Letter
Medicine, General & Internal
Brian W. McCrindle, Ashraf S. Harahsheh, Ryan Handoko, Geetha Raghuveer, Michael A. Portman, Michael Khoury, Jane W. Newburger, Simon Lee, Supriya S. Jain, Manaswitha Khare, Nagib Dahdah, Cedric Manlhiot
Summary: The severity of multisystem inflammatory syndrome in children (MIS-C) has decreased with each subsequent SARS-CoV-2 variant, but critical illness remains prevalent in hospitalized patients with MIS-C.
NEW ENGLAND JOURNAL OF MEDICINE
(2023)
Article
Oncology
Samantha D. Martin, Elizabeth S. Davis, Chen Dai, Lauren H. Boal, Brook Araya, Julienne Brackett, David Dickens, Alissa Kahn, Isaac Martinez, Archana Sharma, Carla Schwalm, Paibel Aguayo-Hiraldo, Smita Bhatia, Jennifer M. Levine, Emily E. Johnston, Julie A. Wolfson
Summary: This study examined the risk of post-COVID-19 multisystem inflammatory syndrome in children (MIS-C) among pediatric oncology patients and identified factors associated with MIS-C. The results showed that children with hematologic cancer, noncancer comorbidities, and previous cancer treatment were more likely to develop MIS-C. The clinical course of MIS-C in these children was found to be more severe.
Article
Immunology
Eisha Jain, Jeffrey R. Donowitz, Elizabeth Aarons, Beth C. Marshall, Michael P. Miller
Summary: This article presents two cases of MIS-C occurring after SARS-CoV-2 vaccination, with one patient showing evidence of recent SARS-CoV-2 infection. The findings suggest that vaccination may modulate the pathogenesis of MIS-C.
EMERGING INFECTIOUS DISEASES
(2022)
Article
Medicine, General & Internal
Mary Beth F. Son, Laura Berbert, Cameron Young, Johnathan Dallas, Margaret Newhams, Sabrina Chen, Stacy P. Ardoin, Matthew L. Basiaga, Susan P. Canny, Hillary Crandall, Sanjeev Dhakal, Anita Dhanrajani, Anna Carmela P. Sagcal-Gironella, Charlotte Hobbs, Livie Huie, Karen James, Madelyn Jones, Susan Kim, Geraldina Lionetti, Melissa L. Mannion, Eyal Muscal, Sampath Prahalad, Grant S. Schulert, Kristen Sexson Tejtel, D. Sofia Villacis-Nunez, Eveline Y. Wu, Laura D. Zambrano, Angela P. Campbell, Manish M. Patel, Adrienne G. Randolph
Summary: This multicenter cohort study evaluated the clinical characteristics and outcomes of postdischarge glucocorticoid treatment in children with multisystem inflammatory syndrome in children (MIS-C). The study found that many patients with MIS-C continued glucocorticoid treatment after discharge, but clinical outcomes were similar in patients prescribed shorter courses. Outpatient weight gain was common, but readmission for cardiovascular dysfunction was infrequent. The findings suggest the need for optimizing postdischarge glucocorticoid regimens for patients with MIS-C.
Review
Rheumatology
Jordan E. Roberts, Lauren A. Henderson
Summary: The diagnosis and treatment of multisystem inflammatory syndrome in children (MIS-C) associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) can be challenging due to varied clinical presentations, but prompt recognition and intervention are crucial for overall reassuring prognosis.
CURRENT OPINION IN RHEUMATOLOGY
(2021)
Article
Virology
Mariawy Riollano-Cruz, Esra Akkoyun, Eudys Briceno-Brito, Shanna Kowalsky, James Reed, Roberto Posada, Emilia Mia Sordillo, Michael Tosi, Rebecca Trachtman, Alberto Paniz-Mondolfi
Summary: Following the outbreak of the COVID-19 pandemic in 2020, children have been reported to develop a systemic inflammatory syndrome related to the virus. The pediatric patients mainly come from Hispanic/Latino and Black/African American backgrounds in New York City.
JOURNAL OF MEDICAL VIROLOGY
(2021)
Article
Medicine, General & Internal
Anuradha Rajamanickam, Pavan Kumar Nathella, Aishwarya Venkataraman, Bindu Dasan, Sulochana Putlibai, Shaik Fayaz Ahamed, Nandhini Selvaraj, Kalaimaran Sadasivam, Balasubramanian Sundaram, Thomas B. Nutman, Subash Babu
Summary: This cross-sectional study suggests that multisystem inflammatory syndrome in children (MIS-C) and COVID-19 in children are associated with the hyperactivation of the complement system. The findings of this study may provide new avenues for research and treatment of MIS-C and COVID-19.
Article
Immunology
Timothy F. Spracklen, Simon C. Mendelsohn, Claire Butters, Heidi Facey-Thomas, Raphaella Stander, Debbie Abrahams, Mzwandile Erasmus, Richard Baguma, Jonathan Day, Christiaan Scott, Liesl J. Zuehlke, George Kassiotis, Thomas J. Scriba, Kate Webb
Summary: This study investigated the expression of immune-related genes in South African MIS-C patients and controls and identified a unique gene signature of MIS-C in South African children. The up-regulation of interleukin-1 and pyroptosis pathway genes highlights the role of the innate immune system in MIS-C. IL-27 expression may distinguish MIS-C from other conditions in this setting.
FRONTIERS IN IMMUNOLOGY
(2022)
Review
Medicine, General & Internal
Pragna Patel, Jennifer DeCuir, Joseph Abrams, Angela P. Campbell, Shana Godfred-Cato, Ermias D. Belay
Summary: This study summarized the descriptive epidemiology and clinical characteristics of multisystem inflammatory syndrome in adults (MIS-A), indicating that MIS-A is a serious hyperinflammatory condition that typically occurs approximately 4 weeks after onset of acute COVID-19, with most patients showing elevated markers of inflammation and a positive SARS-CoV-2 serologic finding.
Review
Virology
Chih-Cheng Lai, Chi-Kuei Hsu, Shun-Chung Hsueh, Muh-Yong Yen, Wen-Chien Ko, Po-Ren Hsueh
Summary: Following the emergence of multisystem inflammatory syndrome in children (MIS-C), a similar clinical scenario called multisystem inflammatory syndrome in adults (MIS-A) has been observed. Although the prevalence of MIS-A is low and likely underdiagnosed, it can be associated with high mortality. MIS-A is a multisystem disease that affects the cardiovascular, respiratory, gastrointestinal, dermatologic, hematologic, and neurologic systems. The diagnosis of MIS-A requires clinical manifestations, laboratory evidence of inflammation, and SARS-CoV-2 infection. The appropriate treatment for MIS-A is still uncertain, but anti-inflammatory agents like intravenous immunoglobulin and corticosteroids are commonly used. Further research is needed to determine the true prevalence, pathogenesis, and effective treatment for MIS-A.
JOURNAL OF MEDICAL VIROLOGY
(2023)
Article
Pediatrics
Maninder Dhaliwal, Veena Raghunathan, Prabhat Maheshwari, Krishan Chugh, Hari Pal, Mukul Satija, Navin Bhatia, Pooja Sharma, Manish Singh, Sunit C. Singhi
Summary: This retrospective study evaluated the clinical features, severity, laboratory findings, and outcomes of children with multisystem inflammatory syndrome (MIS-C) secondary to SARS-CoV-2 infection. The study found that MIS-C should be considered in febrile children, even without a history of documented SARS-CoV-2 infection/contact. High NT-proBNP and thrombocytopenia were identified as significant risk factors for severe MIS-C.
INDIAN JOURNAL OF PEDIATRICS
(2022)
Article
Allergy
Janet Chou, Craig D. Platt, Saddiq Habiballah, Alan A. Nguyen, Megan Elkins, Sabrina Weeks, Zachary Peters, Megan Day-Lewis, Tanya Novak, Myriam Armant, Lucinda Williams, Shira Rockowitz, Piotr Sliz, David A. Williams, Adrienne G. Randolph, Raif S. Geha
Summary: MIS-C is a pediatric complication of severe acute respiratory syndrome coronavirus 2 infection characterized by multiorgan inflammation and cardiovascular dysfunction. Genetic risk factors such as variants in SOCS1, XIAP, and CYBB have been identified in patients with MIS-C, leading to an inflammatory immune cell transcriptome enriched in specific pathways even after recovery. This suggests a potential role for next-generation sequencing in identifying monogenic susceptibility to inflammation in previously healthy children with MIS-C.
JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY
(2021)
Article
Immunology
Juan Carlos Ruiz-Rodriguez, Luis Chiscano-Camon, Clara Palmada, Adolf Ruiz-Sanmartin, Marina Garcia-de-Acilu, Erika Plata-Menchaca, Janire Perurena-Prieto, Manuel Hernandez-Gonzalez, Marcos Perez-Carrasco, Pere Soler-Palacin, Ricard Ferrer
Summary: MIS-C associated with COVID-19 is characterized by hypercytokinemia leading to overwhelming inflammation. The use of a hemadsorption device as part of supportive treatment for cytokine storm has been described.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Dermatology
Sergey Rekhtman, Rachel Tannenbaum, Andrew Strunk, Morgan Birabaharan, Shari Wright, Amit Garg
Summary: This study provides a descriptive analysis of mucocutaneous disease in children with COVID-19 and MIS-C, highlighting its common occurrence and potential for predicting a less severe clinical course in patients with rash. None of the MIS-C patients met criteria for Kawasaki disease.
JOURNAL OF THE AMERICAN ACADEMY OF DERMATOLOGY
(2021)
Article
Immunology
Joseph Y. Abrams, Ermias D. Belay, Shana Godfred-Cato, Angela P. Campbell, Laura D. Zambrano, Amber Kunkel, Allison D. Miller, Michael J. Wu, Lu Meng, Ami B. Shah, Matthew E. Oster
Summary: This study shows that standard treatments for MIS-C patients, including IVIG, steroids, and antiplatelet medication, have become increasingly utilized. Older patients and non-Hispanic Black patients were more likely to receive additional treatments.
CLINICAL INFECTIOUS DISEASES
(2022)
Article
Rheumatology
Stefanie D. Wade, Jason A. Freed, Vasileios C. Kyttaris, Staci Saunders, Jonathan S. Hausmann
Summary: This study aimed to evaluate the use of active learning strategies in graduate medical education through a faculty development workshop and transitioning to a virtual flipped classroom. The results showed an increase in active learning scores and high faculty satisfaction within the Introductory Rheumatology Curriculum.
ARTHRITIS CARE & RESEARCH
(2023)
Article
Rheumatology
Sarah Ringold, Anne C. Dennos, Yukiko Kimura, Timothy Beukelman, Peter Shrader, Thomas A. Phillips, Melanie Kohlheim, Laura E. Schanberg, Rae S. M. Yeung, Daniel B. Horton
Summary: The outcomes of disease recapture in children with well-controlled JIA after medication discontinuation were characterized in this study. The study found that approximately half of the patients experienced a flare within 6 months. The use of biologic drugs and a history of flares were associated with increased odds of successful recapture, while limited range of motion in the joints was associated with decreased odds.
ARTHRITIS CARE & RESEARCH
(2023)
Article
Rheumatology
Emily A. Smitherman, Rouba A. Chahine, Nicole Bitencourt, A. K. M. Fazlur Rahman, Erica F. Lawson, Joyce C. Chang
Summary: This study evaluates the care utilization and outcomes of young adults with JIA, and explores factors associated with complete transfer to adult rheumatology. The study finds that respondents with complete transfer to adult rheumatology are slightly older and report higher disease activity, morning stiffness, and pain. Biologic use is high and does not differ by transfer status. Respondents who completed the transfer are more likely to have private insurance and be pursuing postsecondary education. Majority of the cohort reports problems with pain or discomfort and anxiety or depression.
JOURNAL OF RHEUMATOLOGY
(2023)
Article
Rheumatology
Manuel F. Ugarte-Gil, Graciela S. Alarcon, Andrea M. Seet, Zara Izadi, Anna D. Montgomery, Ali Duarte-Garcia, Emily L. Gilbert, Maria O. Valenzuela-Almada, Leanna Wise, Jeffrey A. Sparks, Tiffany Y. -T. Hsu, Kristin M. D'Silva, Naomi J. Patel, Emily Sirotich, Jean W. Liew, Jonathan S. Hausmann, Paul Sufka, Rebecca Grainger, Suleman Bhana, Zachary Wallace, Lindsay Jacobsohn, Anja Strangfeld, Elsa F. Mateus, Kimme L. Hyrich, Laure Gossec, Loreto Carmona, Saskia Lawson-Tovey, Lianne Kearsley-Fleet, Martin Schaefer, Pedro M. Machado, Philip C. Robinson, Milena Gianfrancesco, Jinoos Yazdany
Summary: A study on individuals with SLE in the US found that Black and Hispanic patients had higher odds of experiencing more severe COVID-19 outcomes compared to White patients, which may be attributed to socioeconomic and health disparities.
ARTHRITIS CARE & RESEARCH
(2023)
Article
Rheumatology
Emily A. Smitherman, Rouba A. Chahine, Timothy Beukelman, Laura B. Lewandowski, A. K. M. Fazlur Rahman, Scott E. Wenderfer, Jeffrey R. Curtis, Aimee O. Hersh
Summary: The objective of this study was to characterize short-term kidney status and describe variation in early care utilization in pediatric systemic lupus erythematosus (cSLE) patients with nephritis. The study found that male sex and older age at cSLE diagnosis were associated with abnormal short-term kidney status. Furthermore, there was substantial variation in contemporary medication use for pediatric lupus nephritis between pediatric rheumatology centers.
ARTHRITIS CARE & RESEARCH
(2023)
Editorial Material
Medicine, General & Internal
Stacy A. Kahn, Maureen M. Leonard, Sjirk J. Westra, Jonathan S. Hausmann, Sarah B. Mueller
NEW ENGLAND JOURNAL OF MEDICINE
(2023)
Article
Immunology
Amelie M. Jule, Ki Pui Lam, Maria Taylor, Kacie J. Hoyt, Kevin Wei, Maria Gutierrez-Arcelus, Siobhan M. Case, Mia Chandler, Margaret H. Chang, Ezra M. Cohen, Fatma Dedeoglu, Olha Halyabar, Jonathan Hausmann, Melissa M. Hazen, Erin Janssen, Jeffrey Lo, Mindy S. Lo, Esra Meidan, Jordan E. Roberts, Holly Wobma, Mary Beth F. Son, Robert P. Sundel, Pui Y. Lee, Peter T. Sage, Talal A. Chatila, Peter A. Nigrovic, Deepak A. Rao, Lauren A. Henderson
Summary: Tph cells play a crucial role in driving B cell maturation and antibody production in seropositive rheumatoid arthritis. In this study, the researchers investigated T cell-B cell interactions in juvenile oligoarthritis and found dysregulated Tph cells promoting B cell antibody production in the synovial fluid of patients with antinuclear antibodies. The single-cell transcriptomics analysis revealed the upregulation of B cell help-associated genes in Tph cells from ANA-positive patients. These findings highlight the central role of disordered T cell-help to B cells in autoantibody-positive arthritides.
FRONTIERS IN IMMUNOLOGY
(2023)
Article
Rheumatology
Holly Wobma, Sage R. Arvila, Maria L. Taylor, Ki Pui Lam, Marina Ohashi, Catherine Gebhart, Helene Powers, Siobhan Case, Mia T. Chandler, Margaret H. Chang, Ezra Cohen, Megan Day-Lewis, Martha P. Fishman, Olha Halyabar, Jonathan S. Hausmann, Melissa M. Hazen, Pui Y. Lee, Mindy S. Lo, Esra Meidan, Jordan E. Roberts, Mary Beth F. Son, Robert P. Sundel, Fatma Dedeoglu, Peter A. Nigrovic, Alicia Casey, Joyce Chang, Lauren A. Henderson
Summary: This study retrospectively evaluated the incidence and risk factors for eosinophilia and lung disease in systemic JIA patients exposed to IL-1/IL-6 inhibitors. The results showed that eosinophilia is common in JIA patients using these inhibitors and may be associated with severe disease and lung manifestations in systemic JIA.
ARTHRITIS CARE & RESEARCH
(2023)
Article
Rheumatology
Jordan E. Roberts, Cordelia Burn, Rebecca E. Sadun, Emily A. Smitherman, Scott E. Wenderfer, Mary Beth F. Son
Summary: This study aimed to evaluate the indications for belimumab use and assess the changes in oral glucocorticoid doses and disease activity scores in children with childhood-onset systemic lupus erythematosus (cSLE) after belimumab initiation. The results showed that in children treated with belimumab for more than 1 year, daily oral glucocorticoid doses significantly decreased and disease activity scores remained stable.
Article
Rheumatology
Holly Wobma, Erin Janssen
Summary: Regulatory T cells (Tregs) are essential for immune tolerance, and disorders of Tregs can lead to immune dysregulation. This review focuses on monogenic inborn errors of immunity (IEIs) affecting Treg development, stability, and function, leading to autoimmune, atopic, and infectious manifestations.
RHEUMATIC DISEASE CLINICS OF NORTH AMERICA
(2023)
Editorial Material
Rheumatology
Erin Janssen
RHEUMATIC DISEASE CLINICS OF NORTH AMERICA
(2023)
Article
Rheumatology
R. E. Borgia, M. J. Gurka, S. L. Filipp, M. Elder, M. Cardel, N. J. Shiff, CARRA Registry Investigators
Summary: This study examines the connection between race/ethnicity and the Patient-Reported Outcomes Measurement Information System (PROMIS) in childhood-onset systemic lupus erythematosus (cSLE) patients. The study found that PROMIS global health was lower among multiethnic cSLE patients compared to the general pediatric population, but there was no significant association between race/ethnicity and PROMIS scores.
CLINICAL AND EXPERIMENTAL RHEUMATOLOGY
(2023)
Article
Rheumatology
Pedro M. Machado, Martin Schaefer, Satveer K. Mahil, Jean Liew, Laure Gossec, Nick Dand, Alexander Pfeil, Anja Strangfeld, Anne Constanze Regierer, Bruno Fautrel, Carla Gimena Alonso, Carla G. S. Saad, Christopher E. M. Griffiths, Claudia Lomater, Corinne Miceli-Richard, Daniel Wendling, Deshire Alpizar Rodriguez, Dieter Wiek, Elsa F. Mateus, Emily Sirotich, Enrique R. Soriano, Francinne Machado Ribeiro, Felipe Omura, Frederico Rajao Martins, Helena Santos, Jonathan Dau, Jonathan N. Barker, Jonathan Hausmann, Kimme L. Hyrich, Lianne Gensler, Ligia Silva, Lindsay Jacobsohn, Loreto Carmona, Marcelo M. Pinheiro, Marcos David Zelaya, Maria de los Angeles Severina, Mark Yates, Maureen Dubreuil, Monique Gore-Massy, Nicoletta Romeo, Nigil Haroon, Paul Sufka, Rebecca Grainger, Rebecca Hasseli, Saskia Lawson-Tovey, Suleman Bhana, Thao Pham, Tor Olofsson, Wilson Bautista-Molano, Zachary S. Wallace, Zenas Z. N. Yiu, Jinoos Yazdany, Philip C. Robinson, Catherine H. Smith
Summary: This study investigated factors associated with severe COVID-19 in patients with psoriasis, psoriatic arthritis, and axial spondyloarthritis. The study found that age, sex, comorbidities, disease activity, and glucocorticoid use were associated with the severity of COVID-19. Additionally, later pandemic time periods, psoriasis, and the use of TNFi, IL17i, and IL-23i/IL-12+23i were associated with a reduced risk of severe COVID-19.
ANNALS OF THE RHEUMATIC DISEASES
(2023)
Article
Medicine, Research & Experimental
Zhengping Huang, Kailey E. Brodeur, Liang Chen, Yan Du, Holly Wobma, Evan E. Hsu, Meng Liu, Joyce C. Chang, Margaret H. Chang, Janet Chou, Megan Day-Lewis, Fatma Dedeoglu, Olha Halyabar, James A. Lederer, Tianwang Li, Mindy S. Lo, Meiping Lu, Esra Meidan, Jane W. Newburger, Adrienne G. Randolph, Mary Beth, Robert P. Sundel, Maria L. Taylor, Huaxiang Wu, Qing Zhou, Scott W. Canna, Kevin Wei, Lauren A. Henderson, Peter A. Nigrovic, Pui Y. Lee
Summary: This study investigated the immunologic characteristics of macrophage activation syndrome (MAS) associated with Still's disease (SD) using RNA sequencing and single-cell RNA sequencing (scRNA-Seq). The results showed that MAS is characterized by excessive production of IFN-I, which, in synergy with IL-15, generates CD38+HLA-DR+ cycling lymphocytes that produce IFN-gamma.
JOURNAL OF CLINICAL INVESTIGATION
(2023)