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Year : 2014  |  Volume : 34  |  Issue : 6  |  Page : 280-282

Lupus flare in a manifestation of consecutive hypotony maculopathy after trabeculectomy

Department of Ophthalmology, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan, Republic of China

Date of Web Publication19-Dec-2014

Correspondence Address:
Da-Wen Lu
Department of Ophthalmology, Tri-Service General Hospital, National Defense Medical Center, No. 325, Sec. 2, Cheng Gong Road, Taipei, 114, Taiwan
Republic of China
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1011-4564.147278

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Systemic lupus erythematosus (SLE) up-raises the surgical risk due to its unpredictable perioperative disease activity. Lupus flare represents an important issue because of its potential threat in organ damage and drug toxicity after adjusting the dosage of immune-modulating agents. A 34-year-old female was referred for trabeculectomy surgery for her poor-controlled steroid-related glaucoma with a 5-year history of SLE under systemic steroids control. Remission status was confirmed with normal serum complement levels. However, lupus flare with clinical ocular findings as hypotony maculopathy presented 1-week after uneventful surgery. Early and effective treatment with pulse corticosteroid therapy was conducted, and this patient recovered her ocular performance 1-month later except preexisting glaucomatous visual field deficits. SLE flare may be seen in any form of postoperative complications, even masked with consecutive hypotony maculopathy. This case reminds early detection of systemic lupus flare and different therapeutic plan is prompt in postoperative follow-up.

Keywords: Macular hypotony, systemic lupus erythematosus, trabeculectomy

How to cite this article:
Chien KH, Lu DW, Tai MC. Lupus flare in a manifestation of consecutive hypotony maculopathy after trabeculectomy. J Med Sci 2014;34:280-2

How to cite this URL:
Chien KH, Lu DW, Tai MC. Lupus flare in a manifestation of consecutive hypotony maculopathy after trabeculectomy. J Med Sci [serial online] 2014 [cited 2020 Jul 6];34:280-2. Available from: http://www.jmedscindmc.com/text.asp?2014/34/6/280/147278

  Introduction Top

Systemic lupus erythematosus (SLE) is a complex immune response related systemic disorders which characterized as end-organ dysfunction by an autoantibody deposition. Majority are women in their second and third decades. The prevalence of SLE in the western countries is higher in black females than in Caucasian females and black SLE patients more often developing severe complications and high mortality. [1] Treatment of SLE is composited with corticosteroid and immune suppressants, and it remains a clinical challenge due to its complications related to life-long medication. Corticosteroid-induced intraocular pressure (IOP) elevation is much often encountered in topical steroids of ophthalmologic medication, but it may be overlooked in systemic steroids. The correlation between systemic steroids and glaucoma remains unclear due to more complex pathophysiologic mechanisms are involved. Herein, we reported a case of steroid glaucoma who developed lupus flare mimicking postoperative hypotony maculopathy.

  Case Report Top

A 34-year-old woman presented with glaucoma in both eyes, who had been under medication with Cosopt (Merck and Co., Inc., Whitehouse Station, NJ, USA) and Xalacom (Pharmacia NV/SA, Purrs, Belgium) for 4-year. She also had a 5-year history of SLE treated with oral prednisolone (10 mg/day). She was referred to our clinic because of a sudden elevation of IOP for 1-week. Ophthalmologic exams showed a best-corrected visual acuity (BCVA) of 6/6 and increased cupping with an IOP of 40 mmHg in both eyes. Other examinations were unremarkable. Good control of the patient's SLE status was affirmed with a rheumatologist based on normal C 3 and C 4 level. 40 mg methylprednisolone was given as an intravenous drip 30 min before the operation as advised. Trabeculectomy with a mitomycin C soaking for 1 min (0.4 mg/mL; Kyowa Hakko Kogyo Co., Ltd., Tokyo, Japan) was performed uneventfully in the right eye. On the 1 st day after surgery, an IOP of 6 mmHg with a well-formed bleb and a clear anterior chamber were noted.

One week later, the patient presented at our emergency department with a sudden onset of blurred vision in the right eye. On ophthalmology, her BCVA was 6/60 in the right eye and 6/6 in the left eye, with an IOP of 7 mmHg in the right eye and 19 mmHg in the left eye. Moderate flares and cells were found in the anterior chamber. A dilated fundus examination demonstrated severe macular edema, with a foveal thickness of 318 nm from optical coherence tomography (OCT) in the right eye [Figure 1] and [Figure 2]. However, there were unremarkable findings in the left eye. A systemic survey revealed an elevated erythrocyte sedimentation rate with reduced C 3 and C 4 levels. Lupus flare was favored, and pulse corticosteroid therapy was applied. This consisted of intravenous methylprednisolone (250 mg every 6 h for 3 d), tapering down to oral prednisolone (1 mg/kg/day) for the following 11 days. The macular edema improved 3 days later with increased foveal thickness up to 294 nm from OCT and IOP remained 7 mmHg in the right eye. Oral prednisolone (60 mg/day) with immune suppressant azathioprine (100 mg/day, Pharmachemie BV, Haarlem, The Netherlands) was then prescribed after the pulse corticosteroid course. The BCVA in the right eye improved to 6/20 1-week later and 6/6 1-month later with a normal foveal thickness of 209 nm from OCT.
Figure 1: Red-free photography revealed diffused retinal edema, especially in the macula region, 7 days after trabeculectomy in this young woman with systemic lupus erythematosus

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Figure 2: Seven days after trabeculectomy, optical coherence tomography revealed severe macular edema with a central foveal thickness of 318 nm in the right eye, whereas the left eye had a normal central foveal thickness 185 nm (upper and middle panel) 1-month after treatment, foveal thickness returned to 209 nm (lower panel)

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  Discussion Top

Steroid glaucoma is one type of secondary glaucoma with significant history of prolonged application of either topical or systemic steroid though previous studies reveal that topical steroids induce sooner and more severe IOP response. [2] The supposed mechanisms responsible to IOP elevation are believed to extracellular matrix deposition in the trabecular meshwork which resulted in reduced outflow. [3] In addition, genetic factors may play a role in steroid glaucoma as they are up-regulated in dexamethosone-treated trabecular meshwork cells. [3] The clinical features of steroid glaucoma are similar to primary open angle glaucoma with characteristics including nerve fiber damage and visual field loss. Though some ocular damages seem to be reversible after cessation of steroid use. However, steroid glaucoma still poses irreversible deficits especially in those with steroid for >4-year. [4] More than 25% of the patients will undergo surgical management such as trabeculectomy for controlling steroid glaucoma. [5] In SLE patients, a higher correlation to cataract (29%) and glaucoma (3%) is proposed recently. [6] Our patient, a case of steroid glaucoma, presented with poor control of IOP (40 mmHg) under dual medication and underwent uneventful trabeculectomy with Mitomycin-C. Severe macular edema with relative hypotony 1-week after operation presented as a therapeutic challenge.

Hypotony maculopathy is defined as clinical hypotony plus fundic abnormalities, including papilledema, vascular tortuosity, and chorioretinal folds. [7] When treatment with antifibrotic agents like mitomycin C is added to glaucoma filtering surgery, hypotony maculopathy is occasionally reported as an increasing complication. [8] Fannin et al. reported their results about risk factors of hypotony maculopathy based on 228 glaucomatous eyes. [9] Young age, male gender and myopia are significant risk factors while antifibrotic agents' usage and which type of antifibrotic agents (5-fluorouracil and mitomycin C) do not provide significant impact. [9] Our patient has had a 5-year history of SLE and remained remission status under 10 mg/day of prednisolone before surgery. No ocular involvement related to SLE was noted before surgery except glaucomatous change secondary to chronic steroids usage. Severe macular edema accompanying with anterior chamber reaction developed and blurred her vision. Lupus flare in the manifestations of acute hypotony maculopathy is confirmed by ophthalmologic examinations plus decreasing C 3 and C 4 level. Severe methods are suggested for hypotony maculopathy including bleb revision, topical steroids, atropine, and flap-suture. We adopted management of pulse corticosteroid therapy in the suspicion of lupus flare, and clinical therapeutic response provided a correspondence.

  Conclusion Top

We provided an unusual experience dealing with ocular lupus flare in the manifestation of acute hypotony maculopathy. Based upon this patient's clinical picture, we treated this patient as SLE flare in evidence of serum finding and clinical satisfying result. This case reminds us that lupus flare should be listed into the differential diagnosis of consecutive hypotony maculopathy, and it needs prompt urgent management which is different from usual hypotony maculopathy to prevent from permanent organ damage.

  Disclosure Top

The authors declare this study has no conflict of interest.

  References Top

Read RW. Clinical mini-review: Systemic lupus erythematosus and the eye. Ocul Immunol Inflamm 2004;12:87-99.  Back to cited text no. 1
Armaly MF. Effect of corticosteroids on intraocular pressure and fluid dynamics. I. The effect of dexamethasone in the normal eye. Arch Ophthalmol 1963;70:482-91.  Back to cited text no. 2
Kersey JP, Broadway DC. Corticosteroid-induced glaucoma: A review of the literature. Eye (Lond) 2006;20:407-16.  Back to cited text no. 3
Espildora J, Vicuna P, Diaz E. Cortisone-induced glaucoma: A report on 44 affected eyes (author's transl). J Fr Ophtalmol 1981;4:503-8.  Back to cited text no. 4
Sihota R, Konkal VL, Dada T, Agarwal HC, Singh R. Prospective, long-term evaluation of steroid-induced glaucoma. Eye (Lond) 2008;22:26-30.  Back to cited text no. 5
Carli L, Tani C, Querci F, Della Rossa A, Vagnani S, Baldini C, et al. Analysis of the prevalence of cataracts and glaucoma in systemic lupus erythematosus and evaluation of the rheumatologists' practice for the monitoring of glucocorticoid eye toxicity. Clin Rheumatol 2013;32:1071-3.  Back to cited text no. 6
Dellaporta A. Fundus changes in postoperative hypotony. Am J Ophthalmol 1955;40:781-5.  Back to cited text no. 7
Bashford KP, Shafranov G, Shields MB. Bleb revision for hypotony maculopathy after trabeculectomy. J Glaucoma 2004;13:256-60.  Back to cited text no. 8
Fannin LA, Schiffman JC, Budenz DL. Risk factors for hypotony maculopathy. Ophthalmology 2003;110:1185-91.  Back to cited text no. 9


  [Figure 1], [Figure 2]

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