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Case Reports
. 2020 Jun 2:19:100763.
doi: 10.1016/j.ajoc.2020.100763. eCollection 2020 Sep.

Visual recovery and vascular reperfusion after vaso-occlusive retinopathy from anti-phospholipid syndrome associated with systemic lupus erythematosus

Jordan D Deaner  1 , Andrew S Zeft  2 , Parisa Emami-Naeini  1 , Careen Y Lowder  1
Affiliations
Case Reports

Visual recovery and vascular reperfusion after vaso-occlusive retinopathy from anti-phospholipid syndrome associated with systemic lupus erythematosus

Jordan D Deaner et al. Am J Ophthalmol Case Rep. .

Abstract

Purpose: To report a case of visual recovery and vascular reperfusion after vaso-occlusive retinopathy from anti-phospholipid syndrome associated with systemic lupus erythematosus.

Observations: A 15-year-old boy with a known diagnosis of systemic lupus erythematosus and a clinically significant anti-phospholipid panel presented with sudden vision loss in the left eye. Examination and ocular imaging revealed signs of vaso-occlusive retinopathy. The patient was immediately started on high dose intravenous steroids, followed by mycophenolate mofetil. He remained on aspirin. After showing no improvement in retinal arteriole and capillary perfusion he was started on therapeutic anti-coagulation with enoxaparin. He regained 20/20 vision. Intravenous fluorescein angiography demonstrated reperfusion of retinal arterioles. Optical coherence tomography angiography showed return of flow in the capillary networks.

Conclusions: We present a case of vaso-occlusive retinopathy in a patient with known systemic lupus erythematosus and a clinically significant anti-phospholipid panel, thus meeting criteria for anti-phospholipid syndrome. He was treated with intravenous methylprednisolone, mycophenolate motefil, aspirin, and enoxaparin. The patient not only had great recovery of visual acuity, but also demonstrated reperfusion of arterioles and reconstitution of flow in the retinal capillary network. These findings suggest that the vaso-occlusive disease is reversible if the diagnosis is made promptly and intensive therapy is initiated.

Importance: Currently there are no reported cases of vaso-occlusive retinopathy from APLS and SLE with visual recovery, reperfusion, and return of capillary flow.

Keywords: APLS; Case report; Systemic lupus erythematosus; Vaso-occlusive retinopathy.

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Figures

Fig. 1
Fig. 1
Fundus photograph of the left eye at the time of patient presentation showing numerous cotton wools spots in the macula and posterior pole.
Fig. 2
Fig. 2
OCT of the left eye macula showing retinal thickening, inner retinal hyperreflectivity, cystoid edema, and subretinal fluid.
Fig. 3a
Fig. 3a
Late fluorescein angiogram of the right eye demonstrating normal vascular perfusion.
Fig. 3b
Fig. 3b
Late fluorescein angiogram of the left eye demonstrating multiple branch retinal artery occlusions, diffuse vascular leakage in the macula, and macular capillary non-perfusion.
Fig. 4a
Fig. 4a
OCT angiography of the unaffected right eye showing a normal flow pattern.
Fig. 4b
Fig. 4b
OCT angiography of the left eye showing significant loss of capillary flow signal.
Fig. 5a
Fig. 5a
OCT angiography of the left eye at 3-month follow up showing reconstitution of flow signal in the macula.
Fig. 5b
Fig. 5b
OCT of the left eye at 3-month follow up showing resolution of CME and SRF, along with improvement in retina thickening.
Fig. 5c
Fig. 5c
Late fluorescein angiography of the left eye showing reperfusion of previously occluded arterioles and improved vascular leakage.
Fig. 6
Fig. 6
Fundus photo of the left eye at 4-month follow up showing resolution of cotton wool spots.
Fig. 7
Fig. 7
Late fluorescein angiography of the left eye at 10-month follow up demonstrating complete resolution of vascular leakage.
See this image and copyright information in PMC

References

    1. Silpa-archa S., Lee J.J., Foster C.S. Ocular manifestations in systemic lupus erythematosus. Br J Ophthalmol. 2016;100(1):135–141. - PubMed
    1. Peponis V., Kyttaris V.C., Tyradellis C., Vergados I., Sitaras N.M. Ocular manifestations of systemic lupus erythematosus: a clinical review. Lupus. 2006;15(1):3–12. - PubMed
    1. Acharya N., Pineda R., Uy H.S., Foster C.S. Discoid lupus erythematosus masquerading as chronic blepharoconjunctivitis. Ophthalmology. 2005;112(5):e19–23. - PubMed
    1. Miyakis S., Lockshin M.D., Atsumi T. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS) J Thromb Haemost JTH. 2006;4(2):295–306. - PubMed
    1. Taraborelli M., Leuenberger L., Lazzaroni M.G. The contribution of antiphospholipid antibodies to organ damage in systemic lupus erythematosus. Lupus. 2016;25(12):1365–1368. - PubMed

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