Systemic lupus erythematosus presenting with status epilepticus and acute cardiomyopathy with acute heart failure:
H M M T B Herath1, Aruna Kulatunga1
1National Hospital of Sri Lanka, Colombo, Sri Lanka.
Insights
This case highlights uncommon but serious complications of systemic lupus erythematosus (SLE), including status epilepticus and acute myocarditis with heart failure in a young female. Early recognition of these atypical SLE presentations is crucial for better patient outcomes.
Area of Science:
- Rheumatology
- Neurology
- Cardiology
Background:
- Systemic lupus erythematosus (SLE) is a complex connective tissue disorder with multi-organ involvement.
- While neurological and cardiac issues are known SLE manifestations, status epilepticus and acute myocarditis with heart failure at initial presentation are rare.
- This case underscores the diverse and sometimes atypical clinical spectrum of SLE.
Observation:
- A 15-year-old female presented with refractory status epilepticus.
- She subsequently developed tachycardia, shortness of breath, and heart failure with reduced ejection fraction.
- Diagnostic workup revealed positive ANA, high anti-ds-DNA, low C3/C4, and urinary sediment, confirming SLE.
Findings:
- The patient's presentation with both neurological (status epilepticus) and cardiac (myocarditis) complications is atypical for SLE.
- This case underscores the diverse clinical spectrum of SLE, particularly in young females.
- Prompt diagnosis and management are critical due to the potential for severe outcomes.
Implications:
- Clinicians must maintain a high index of suspicion for SLE in young patients presenting with unusual multi-organ involvement.
- Early identification of rare SLE manifestations like status epilepticus and myocarditis is vital.
- Timely and appropriate management can improve prognosis and reduce mortality in SLE patients.
Introduction:
Systemic lupus erythematosus is a connective tissue disorder, which causes complex multi organ involvement. Neurological and cardiac manifestations have been well noted but complications such as status epilepticus and acute myocarditis with heart failure at presentation remains uncommon.
Case Description:
A 15-year-old, previously healthy, South Asian, Sri Lankan female presented with status epilepticus and the seizures only responded to intravenous midazolam and thiopentone sodium. On the fourth day, she developed tachycardia and shortness of breath and was found to have cardiomyopathy with heart failure with an ejection fraction 40%. Along with a positive urinary sediment, a positive ANA with a very high level of ds-DNA and low C3 and C4 levels confirmed our suspicion of systemic lupus erythematosus.
Discussion And Evaluation:
Systemic lupus erythematosus presents in a variety of clinical presentations and the spectrum may range from unique to ubiquitous. Clinicians should have a high index of suspicion specially when encountering atypical presentations with multi-organ involvement, especially when patients tend to be young females. Status epilepticus and myocarditis are uncommon manifestations of systemic lupus erythematosus, and should be appreciated early, as if inappropriately managed would have a deleterious impact on mortality and morbidity.
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