Statistics from Altmetric.com
Editor,—Central serous chorioretinopathy is thought to be an idiopathic disease and such cases are not usually investigated for an underlying cause. Bullous exudative retinal detachment is a recognised complication of central serous chorioretinopathy (CSR).1 There are a number of reports of exudative retinal detachments occurring in the context of generalised coagulopathies.2 3 We present a patient with central serous chorioretinopathy who on further investigation was found to have the primary antiphospholipid syndrome.
A 42 year old white man presented in December 1992 with difficulty in reading and a grey “circle” in his right central field of vision of 6 weeks' duration. His past medical history consisted of widespread burns requiring skin grafting and complicated by septicaemia 20 years previously. Apart from two short courses of antimalarial tablets within the past 6 years, he had been taking no other medication. He was a non-smoker, but had been a heavy drinker. Corrected visual acuity was 6/9 N12 right eye, 6/9 N5 left eye. Funduscopy revealed bilateral retinal pigment epithelial (RPE) disturbances with no dye leakage on fluorescein angiography. Over subsequent months, transient serous elevation of the neurosensory retina at the right macula was noted. In October bilateral pigment epithelial detachments were noted with visual acuities of right eye 6/24 N10, left eye 6/60 N48. A trial of oral prednisolone seemed to halt the deterioration in vision and subsequently the patient was given 1 g daily of intravenous methylprednisolone for 3 days. A subsequent autoimmune screen revealed a positive lupus anticoagulant test (Russell viper venom with Cephalin 1.61 (0.95–1.05), Russell viper venom with platelets 1.12 (0.95- 1.05)) and the presence of anticardiolipin antibodies (anticardiolipin IgG 46.3 gpl units (0–13.3)). Antinuclear antibodies were weakly positive at 1:20 with a homogeneous/speckled pattern but anti-double stranded DNA, complement, extractable nuclear antigens and rheumatoid factor were negative. Steroids were stopped and the patient was given aspirin.
On subsequent review the patient had developed a large bullous inferior exudative retinal detachment in the right eye (Fig 1). B-scan ultrasound revealed retinal detachment with presumed subretinal fibrosis (Fig 2). Eventually the retinal detachment resolved spontaneously with the patient taking aspirin alone. Visual acuity is currently right eye 1/60, left eye 6/36. The patient manages N8 with a reading loupe.
Idiopathic CSR tends to be a self limiting disease4 which can be complicated by the development of exudative retinal detachments.1 The exact aetiology of CSR remains obscure. Indocyanine green angiography findings suggest that perfusion changes in the choriocapillaris may be responsible.5 Occlusive ocular vascular disease involving the retinal and choroidal vessels has been reported in up to 8% of patients with raised anticardiolipin antibodies.6 Serous detachment of the macula has been reported with the primary antiphospholipid antibody syndrome; however, other ocular and systemic features were always noted.2
The presence of antiphospholipid antibodies (anticardiolipin and lupus anticoagulant) can result in abnormalities of coagulation leading to venous and arterial thrombosis although exactly why this happens is not fully understood. Putative mechanisms include inhibition of antithrombin and activated protein C dependent anticoagulant systems, inhibition of fibrinolysis, and interference with the anticoagulant properties of β2 glycoprotein I.7
We postulate that some cases of presumed idiopathic CSR may reflect thrombosis of the choroidal circulation secondary to the primary antiphospholipid antibody syndrome. Further studies are warranted to prove or disprove this hypothesis.
If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.