Baseline Clinical and Serological Findings in Pediatric-Onset Discoid Lupus Erythematosus: Analysis of a Multicenter Retrospective Cohort Study

Nnenna Ezeh¹, Kevin Buhr², Cordellia Nguyen³, Ohoud Al Ahmed⁴, Stacy Ardoin⁵, Virginia Barton⁶, Samantha Bell⁷, Heather Brandling-Bennett⁸, Leslie Castelo-Soccio⁹, Yvonne Chiu¹⁰, Benjamin Chong¹¹, Dominic Co¹², Irene Lara-Corrales¹³, Amber Cintosun¹³, Lucia Diaz¹⁴, Scott Andrew Elman¹⁵, Esteban Fernandez Faith⁴, Maria Teresa Garcia-Romero¹⁶, Julie Grossman-Kranseler⁸, Aimee Hersh¹⁷, Marcia Hogeling¹⁸, Andrew Hudson¹⁹, Raegan Hunt²⁰, Erin Ibler¹⁰, Mariana Marques²¹, Reesa Monir²², Vikash Oza²³, Amy Paller²⁴, Elana Putterman⁹, Pamela Rodriguez-Salgado¹⁶, Jennifer Schoch²², Allison Truong¹⁸, Jason Wang²³, Lara Wine Lee⁶, Ruth Ann Vleugels¹⁵, Marisa Klein-Gitelman²⁵, Emily von-Scheven²⁶, Victoria Werth²⁷, Kaveh Ardalan²⁸and Lisa Arkin²⁹,¹University of Wisconsin School of Medicine and Public Health, Madison, WI,²Department of Biostatistics & Medical Informatics, University of Wisconsin School of Medicine and Public Health, Madison,³University of Wisconsin School of Medicine and Public Health, Madison,⁴Department of Pediatrics, Division of Dermatology, Nationwide Children's Hospital/Ohio State University, Columbus,⁵Nationwide Children's Hospital, Columbus, OH,⁶Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina, Charleston,⁷Department of Pediatrics, Division of Rheumatology, University of California at San Francisco, San Francisco,⁸Division of Dermatology; Department of Pediatrics, Seattle Children's Hospital/University of Washington School of Medicine, Seattle,⁹Department of Pediatrics, Section of Dermatology, Children's Hospital of Philadelphia, Philadelphia,¹⁰Departments of Dermatology and Pediatrics, Medical College of Wisconsin, Milwaukee,¹¹University of Texas Southwestern, Dallas,¹²Department of Pediatrics, Division of Rheumatology, University of Wisconsin School of Medicine and Public Health, Madison,¹³Department of Pediatrics, Division of Dermatology, The Hospital for Sick Children/University of Toronto, Toronto, Canada,¹⁴Department of Pediatrics, Division of Dermatology, Dell Medical School/Dell Children's Hospital, Austin, Texas,¹⁵Department of Dermatology, Boston's Children Hospital/Harvard Medical School, Boston,¹⁶Department of Dermatology, National Institute for Pediatrics, Mexico City, Mexico,¹⁷University of Utah, Salt Lake City, UT,¹⁸Department of Dermatology, University of California at Los Angeles, Los Angeles,¹⁹Department of Dermatology, University of Texas Southwestern Medical Center, Dallas,²⁰Departments of Pediatrics and Dermatology, Texas Children's Hospital, Houston, Texas,²¹Division of Rheumatology, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago,²²Department of Dermatology, University of Florida School of Medicine, Gainesville,²³Departments of Pediatrics and Dermatology, New York University School of Medicine, New York,²⁴Departments of Dermatology and Pediatrics, Northwestern University Feinberg School of Medicine/Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago,²⁵Department of Pediatrics, Division of Rheumatology, Northwestern University Feinberg School of Medicine/Ann & Robert H. Lurie Children's Hospital, Chicago,²⁶University of California San Francisco, San Francisco,²⁷Corporal Michael J. Crescenz VAMC, Philadelphia, PA, USA and Department of Dermatology, University of Pennsylvania, Philadelphia, PA, USA, Philadelphia, PA,²⁸Division of Pediatric Rheumatology, Department of Pediatrics, Northwestern University, Chicago, Illinois,²⁹Department of Dermatology, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin

Meeting: 2019 ACR/ARP Annual Meeting

Keywords: Lupus, pediatrics and interdisciplinary rheumatology team

Session Information

Date: Wednesday, November 13, 2019

Title: 6W010: Pediatric Rheumatology – Clinical III: Juvenile SLE & Dermatomyositis (2864–2869)

Session Type: ACR Abstract Session

Session Time: 9:00AM-10:30AM

Background/Purpose: DLE is a rare, disfiguring disorder in children. Small retrospective studies suggest 20-25% of patients progress to SLE. Progression risk factors are poorly understood, but DLE has been associated with delay in SLE diagnosis and reduced access to care. This multicenter retrospective cohort study aimed to describe baseline characteristics and clinical phenotypes of pediatric DLE patients at diagnosis.

Methods: Medical records at eighteen sites were reviewed for pediatric dermatology and rheumatology patients with DLE. For inclusion, patients required clinical and/or histopathologic findings consistent with DLE. Baseline data were collected at the first documented visit including sociodemographic data, ACR/SLICC SLE criteria (i.e. DLE+SLE), date of DLE onset/diagnosis, DLE distribution, family history, comorbidities, and treatment. Outcome variables included ACR (primary outcome) /SLICC SLE criteria. Rates of progression from skin-limited DLE (DLE) to SLE (DLE+SLE) were evaluated. Analysis included descriptive statistics, chi-square and Wilcoxon tests.

Results: Out of >1,000 patients reviewed, 441 met inclusion criteria. The cohort was predominantly female (72%) and racially/ethnically diverse (Table 1). A minority presented at baseline with SLE based on ACR and SLICC criteria, respectively (n=165, 37%; n=183, 42%). DLE+SLE patients were older (median 13.7y vs 10.2y) with shorter time from DLE onset to diagnosis (median 2 mo vs 7 mo), compared to DLE patients (p< 0.001). DLE patients presented with low incidence of renal involvement, serositis, seizures or psychosis (p< 0.001, Table 2). DLE+SLE patients had more positive serologies and higher-titer ANAs (p< 0.001, Table 3), although 5% were ANA negative. Among 231 DLE patients with³1 follow up visit, median follow-up was 2.7 y (range 0-13.9y) with 747 total subject-years. Progression to SLE occurred in 20% and 25% of patients based on ACR and SLICC criteria, respectively.

Conclusion: To date, this is the largest investigation of pediatric DLE. Patients with DLE + SLE were most likely to present in adolescence with abnormal serologies and end-organ disease. Progression of DLE to SLE occurred at rates consistent with previous literature. All patients with DLE require SLE surveillance at diagnosis and regular follow-up, particularly during adolescence. Limitations include the retrospective study design with potential for misclassification, and analysis restricted to the baseline visit. Further analysis of follow up visits will evaluate for baseline risk factors and biomarkers of evolving SLE, as well as timing of progression, identifying DLE patients at highest risk for systemic disease.

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N. Ezeh, None; K. Buhr, None; C. Nguyen, None; O. Al Ahmed, None; S. Ardoin, None; V. Barton, None; S. Bell, None; H. Brandling-Bennett, None; L. Castelo-Soccio, None; Y. Chiu, Novan, 1; B. Chong, Celgene, 1, Viela Bio, 1, Daavlin Corporation, 1, Biogen, 1, Pfizer Incorporated, 1; D. Co, None; I. Lara-Corrales, Sanofi Genzyme, 1, Abbvie, 1, 2, 3, L'Oréal, 1, Pfizer, 1, Fuse Health, 1, Jansen, 1, Novartis, 1, Amgen, 1, 2, Eli Lilly, 1, Avicanna, 1; A. Cintosun, None; L. Diaz, None; S. Elman, None; E. Fernandez Faith, None; M. Garcia-Romero, None; J. Grossman-Kranseler, None; A. Hersh, None; M. Hogeling, None; A. Hudson, None; R. Hunt, Dermavant, 1, Up To Date, Inc, 1, Medscape Expert Consult, 1; E. Ibler, None; M. Marques, None; R. Monir, None; V. Oza, Pfizer, 1, Regeneron, 1, Cuteness Life Sciences, 1; A. Paller, None; E. Putterman, None; P. Rodriguez-Salgado, None; J. Schoch, Janssen Biotech, Inc, 1; A. Truong, None; J. Wang, None; L. Wine Lee, None; R. Vleugels, None; M. Klein-Gitelman, None; E. von-Scheven, CARRA, 6, 9; V. Werth, Biogen, 2, 5, Corbus Pharmaceuticals, 2, 9, University of Pennsylvania, 9; K. Ardalan, None; L. Arkin, None.

To cite this abstract in AMA style:

Ezeh N, Buhr K, Nguyen C, Al Ahmed O, Ardoin S, Barton V, Bell S, Brandling-Bennett H, Castelo-Soccio L, Chiu Y, Chong B, Co D, Lara-Corrales I, Cintosun A, Diaz L, Elman S, Fernandez Faith E, Garcia-Romero M, Grossman-Kranseler J, Hersh A, Hogeling M, Hudson A, Hunt R, Ibler E, Marques M, Monir R, Oza V, Paller A, Putterman E, Rodriguez-Salgado P, Schoch J, Truong A, Wang J, Wine Lee L, Vleugels R, Klein-Gitelman M, von-Scheven E, Werth V, Ardalan K, Arkin L. Baseline Clinical and Serological Findings in Pediatric-Onset Discoid Lupus Erythematosus: Analysis of a Multicenter Retrospective Cohort Study [abstract]. Arthritis Rheumatol. 2019; 71 (suppl 10). https://acrabstracts.org/abstract/baseline-clinical-and-serological-findings-in-pediatric-onset-discoid-lupus-erythematosus-analysis-of-a-multicenter-retrospective-cohort-study/. Accessed .

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