Severity of Childhood-onset Systemic Lupus Erythematosus: Impact of Preceding and Co-existing Autoimmune Cytopenias Restricted; Files Only

Abstract

Background: Autoimmune cytopenias may precede or occur with childhood-onset systemic lupus erythematosus (cSLE). Adult studies suggest that lupus patients with concurrent autoimmune cytopenias have relatively lower prevalence of lupus nephritis (LN) and are a unique sub-population. Therefore, the objectives of our study were to assess whether in cSLE, autoimmune cytopenias decrease the 2-year risk and severity of LN; to assess associated serological differences in those with and without autoimmune cytopenias and the effect of prior immune therapy for autoimmune cytopenia on 2-year risk of LN; and to perform descriptive analyses of these pediatric patients without LN at cSLE diagnosis.

Methods: Ours was a retrospective cohort study of incident cSLE cases over a 16-year period. We included patients aged less than 17 years who met the classification criteria for SLE. We excluded patients diagnosed outside our institution and those with LN at cSLE diagnosis. Our follow-up period was 2 years. We defined autoimmune cytopenia as either autoimmune hemolytic anemia, Coombs positive anemia without hemolysis, immune thrombocytopenia or Evan’s syndrome.

Results: Our study included 130 incident cSLE patients. Of these, 43 (33%) had autoimmune cytopenia before or at cSLE diagnosis. Those with autoimmune cytopenia had significantly more neuropsychiatric symptoms and higher mean ESR versus those without autoimmune cytopenia. However, they had less arthritis, malar rash and myositis. 2-year incidence of LN was 12% in our cohort. Patients with autoimmune cytopenia had lower 2-year risk of LN compared to other cSLE patients (7% vs 15%). Of the 16 patients who developed LN, those with autoimmune cytopenia had mostly class V (2 of 3 patients) versus mostly class III and IV in those without autoimmune cytopenia (6 of 12 patients). None of the 13 patients pre-treated for autoimmune cytopenia prior to cSLE diagnosis developed LN.

Conclusion: Patients with autoimmune cytopenia before or at cSLE diagnoses have significant and clinically relevant differences in their presentation from other cSLE patients. Our findings call for further studies on the immunologic and genetic bases of these differences.

Table of Contents

INTRODUCTION..………………………………………………………………………....1

BACKGROUND……………………………………………………………………………...3

METHODS…………………………………………………………………………………...7

RESULTS…………………………………………………………………………………….13

DISCUSSION……………………………………………………………………………….17

REFERENCES...……………………………………………………………………………21

TABLES AND FIGURES…..……………………………………………………………...27

Figure 1. Flow diagram showing selection of study population

Table 1. Comparison of demographic, clinical and laboratory characteristics of 130 incident patients with childhood-onset systemic lupus erythematosus from January 1, 2000 and June 30, 2016

Table 2. Comparison of demographic, clinical and laboratory characteristics of 130 incident patients with childhood-onset systemic lupus erythematosus from January 1, 2000 and June 30, 2016 by baseline autoimmune cytopenia status

Table 3. Comparison of baseline demographic, clinical and laboratory characteristics of 130 incident patients with childhood-onset systemic lupus erythematosus from January 1, 2000 and June 30, 2016 by race

Table 4. Comparison of baseline demographic, clinical and laboratory characteristics of 130 incident patients with childhood-onset systemic lupus erythematosus from January 1, 2000 and June 30, 2016 by age

Table 5. Comparison of baseline demographic, clinical and laboratory characteristics of 130 incident patients with childhood-onset systemic lupus erythematosus from January 1, 2000 and June 30, 2016 by sex

Table 6. Univariate logistic regression analysis examining the association of characteristics of cSLE patients with incident lupus nephritis

Table 7. Final multivariable logistic regression model examining the association of patient characteristics with incident lupus nephritis

Table 8. Comparing demographics and characteristics at time of cSLE diagnosis of patients who developed lupus nephritis within the first 2 years of diagnosis by baseline autoimmune cytopenia status

Table 9. Comparing characteristics of patients who developed lupus nephritis within the first 2 years of diagnosis by baseline autoimmune cytopenia status.

Table 10. Comparing 2-year risk of lupus nephritis in 130 patients with childhood-onset systemic lupus erythematosus.

About this Master's Thesis

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School	Laney Graduate School
Department	Clinical Research
Degree	M.S.
Submission	Master's Thesis
Language	English
Research Field	Health Sciences, Medicine and Surgery
Mot-clé	Immune thrombocytopenia Evans syndrome Systemic Lupus Erythematosus Lupus nephritis Autoimmune cytopenia Autoimmune hemolytic anemia
Committee Chair / Thesis Advisor	Sampath Prahalad, MD MSc, Emory University
Committee Members	Mitchel Klein, PhD, Emory University Shanmuganathan Chandrakasan, MD, Emory University Laura Plantinga, PhD, Emory University

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