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Bilateral optic neuritis in pediatric systemic lupus erythematosus with antiphospholipid antibody syndrome
Soumya Patra1, Sriram Krishnamurthy, Anju Seth
1Department of Pediatrics, Lady Hardinge Medical College and associated Kalawati Saran Children's Hospital, New Delhi, 110001, India. dr_soumyapatra@rediffmail.com
Insights
Bilateral optic neuritis is a rare complication in pediatric systemic lupus erythematosus. This case highlights severe vision loss despite treatment, emphasizing the challenges in managing this condition.
Area of Science:
- Pediatric Rheumatology
- Neuro-ophthalmology
Background:
- Pediatric systemic lupus erythematosus (SLE) can present with diverse neurological manifestations.
- Bilateral optic neuritis is an exceptionally rare complication of pediatric SLE.
Observation:
- An 11-year-old girl with SLE presented with fever, anemia, hepatosplenomegaly, and headache.
- She rapidly developed vision loss to no light perception with bilateral optic neuritis.
- Cerebral venous sinus thrombosis was also identified.
Findings:
- The patient exhibited autoimmune hemolytic anemia, thrombocytopenia, and positive anti-dsDNA and anti-phospholipid antibodies.
- Treatment included methylprednisolone, cyclophosphamide, and anticoagulation.
- Despite achieving remission, the patient developed irreversible bilateral optic atrophy and persistent vision loss.
Implications:
- This case underscores the severe visual morbidity associated with optic neuritis in pediatric SLE.
- Early recognition and aggressive immunosuppressive therapy are crucial, though visual outcomes can be poor.
- Further research into novel therapeutic strategies for neuro-SLE complications is warranted.
Abstract:
Bilateral optic neuritis is an extremely uncommon complication of pediatric systemic lupus erythematosus and sporadic cases are reported in the literature. The authors describe an 11-yr-old girl who presented with fever and progressively increasing pallor for 4 months, headache for 7 days, severe anemia and hepatosplenomegaly. Soon after admission, she developed rapid deterioration of vision, worsening to no perception of light with afferent pupillary defect. Fundoscopy showed bilateral optic neuritis. Investigations revealed autoimmune hemolytic anemia and thrombocytopenia. Anti-dsDNA and anti-phospholipid antibodies were positive. Magnetic resonance venography showed multiple thrombi in the cerebral venous sinuses, for which anticoagulant therapy was initiated. She was managed with intravenous methylprednisolone followed by cyclophosphamide pulse therapy for 6 months along with oral prednisolone. Though she went into remission, visual outcome has been dismal, with development of bilateral optic atrophy, and absence of perception of light.
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