Case report: Multifocal central serous retinopathy associated with lupus
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Central serous retinopathy is a condition involving central, localized serous detachment of the sensory retina that may also feature alterations of the retinal pigment epithelium and focal pigment epithelial detachments.
Multifocal central serous retinopathy (CSR) is designated when there are multiple areas of involvement. CSR typically affects middle-aged men, is associated with stress or type “A” personality and is self-limiting with good visual recovery. This case report will discuss a patient with multifocal CSR in the setting of systemic lupus erythematosus (SLE).
Presentation
A 44-year-old man presented with a chief complaint of reduced vision in both eyes 1 week prior without other associated factors.
Medical history included SLE and controlled hypertension. He had previous SLE treatment with Plaquenil (hydroxychloroquine, Sanofi-Aventis) for 3 years, but it was discontinued 8 years ago; he was currently being treated with Cellcept (mycophenolate mofetil, Genentech). He had historically been treated with but was not currently taking oral steroids.
Best corrected visual acuities were 20/70 OD and 20/60 OS. Pupil testing, extraocular motilities and confrontation visual fields were normal. Amsler grid showed a central distortion for the right eye; the left eye was normal. External slit lamp exam and tonometry were normal. Early lenticular changes were non-contributory to the vision reduction. On dilated fundus exam, optic nerves were healthy and well-perfused with CD ratios of 0.3. The peripheral retina was flat and intact in each eye.
At the macula of the right eye a central serous detachment and pigment epithelial detachments (PED) were noted. In the left eye, a large PED with adjacent serous detachment was noted. OCT demonstrated serous detachment with PED inferior to fixation in the right eye and PED adjacent to fixation in the left eye. Fluorescein angiography (FA) showed scattered “hot spots” of serous detachment in each eye. The patient was diagnosed with bilateral multifocal CSR and asked to return for follow-up in 2 months.
Follow-up visit
At the subsequent visit, macular presentation and vision improved. Best corrected vision was 20/60 OD and 20/50 OS. The central serous detachment of the right eye had resolved; the PED persisted. The serous fluid and PED were improved in the left eye.
If there is no improvement at the next follow-up, treatment with photodynamic therapy (PDT) will be initiated.
Discussion, treatment
Retinal pigment epithelium (RPE) disruptions associated with CSR are caused by circulatory abnormalities and vessel stasis (visualized as mid-phase staining on indocyanine green angiography or ICGA). Hypofluorescence on ICGA highlights another possible cause as choriocapillaris non-perfusion leading to venous dilation and congestion.
Enhanced depth imaging OCT typically shows a thickened choroid. It is thought that the hyperpermeability of the choroidal vessels leads to increased hydrostatic pressure that overcomes the RPE cell barrier, allowing leakage into subretinal space. Its propensity for the posterior pole is due to the anatomically high density of choroidal vasculature residing there.
Endogenous steroid exposure via Cushing’s syndrome or stress as well as exogenous steroid use potentiates choroidal vasospasm leading to choroidal vessel congestion, pachychoroid and increased choroidal hydrostatic pressure and permeability. Up to 52% of CSR patients have some form of exogenous corticosteroid exposure (Colucciello). When possible, the suspension of steroid treatment can improve the course of the CSR.
Because most CSR cases resolve in 2 to 4 months, patients are typically observed without treatment initially. A more chronic form of the disease can lead to vision loss, and, thus, treatment may be necessary. In such cases, half-dose PDT proved more efficacious than high-density subthreshold micropulse laser (HSML). The PLACE Trial showed 51.2% of participants treated with PDT had complete resolution of subretinal fluid in 6 to 8 weeks, and 67.2% of patients at 7 to 8 months. Comparatively, with HSML treatment only 13.8% of participants had resolution of subretinal fluid at initial follow-up and 28.8% at final follow up (van Dijk et al.). In patients with persistent serous macula detachment lasting more than 3 months, low dose PDT may provide benefit.
SLE is an autoimmune disease associated with multisystem involvement. It is thought that the systemic vasculitis and the likelihood of systemic hypertension associated with SLE contributes to the pathogenesis of the choroidal vasculature leading to CSR.
Hannouche and colleagues reported a case of bilateral CSR in a female SLE patient; as the systemic condition was controlled, the CSR resolved, and initial visual acuity was restored. The authors proposed immune-complex mediated CSR or the presence of anti-RPE antibodies allowing fluid leakage into the subretinal space.
Eckstein reported two cases of CSR in female SLE patients who did not experience visual recovery over 2-year follow up. Khng and colleagues presented a case series of four female SLE patients with unilateral CSR that self-resolved. Vision was restored to initial level in three of the four cases; the visual outcomes were comparable to that expected in typical CSR cases.
CSR with SLE is rare
CSR associated with SLE is rare and does not often present alongside more typical signs of retinopathy (ie, cotton wool spots or hemorrhaging). Underlying SLE does not seem to affect the course or severity of the condition; therefore, observation and intervention in SLE-associated CSR should follow the same parameters as non-complicated disease.
SLE-associated CSR should serve as an indication to providers that the systemic condition may require additional or alternative treatment.
References
- Colucciello M. Central Serous Chorioretinopathy: Treatment, timing and options. Retinal Physician. 2017;14:24-29.
- Eckstein MB, et al. Visual loss from central serous retinopathy in systemic lupus erythematosus. Br J Ophthalmol. 1993;77(9):607-609.
- Hannouche D, et al. Systemic lupus erythematosus with choroidopathy and serous retinal detachment. Int Ophthalmol. 1995;19(2):125-127.
- Iacono P, et al. Pharmacotherapy of central serous chorioretinopathy: A review of the current treatments. Curr Pharm Des. 2018;24(41):4864-4873.
- Khairallah M, et al. Central serous chorioretinopathy, corticosteroids, and uveitis. Ocul Immunol Inflamm. 2012;20:76-85.
- Khng CG, et al. Central serous retinopathy complicating systemic lupus erythematosus: a case series. Clin Exp Ophthalmol. 2000;28(4):309-313.
- Sadda, et al. Ryan's Retina. Elsevier; 2018.
- van Dijk E, et al. Half-dose photodynamic therapy versus high-density subthreshold micropulse laser treatment in patients with chronic central serous chorioretinopathy: The PLACE trial. Ophthalmol. 2018;125(10):1547-1555.
For more information:
Lynn Finnegan, OD, practices at the Northport Veterans Affairs Medical Center and is an adjunct professor at the SUNY College of Optometry, New England College of Optometry and Salus University College of Optometry. She can be reached at lynn.finnegan@va.gov.
Danielle Kalberer, OD, FAAO, practices at the Northport Veterans Affairs Medical Center and is an adjunct assistant clinical professor at the SUNY College of Optometry. She can be reached at danielle.kalberer@gmail.com.
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