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Clinical and immunological profile of SLE: some unusual features
S Singh1, L Kumar, R Khetarpal
1Department of Pediatrics, Post Graduate Institute of Medical Education and Research, Chandigarh.
Insights
Systemic lupus erythematosus (SLE) in children often presents with multisystem involvement, including fever, rash, and arthritis. Early consideration of SLE is crucial due to potentially unusual presentations and significant morbidity.
Area of Science:
- Pediatric Rheumatology
- Immunology
- Clinical Medicine
Background:
- Systemic lupus erythematosus (SLE) is a chronic autoimmune disease affecting multiple organ systems.
- Pediatric SLE presents unique challenges in diagnosis and management.
- Understanding the clinical and immunological profile is vital for early intervention.
Purpose of the Study:
- To elucidate the clinical and immunological characteristics of pediatric SLE.
- To compare findings with existing Indian cohorts.
Main Methods:
- Retrospective hospital-based study at a tertiary center in North India.
- Analysis of medical records of 16 children (aged 4-12 years) diagnosed with SLE.
- Comparison of clinico-pathological features with two other Indian series.
Main Results:
- Common manifestations included rash (87%) and arthritis (87%), with fever in 56.2%.
- Renal involvement occurred in 56.2%; cardiac and pulmonary hypertension were noted in individual cases.
- All patients tested positive for antinuclear antibodies (ANA); mortality was 31.25%.
Conclusions:
- SLE should be suspected in children presenting with multisystemic disease.
- Unusual presentations of pediatric SLE necessitate a high index of suspicion.
- Timely diagnosis and management, potentially following protocols like the NIH Bethesda protocol, can improve outcomes.
Objective:
To study the clinical and immunological profile of children with systemic lupus erythematosus (SLE).
Design:
Retrospective hospital based study.
Setting:
Tertiary level center of North India.
Subjects:
Sixteen children in the age group 4-12 years.
Methods:
Medical records of children with SLE were analyzed. Clinico pathological features were compared with 2 other series from India.
Results:
Mean age of children at the time of diagnosis was 10 yr and 8 (50%) children were less than 10 yr of age. The female to male ratio was 7:1. Fever (56.2%), rash (87%) and arthritis (87%) were the common clinical manifestations, Renal involvement was noted in 56.2% of cases. Other clinical features included hemolytic anemia (31.2%), thrombocytopenia (18.6%) and Raynaud's phenomenon (12.5%). Cardiac involvement in the form of severe myocarditis and endocarditis occurred in one patient each. Pulmonary hypertension was the presenting feature in one child with right heart failure. One child had multiple sclerosis along with SLE--a rare combination. ANA positivity was seen in all children. Five children died; two had severe cardiac involvement. Three children had renal involvement and one died of pulmonary hypertension. Two-thirds of subjects with renal involvement improved after therapy according to NIH, Bethesda protocol.
Conclusions:
SLE must be considered in any child with multisystem disease, as the disease may have certain unusual presentations.