4.6 Article

Favorable Outcome of Juvenile Dermatomyositis Treated without Systemic Corticosteroids

Journal

JOURNAL OF PEDIATRICS
Volume 156, Issue 2, Pages 302-307

Publisher

MOSBY-ELSEVIER
DOI: 10.1016/j.jpeds.2009.09.008

Keywords

-

Categories

Funding

  1. NIAMS NIH HHS [K23 AR053202-02, K23 AR053202] Funding Source: Medline

Ask authors/readers for more resources

Objective To describe the course of patients with juvenile dermatomyositis (JDM) treated effectively without systemic corticosteroids. Study design A retrospective study of 38 patients with JDM treated at a tertiary care children's hospital identified 8 patients who had never received corticosteroids. Disease presentation and course, pharmacologic, and ancillary treatments were recorded. Results Patients in the no corticosteroid group were followed for a median of 2.8 years (range, 2.1 to 9.5 years). Treatment was primarily with intravenous immunoglobulin (IVIG) (75%) and methotrexate (50%), with favorable response in all. No serious treatment complications were observed; headaches were reported by 3 patients receiving IVIG. Two patients had a myositis flare after discontinuing all medications for more than 1 year; complete resolution of symptoms was observed after either 1 or 2 further doses of IVIG. Two patients had calcinosis ( at 1 and 9 years of disease); however, no patient had joint contractures, muscle atrophy, lipodystrophy, or functional limitations. Conclusions Systemic corticosteroids can be avoided in a select group of patients with JDM. Alternative agents such as methotrexate and IVIG may be prescribed to effectively treat JDM and prevent complications. (J Pediatr 2010; 156: 302-7).

Authors

I am an author on this paper
Click your name to claim this paper and add it to your profile.

Reviews

Primary Rating

4.6
Not enough ratings

Secondary Ratings

Novelty
-
Significance
-
Scientific rigor
-
Rate this paper

Recommended

Article Rheumatology

Timing of Childhood-Onset Systemic Lupus Erythematosus Diagnosis Relative to Menarche and the Impact on Final Adult Height

Watchareewan Sontichai, Fangming Liao, Daniela Dominguez, Deborah M. Levy, Muna Al Mutairi, Lawrence Ng, Frank Silverio, Earl D. Silverman, Jonathan D. Wasserman, Linda T. Hiraki

Summary: This study aimed to evaluate the impact of timing of childhood-onset systemic lupus erythematosus (SLE) diagnosis on the final height of female patients. The results showed that patients diagnosed after menarche achieved a taller final height, even after adjusting for ethnicity and disease severity.

ARTHRITIS CARE & RESEARCH (2022)

Article Rheumatology

Variations in Pediatric Rheumatology Workforce and Care Processes Across Canada

Jennifer J. Y. Lee, Ronald M. Laxer, Brian M. Feldman, Claire E. H. Barber, Michelle Batthish, Roberta Berard, Lori B. Tucker, Deborah M. Levy

Summary: This study examines the Canadian pediatric rheumatology workforce and care processes. The findings reveal a national deficit of pediatric rheumatologists at the recommended benchmark, with a regional maldistribution of these specialists. Most rheumatologists work in multidisciplinary teams, but there may be inadequate support for allied health professionals (AHPs).

JOURNAL OF RHEUMATOLOGY (2022)

Article Rheumatology

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

Nathalie E. Chalhoub, Scott E. Wenderfer, Deborah M. Levy, Kelly Rouster-Stevens, Amita Aggarwal, Sonia Savani, Natasha M. Ruth, Thaschawee Arkachaisri, Tingting Qiu, Angela Merritt, Karen Onel, Beatrice Goilav, Raju P. Khubchandani, Jianghong Deng, Adriana R. Fonseca, Stacy P. Ardoin, Coziana Ciurtin, Ozgur Kasapcopur, Marija Jelusic, Adam M. Huber, Seza Ozen, Marisa S. Klein-Gitelman, Simone Appenzeller, Andre Cavalcanti, Lampros Fotis, Sern Chin Lim, Rodrigo M. Silva, Julia Ramirez-Miramontes, Natalie L. Rosenwasser, Claudia Saad-Magalhaes, Dieneke Schonenberg-Meinema, Christiaan Scott, Clovis A. Silva, Sandra Enciso, Maria T. Terreri, Alfonso-Ragnar Torres-Jimenez, Maria Trachana, Sulaiman M. Al-Mayouf, Prasad Devarajan, Bin Huang, Hermine Brunner

Summary: This study aimed to develop a standardized steroid dosing regimen for physicians treating childhood-onset systemic lupus erythematosus complicated by lupus nephritis. By evaluating patient course and various parameters, a consensus-based dosing regimen was developed and validated.

ARTHRITIS & RHEUMATOLOGY (2022)

Article Allergy

TNF inhibition in vasculitis management in adenosine deaminase 2 deficiency (DADA2)

Natalie T. Deuitch, Dan Yang, Pui Y. Lee, Xiaomin Yu, Natalia Sampaio Moura, Oskar Schnappauf, Amanda K. Ombrello, Deborah Stone, Hye Sun Kuehn, Sergio D. Rosenzweig, Patrycja Hoffmann, Cornelia Cudrici, Deborah M. Levy, Elizabeth Kessler, Jennifer B. Soep, Arielle D. Hay, Austin Dalrymple, Yu Zhang, Li Sun, Qiuye Zhang, Xuemei Tang, Yuan Wu, Koneti Rao, Haibo Li, Hong Luo, Yao Zhang, Jon M. Burnham, Manfred Boehm, Karyl Barron, Daniel L. Kastner, Ivona Aksentijevich, Qing Zhou

Summary: This study investigates the cellular mechanisms underlying the response to TNF inhibitor therapy in patients with DADA2 and identifies the pathological features associated with treatment response. TNFi treatment reduces inflammation, corrects skewed macrophage differentiation, and restores endothelial cell integrity.

JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY (2022)

Article Rheumatology

Schizophrenia Genetics and Neuropsychiatric Features in Childhood-onset Systemic Lupus Erythematosus

Ana C. Ulloa, Fangming Liao, Raffaella L. Carlomagno, Talia Diaz, Daniela Dominguez, Deborah M. Levy, Lawrence Ng, Andrea M. Knight, Linda T. Hiraki

Summary: The association between schizophrenia genetic susceptibility loci and NPSLE features in multiethnic childhood-onset SLE patients was examined. The results suggested that there was no significant association between schizophrenia risk loci and NPSLE in this cohort.

JOURNAL OF RHEUMATOLOGY (2022)

Article Pediatrics

Acute kidney injury requiring kidney replacement therapy in childhood lupus nephritis: a cohort study of the Pediatric Nephrology Research Consortium and Childhood Arthritis and Rheumatology Research Alliance

Brian R. Stotter, Ellen Cody, Hongjie Gu, Ankana Daga, Larry A. Greenbaum, Minh Dien Duong, Alexandra Mazo, Beatrice Goilav, Alexis Boneparth, Mahmoud Kallash, Ahmed Zeid, Wacharee Seeherunvong, Rebecca R. Scobell, Issa Alhamoud, Caitlin E. Carter, Siddharth Shah, Caroline E. Straatmann, Bradley P. Dixon, Jennifer C. Cooper, Raoul D. Nelson, Deborah M. Levy, Hermine Brunner, Priya S. Verghese, Scott E. Wenderfer

Summary: Children with lupus nephritis and stage 3 acute kidney injury have a high long-term risk of kidney failure. The severity of tubular atrophy and interstitial fibrosis at the time of acute kidney injury predicts kidney disease progression.

PEDIATRIC NEPHROLOGY (2023)

Article Rheumatology

Genome-Wide Sequencing Identified Rare Genetic Variants for Childhood-Onset Monogenic Lupus

Melissa C. Misztal, Fangming Liao, Madeline Couse, Jingjing Cao, Daniela Dominguez, Lynette Lau, Christian R. Marshall, Sergey Naumenko, Andrea M. Knight, Deborah M. Levy, Linda T. Hiraki

Summary: This study conducted genome-wide sequencing in children suspected of having monogenic lupus, and found that 13% of patients carried rare predicted damaging variants for monogenic lupus. Further studies are needed to validate these findings.

JOURNAL OF RHEUMATOLOGY (2023)

Letter Rheumatology

Supply and Services of Pediatric Rheumatologists in Ontario, Canada

Jennifer J. Y. Lee, Roberta Berard, Deborah M. Levy, Vicki Ling, Jodi M. Gatley, Jessica Widdifield

JOURNAL OF RHEUMATOLOGY (2023)

Meeting Abstract Rheumatology

Improving Completion Rates of Routine Mental Health Screening for Depression and Anxiety in Paediatric Lupus Outpatient Clinic to Enhance Patient Mental Health Care

Tala El Tal, Avery Longmore, Abdulaziz el Mutairi, Amani Al Bijadi, Audrea Chen, Holly Convery, Dinah Finkelstein, Linda Hiraki, Chetana Kulkarni, Justine Ledochowski, Neely Lerman, Karen Leslie, Deborah Levy, Sharon Lorber, Jayne MacMahon, Jeanine McColl, Sarah Mossad, Oscar Mwizerwa, Lawrence Ng, Luana Flores Pereira, Vandana Rawal, Alaa Shehab, Evelyn Smith, Alene Toulany, Andrea Knight

ARTHRITIS & RHEUMATOLOGY (2022)

Meeting Abstract Rheumatology

Poor Executive Function Correlates with Increased Disease Damage and Impaired Patient-Reported Outcomes in Youth with Childhood-Onset Lupus: A Cross-Sectional Study

Tala El Tal, Santiago Arciniegas, Sarah Mossad, Ibrahim Mohamed, Victoria Lishak, Stephanie Fevrier, Lawrence Ng, Paris Moaf, Joanna Law, Ashley Danguecan, Linda Hiraki, Deborah Levy, Andrea Knight

ARTHRITIS & RHEUMATOLOGY (2022)

Meeting Abstract Rheumatology

Association of HLA Alleles with Specific Auto-antibodies in an Ancestrally Diverse Population of Childhood Systemic Lupus Erythematosus (SLE)

Nicholas Gold, Fangming Liao, JingJing Cao, Daniela Dominguez, Andrea Knight, Deborah Levy, Linda Hiraki

ARTHRITIS & RHEUMATOLOGY (2022)

Meeting Abstract Rheumatology

Potential Biomarkers of Cognitive Impairment in the Context of Childhood-onset Systemic Lupus Erythematosus

Santiago Arciniegas, Sarah Mossad, Tala El Tal, Lawrence Ng, Paris Moaf, Helen Branson, Adrienne Davis, Linda Hiraki, Deborah Levy, Ashley Danguecan, George Ibrahim, Andrea Knight

JOURNAL OF RHEUMATOLOGY (2022)

Meeting Abstract Rheumatology

High Adolescent Health Needs and Relationship to Disease in Patients With Childhood-onset Systemic Lupus Erythematosus

Chelsea DeCoste, Paris Moaf, Lawrence Ng, Dragana Ostojic-Aitkens, Fatima Faruq, Bryan Maguire, Deborah Levy, Linda Hiraki, Alene Toulany, Andrea Knight

JOURNAL OF RHEUMATOLOGY (2022)

Meeting Abstract Rheumatology

Assessing the Relationship Between Health Outcomes and Ethnic Concentration in Patients With Childhood-onset Systemic Lupus Erythematosus

Ibrahim Mohamed, Chelsea DeCoste, Lawrence Ng, Paris Moaf, Hardil Bhatt, Dragana Ostojic-Aitkens, Bryan Maguire, Linda Hiraki, Deborah Levy, Alene Toulany, Andrea Knight

JOURNAL OF RHEUMATOLOGY (2022)

Meeting Abstract Rheumatology

Structural Neural Underpinnings of Low Mood and Anxiety in Childhood Onset Systemic Lupus Erythematosus

Sarah Mossad, Santiago Arciniegas, Tala El Tal, Lawrence Ng, Paris Moaf, Helen Branson, Adrienne Davis, Linda Hiraki, Deborah Levy, Ashley Danguecan, George Ibrahim, Andrea Knight

JOURNAL OF RHEUMATOLOGY (2022)

Article Pediatrics

Multicomponent Strategy Improves Human Papillomavirus Vaccination Rates Among Adolescents with Sickle Cell Disease

Tarun Aurora, Audrey Cole, Parul Rai, Paul Lavoie, Carrie Mcivor, Lisa M. Klesges, Guolian Kang, Janaka S. S. Liyanage, Heather M. Brandt, Jane S. Hankins

Summary: This study evaluated the effectiveness of a vaccine strategy bundle in increasing HPV vaccine initiation and completion rates in a specialty clinic setting. By implementing the bundle, which included staff education, provider incentives, offering vaccines in clinics, and verifying vaccine completion, the clinic successfully improved HPV vaccine initiation and completion rates among sickle cell disease patients.

JOURNAL OF PEDIATRICS (2024)

Article Pediatrics

Effect of Systemic Hydrocortisone on Brain Abnormalities and Regional Brain Volumes in Ventilator-dependent Infants Born Preterm: Substudy of the SToP-BPD Study

Nienke M. Halbmeijer, Wes Onland, Jeroen Dudink, Filip Cools, Anne Debeer, Anton H. van Kaam, Manon J. N. L. Benders, Niek E. van der Aa

Summary: In ventilated infants born preterm, high dose systemic hydrocortisone initiated between 7 and 14 days after birth did not have a significant impact on brain development.

JOURNAL OF PEDIATRICS (2024)

Article Pediatrics

Predictors of Transition Outcomes in Cystic Fibrosis: Analysis of National Patient Registry and CF RISE (Responsibility. Independence. Self-care. Education) Data

Katherine Melton, Jianfang Liu, Hossein Sadeghi, Maureen George, Arlene Smaldone

Summary: This study aims to identify predictors of change in lung function and body weight during health care transition in cystic fibrosis (CF) patients. The study findings highlight the importance of CF RISE program engagement and reducing gaps in care for improving the transition of adolescents and young adults with CF.

JOURNAL OF PEDIATRICS (2024)

Article Pediatrics

Quantification of Enteric Dysfunction in Cystic Fibrosis: Inter- and Intraindividual Variability

Laura A. Duckworth, Kimberly A. Sutton, Nurmohammad Shaikh, Jinli Wang, Carla Hall-Moore, Lori R. Holtz, Phillip I. Tarr, Ronald C. Rubenstein

Summary: The study tested the usefulness of various biomarkers as indicators of gut dysfunction in cystic fibrosis (CF) and investigated the repeatability of these measures in individuals over short periods and their correlation with clinical outcomes. The results showed that elevated levels of fLcn2 in individuals with CF may predict worsened pulmonary function.

JOURNAL OF PEDIATRICS (2024)

Article Pediatrics

Right Atrial Enlargement on Electrocardiogram in Previously Healthy Young

Lindsey Haack, Nikkan Das, Arvind Hoskoppal, Mark Debrunner, Tarek Alsaied, Gaurav Arora

Summary: RAE on ECG has a low positive predictive value for RAE on echocardiogram in previously healthy young patients. The highest yield for RAE on echocardiogram was observed in patients who were <1 year of age, had RAE in the anterior precordial leads, or displayed right ventricular hypertrophy on ECG.

JOURNAL OF PEDIATRICS (2024)

Article Pediatrics

Spectrum of Disease in Hospitalized Newborns with Congenital Micrognathia: A Cohort of 3,236 Infants at North American Tertiary-Care Intensive Care Units

Michael A. Padula, Khatija Naing, Tara L. Wenger, Irfan Ahmad, Carl H. Coghill, K. Taylor Wild, S. Alex Rottgers, Cory M. Resnick, Jeffrey Goldstein, Zarmina Ehsan, Donna Watkins, Nicole Deptula, Kuan-Chi Lai, Janet Lioy, Semsa Gogcu, Christopher M. Cielo

Summary: This study describes the spectrum of disease and burden of care in infants with congenital micrognathia. The results show that these infants commonly require surgical intervention and tube feedings, and disparities based on race and among centers were identified.

JOURNAL OF PEDIATRICS (2024)

Article Pediatrics

Opioid Utilization after Cardiac Surgery in the Pediatric Medicaid-Insured Population

Michael P. Fundora, Manvitha Kalicheti, Guantao Zhao, Kevin O. Maher, Nicoleta Serban

Summary: This study investigated the variation of outpatient opioid prescribing in postoperative pediatric cardiac patients across the US. The results showed that there were significant differences in opioid prescribing by race, ethnicity, sex, and region.

JOURNAL OF PEDIATRICS (2024)

Article Pediatrics

Tracking of Cardiorespiratory Fitness from Childhood to Mid-adulthood

Jia Guo, Brooklyn J. Fraser, Leigh Blizzard, Michael D. Schmidt, Terence Dwyer, Alison J. Venn, Costan G. Magnussen

Summary: There is a correlation between childhood and adulthood cardiorespiratory fitness.

JOURNAL OF PEDIATRICS (2024)

Article Pediatrics

Blood Pressure Outcomes in NICU-Admitted Infants with Neonatal Hypertension: A Pediatric Nephrology Research Consortium Study

Nianzhou Xiao, Michelle Starr, Adrienne Stolfi, Gilad Hamdani, Shireen Hashmat, Stefan G. Kiessling, Christina Sethna, Mahmoud Kallash, Robyn Matloff, Robert Woroniecki, Keia Sanderson, Ikuyo Yamaguchi, Stephen D. Cha, Michael G. Semanik, Rahul Chanchlani, Joseph T. Flynn, Mark Mitsnefes

Summary: This multicenter study reports that most infants diagnosed with idiopathic hypertension in the NICU will discontinue antihypertensive treatment within 2 years of discharge. Antenatal steroid treatment is associated with a decreased likelihood of needing antihypertensive therapy for more than 1 year.

JOURNAL OF PEDIATRICS (2024)

Editorial Material Pediatrics

Real-World Evidence for Neonatal Drug Development: Challenges and Opportunities

Kanwaljit Singh, John Concato, Jonathan M. Davis

JOURNAL OF PEDIATRICS (2024)

Article Pediatrics

The Influence of Dietary Counseling Over 20 Years on Tracking of Non-High-Density Lipoprotein Cholesterol from Infancy to Young Adulthood

Yaxing Meng, Harri Niinikoski, Suvi P. Rovio, Brooklyn J. Fraser, Feitong Wu, Antti Jula, Tapani Ronnemaa, Jorma S. A. Viikari, Olli T. Raitakari, Katja Pahkala, Costan G. Magnussen

Summary: This 26-year study shows a correlation between early-life non-HDL-C levels and future levels. Early dietary counseling can reduce the risk of high pediatric non-HDL-C, emphasizing the importance of early interventions in preventing cardiovascular risks.

JOURNAL OF PEDIATRICS (2024)

Article Pediatrics

One Step Closer to Safer: Counseling Outcomes from American Academy of Pediatrics Firearm Safe Storage Education Training

Kelsey A. B. Gastineau, Rebecca Bell, Allison Hanes, Sandra Mckay, Eric Sigel, Filoteia Popescu, Evan C. Sommer, Shari Barkin

Summary: This study aimed to assess the self-reported counseling outcomes for a firearm safe storage counseling training program provided by the American Academy of Pediatrics. The results demonstrated significant improvement in counseling self-efficacy and frequency one month after the training.

JOURNAL OF PEDIATRICS (2024)

Article Pediatrics

Health-Related Quality of Life for Parents of Infants with Bronchopulmonary Dysplasia

Kathryn E. K. Berlin, William Scott, Sara Dawson, David Brousseau, Joanne M. Lagatta

Summary: This prospective cohort study aimed to investigate the impact of bronchopulmonary dysplasia (BPD) on the health-related quality of life (HRQL) of infants from NICU hospitalization to one year post-discharge. The study found that lower HRQL during NICU stay was associated with earlier gestational age, postnatal corticosteroid usage, outborn status, and gastrostomy tube placement. Lower HRQL at 3 and 12 months post-discharge was associated with readmissions, home oxygen use, parent-reported difficulty breathing, lower developmental scores, and not playing with other children. Most parents reported similar or improved HRQL after discharge, but parents of infants with respiratory symptoms experienced less improvement. Efforts to improve parent HRQL should focus on respiratory symptoms and social isolation.

JOURNAL OF PEDIATRICS (2024)

Article Pediatrics

Reference Values for Cardiorespiratory Fitness in Patients Aged 6 to 18 Years

Garett J. Griffith, Alan P. Wang, Robert I. Liem, Michael R. Carr, Tyler Corson, Kendra Ward

Summary: This study developed reference values for cardiorespiratory fitness in children aged 6-18 years without underlying heart disease, measured by peak oxygen uptake and treadmill time. Fitness levels increased with age in males but not females. Males generally exhibited higher fitness levels compared to females in the same age groups.

JOURNAL OF PEDIATRICS (2024)

Article Pediatrics

Early Elbow Flexion Contracture Predicts Shoulder Contracture in Infants with Brachial Plexus Birth Injury

David S. Liu, Patricia Miller, Anna Rothenberg, Carley Vuillermin, Peter M. Waters, Andrea S. Bauer

Summary: This study aims to determine if children with elbow flexion contracture (EFC) caused by brachial plexus birth injury (BPBI) are more likely to develop shoulder contracture and undergo surgical treatment. A retrospective review was conducted on children under 2 years old with BPBI who presented to a single children's hospital. The results showed that patients with EFC had reduced shoulder range of motion and higher odds of shoulder contracture and surgical treatment. Prompt referral to a BPBI specialty clinic is recommended for evaluation and potential surgery.

JOURNAL OF PEDIATRICS (2024)