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Clinical and Immunological Profile of Pediatric-Onset Systemic Lupus Erythematosus in Eastern India: A Prospective
Soumya Mishra1, Jyoti Ranjan Behera2, Amit R Rup2
1Pediatrics, Institute of Medical Sciences and SUM Hospital, Bhubaneswar, IND.
Insights
Pediatric Systemic Lupus Erythematosus (pSLE) is a severe autoimmune condition affecting multiple systems in children. This study highlights its clinical features and outcomes in Eastern India, emphasizing early diagnosis for better management.
Area of Science:
- Pediatric Rheumatology
- Autoimmune Diseases
- Clinical Immunology
Background:
- Systemic Lupus Erythematosus (SLE) is a multisystem autoimmune disease with a known female predominance.
- Pediatric SLE (pSLE) often presents with a more severe prognosis than adult-onset SLE.
- Limited data exists on pSLE in the Indian subcontinent.
Purpose of the Study:
- To characterize the clinical manifestations, laboratory findings, and serological markers of pSLE.
- To document the management strategies and patient outcomes in pSLE.
- To provide insights into pSLE from a tertiary care center in Eastern India.
Main Methods:
- A prospective observational study was conducted from September 2020 to October 2023.
- Included children aged 1-14 years meeting the Systemic Lupus International Collaborating Clinics (SLICC) criteria for SLE.
- Detailed clinical assessments, laboratory investigations, and outcome data were collected and analyzed.
Main Results:
- The incidence of pSLE was 0.35 per 1000 children, with a female-to-male ratio of 7:1.
- Mucocutaneous (97.5%), hematological (90%), and renal (47.5%) systems were most commonly involved.
- Lupus nephritis occurred in 47.5% of patients; complete remission was achieved in 35%.
Conclusions:
- pSLE is an uncommon yet severe autoimmune disease with significant multi-systemic impact.
- Early diagnosis and prompt intervention are critical for improving outcomes in pSLE.
- This study offers valuable data on pSLE in Eastern India, advocating for larger multicentric research.
Background:
Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease with a strong female predisposition. pSLE often results in a worse prognosis compared to adult SLE. Studies on pSLE from the Indian subcontinent are scarce.
Objective:
This study aims to describe the clinical manifestations, laboratory and serological parameters, management, and outcomes of pSLE patients from a premier tertiary care institute in Eastern India.
Methods:
This prospective observational study was conducted at Kalinga Institute of Medical Sciences, Bhubaneswar, from September 2020 to October 2023. Children aged 1-14 years fulfilling the Systemic Lupus International Collaborating Clinics criteria for SLE were included. A detailed history, clinical examination, and laboratory investigations were performed. Data on complications, treatment, and outcomes were collected. Statistical analysis was done using SPSS Statistics version 21 (IBM Corp. Released 2012. IBM SPSS Statistics for Windows, Version 21.0. Armonk, NY: IBM Corp.).
Results:
Out of 114,009 patients (outdoor and indoor), 40 were diagnosed with pSLE, resulting in an incidence of 0.35 per 1000 children. The female-to-male ratio was 7:1. The mean age of presentation was 11.67 ± 2.37 years. Among the predominant symptoms observed, mucocutaneous manifestations were seen in 39 (97.5%), followed by pallor in 36 (90%), and fever in 33 (82.5%). The most common organ system involved was mucocutaneous, observed in 39 (97.5%) patients, followed by hematological in 36 (90%) and renal in 19 (47.5%). Lupus nephritis was observed in 19 (47.5%) patients, with class IV being the most common. Anti-nuclear antibody and anti-double-stranded DNA were positive in 39 (97.5%) and 27 (68%) of children, respectively. Complete remission was achieved in 14 (35%), improvement in 16 (40%), and flare-ups in 3 (7.5%) patients.
Conclusion:
pSLE is an uncommon but severe autoimmune disease with significant multi-system involvement. Early identification and prompt treatment are crucial to minimizing adverse outcomes. This study provides detailed insights into the clinical and immunological profile of pSLE in Eastern India, underscoring the need for larger multicentric studies with long-term follow-ups.
