05_Retinal Vasculitis in SLE_Kessara

8/22/2019 05_Retinal Vasculitis in SLE_Kessara

1/4

Chiang Mai Med J 2009;48(4):159-162.

Address requests for reprints: Kessara Pathanapitoon, M.D., Department of Ophthalmology, Faculty of Medicine,

Chiang Mai University, Chiang Mai 50200, Thailand: E-mail:[email protected]

Received 15 June 2009, and in revised form 15 July 2009.

Case report

SEVERE OCCLUSIVE RETINAL VASCULITIS IN

SYSTEMIC LUPUS ERYTHEMATOSUS: A CASE REPORT

Paradee Kunavisarut, M.D.,1 Kessara Pathanapitoon, M.D.,1

Yodpong Chantarasorn, M.D.,1 Janejit Choovuthayakorn, M.D.,1 Aniki Rothova, Ph.D.2

1Department of Ophthalmology, Faculty of Medicine, Chiang Mai University,2Uveitis Centre, Department of Ophthalmology, University Medical Centre Utrecht,

The Netherlands

Abstract

Severe vaso-occlusive retinopathy, as the primary manifestation of systemic lupus

erythematosus (SLE), is relatively rare. We report a 30-year-old female, who gradually

suffered bilateral visual loss. Fundus examination revealed bilateral severe occlusive

retinal vasculitis and development of neovascularizations. Her clinical manifestations and

laboratory findings were compatible with the diagnosis of systemic lupus erythematosus.

Chiang Mai Medical Journal 2009;48(4):159-162.

Keywords: occlusive retinal vasculitis, systemic lupus erythematosus, bevacizumab

Systemic lupus erythematosus (SLE) is a

chronic systemic autoimmune disease, which

may affect multiple organ systems. It is char-

acterized by the production of pathological

autoantibodies that lead to end-organ damage

via inflammatory mechanisms. Keratocon-

junctivitis sicca is the most common mani-festation in ophthalmic lupus, while the pos-

terior segment involvements have important

implications for visual function. The retinal

arterial occlusion is less common than

office ocular complication.(1) The presence

of retinal manifestations parallels disease

activity elsewhere.(2-4) We, herein, describe a

case of systemic lupus erythematosus, which

who initially presented with bilateral severe

vaso-occlusive retinopathy.

Case report

A previously healthy 30-year-old Thaifemale presented with gradual bilateral visual

loss for 2 months. Visual acuity was finger

counting in both eyes; retinal examination

revealed severe occlusive vasculitis with

diffuse sheathing of the arteries and develop-

ment of neovascularizations located on both


2/4

160 Kunavisarut P, et al.

optic discs. Multiple areas of retinal edema

with hemorrhages and lipid exudates were

present and all vessels exhibited gross caliber

changes (Fig. 1). She had malar rash, discoid

rash, oral ulcer and a history of photosensi-

tivity, arthralgia, and alopecia for one year.

She used no medications. Systemic lupus

erythematosus was initially suspected, so

she consulted a rheumatologist for further

investigations. Complete blood count in-

cluded hemoglobin at 8.3 g/dL without

hemolytic blood features, and normal white

blood cell and platelet count. Erythrocyte

sedimentation rate (ESR) was 139 mm/hour.

Urine analysis found trace proteinuria, with-

out granular cast, and urine protein = 0.35 g

per day. An antinuclear antibody test was

positive at a titer of 1:1280. Renal and liver

function tests were normal. The diagnosis of

systemic lupus erythematosus was made

following the American College of Rheu-matology criteria. The results of serologic

evaluation for HIV and syphilis were nega-

tive. Anticardiolipin antibodies, both Ig G

and Ig M, were negative. Her blood pressure

was normal. The patient was treated with

systemic corticosteroid (1 mg/kg/day) and

posterior subtenon injection with triamcino-

lone (20 mg/0.5 cc), panretinal laser photo-

coagulation and intravitreal injection of 1.25

mg of bevacizumab in both eyes. Within

6 weeks, retinal edema decreased and the

majority of exudates and hemorrhages had

been absorbed. Retinal neovascularizations

disappeared in the right eye and were grossly

reduced in the left one (Fig. 2). Optic disc

pallor developed and arterial sheathing as

well as extremely variable caliber of the

retinal vessels persisted. In the left eye,

adjacent to the optic disc, a development of

fibrous tissue in previous neovascular mem-

branes could seen, which caused traction on

the retina. Unfortunately, within one year, the

neovascularizations of optic discs in both eyes

reappeared. The patient was subsequently

treated with extensive laser photocoagulation

as well as intravitreal bevacizumab injectionsin both eyes. However, vitreous hemorrhage

developed in both eyes, and pars plana vi-

trectomy was required to remove vitreous

hemorrhage and release macular traction.

During the surgery, additional endophoto-

Figure 1. Fundus photograph at the onset of the disease demonstrating severe occlusive vasculitis in

systemic lupus erythematosus.


3/4

Retinal Vasculitis in systemic lupus erythematosus 161

coagulation was also performed. Several

months later, the neovascularizations of optic

discs regressed. Herfinal visual acuity was

0.1 in both eyes

Discussion

Retinal vascular lesions are one of the most

common forms of intraocular involvement in

patients with SLE, and present in 2-30% of

all SLE patients, depending on the activity

and severity of the disease.(5) The retinopathy

in SLE usually consists of cotton-wool spots,

with or without intraretinal hemorrhage, and

does not compromise visual acuity unless it is

located in the macular area. A more severe

variant of retinal vaso-occlusive disease is

less common, and may present with localized

features as central and branch retinal vas-

cular occlusions or diffuse occlusive retinal

disease, as in our patient. Clinicians should

be aware of clinical pictures that resemble

hypertensive retinopathy, which could be

present secondary to lupus renal hyperten-

sion. Still, this patient had lupus retinopathy,

which occurred as an independent manifes-

tation of the underlying disease process in

the absence of hypertension.(6) Severe occlu-

sive SLE retinopathy is associated with CNS

involvement, which did not present in our

patient however. The presence of antiphos-

pholipid antibodies is mostly found when

a search is made in patients with severe

occlusive SLE retinopathy.(7) However, the

test was negative in our patient. The visual

prognosis of diffuse occlusive retinal vascu-

lar disease is poor, and retinal neovascular-

izations with subsequent vitreous hemorrhage

and tractional retinal detachment commonly

develop.(1,5,8) In our patient, visual acuity was

slightly improved in both eyes after treat-

ment. Systemic SLE treatment combined

with scatter laser photocoagulation, and/or

vitreoretinal surgery, contributed to the treat-

ment of retinal neovascularizations. The role

of anti-VEGF agents in the treatment of

SLE retinal vasculopathy or further autoim-

mune diseases have not been systematically

evaluated yet.(9) We illustrated the promising

role of anti-VEGF agents as an adjuvant in

the treatment of retinal neovascularizations

from severe occlusive retinal vasculitis.

In conclusion, this study demonstrated

Figure 2. Fundus photograph six weeks after treatment with systemic and periocular corticosteroids,

panretinal laser photocoagulation and intravitreal injection of 1.25 mg of bevacizumab in both eyes

demonstrating decreased neovascularizations and hemorrhages.


4/4

162 Kunavisarut P, et al.

that SLE might initially manifest with ocular

symptoms, which are potentially severe, and

it also illustrated novel treatment possibilities

with anti-VEGF agents.

References1. Jabs DA, Fine SL, Hochberg MC, Newman SA,

Heiner GG, Stevens MB. Severe retinal vaso-

occlusive disease in systemic lupus erythema-

tous. Arch Ophthalmol 1986;104:55863.

2. Read RW. Clinical mini-review: systemic lupus

erythematosus and the eye. Ocul Immunol In-

flamm 2004;12:87-99.

3. Arevalo JF, Lowder CY, Muci-Mendoza R. Ocu-

lar manifestations of systemic lupus erythemato-

sus. Curr Opin Ophthalmol 2002;13:404-10.

4. Yap EY, Au Eong KG, Fong KY, et al. Oph-

thalmic manifestations in Asian patients with

systemic lupus erythematosus. Singapore Med J

1998;39:557-9.

5. Foster CS. Systemic Lupus Erythematosus In:

Albert & Jakobiec, editors. Principles and Prac-

tice of Ophthalmology. 2nd ed. Philadelphia;

Saunders 2000: 4563-70.

6. Ushiyama O, Ushiyama K, Koarada S, et al.

Retinal disease in patients with systemic lupus

erythematosus. Ann Rheum Dis 2000;59:7058.

7. Montehermoso A, Cervera R, Font J, et al. Asso-

ciation of antiphospholipid antibodies with reti-

nal vascular disease in systemic lupus erythema-

tosus. Semin Arthritis Rheum 1999;28:32632.

8. Au A, ODay J. Review of severe vaso-occlusive

retinopathy in systemic lupus erythematosus and

the antiphospholipid syndrome: associations, vi-sual outcomes, complications and treatment. Clin

Experiment Ophthalmol 2004;32:87-100.

9. Carvalho JF, Blank M, Shoenfeld Y. Vascular

endothelial growth factor (VEGF) in autoim-

mune diseases. J Clin Immunol 2007;27:246-56.

:

systemic lupus erythematosus

, ..,1 , ..,1 , ..,1, ..,1 Aniki Rothova, Ph.D.2

1, 2Uveitis Centre, Department ofOphthalmology, University Medical Centre Utrecht, The Netherlands

Systemic LupusErythematosus 30 systemic lupus erythematosus 2552;48(4):159-162.

: occlusive retinal vasculitis, systemic lupus erythematosus, bevacizumab

Documents

05_Retinal Vasculitis in SLE_Kessara