Impact of autoimmune cytopenias on severity of childhood-onset systemic lupus erythematosus: A single-center retrospective cohort study

• Type of Material: Article
• Authors: Ekemini A. Ogbu, Johns Hopkins University; Shanmuganathan Chandrakasan, Emory University; Kelly Rouster Stevens, Emory University; Larry Greenbaum, Emory University; Ignacio Sanz, Emory University; Scott E. Gillespie, Emory University; Chelsea Marion, Children’s Healthcare of Atlanta; Karli Okeson, Emory University; Sampath Prahalad, Emory University
• Language: English
• Date: 2021-01-01
• Publisher: SAGE PUBLICATIONS LTD
• Publication Version: Author Accepted Manuscript (After Peer Review)
• Copyright Statement: 2020
• Final Published Version (URL): http://dx.doi.org/10.1177/0961203320969806
• Title of Journal or Parent Work: LUPUS
• Volume: 30
• Issue: 1
• Start Page: 109
• End Page: 117
• Grant/Funding Information: Dr. Ogbu was supported in part by the National Center for Advancing Translational Sciences of the National Institutes of Health under Award Number UL1TR002378 and TL1TR002382. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health. Dr. Prahalad is supported in part by a grant from the Marcus Foundation Inc., Atlanta.
• Keywords: Systemic lupus erythematosus; Rheumatology; nephritis; pediatrics; Science & Technology; Life Sciences & Biomedicine; anemia; thrombocytopenia
• Research Categories: Health Sciences, Medicine and Surgery; Health Sciences, Immunology

Abstract

Objective: To assess whether children with autoimmune cytopenias prior to or at diagnosis of systemic lupus erythematosus (cSLE), differ phenotypically from other cSLE patients; and have a lower risk and severity of lupus nephritis (LN) as observed in prior adult studies. To assess the effect of prior immune therapy for autoimmune cytopenias on 2-year risk of LN. Methods: This was a retrospective cohort study of incident cSLE cases. We included patients aged less than 17 years at diagnosis. We excluded patients with LN at cSLE diagnosis. Our follow-up period was 2 years. We defined autoimmune cytopenias as either autoimmune hemolytic anemia, immune thrombocytopenia or Evan’s syndrome. Results: Forty-three (33%) of the 130 patients had autoimmune cytopenias before or at cSLE diagnosis. Those with autoimmune cytopenias had significantly more neuropsychiatric symptoms and higher mean ESR but less arthritis, malar rash and myositis versus those without autoimmune cytopenias. They had lower 2-year incidence proportion of LN compared to other cSLE patients (7% vs 15%). Of the 16 patients who developed LN, those with autoimmune cytopenias had mostly class V (2 of 3 patients) versus mostly class III and IV in those without autoimmune cytopenias (6 of 12 patients). None of the 13 patients pre-treated for autoimmune cytopenias developed LN. Conclusion: Patients with autoimmune cytopenias before or at cSLE diagnosis have intriguing differences from other cSLE patients. They may represent a unique sub-type of cSLE patients and should be further explored.