Bilateral optic neuritis in pediatric systemic lupus erythematosus with antiphospholipid antibody syndrome

Indian J Pediatr. 2011 Feb;78(2):234-6. doi: 10.1007/s12098-010-0228-5. Epub 2010 Sep 30.

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.

Publication types

  • Case Reports

MeSH terms

  • Anemia, Hemolytic, Autoimmune / diagnosis
  • Anemia, Hemolytic, Autoimmune / therapy
  • Anti-Inflammatory Agents / therapeutic use
  • Anticoagulants / therapeutic use
  • Antiphospholipid Syndrome / complications*
  • Antiphospholipid Syndrome / diagnosis
  • Antiphospholipid Syndrome / drug therapy
  • Cerebral Veins / physiopathology
  • Child
  • Cyclophosphamide / therapeutic use
  • Female
  • Heparin / therapeutic use
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Lupus Erythematosus, Systemic / complications*
  • Lupus Erythematosus, Systemic / diagnosis
  • Lupus Erythematosus, Systemic / drug therapy
  • Methylprednisolone / therapeutic use
  • Optic Neuritis / complications*
  • Optic Neuritis / diagnosis
  • Optic Neuritis / drug therapy
  • Prednisolone / therapeutic use
  • Thrombocytopenia / diagnosis
  • Thrombocytopenia / therapy
  • Treatment Outcome
  • Venous Thrombosis / diagnosis
  • Venous Thrombosis / drug therapy
  • Warfarin / therapeutic use

Substances

  • Anti-Inflammatory Agents
  • Anticoagulants
  • Immunosuppressive Agents
  • Warfarin
  • Cyclophosphamide
  • Heparin
  • Prednisolone
  • Methylprednisolone

Supplementary concepts

  • Evans Syndrome