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Central retinal venous occlusion usually occurs in elderly patients with known risk factors such as raised intraocular pressure, arterial hypertension, atherosclerotic disease, or diabetes mellitus. In addition, central retinal venous occlusion infrequently occurs in individuals with hyperviscosity from leukaemia, polycythaemia, Waldenstrom’s macroglobulinaemia, sickle cell disease, and periphlebitis.1 However, it has also been described in patients with antiphospholipid antibodies2 and rarely in patients with thrombotic thrombocytopenic purpura (TTP) (pentad of fever, microangiopathic haemolytic anaemia, thrombocytopenia, neurological abnormalities, and renal impairment).3 We describe central retinal venous occlusion in a 26 year old female patient with systemic lupus erythematous (SLE), in whom both TTP and antiphospholipid syndrome (APS) appear to have contributed to the pathogenesis. To our knowledge, co-existence of TTP and APS has not been previously reported in a case of central retinal venous occlusion.
The patient presented in 1994, aged 19, with arthralgia and fatigue. Haemoglobin was 3.5 g/dl and platelet count 15 × 109/l. Investigations revealed SLE (antinuclear antibodies were positive at a titre of 1:640 and anti-double stranded DNA antibodies were 1:320) with Evans’ syndrome (combined autoimmune haemolytic anaemia and immune thrombocytopenia). Tests for APS were not performed. The patient responded to corticosteroids and azathioprine, which were reduced and finally stopped in April 1997.
She remained well until hospitalised in February 2001 with fever of 38°C, gross haematuria, malaise, poor concentration, and headache. Haemoglobin was 7.5 g/dl (Direct Coombs’ test negative) and platelet count 1 × 109/l. Serum creatinine was 244 μmol/l and activated partial thromboplastin time was prolonged at 93 seconds. IgG anticardiolipin antibodies were elevated (18 GPL units/ml) and lupus anti-coagulant was detected by a positive kaolin clotting time index and a dilute Russell viper venom test. C3 and C4 complement components were reduced at 6.6 g/l (normal, 7.5–17 g/l) and 0.4 g/l (normal, 1.4–5.4 g/l) respectively, indicating activation of the classic complement pathway by immune complexes.
She was treated with intravenous antibiotics, blood transfusion and, for the presumptive diagnosis of immune thrombocytopenia, high dose corticosteroids. Early next morning, she complained of sudden onset loss of vision in the right eye. On ophthalmic examination, her visual acuity was hand movements in the right eye and 6/6 in the left eye. She had a dense right relative afferent pupillary defect and right fundal examination revealed completely frosted retinal veins and a severe haemorrhagic central retinal vein occlusion with diffuse superficial and deep retinal haemorrhages (Figs 1 and 2). As the platelet count remained low at 2 × 109/l, a 3 day course of intravenous immunoglobulins (400 mg/kg/day) was commenced. On the third day of hospitalisation, vision in the right eye had not improved and the platelet count remained <5 × 109/l. Review of daily blood films revealed increased numbers of fragmented red cells (schizocytes) while serum LDH was 2049 units/l (normal <450 units/l). A diagnosis of TTP secondary to SLE was made and treatment with 1 litre of cryoprecipitate poor fresh frozen plasma (FFP) twice daily was started. Within 36 hours, the platelet count had risen to 39 × 109/l and aspirin and azathioprine were added. After a week’s therapy with cryoprecipitate poor FFP, platelet count, serum creatinine, and serum LDH were all within normal range with normal blood film.
Three months later, the right eye had developed rubeosis irides, raised intraocular pressure of 34 mm Hg, disc new vessels, and early vitreous haemorrhage. Following treatment with topical β blockers and multiple sessions of panretinal argon laser photocoagulation, the disc new vessels and rubeosis resolved but visual acuity remained poor at hand movements. Ten months later, the patient is taking prednisolone, azathioprine, and aspirin without evidence of TTP but without recovery of vision in the right eye.
In a recent review, anticardiolipin antibodies were detected in eight of 17 patients with SLE and TTP.4 Antiphospholipid antibodies, therefore, may contribute to the pathogenesis of some cases of TTP in association with SLE, possibly by causing endothelial damage in the microvasculature. TTP is a rare but recognised association with central retinal venous occlusion3 while a high prevalence of anticardiolipin antibodies has been reported in patients with vaso-occlusive retinopathy exempt from conventional risk factors.2 However, to our knowledge, this present case of central retinal venous occlusion is unique because of co-existent APS and TTP secondary to SLE. Although aggressive immunosuppressive therapy, aspirin and cryoprecipitate poor FFP led to control of APS and TTP, the severe visual impairment due to central retinal venous occlusion failed to recover.
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