# Pericardial Effusion as the First Presentation of Systemic Lupus Erythematosus in a 22-Month-Old Infant: A Case Report and Literature Review

## TL;DR

A 22-month-old infant presented with pericardial effusion as the first sign of systemic lupus erythematosus, the youngest case reported, highlighting the need for early diagnosis in infants.

## Contribution

This is the youngest reported case of SLE presenting with pericardial effusion, expanding the understanding of early infant manifestations of the disease.

## Key findings

- Pericardial effusion was the initial manifestation of SLE in a 22-month-old infant.
- The infant's condition improved significantly with immunosuppression therapy.
- This case is the youngest reported instance of SLE presenting with pericardial effusion.

## Abstract

Childhood-onset systemic lupus erythematosus (cSLE) is a multi-systemic, inflammatory autoimmune disease that affects many organs including the heart. Pericardial effusion as a primary manifestation of SLE in early infancy is very rare. It has been reported as the first symptom of SLE in adult and adolescent case reports only and the youngest reported case was a three-year-old. We report a case of a 22-month-old infant who had previously been healthy but presented with pericardial effusion and a reduced ejection fraction of 20%. She progressed to cardiogenic shock and acute renal failure and required invasive ventilation, inotropic support and temporary dialysis. She was diagnosed with SLE that was genetically confirmed as autosomal recessive SLE. Her condition improved significantly after starting SLE management with immunosuppression therapy. Pericardial effusion has resolved with medical therapy only and cardiac dysfunction has recovered. According to the available literature, this is the youngest reported case of SLE manifesting as pericardial effusion. This case highlights the importance of including SLE in the differential diagnosis for infants presenting with pericarditis, myocarditis, or pericardial effusion to guide early intervention and reduce risks associated with the disease.