Association of systemic lupus erythematosus disease activity index score with clinical and laboratory parameters in pediatric onset systemic lupus erythematosus.

Roshila Shamim,Saira Elaine Anwer Khan,Sumaira Farman,Shabnam Batool,Muhammad Kamil Hussain Raja

doi:10.12669/pjms.36.3.1480

Abstract

Objective:To determine the association of systemic lupus erythematosus disease activity index (SLEDAI) score in pediatric onset SLE (p-SLE) with clinical and laboratory parameters.Methods:This cross sectional observational study was conducted at Division of Rheumatology, Fatima Memorial Hospital, Lahore from November 2018 to January 2019. Total 23 patients diagnosed with p-SLE having onset of symptoms at ≤ 18 years of age, irrespective of their current age at presentation, of either gender, fulfilling criteria of 2012 Systemic Lupus International Collaborating Clinics (SLICC) criteria were enrolled. Patients’ clinical symptoms and laboratory parameters were reviewed, SLEDAI scores were calculated. Collected Data were entered in proforma and analyzed on SPSS version 23.Results:There were 91.3% females. Mean age at diagnosis was 11years ± 4years. At presentation patients had hematological involvement 69.6% followed by mucocutaneous symptoms 65.2% and renal involvement 21.6%. ANA by IFA was positive in all, while anti-ds-DNA was positive in 78.3% patients. SLEDAI score was ≥6 in 87% patients, average SLEDAI score was higher in patients with renal involvement (p=0.06). Elevated ESR (r=0.48, p=0.02), Anti-dsDNA (r=0.44, p=0.05) and low complement levels (p=0.03) were significantly positively correlated, while hemoglobin (r= -0.43, p=0.04) was negatively correlated with the SLEDAI score.Conclusion:In this study, patients with Lupus Nephritis had high SLEDAI scores. Elevated Anti-dsDNA titer, ESR, low complement levels and hemoglobin were significantly associated with high SLEDAI scores. We recommend that SLEDAI score should be calculated in p-SLE patients for stringent disease monitoring and treatment.

Highlights

Systemic Lupus Erythematosus (SLE) is an autoimmune multi system disease that causes dysregulation of immune system leading to damage of various tissues and organs.[1]
No laboratory tests/ intervention has been done for the purpose of this study, it was a standard of care provided to the patients and after informed consent (Appendix I) from patient or parent/ guardian, their medical records were reviewed for clinical symptoms, and laboratory investigations comprising of erythrocyte sedimentation rate (ESR), hemoglobin level, total leucocyte count (TLC), platelet count, urine complete analysis for proteinuria, hematuria (> 5 RBCs), pyuria (>5 WBCs) on high power field and spot urine for protein to creatinine ratio; SLE related specific tests, including Anti-nuclear antibodies (ANA) by IFA, Anti-dsDNA by ELISA technique, complement C3 and C4 levels and histopathology
In this study we have found significant differences in systemic lupus erythematosus disease activity index (SLEDAI) scores of different clinical manifestations

Summary

Introduction

Systemic Lupus Erythematosus (SLE) is an autoimmune multi system disease that causes dysregulation of immune system leading to damage of various tissues and organs.[1] it predominantly affects women of childbearing age, it can occur in all age groups, with pediatric onset SLE (p-SLE) comprising 15-20% of all cases.[2] p-SLE is often a more severe disease with involvement of renal, neurological and hematologic systems[1] leading to permanent damage when compared to adult-onset SLE patients (a-SLE).[1] The upper age limit for p-SLE is not clearly defined, with most studies quoting the. Pak J Med Sci March - April 2020 Vol 36 No 3 www.pjms.org.pk 467 upper age limit cut off of 16 or 18 years. Anti-double stranded DNA antibody (AntidsDNA) another antibody commonly detected in SLE, it has specificity of 97% but sensitivity of 60%.5. By combining these two antibody, we get a higher sensitivity and specificity for the diagnosis of SLE For the diagnosis of SLE, Anti-nuclear antibodies (ANA) by immunofluorescence technique (IFA), with a sensitivity of 90-98%, is considered a gold standard screening test, its specificity is only 7883%.4 Anti-double stranded DNA antibody (AntidsDNA) another antibody commonly detected in SLE, it has specificity of 97% but sensitivity of 60%.5 By combining these two antibody, we get a higher sensitivity and specificity for the diagnosis of SLE

Methods

Results

Conclusion