5 Discussion
This case report presents a 12year old girl, whose SLE diagnosis was
revealed by a new onset chorea as part of acute neurological
manifestation of SLE along with, bicytopenia, elevated titers of
anti-dsDNA antibodies, positive antinuclear antibodies, and positive
antiphospholipid antibodies.
While chorea can manifest as a symptom in various diseases, when a child
exhibits generalized chorea as the sole symptom, Sydenham’s chorea is
typically the initial diagnosis in regions including Nepal with a high
prevalence of Rheumatic Heart Disease.5The observation
of leukopenia, lymphopenia, and an elevated erythrocyte sedimentation
rate (ESR) raised concerns, leading to the search for an alternative
diagnosis that could encompass the multisystem manifestations. Hence,
the investigations for autoimmune disease were performed, ultimately
revealing a diagnosis of Systemic Lupus Erythematosus (SLE).
SLE is a common diagnosis in the pediatric population.However it mostly
presents in the form of lupus nephritis.6 The American
College of Rheumatology (ACR) nomenclature for neuropsychiatry syndromes
in SLE includes 12 central nervous system syndromes and 7 peripheral
nervous system syndromes.There is a wide variation in the frequency of
NPSLE in various studies. Mild cognitive dysfunction and mood disorders
are frequently seen as manifestations of NPSLE (6-80%) whereas movement
disorders including chorea are extremely rare (< 1% of SLE
patients.7
Neuropsychiatric(NP) manifestations are a rare presentation of Lupus.In
a study carried out in Turkey among 1107 juvenile SLE patients 149
patients had NP involvement (13.5%). The most common NPSLE findings
were headache (50.3%), seizure (38.3%), and acute confusional state
(33.6%). A study found the prevalence of movement disorders in SLE to
be 9.4%.8
Chorea as the sole initial presentation of SLE remains rare across all
age groups. It manifests in only around 1–3% of cases of SLE.9
The exact mechanism behind lupus-associated chorea remains uncertain,
but it is potentially linked to damage in the vascular, neuronal, and
glial components. There is a high incidence of antiphospholipid
antibodies (aPL) in studies of SLE patients with neuropsychiatric
manifestations, between 25 and 90%, perhaps implicating a role of these
antibodies in the pathogenesis.10Vascular injury may
involve both inflammatory and thrombotic processes. The presence of aPL
appears to have a significant impact on the pathogenesis of
neuropsychiatric systemic lupus erythematosus (SLE)11
The European League Against Rheumatism (EULAR) recommends antiplatelet
therapy for SLE patients with movement disorders and positive aPL
antibodies. Anticoagulation agents are only suggested for patients with
thrombotic manifestations. As there are no specific guidelines for
children with neuropsychiatric SLE, the management should be
individualized and based on the decision of the treating
clinician.12
As chorea is a frequent manifestation of rheumatic fever, the diagnosis
of systemic lupus erythematosus (SLE) can be delayed, particularly in
regions where rheumatic fever is widespread. Despite its rarity, it is
essential to consider the possibility of childhood SLE in the
differential diagnosis of any child presenting with unexplained
neurological symptoms.