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Abstract Number: 468

Baseline Features and Outcomes of Pediatric-Onset Discoid Lupus Erythematosus: Interim Data Analysis of a Multicenter Retrospective Cohort Study

Lisa Arkin1, Kevin A. Buhr2, Cordellia Nguyen3, Heather Brandling-Bennett4, Leslie Castelo-Soccio5, Yvonne Chiu6, Benjamin F. Chong7, Lucia Diaz8, Marisa S. Klein-Gitelman9, Amy Paller10, Jennifer Schoch11, Emily von Scheven12, Victoria P. Werth13, Julie Grossman-Kranseler14, Andrew D. Hudson15, Erin M. Ibler16, Mariana C. Marques17, Reesa L. Monir11, Elana Putterman18 and Kaveh Ardalan19, 1Departments of Dermatology and Pediatrics, University 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, WI, 3University of Wisconsin School of Medicine and Public Health, Madison, WI, 4Division of Dermatology; Department of Pediatrics, Seattle Children’s Hospital/University of Washington School of Medicine, Seattle, WA, 5Pediatrics, Section of Dermatology, Children's Hospital of Philadelphia, Philadelphia, PA, 6Pediatrics and Dermatology, Medical College of Wisconsin, Milwaukee, WI, 7Dermatology, University of Texas Southwestern Medical Center, Dallas, TX, 8Department of Pediatrics, Division of Dermatology, Dell Children’s Hospital, Austin, TX, 9Department of Pediatrics, Division of Rheumatology, Northwestern University Feinberg School of Medicine/Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, 10Departments of Dermatology and Pediatrics, Northwestern University Feinberg School of Medicine/Ann & Robert H. Lurie Children’s Hospital of Chicago, Chicago, IL, 11Department of Dermatology, University of Florida School of Medicine, Gainesville, FL, 12Department of Pediatrics, Division of Rheumatology, University of California at San Francisco, San Francisco, CA, 13Department of Dermatology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, 14Departments of Pediatrics, Division of Dermatology, University of Washington School of Medicine, Seattle, WA, 15Texas Tech University Health Sciences Center School of Medicine, Lubbock, TX, 16Departments of Dermatology and Pediatrics, Medical College of Wisconsin, Milwaukee, WI, 17Division of Rheumatology, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, 18Children’s Hospital of Philadelphia, Department of Pediatrics, Section of Dermatology, Children’s Hospital of Philadelphia, Philadelphia, PA, 19Departments of Pediatrics and Medical Social Sciences, Division of Rheumatology, Northwestern University Feinberg School of Medicine/Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL

Meeting: 2018 ACR/ARHP Annual Meeting

Keywords: Lupus, lupus dermatitis, outcomes and pediatric rheumatology

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Session Information

Date: Sunday, October 21, 2018

Title: Pediatric Rheumatology – Clinical Poster I: Lupus, Sjögren’s Disease, and Myositis

Session Type: ACR Poster Session A

Session Time: 9:00AM-11:00AM

Background/Purpose:

Discoid lupus erythematosus (DLE) is rare in children. Prior studies suggest 25-30% of children with skin-limited DLE are diagnosed with systemic lupus erythematosus (SLE) over time. Biomarkers and risk factors to identify those at highest risk are unknown. This multicenter, retrospective study aims to characterize baseline features and outcomes in pediatric patients with skin-limited DLE, as well as risk factors for the progression of DLE to SLE.  Baseline characteristics of all patients with DLE and initial study findings are presented in this interim analysis.

Methods:

Nine of 18 committed clinical sites, including pediatric dermatologists and rheumatologists, retrospectively reviewed all medical records of patients ≤18 years of age with clinical and/or histopathologic findings consistent with DLE. Baseline data were collected on all patients, including demographics, dates of DLE onset and diagnosis, distribution of DLE, and family history of SLE. For patients with skin-limited DLE, rates of progression to SLE based on American College of Rheumatology (ACR) and Systemic Lupus International Collaborating Clinics (SLICC) classification criteria were evaluated.

Results:

Clinical records for 205 patients have been reviewed to date, with 50% of participating sites reporting. Baseline data are presented in Table 1. African-American females were most commonly affected. Median age at DLE diagnosis was 11.8 years, with median time from DLE onset to diagnosis of 0.5 years.  Most patients (76%) had localized disease (i.e. head/neck only); 20% had a family history of SLE in a 1st degree relative. Most patients had skin-limited DLE at baseline, with only a minority exhibiting ≥4 ACR classification criteria (n = 56; 27%) or ≥4 SLICC classification criteria (n = 46; 22%). Initial treatments are presented in Table 2. Patients with skin-limited DLE and at least one follow-up visit (n=115) had median follow up of 3.1 years (range 0.1-12.5 years, 393 total patient-years). During this period, a minority met criteria for SLE diagnosis, utilizing ≥4 ACR classification criteria (n = 16; 14%) and ≥4 SLICC classification criteria  (n = 24; 22%).

Conclusion:

This study represents the largest investigation of pediatric DLE performed to date. Utilizing both ACR and SLICC classification criteria for SLE, most patients (>73%) presented with skin-limited DLE, with a low cumulative incidence of SLE using both ACR and SLICC classification criteria.  Further analysis may help to determine risk factors for progression to SLE and inform the creation of consensus guidelines for treating children with DLE.

Table 1. Baseline data for 205 patients with DLE (skin-limited DLE and SLE)

                                                                        N (%)

Gender

   Female

    Male

142 (69%)

63 (31%)

Race/Ethnicity*

   Black or African American

    White

    Hispanic or Latino

    Asian

    Native Hawaiian or Other Pacific Islander

    American Indian or Alaskan Native

    Unknown or not reported

82 (40%)

61 (30%)

34 (17%)

14 (7%).

2 (1%)

1 (0.5%)

15 (7%)

1st degree family member with diagnosis of SLE

42 (20%)

Distribution of DLE lesions

     Localized (head/neck only)

     Generalized (both above/below the neck)

     Isolated (below the neck ONLY)

155 (76%)

46 (22%)

4 (2%)

                                                                    Median (IQR)

Age at presentation of DLE, years

10.8

(7.2-13.6)

Age at DLE diagnosis, years

11.8

(8.0-14.4)

*Participants could designate more than 1 race/ethnicity category

Table 2. Medications at baseline for patients with DLE (Skin-Limited and SLE)

Medication

N, (%)

Topical steroids

132 (65%)

Hydroxychloroquine

118 (58%)

Other immunomodulatory medications

á       Prednisone/Methylprednisolone

á       Mycophenole mofetil

á       Rituximab

á       Methotrexate

á       Dapsone

á       Azathioprine

á       Quinacrine

á       Thalidomide

59 (29%)

46 (22%)

16 (8%)

5 (2%)

4 (2%)

4 (2%)

4 (2%)

1 (0.5%)

1 (0.5%)

Topical calcineurin inhibitors

40 (20%)

None

13 (6%)


Disclosure: L. Arkin, None; K. A. Buhr, None; C. Nguyen, None; H. Brandling-Bennett, None; L. Castelo-Soccio, None; Y. Chiu, None; B. F. Chong, Biogen Incorporated, 2,Daavlin Incorporated, 2,Celgene Corporation, 5,Pfizer Incorporated, 9; L. Diaz, None; M. S. Klein-Gitelman, None; A. Paller, None; J. Schoch, None; E. von Scheven, None; V. P. Werth, None; J. Grossman-Kranseler, None; A. D. Hudson, None; E. M. Ibler, None; M. C. Marques, None; R. L. Monir, None; E. Putterman, None; K. Ardalan, None.

To cite this abstract in AMA style:

Arkin L, Buhr KA, Nguyen C, Brandling-Bennett H, Castelo-Soccio L, Chiu Y, Chong BF, Diaz L, Klein-Gitelman MS, Paller A, Schoch J, von Scheven E, Werth VP, Grossman-Kranseler J, Hudson AD, Ibler EM, Marques MC, Monir RL, Putterman E, Ardalan K. Baseline Features and Outcomes of Pediatric-Onset Discoid Lupus Erythematosus: Interim Data Analysis of a Multicenter Retrospective Cohort Study [abstract]. Arthritis Rheumatol. 2018; 70 (suppl 9). https://acrabstracts.org/abstract/baseline-features-and-outcomes-of-pediatric-onset-discoid-lupus-erythematosus-interim-data-analysis-of-a-multicenter-retrospective-cohort-study/. Accessed .
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