsterdam University Medical Center Red Cross War Memorial Children's Hospital and University of Cape Town Universidade de São Paulo Hospital de la Beneficencia Española Universidade Federal de São Paulo National Medical Center La Raza Aristotle University of Thessaloniki King Faisal Specialist Hospital and Research Center and Alfaisal University University of Cincinnati College of Medicine and Cincinnati Children's Hospital Medical Center São Paulo State University CNPq: 303422/2015-7 FAPESP: FAPESP 2015/03756-4 National Institute of Arthritis and Musculoskeletal and Skin Diseases: P30-AR-076316 National Institute of Diabetes and Digestive and Kidney Diseases: P50-DK-096418 National Institute of Arthritis and Musculoskeletal and Skin Diseases: R34-AR-071651 National Center for Advancing Translational Sciences: T32-AR-050958

Published
2021

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