A 44-year-old African-American female presents to the emergency department having lost vision in her right eye 4 days previously. She reports fatigue and photophobia, but no eye pain or headache. On questioning, the patient also denies having any joint pain or chest pain, skin rash, or constitutional symptoms. Her medical history includes stable systemic lupus erythematosus (SLE) – as a result of her lupus, she developed kidney inflammation, followed by end stage renal disease. She explains that she recently had a failed renal transplant.
Findings of cardiac, lung, and abdominal examinations are unremarkable. Neurological examination findings are also normal, and cranial nerves I and III-XI are grossly intact. The patient has no clinical or historical signs of systemic infection.
- Right eye: Unable to perceive light; nil near vision and nil color
- Visual fields to confrontation in temporal field only
- Left eye: Visual acuity 20/25; near vision a brisk Jaeger 1; color vision intact. Visual fields were full to confrontation; normal optic disc and retinal vasculature
- Pupils are 3 mm being nonreactive on the right and 1+ reactive on the left, and there is a 2+ right relative afferent pupillary defect; the patient is orthophoric at distance with full ductions, versions, pursuit, and saccadic eye movements
- Examination shows no significant ptosis, enhanced ptosis, lid lag, or lid retraction
- Cranial nerves V and VII are intact with normal corneal reflexes; ocular tensions are 12 and 17, respectively, and each eye is normal on slit lamp examination
- Fundoscopic examination reveals marked optic disc swelling with a markedly diffusely pale optic disc and 1+ retinal venous tortuosity; there are no vitreous cells and no papillary or retinal hemorrhages
Computerized tomography scan does not detect any neoplastic masses, acute infection/brain abscess, or evidence of optic nerve compression. Drug screening and serum electrolytes, as well as complement (C3 and C4) levels, are all within normal limits.
Lab Test Results (Test Patient (Normal reference range)
- Erythrocyte sedimentation rate: 61 mm/hour (normal reference range is 0-29 mm/hour)
- Anti-nuclear ab (antibody) >12 U (<1 U)
- Anti-ds-DNA ab 294 IU/mL (<30 IU/mL)
- Anti-Sm ab >8 U (<1 U)
- Anti-phospholipid IgG ab 20.9 GPL (<10 GPL)
Diagnosis and Treatment
Based on the clinical examination findings, the severe vision loss, including no light perception, and elevated titers of several inflammatory markers consistent with a lupus flare, clinicians diagnose the patient with acute optic neuropathy.
The patient is started on high-dose intravenous dexamethasone (250 mg every 6 hours), and within a few hours she begins to recover the vision in her right eye. Within 3 days, she can perceive a hand two feet away in all visual fields.
She is transitioned from IV dexamethasone to oral prednisone (80 mg/day), which is tapered over 3 weeks. However, vision in her right eye never returns to its pre-hospitalization baseline of 20/60-1; a later assessment finds it to be 20/200.
This case1 reports a patient with an active lupus flare who presents with painless vision loss affecting only her right eye, with optic disc edema and elevated antiphospholipid antibody titers. SLE is a complex inflammatory disease involving formation of both antibodies and immune complexes that can trigger a systemic inflammatory reaction. This widespread inflammation is responsible for the multiple organ changes associated with SLE.2,3
Clinical manifestations that can differentiate SLE patients from healthy people include skin lesions, arthritis, renal disorder, neurologic disorder, and hematologic changes, among others.2,3
Diagnosis of SLE involves confirmation of four out of 11 diagnostic criteria for SLE developed by the American College of Rheumatology.2,3 These may be identified on present history and physical or documented at some time in the past.
Test Sensitivity for SLE diagnosis
Blood tests for lupus vary in sensitivity and specificity, and were as follows.4
- Antinuclear antibody: Screening test; sensitivity 95%; not diagnostic without clinical features
- Anti-double-stranded DNA: High specificity; sensitivity only 70%; level is variable based on disease activity
- Anti-Smith: Most specific antibody for SLE; only 30-40% sensitivity
- Anti-Sjögren syndrome (SS) A (Ro)/Anti-SSB (La): Present in 15% of patients with SLE and other connective-tissue diseases
Ocular Manifestations of SLE Common
SLE has ocular manifestations in up to one-third of people affected; because of the thrombotic and inflammatory nature of the disease, almost every anatomical structure of the eye may be affected.2,3
Symptoms can range from dry eyes (i.e., in secondary SS) to complete vision loss when the retina and optic nerve are affected. Optic neuritis and ischemic optic neuropathy (anterior and posterior) affect only 1% of SLE patients,5 but can result in complete loss of vision.
Patients with confirmed lupus should have a thorough ophthalmologic examination, including a detailed fundoscopic examination of the blood vessels and nerves. Various findings include eyelid abnormalities, ocular adnexa, keratoconjunctivitis sicca, iridocyclitis, retinal vasculitis, vaso-occlusive disease, choroidopathy, and optic neuropathy.6
SLE-associated Ocular Diseases
Optic neuritis is usually acute, with painful loss of vision, and due to infarction of the optic nerve secondary to arteriolar fibrinoid necrosis.7
Optic neuropathy is bilateral, with painless vision loss, with or without optic disc swelling, and due to ischemia affecting the optic nerve head and retrobulbar nerve.6,7 Conversely (as in this rare case), unilateral vision loss in the presence of antiphospholipid antibodies, secondary to a focal thrombotic event that affects microcirculation rather than a global, vasculitic process.8
In SLE patients with suspected optic neuritis, advance testing with fundus fluorescein angiography (FFA) can detect retinal vasculature changes,5 including vasodilation of the peripapillar capillaries and hyperfluorescence.9 Ischemic optic neuropathies are marked by incomplete capillary and choroidal filling, followed by hyperfluorescence leakage (Figure).
Optic neuropathies due to retrobulbar lesions may appear normal on fundoscopy and FFA, necessitating magnetic resonance imaging with gadolinium contrast to diagnose optic nerve enlargement and enhancement.5,10
Immunosuppressive therapy is the mainstay of treatment for both systemic and ocular SLE.11 SLE optic neuropathy is typically treated with high-dose intravenous methylprednisolone (1 g/day for 3 days) followed by oral prednisone (1 mg/kg/day).12 Pulse-dosed intravenous cyclophosphamide for 6 months may be an effective alternative13 in the up to 29% of patients refractory to corticosteroids.14
Treatment for ischemic optic neuropathy is controversial, especially in the presence of antiphospholipid antibodies, noted the authors of the case report, Alexander J. Heckman, MD, of the Mayo Clinic in Jacksonville, Florida, and colleagues. They said that when visual loss is likely caused by an acute thrombotic event, anticoagulation with warfarin or aspirin should be considered.11,15
In addition, to prevent thrombosis in the presence of antiphospholipid antibodies in patients with SLE, hydroxychloroquine may be considered.16
High-dose corticosteroids may be given empirically to treat the systemic autoimmune illness, the team added.15 Heckman and co-authors explained that they decided to use dexamethasone rather than the standard methylprednisolone due to the higher free plasma levels and central nervous system penetration.12
Optic neuropathy is a rare, acute, vision-threatening ophthalmic complication of SLE. A thorough patient history and funduscopic examination combined with serological studies are crucial to accurate diagnosis. The authors concluded that understanding the workup and the importance of rapid immunosuppressive treatment, along with awareness of the disease’s rare unilateral presentation, may prevent vision loss in SLE patients.
1. Heckman AJ, et al “Acute Unilateral Vision Loss Due to Optic Neuropathy in a Patient with Systemic Lupus Erythematosus” Am J Case Rep 2019; 20: 97-100.
2. Tan EM, et al “The 1982 revised criteria for the classification of systemic lupus erythematosus” Arthritis Rheum 1982; 25: 1271–1277.
3. Yu C, et al “Diagnostic criteria for systemic lupus erythematosus: A critical review” J Autoimmun 2014; 48-49: 10-13.
4. Bartels CM “Systemic Lupus Erythematosus (SLE) Workup” https://emedicine.medscape.com/article/332244-workup, Updated: Dec 12, 2018.
5. de Andrade FA, et al “Neuro-ophthalmologic manifestations in systemic lupus erythematosus” Lupus 2017; 26(5): 522-528.
6. Palejwala NV, et al “Ocular manifestations of systemic lupus erythematosus: A review of the literature” Autoimmune Dis 2012; 2012: 290898.
7. Sivaraj RR, et al “Ocular manifestations of systemic lupus erythematosus” Rheumatology (Oxford) 2007; 46(12): 1757-1762.
8. Giorgi D, et al “Optic neuropathy in systemic lupus erythematosus and antiphospholipid syndrome (APS): Clinical features, pathogenesis, review of the literature and proposed ophthalmological criteria for APS diagnosis” Clin Rheumatol 1999; 18(2): 124-131.
9. Vine AK, Barr CC “Proliferative lupus retinopathy” Arch Ophthalmol 1984; 102: 852-854.
10. Uy HS, Chan PS “Systemic lupus erythematosus. In: Foster CS, Vitale A (eds.), Diagnosis & Treatment of Uveitis, 2nd ed” New Delhi: Jaypee Brothers Medical Publishers 2013.
11. Bajwa A, Foster SC “Ocular manifestations of systemic lupus erythematosus” J Clin Cell Immunol 2014; 5: 191.
12. Balis FM, et al “Differences in cerebrospinal fluid penetration of corticosteroids: Possible relationship to the prevention of meningeal leukemia” J Clin Oncol 1987; 5(2): 202-207.
13. Frigui M, et al “Optic neuropathy as a presenting feature of systemic lupus erythematosus: Two case reports and literature review” Lupus 2011; 20(11): 1214-1218.
14. Galindo-Rodriguez G, et al “Cyclophosphamide pulse therapy in optic neuritis due to systemic lupus erythematosus: An open trial” Am J Med 1999; 106(1): 65–69.
15. Tugcu B, et al “Nonarteritic anterior ischemic optic neuropathy as the presenting manifestation of primary antiphospholipid syndrome” Indian J Ophthalmol 2014; 62(5): 642-644.
16. Szymezak J, et al “Hydroxychloroquine: A new therapeutic approach to the thrombotic manifestations of antiphospholipid syndrome” Rev Med Interne 2010; 31(12): 854-857 [in French].
No disclosures were reported.