Clinical course after Rituximab RITUXIMAB IN THE MANAGEMENT OF ANTIPHOSPHOLIPID ANTIBODIES ASSOCIATED CHOREA IN A CHILD WITH LUPUS Vignesh P, Sikha Agarwal, Anju Gupta, Jasmina Ahluwalia#, Deepti Suri, Amit Rawat, Surjit Singh Pediatric Allergy and Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, PGIMER, Chandigarh, India #Department of Hematopathology, PGIMER, Chandigarh, India Introduction Clinical course after Rituximab Variable prevalence of neuropsychiatric manifestations in children with lupus, reported to be between 20%-95%1 Chorea as an isolated manifestation in lupus children is rare Result of both thrombotic and nonthrombotic immune mediated damage2 Strongly linked to coexisting antiphospholipid antibodies (APLA) (4%) Showed complete symptom recovery after rituximab infusions. APLA was negative six months down the line Done well for next one year but later succumbed to pneumonia and acute respiratory distress syndrome MRI Brain Clinical Profile YEAR 2001- 10 year girl diagnosed to have lupus3 Presented with spastic quadriparesis, constitutional symptoms, malar rash, anemia thrombocytopenia, ANA 4+ diffuse pattern, raised anti- ds DNA titres. APLA- Lupus anticoagulant (LA) positive. IgM anti cardiolipin (aCL) antibodies elevated. T2 MRI brain and spine images- multiple increased signal intensity changes, [Fig. 1A]. Started on oral prednisolone, hydroxychloroquine, aspirin. Later Prednisolone tapered, started on azathioprine maintenance. Remarkable improvement in two months. YEAR 2007- Relapsed with multiple joint pains, seizures and foot drop related to mononeuritis multiplex. MRI brain- T2 FLAIR periventricular hyperintensities, [Fig. 1B]. Improved with oral prednisolone 2 mg/kg. Azathioprine restarted. YEAR 2008- Relapsed with seizures and chorea. MRI- T2 white matter hyperintensities and no evidence of thrombus. LA positive. Responded to IV methylprednisolone and cyclophosphamide pulses. YEAR 2009- LA negative, aCL- negative, anti-beta2 glycoprotein I antibody (beta2GPI)- negative. YEAR 2010- LA negative, aCL- negative, anti-beta2GPI- negative. YEAR 2011- Relapsed with choreiform movements and aggressive behavior. LA positive. MRI brain- normal. No response to haloperidol, steroids and warfarin. Administered 3 doses of rituximab (500 mg/m2 at one weekly interval). B A Figure 1. MRI Brain showing A) T2 weighted hyperintensities in corona radiata and centrum semiovale. B) T2 FLAIR hyperintensities in periventricular white matter Discussion High dose steroids and cyclophosphamide consitute the initial primary treatment of neuropsychiatric lupus Rituximab has been used successfully in management of nephritis and hematological manifestations of lupus Few case reports suggest some efficacy of rituximab in neuropsychiatric manifestations4 Moreover, early remission following rituximab in the index patient suggests its promising role in neuropsychiatric lupus Conclusion Rituximab can be considered as an effective treatment modality in APLA related chorea where conventional measures have failed References 1. Singh S, Gupta M, Ahluwalia J, et al. Neuropsychiatric manifestations and antiphospholipid antibodies in pediatric onset lupus: 14 years of experience from a tertiary center of North India. Rheumatol Int 2009; 29: 1455–1461. 2. Avcin T, Silverman ED. Antiphospholipid antibodies in pediatric systemic lupus erythematosus and the antiphospholipid syndrome. Lupus 2007; 16: 627–633. 3. Gupta A, Singh S, Singh P, et al. Spastic quadriparesis: an unusual early manifestation of systemic lupus erythematosus. Scand J Rheumatol 2003; 32: 189–190. 4. Brogna C, Mariotti P, Manna R. Conventional and intravenous immunoglobulin therapy in paediatric antiphospholipid antibodies-related chorea. Lupus 2014; 23: 1449–1451.