Correspondence

Primary Antiphospholipid Antibody Syndrome with Optic Neuritis

D Rajasekaran*, P Jayapandian**, G Subbaraghavalu***

*Addl. Professor of Medicine; **Postgraduate; ***Asst.Professor of Medicine, Institute of Internal Medicine, Madras Medical College, Chennai. Received: 04.12.2008; Revised: 13.03.2008; Accepted: 14.03.2008

Sir,

“Primary” antiphospholipid syndrome (PAPS) occurs in patients without clinical evidence of any other autoimmune disease, whereas “secondary” antiphospholipid syndrome occurs in association with autoimmune or other diseases1. Ocular manifestations of antiphospholipid syndrome (APS) are usually due to arterial or venous occlusion and are commonly associated with secondary APS. The manifestations include amaurosis fugax, acute ischemic atrophy, progressive optic atrophy and optic neuritis. PAPS are very rarely associated with optic neuritis.

We report a case of PAPS associated with bilateral optic neuritis. A 21 year old unmarried girl was admitted with seizures, left side hemiparesis, and symptoms of raised intracranial hypertension. There was no history suggestive of any connective tissue disorders in the past. On examination she had bilateral lateral rectal paralysis and left side hemiparesis. Her vitals and the other system examination were normal. Her Hb was 7.2 gm%, ESR 78 mm/hr. All other biochemical values and CSF analysis were normal. MRI/MRV of the brain showed cortical venous thrombosis of superior sagittal, transverse, sigmoid sinus and internal jugular veins.

CRP was 12 mg/L, VDRL positive with negative TPI (indicating false positive VDRL), ANA negative (by immunofluorescent method), anticardiolipin antibody IgG 28 GPL (moderate positive), IgM 10.2 MPL (mild positive), APTT 46 seconds (control 36 seconds), dilute Russell viper venom time prolonged, and homocysteine, protein C and S levels were normal. She was treated with antiedema measures, anticonvulsants and low molecular weight heparin.

On the 4th day of admission while on treatment she developed acute loss of vision in both eyes. Examination by an ophthalmologist revealed an absent pupilary reflex, mild papilledema with few cells and normal optic cup. Fundus fluorescent angiography showed a vascular leakage. All the above features were suggestive of optic neuritis. Treatment with adequate dose of methylprednisolone did not improve the vision. Six weeks later, repeat values of anticardiolipin antibody were IgG 28 GPL and IgM 10.8 MPL with prolongation of APTT and dilute Russell viper venom time.

The patient had bilateral optic neuritis with anticardiolipin IgG and IgM positive, LA (APTT of dilute Russell venom time prolongation) on two different occasions 6 weeks apart. She was also ANA negative without features of other connective tissue disorders. Hence, we concluded that the patient is a case of primary antiphospholipid syndrome (PAPS) with optic neuritis. The outcome of the treatment was poor since bilateral optic neuritis tends to relapse even if it responds to prednisolone.

APS is a disease constellation of venous and/or arterial thrombosis, fetal loss (at or after 10 weeks of gestation), thrombocytopenia, anticardiolipin antibodies and lupus anticoagulant that are directed against β2 glycoprotein. Optic neuritis is uncommon in PAPS compared to secondary APS that are associated with SLE. Antibody dependent cytotoxicity may be the cause of retinal cell death and demyelination of optic nerve.2 Intravenous cyclophosphomide pulse therapy or oral immunosuppressants are effective for optic neuritis refractory to corticosteroids treatment. Optic neuritis is very rarely associated with PAPS and the outcome of bilateral optic neuritis is poor compared to unilateral.3

References

1. Jerrolds. Levine, D. Warebranch, Joycerauch The Antiphospholipid Syndrome. New England J Med 2002 ; 346: 752 -763.
2. R.R., Sivarj, O.M. Durani, A.K. Denniston, P.I. Murray, Caroline Gordon. Ocular manifestations of systemic lupus erythematosus.Rheumatology 2007; 46:1757-1762.
3. Munther A, Khamashta. Ocular syndromes. Rheumatic Disease Clinics of North America 2006;32: 476.