Background and aims Neuropsychiatric manifestations are noted in 50.94% of children with lupus. Chorea, a non-thrombotic complication associated with antiphospholipid antibodies (APLA), is seen in 4% cases.
Aim- To describe the clinical course of a child with lupus who had chorea on follow up and was treated with rituximab
Methods A 10 year old girl presented with spastic quadriparesis, fever, weight loss, alopecia, oral ulcers, anaemia and thrombocytopenia. Antinuclear antibody was 4+ diffuse pattern; and anti-dsDNA titres were 220.25 IU/ml (normal <55 IU/ml). T2 weighted magnetic resonance imaging (MRI) brain and spine showed multiple foci of increased signal intensity in bilateral corona radiata and centrum semiovale. She also had positive lupus anticoagulant and elevated anticardiolipin antibody titres. She received steroids, azathioprine, anticoagulants and aspirin, and showed remarkable improvement. Five years later, she was admitted with multiple joint pains and foot drop related to mononeuritis multiplex. She developed seizures and MRI brain showed diffuse cerebral atrophy with T2/FLAIR white matter hyperintensities. At 18 years of age, she presented with chorea and MRI showed periventricular T2/FLAIR hyperintensities. She responded to intravenous pulses of methylprednisolone and cyclophosphamide. Three years later, she again had similar choreiform movements with aggressive behaviour, which did not respond to haloperidol, steroids and warfarin. At this point of time, she was administered 3 doses of rituximab (500 mg/m2 at one weekly interval).
Results She showed complete recovery after rituximab.
Conclusions Rituximab can be considered as an effective treatment modality in APLA related chorea where conventional measures have failed.
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