Spotlight Case: What a Baseline Examination Uncovered in a Systemic Lupus Erythematosus Patient
A 28-year-old African American female with systemic lupus erythematosus (SLE) and autoimmune lymphoproliferative syndrome is referred to the eye clinic by her rheumatologist for a baseline hydroxychloroquine (HCQ) eye examination 3 months after starting HCQ. The patient reports no vision changes since starting HCQ, but states she has always seen better out of her right eye. Best-corrected visual acuity (BCVA) is 20/20 OD and 20/25 OS with normal pupil reactions, extraocular motility, and intraocular pressure (IOP).
As part of her baseline HCQ screening, 10-2 Humphrey visual field testing is performed (Figure 1). Dilated fundoscopic examination is normal OD, but vascular changes and atrophy OS are noted (Figure 2A-D). Optical coherence tomography (OCT) (Figure 3) and fluorescein angiography (FA) are also performed (Figure 4A-C).
Figure 1: 10-2 Automated visual field test on left eye with a superior scotoma
What’s your diagnosis?
The patient was diagnosed with SLE with remote branch retinal vein occlusion (BRVO) of the left eye and associated collateral formation without macular edema or neovascularization. There was no evidence of HCQ toxicity on examination, OCT, angiography, or visual-field testing.
This patient was referred for HCQ screening, but was found to have a BRVO in the left eye, as evidenced by sheathing of the retinal veins and the formation of collaterals, which cross the horizontal raphe. The appearance of collaterals and the patient’s denial of recent changes in vision suggest the BRVO occurred remotely.
There was no evidence of neovascularization of the iris or angle and no neovascularization of the retina on examination. Inner-retinal thinning on OCT was also consistent with the diagnosis of BRVO. FA confirmed nonperfusion in the distribution of the BRVO and the presence of collateral vessels that demonstrate low-grade leakage without leakage involving the fovea.
BRVO is the most common of the RVOs and can cause significant visual-field loss. The pathogenesis of BRVO can be multifactorial, including:
- Degenerative changes of the blood vessel wall
- Abnormal hematological factors  (hypercoagulability)
- Compression of at the arterial-vein crossing
As a result, macular edema, retinal neovascularization, and macular nonperfusion can develop. Risk factors related to BRVO include:
- Diabetes mellitus
- Systemic inflammatory conditions (as in the case of this patient)
Average reduction in VA for ischemic BRVO is 20/50 and for nonischemic BRVO is 20/60. Acutely, flame hemorrhages, dot and blot hemorrhages, cotton-wool spots, hard exudates, retinal edema, and dilated tortuous veins can be observed. Macula-involving BRVO may cause a central scotoma, though the majority of patients with BRVO have peripheral visual-field loss.
Venous collateral formation and vascular sheathing are often seen in chronic BRVO, as in the case of this patient. OCTs are useful to visualize retinal thinning and macular edema. Lastly, FA permits visualization of areas of ischemia, lack of perfusion, and leakage in retinal blood vessels. The most common complications of BRVO are macular edema and neovascularization, neither of which were observed in the patient.
Depending on VA, the presence or absence of macular edema, and the presence or absence of neovascularization, patients may be offered anti-VEGF therapy, steroids, or retinal laser. They should also be monitored for the development of neovascularization of the angle and glaucoma.
HCQ is used in the treatment of systemic inflammatory disease such as rheumatoid arthritis and systemic lupus erythematosus. Although the method of toxicity has not been clarified, HCQ is known to have effects on the metabolism of the retinal photoreceptors in the retinal pigment epithelium (RPE). The recommended clinical assessment tools in HCQ toxicity screening to assist in documenting possible anatomical and subjective visual loss are automated visual field testing, OCT, and FA initially in the first year of HCQ treatment and then annually. The patient was advised to follow closely every 2 months due to history of lupus, and her rheumatologist was advised to keep her HCQ dose below 6.5 mg/kg/d as a precautionary step.
1. Jaulim A, Ahmed B, Khanam T, Chatziralli IP. Branch retinal vein occlusion: epidemiology, pathogenesis, risk factors, clinical features, diagnosis, and complications. An update of the literature. Retina. 2013;33(5):901-910. doi:10.1097/IAE.0b013e3182870c15.
2. Rehak J, Rehak M. Branch retinal vein occlusion: pathogenesis, visual prognosis, and treatment modalities. Curr Eye Res. 2008;33(2):111-131. doi:10.1080/02713680701851902.
3. Marmor MF, Kellner U, Lai TY, Lyons JS, Mieler WF; American Academy of Ophthalmology. Revised recommendations on screening for chloroquine and hydroxychloroquine retinopathy. Ophthalmol. 2011;118(2):415-422. doi:10.1016/j.ophtha.2010.11.017.
Dr. Reddy - None.
Ms. Aman - None.
Dr. Hau - SEQUENOM: Speaker, Honoraria; THROMBOGENICS, INC: Other, Honoraria.
Dr. Choudhry - ALLERGAN, INC: Advisory Board, Speaker, Honoraria; BAUSCH + LOMB, INC: Advisory Board, Honoraria; BAYER HEALTHCARE: Consultant, Speaker, Honoraria; NOVARTIS: Advisory Board, Speaker, Grants, Honoraria; OPTOS PLC: Speaker, Honoraria.