POSTERIOR SCLERITIS IN PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS Robert W. Wong, MD,* Annie Chan, MD,† Robert N. Johnson, MD,* H. Richard McDonald, MD,* Abha Kumar, MD,† Ray Gariano, MD, PhD,† Emmett Cunningham, Jr, MD, PhD, MPH†
Purpose: To describe three cases of posterior scleritis associated with systemic lupus erythematosus. Methods: Retrospective chart review including ophthalmic examination, fundus photography, optical coherence tomography, angiography, ultrasonography, and/or magnetic resonance imaging. Results: Three patients presented with blurred vision, red eye, and/or eye pain in one or both eyes. The diagnosis of posterior scleritis was confirmed in each patient using B-scan ultrasonography. One patient presented with giant nodular scleritis. In two patients, posterior scleritis was the presenting sign of systemic lupus erythematosus. Two patients received systemic corticosteroids together with noncorticosteroid immunosuppressive medications. One patient received topical and periocular injections of corticosteroids. All three patients showed prompt resolution of their posterior scleritis in response to treatment. Conclusion: While uncommon, posterior scleritis may occur in patients with systemic lupus erythematosus and, in some instances, may be the presenting sign of the disease. Prompt diagnosis and treatment was associated with good visual outcomes in our patients. RETINAL CASES & BRIEF REPORTS 4:326–331, 2010
From the *Pacific Vision Foundation, California Pacific Medical Center, San Francisco, California; and †Department of Ophthalmology, Stanford University School of Medicine, Stanford, California.
Ocular complications are found in up to one third of patients with SLE.4 Anterior segment findings in SLE include keratoconjunctivitis sicca,5 conjunctivitis,6 episcleritis,7,8 anterior scleritis,8,9 and keratitis.10 Posterior segment findings of SLE include intraretinal hemorrhages,11 retinal vascular occlusions,11–13 choroidopathies,14 central serous chorioretinopathy,15,16 optic neuritis,17 and papilledema secondary to increased intracranial hypertension.18 While isolated cases of SLE have been listed in two published series of patients with posterior scleritis,19,20 to the best of our knowledge, no detailed reports of patients with both posterior scleritis and SLE have appeared in the literature. We report three cases of posterior scleritis in patients with SLE, including 2 for whom posterior scleritis was the presenting sign of their underlying condition (Table 1). One patient presented with giant nodular scleritis, which has not been previously reported. The collected cases suggest that the visual prognosis of posterior scleritis associated with SLE is favorable.
S
ystemic lupus erythematosus (SLE) is a chronic systemic autoimmune disease characterized by the production and deposition of pathologic autoantibody immune complexes in tissues throughout the body.1 The prevalence of SLE ranges from approximately 40 cases per 100,000 persons among northern Europeans to more than 200 per 100,000 persons among blacks2 and is more common among women.3
Supported in part by the San Francisco Retina Foundation and the Pacific Vision Foundation. The authors report no conflicts of interest. Reprint requests: Emmett T. Cunningham, Jr, MD, PhD, MPH, West Coast Retina Medical Group, 185 Berry Street, Lobby 2, Suite 130, San Francisco, CA 94107-1739; e-mail: emmett_ [email protected].
326
327
POSTERIOR SCLERITIS AND LUPUS Table 1. Clinical Features of Patients with Posterior Scleritis and SLE
Age Sex Ethnicity Eye 72
F
AA
56
F
AA
50
F
L
Symptoms
OD Headache, blurred vision, red eye OU Blurred vision, eye pain
OS Eye pain
Posterior Segment Findings Giant nodular scleritis, exudative retinal detachment Vitritis, optic disk edema, exudative retinal detachment, thickened posterior sclera, fluid in sub-Tenon space Thickened posterior sclera, fluid in sub-Tenon space
Presenting VA OD: 20/32 OS: 20/25
Final VA OD: 20/ 32 OS: 20/ 25
OD: 20/200 OD: 20/ 20 OS: 20/200 OS: 20/ 20
OD: 20/40 OS: 20/40
OD: 20/ 20 OS: 20/ 20-
Treatment
Complete Recovery
Topical corticosteroids, periocular corticosteroid injection 32 Systemic corticosteroids, methotrexate
Yes
Systemic corticosteroids, cyclophosphamide
Yes
Yes
F, female; AA, African American; L, Latina; OD, right eye; OS, left eye; OU, both eyes; VA, visual acuity.
Case Reports Case One A 72-year-old African American woman presented with decreased vision and redness in the right eye associated with a right-sided headache for 1 week. Medical history was notable for renal failure and for systemic hypertension. Review of systems was notable for mild alopecia and for recurrent angioedema. Best-corrected Snellen visual acuity measured 20/32 in the right eye and 20/25 in the left eye. External and anterior segment examination of the right eye revealed conjunctival injection, chemosis, and moderate anterior chamber inflammation. Posterior segment examination revealed a large, bilobular, choroidal mass extending from the temporal macula to the ora serrata associated with a shallow exudative retinal detachment (Figure 1A). Fluorescein angiography failed to demonstrate any retinal vascular or choroidal leakage. Optical coherence tomography scan showed the mass extending to just beneath the center of the fovea without evidence of subfoveal fluid or intraretinal edema. B-scan ultrasonography revealed a mass with medium to high internal reflectivity with nearby subretinal fluid (Figure 1B). No echographic evidence of a classic ‘‘T’’ sign was observed. Examination of the left eye was normal. The patient was given the clinical diagnosis of giant nodular scleritis in the right eye, and subsequent physical and laboratory examination by a rheumatologist confirmed the diagnosis of SLE. Treatment with multiple periocular injections (triamcinolone acetonide, 40 mg/mL) and topical prednisolone acetate (1%) resulted in subsequent relief of pain, redness, and complete resolution of the nodular scleritis. Topical corticosteroids were quickly tapered, and the patient has remained stable without immunosuppressive medications.
Case Two A 56-year-old African American woman presented with decreased vision and aching eye pain bilaterally for 5 days. Review of systems revealed intermittent arthralgias. Medical and surgical histories were notable for moderate obesity but were otherwise unremarkable. Best-corrected visual acuity was 20/200 in each eye. External and anterior segment examinations were normal. Posterior segment examination revealed trace vitreous cell, optic disk edema, and retinal folds in the macula of each eye (Figure 2A). Fluorescein angiography showed leakage from the optic nerve (Figure 2B). B-scan ultrasonography revealed thickening of the posterior sclera with fluid in the sub-Tenon space (T-sign) bilaterally (Figure 2C). Physical and laboratory examination by a rheumatologist confirmed the diagnosis of SLE. Treatment with high-dose oral prednisone (0.1 mg/kg/day) resulted in prompt relief of pain, resolution of the disk edema and retinal folds, and recovery of vision to 20/20 bilaterally. The corticosteroids were tapered, and the patient remained stable on 12.5-mg methotrexate per week.
Case Three A 50-year-old Latin American woman presented with acute severe pain of her left eye on awaking. The pain was worse with eye movement. Review of systems revealed recent nausea, vomiting, and headaches. Medical history was notable for a 5-year history of SLE complicated by previous episodes of episcleritis and lacrimal gland inflammation. She was receiving maintenance therapy consisting of 10 mg daily prednisone and 1-g cyclophosphamide every 3 months by her rheumatologist. Best-corrected visual acuity
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Discussion
Fig. 1. Patient depicted in Case 1. A. Fundus photograph of the right eye showing giant nodular scleritis and associated exudative retinal detachment (outlined by white arrowheads) in the temporal periphery, extending posterior to the equator and into the temporal macula. B. Transverse cut of a B-scan ultrasound showing medium to high reflectivity of the choroidal thickening with subretinal fluid superior to the lesion.
was 20/40 in each eye. Intraocular pressures were normal in each eye. She had moderate ptosis, chemosis, and moderate injection of her left eye. External and anterior segment examinations of the right eye were normal. Dilated fundus examination was normal. Ocular ultrasonography revealed thickening of the posterior sclera with fluid in the sub-Tenon space (Figure 3A). Computed tomography demonstrated retroorbital fat stranding. Because of concern for orbital cellulitis, the patient was admitted to the hospital and received intravenous antibiotic therapy, as well as intravenous 10-mg methylprednisolone every 8 hours as recommended by her rheumatologist. Magnetic resonance imaging with gadolinium later revealed abnormal enhancement of the superior and lateral rectus muscles, the optic nerve sheath, and lacrimal gland (Figure 3B). The patient clinically improved over the next 24 hours and was discharged 5 days later with a tapering dose of oral prednisone and antibiotics.
Posterior scleritis is an uncommon complication of SLE. To the best of our knowledge, only two cases of posterior scleritis associated with SLE have been cited in the literature. Sainz de la Maza et al20 reviewed 82 patients with scleritis, including 7 patients with a medical history of SLE and only 1 patient had posterior scleritis associated with SLE. McCluskey et al19 published a retrospective review of 99 patients with posterior scleritis and found that 29 (29%) of their patients had an associated systemic disease, with the most common systemic associations being rheumatoid arthritis (5%), systemic vasculitis (4%), and Wegener granulomatosus (4%). This series included a single case of posterior scleritis associated with SLE. In neither report were detailed descriptions of the patients with posterior scleritis and SLE provided, however, and no mention was made of whether posterior scleritis was the presenting sign of SLE in these particular cases. We have described three patients with posterior scleritis associated with SLE, including two for whom posterior scleritis was the presenting sign for their underlying condition and one who had giant nodular posterior scleritis, a finding previously unreported in patients with SLE. Giant nodular posterior scleritis appears to be uncommon, regardless of cause. When it does occur, however, it often mimics choroidal melanoma. Of 400 cases referred to the Ocular Oncology Service of Wills Eye Hospital with the diagnosis of choroidal melanoma, for example, 6 cases (1.5%) proved clinically to be posterior scleritis.21 Although several other case reports have been published describing nodular posterior scleritis masquerading as choroidal melanoma,22–30 none were associated with SLE. Typical features of giant nodular scleritis include a domeshaped acoustically solid subretinal mass with normal choroidal circulation and a lack of a ‘‘double circulation’’ on fluorescein angiography. There may be associated subretinal fluid, choroidal folds, or retrobulbar echolucent fluid seen on echography.30 Patients with posterior scleritis most commonly present with periocular pain, headache, visual loss, or anterior scleritis.19 Our patient presented with pain, redness, anterior chamber inflammation, and a large acoustically solid subretinal mass with an associated exudative retinal detachment, choroidal folds, and lack of double circulation on angiography. It is important to distinguish posterior scleritis from choroidal melanoma because clinical misinterpretation has led to therapeutic misdirection, including enucleation.24,31,32
POSTERIOR SCLERITIS AND LUPUS
329
Fig. 2. Patient depicted in Case 2. A. Fundus photograph showing optic disk edema and retinal folds in the macula of the left eye. B. Fluorescein angiogram showing early disk leakage. C. B-scan ultrasound showing thickening of the posterior sclera with juxtascleral fluid (white arrowheads).
Ancillary testing is helpful in diagnosing posterior scleritis. Ultrasonography is key in the diagnosis of posterior scleritis, and it may show increased thickness of the posterior eye wall, fluid in the Tenon space, swelling of the optic disk, distended optic nerve sheath, retinal detachment, and scleral nodules.19 Measurements of eyewall thickness greater than 2.0 mm are considered abnormal.19 In select patients, magnetic resonance imaging33 and computed tomography34 with contrast enhancement may be helpful as adjunct tests. Treatment of posterior scleritis should be targeted toward the underlying etiology if known. Although most patients with idiopathic posterior scleritis respond well with nonsteroid antiinflammatory medications, patients with posterior scleritis and
systemic disease may need more aggressive treatment, including both systemic corticosteroids and noncorticosteroid immunosuppressive medication.19 In our series, each patient benefited initially from local or systemic corticosteroids, and two patients required additional noncorticosteroid immunosuppressive medications to achieve long-term control. In summary, while SLE is a relatively uncommon cause of posterior scleritis, it should be included among the list of possible causes because prompt diagnosis and treatment may result in good visual recovery. Key words: autoimmune disease, exudative retinal detachment, giant nodular scleritis, inflammation, vitritis.
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Fig. 3. Patient depicted in Case 3. A. B-scan ultrasound showing thickening of the posterior sclera with juxtascleral fluid (white arrowheads). B. T1-weighted magnetic resonance imaging with fat suppression and gadolinium enhancement. Axial section through the left orbit with enhancement seen along the posterior sclera, lateral rectus muscle, and optic nerve sheath.
References 1. Rahman A, Isenberg DA. Systemic lupus erythematosus. N Engl J Med 2008;358:929–939. 2. Johnson AE, Gordon C, Palmer RG, Bacon PA. The prevalence and incidence of systemic lupus erythematosus in Birmingham, England. Relationship to ethnicity and country of birth. Arthritis Rheum 1995;38:551–558. 3. D’Cruz DP, Khamashta MA, Hughes GR. Systemic lupus erythematosus. Lancet 2007;369:587–596. 4. Read RW. Clinical mini-review: systemic lupus erythematosus and the eye. Ocul Immunol Inflamm 2004;12:87–99. 5. Frith P, Burge SM, Millard PR, Wojnarowska F. External ocular findings in lupus erythematosus: a clinical and immunopathological study. Br J Ophthalmol 1990;74:163–167.
6. Heiligenhaus A, Dutt JE, Foster CS. Histology and immunopathology of systemic lupus erythematosus affecting the conjunctiva. Eye 1996;10:425–432. 7. Read RW, Weiss AH, Sherry DD. Episcleritis in childhood. Ophthalmology 1999;106:2377–2379. 8. Jabs DA, Mudun A, Dunn JP, Marsh MJ. Episcleritis and scleritis: clinical features and treatment results. Am J Ophthalmol 2000;130:469–476. 9. Watson PG, Hayreh SS. Scleritis and episcleritis. Br J Ophthalmol 1976;60:163–191. 10. Adan CB, Trevisani VF, Vasconcellos M, de Freitas D, de Souza LB, Mannis M. Bilateral deep keratitis caused by systemic lupus erythematosus. Cornea 2004;23: 207–209. 11. Jabs DA, Fine SL, Hochberg MC, Newman SA, Heiner GG, Stevens MB. Severe retinal vaso-occlusive disease in systemic lupus erythematous. Arch Ophthalmol 1986;104: 558–563. 12. Au A, O’Day J. Review of severe vaso-occlusive retinopathy in systemic lupus erythematosus and the antiphospholipid syndrome: associations, visual outcomes, complications and treatment. Clin Exp Ophthalmol 2004; 32:87–100. 13. Shein J, Shukla D, Reddy S, Yannuzzi LA, Cunningham ET Jr. Macular infaction as a presenting sign of systemic lupus erythematosus. Retinal Cases 2:55–60. 14. Nguyen QD, Uy HS, Akpek EK, Harper SL, Zacks DN, Foster CS. Choroidopathy of systemic lupus erythematosus. Lupus 2000;9:288–298. 15. Cunningham ET Jr, Alfred PR, Irvine AR. Central serous chorioretinopathy in patients with systemic lupus erythematosus. Ophthalmology 1996;103:2081–2090. 16. Jabs DA, Hanneken AM, Schachat AP, Fine SL. Choroidopathy in systemic lupus erythematosus. Arch Ophthalmol 1988; 106:230–234. 17. Jabs DA, Miller NR, Newman SA, Johnson MA, Stevens MB. Optic neuropathy in systemic lupus erythematosus. Arch Ophthalmol 1986;104:564–568. 18. Bettman JW Jr, Daroff RB, Sanders MD, Joyt WF. Papilledema and asymptomatic intracranial hypertension in systemic lupus erythematosus. A fluorescein angiographic study of resolving papilledema. Arch Ophthalmol 1968;80: 189–193. 19. McCluskey PJ, Watson PG, Lightman S, Haybittle J, Restori M, Branley M. Posterior scleritis: clinical features, systemic associations, and outcome in a large series of patients. Ophthalmology 1999;106:2380–2386. 20. Sainz de la Maza M, Foster CS, Jabbur NS. Scleritis associated with systemic vasculitic diseases. Ophthalmology 1995;102: 687–692. 21. Shields JA, Augsburger JJ, Brown GC, Stephens RF. The differential diagnosis of posterior uveal melanoma. Ophthalmology 1980;87:518–522. 22. Arevalo JF, Shields CL, Shields JA. Giant nodular posterior scleritis simulating choroidal melanoma and birdshot retinochoroidopathy. Ophthalmic Surg Lasers Imaging 2003;34: 403–405. 23. Brod RD, Saul RF. Nodular posterior scleritis. Arch Ophthalmol 1990;108:1170–1171. 24. Finger PT, Perry HD, Packer S, Erdey RA, Weisman GD, Sibony PA. Posterior scleritis as an intraocular tumour. Br J Ophthalmol 1990;74:121–122. 25. Gedde SJ, Augsburger JJ. Posterior scleritis as a fundus mass. Ophthalmic Surg 1994;25:119–121.
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POSTERIOR SCLERITIS AND LUPUS 26. Gupta A, Bansal RK, Bambery P. Posterior scleritis related fundal mass in a patient with rheumatoid arthritis. Scand J Rheumatol 1992;21:254–256. 27. Pe´rez-Campagne E, Guex-Crosier Y, Schalenbourg A, Uffer S, Zografos L. [Giant nodular posterior scleritis compatible with ocular sarcoidosis simulating choroidal melanoma]. Arch Soc Esp Oftalmol 2007;82:563–566. 28. Shukla D, Kim R. Giant nodular posterior scleritis simulating choroidal melanoma. Indian J Ophthalmol 2006;54:120–122. 29. Wang JK, Lai PC, Yang CH. Subretinal mass as a presenting sign of posterior scleritis: a case report. Kaohsiung J Med Sci 2003;19:522–525. 30. Demirci H, Shields CL, Honavar SG, Shields JA, Bardenstein DS. Long-term follow-up of giant nodular posterior scleritis
31.
32.
33.
34.
simulating choroidal melanoma. Arch Ophthalmol 2000;118: 1290–1292. Accuracy of diagnosis of choroidal melanomas in the Collaborative Ocular Melanoma Study. COMS report no. 1. Arch Ophthalmol 1990;108:1268–1273. Feldon SE, Sigelman J, Albert DM, Smith TR. Clinical manifestations of brawny scleritis. Am J Ophthalmol 1978;85: 781–787. Osman Saatci A, Saatci I, Kocak N, Durak I. Magnetic resonance imaging characteristics of posterior scleritis mimicking choroidal mass. Eur J Radiol 2001;39:88–91. Chaques VJ, Lam S, Tessler HH, Mafee MF. Computed tomography and magnetic resonance imaging in the diagnosis of posterior scleritis. Ann Ophthalmol 1993;25: 89–94.
Purpose: To describe three cases of posterior scleritis associated with systemic lupus erythematosus. Methods: Retrospective chart review including ophthalmic examination, fundus photography, optical coherence tomography, angiography, ultrasonography, and/or magnetic resonance imaging. Results: Three patients presented with blurred vision, red eye, and/or eye pain in one or both eyes. The diagnosis of posterior scleritis was confirmed in each patient using B-scan ultrasonography. One patient presented with giant nodular scleritis. In two patients, posterior scleritis was the presenting sign of systemic lupus erythematosus. Two patients received systemic corticosteroids together with noncorticosteroid immunosuppressive medications. One patient received topical and periocular injections of corticosteroids. All three patients showed prompt resolution of their posterior scleritis in response to treatment. Conclusion: While uncommon, posterior scleritis may occur in patients with systemic lupus erythematosus and, in some instances, may be the presenting sign of the disease. Prompt diagnosis and treatment was associated with good visual outcomes in our patients. RETINAL CASES & BRIEF REPORTS 4:326–331, 2010
From the *Pacific Vision Foundation, California Pacific Medical Center, San Francisco, California; and †Department of Ophthalmology, Stanford University School of Medicine, Stanford, California.
Ocular complications are found in up to one third of patients with SLE.4 Anterior segment findings in SLE include keratoconjunctivitis sicca,5 conjunctivitis,6 episcleritis,7,8 anterior scleritis,8,9 and keratitis.10 Posterior segment findings of SLE include intraretinal hemorrhages,11 retinal vascular occlusions,11–13 choroidopathies,14 central serous chorioretinopathy,15,16 optic neuritis,17 and papilledema secondary to increased intracranial hypertension.18 While isolated cases of SLE have been listed in two published series of patients with posterior scleritis,19,20 to the best of our knowledge, no detailed reports of patients with both posterior scleritis and SLE have appeared in the literature. We report three cases of posterior scleritis in patients with SLE, including 2 for whom posterior scleritis was the presenting sign of their underlying condition (Table 1). One patient presented with giant nodular scleritis, which has not been previously reported. The collected cases suggest that the visual prognosis of posterior scleritis associated with SLE is favorable.
S
ystemic lupus erythematosus (SLE) is a chronic systemic autoimmune disease characterized by the production and deposition of pathologic autoantibody immune complexes in tissues throughout the body.1 The prevalence of SLE ranges from approximately 40 cases per 100,000 persons among northern Europeans to more than 200 per 100,000 persons among blacks2 and is more common among women.3
Supported in part by the San Francisco Retina Foundation and the Pacific Vision Foundation. The authors report no conflicts of interest. Reprint requests: Emmett T. Cunningham, Jr, MD, PhD, MPH, West Coast Retina Medical Group, 185 Berry Street, Lobby 2, Suite 130, San Francisco, CA 94107-1739; e-mail: emmett_ [email protected].
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POSTERIOR SCLERITIS AND LUPUS Table 1. Clinical Features of Patients with Posterior Scleritis and SLE
Age Sex Ethnicity Eye 72
F
AA
56
F
AA
50
F
L
Symptoms
OD Headache, blurred vision, red eye OU Blurred vision, eye pain
OS Eye pain
Posterior Segment Findings Giant nodular scleritis, exudative retinal detachment Vitritis, optic disk edema, exudative retinal detachment, thickened posterior sclera, fluid in sub-Tenon space Thickened posterior sclera, fluid in sub-Tenon space
Presenting VA OD: 20/32 OS: 20/25
Final VA OD: 20/ 32 OS: 20/ 25
OD: 20/200 OD: 20/ 20 OS: 20/200 OS: 20/ 20
OD: 20/40 OS: 20/40
OD: 20/ 20 OS: 20/ 20-
Treatment
Complete Recovery
Topical corticosteroids, periocular corticosteroid injection 32 Systemic corticosteroids, methotrexate
Yes
Systemic corticosteroids, cyclophosphamide
Yes
Yes
F, female; AA, African American; L, Latina; OD, right eye; OS, left eye; OU, both eyes; VA, visual acuity.
Case Reports Case One A 72-year-old African American woman presented with decreased vision and redness in the right eye associated with a right-sided headache for 1 week. Medical history was notable for renal failure and for systemic hypertension. Review of systems was notable for mild alopecia and for recurrent angioedema. Best-corrected Snellen visual acuity measured 20/32 in the right eye and 20/25 in the left eye. External and anterior segment examination of the right eye revealed conjunctival injection, chemosis, and moderate anterior chamber inflammation. Posterior segment examination revealed a large, bilobular, choroidal mass extending from the temporal macula to the ora serrata associated with a shallow exudative retinal detachment (Figure 1A). Fluorescein angiography failed to demonstrate any retinal vascular or choroidal leakage. Optical coherence tomography scan showed the mass extending to just beneath the center of the fovea without evidence of subfoveal fluid or intraretinal edema. B-scan ultrasonography revealed a mass with medium to high internal reflectivity with nearby subretinal fluid (Figure 1B). No echographic evidence of a classic ‘‘T’’ sign was observed. Examination of the left eye was normal. The patient was given the clinical diagnosis of giant nodular scleritis in the right eye, and subsequent physical and laboratory examination by a rheumatologist confirmed the diagnosis of SLE. Treatment with multiple periocular injections (triamcinolone acetonide, 40 mg/mL) and topical prednisolone acetate (1%) resulted in subsequent relief of pain, redness, and complete resolution of the nodular scleritis. Topical corticosteroids were quickly tapered, and the patient has remained stable without immunosuppressive medications.
Case Two A 56-year-old African American woman presented with decreased vision and aching eye pain bilaterally for 5 days. Review of systems revealed intermittent arthralgias. Medical and surgical histories were notable for moderate obesity but were otherwise unremarkable. Best-corrected visual acuity was 20/200 in each eye. External and anterior segment examinations were normal. Posterior segment examination revealed trace vitreous cell, optic disk edema, and retinal folds in the macula of each eye (Figure 2A). Fluorescein angiography showed leakage from the optic nerve (Figure 2B). B-scan ultrasonography revealed thickening of the posterior sclera with fluid in the sub-Tenon space (T-sign) bilaterally (Figure 2C). Physical and laboratory examination by a rheumatologist confirmed the diagnosis of SLE. Treatment with high-dose oral prednisone (0.1 mg/kg/day) resulted in prompt relief of pain, resolution of the disk edema and retinal folds, and recovery of vision to 20/20 bilaterally. The corticosteroids were tapered, and the patient remained stable on 12.5-mg methotrexate per week.
Case Three A 50-year-old Latin American woman presented with acute severe pain of her left eye on awaking. The pain was worse with eye movement. Review of systems revealed recent nausea, vomiting, and headaches. Medical history was notable for a 5-year history of SLE complicated by previous episodes of episcleritis and lacrimal gland inflammation. She was receiving maintenance therapy consisting of 10 mg daily prednisone and 1-g cyclophosphamide every 3 months by her rheumatologist. Best-corrected visual acuity
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Discussion
Fig. 1. Patient depicted in Case 1. A. Fundus photograph of the right eye showing giant nodular scleritis and associated exudative retinal detachment (outlined by white arrowheads) in the temporal periphery, extending posterior to the equator and into the temporal macula. B. Transverse cut of a B-scan ultrasound showing medium to high reflectivity of the choroidal thickening with subretinal fluid superior to the lesion.
was 20/40 in each eye. Intraocular pressures were normal in each eye. She had moderate ptosis, chemosis, and moderate injection of her left eye. External and anterior segment examinations of the right eye were normal. Dilated fundus examination was normal. Ocular ultrasonography revealed thickening of the posterior sclera with fluid in the sub-Tenon space (Figure 3A). Computed tomography demonstrated retroorbital fat stranding. Because of concern for orbital cellulitis, the patient was admitted to the hospital and received intravenous antibiotic therapy, as well as intravenous 10-mg methylprednisolone every 8 hours as recommended by her rheumatologist. Magnetic resonance imaging with gadolinium later revealed abnormal enhancement of the superior and lateral rectus muscles, the optic nerve sheath, and lacrimal gland (Figure 3B). The patient clinically improved over the next 24 hours and was discharged 5 days later with a tapering dose of oral prednisone and antibiotics.
Posterior scleritis is an uncommon complication of SLE. To the best of our knowledge, only two cases of posterior scleritis associated with SLE have been cited in the literature. Sainz de la Maza et al20 reviewed 82 patients with scleritis, including 7 patients with a medical history of SLE and only 1 patient had posterior scleritis associated with SLE. McCluskey et al19 published a retrospective review of 99 patients with posterior scleritis and found that 29 (29%) of their patients had an associated systemic disease, with the most common systemic associations being rheumatoid arthritis (5%), systemic vasculitis (4%), and Wegener granulomatosus (4%). This series included a single case of posterior scleritis associated with SLE. In neither report were detailed descriptions of the patients with posterior scleritis and SLE provided, however, and no mention was made of whether posterior scleritis was the presenting sign of SLE in these particular cases. We have described three patients with posterior scleritis associated with SLE, including two for whom posterior scleritis was the presenting sign for their underlying condition and one who had giant nodular posterior scleritis, a finding previously unreported in patients with SLE. Giant nodular posterior scleritis appears to be uncommon, regardless of cause. When it does occur, however, it often mimics choroidal melanoma. Of 400 cases referred to the Ocular Oncology Service of Wills Eye Hospital with the diagnosis of choroidal melanoma, for example, 6 cases (1.5%) proved clinically to be posterior scleritis.21 Although several other case reports have been published describing nodular posterior scleritis masquerading as choroidal melanoma,22–30 none were associated with SLE. Typical features of giant nodular scleritis include a domeshaped acoustically solid subretinal mass with normal choroidal circulation and a lack of a ‘‘double circulation’’ on fluorescein angiography. There may be associated subretinal fluid, choroidal folds, or retrobulbar echolucent fluid seen on echography.30 Patients with posterior scleritis most commonly present with periocular pain, headache, visual loss, or anterior scleritis.19 Our patient presented with pain, redness, anterior chamber inflammation, and a large acoustically solid subretinal mass with an associated exudative retinal detachment, choroidal folds, and lack of double circulation on angiography. It is important to distinguish posterior scleritis from choroidal melanoma because clinical misinterpretation has led to therapeutic misdirection, including enucleation.24,31,32
POSTERIOR SCLERITIS AND LUPUS
329
Fig. 2. Patient depicted in Case 2. A. Fundus photograph showing optic disk edema and retinal folds in the macula of the left eye. B. Fluorescein angiogram showing early disk leakage. C. B-scan ultrasound showing thickening of the posterior sclera with juxtascleral fluid (white arrowheads).
Ancillary testing is helpful in diagnosing posterior scleritis. Ultrasonography is key in the diagnosis of posterior scleritis, and it may show increased thickness of the posterior eye wall, fluid in the Tenon space, swelling of the optic disk, distended optic nerve sheath, retinal detachment, and scleral nodules.19 Measurements of eyewall thickness greater than 2.0 mm are considered abnormal.19 In select patients, magnetic resonance imaging33 and computed tomography34 with contrast enhancement may be helpful as adjunct tests. Treatment of posterior scleritis should be targeted toward the underlying etiology if known. Although most patients with idiopathic posterior scleritis respond well with nonsteroid antiinflammatory medications, patients with posterior scleritis and
systemic disease may need more aggressive treatment, including both systemic corticosteroids and noncorticosteroid immunosuppressive medication.19 In our series, each patient benefited initially from local or systemic corticosteroids, and two patients required additional noncorticosteroid immunosuppressive medications to achieve long-term control. In summary, while SLE is a relatively uncommon cause of posterior scleritis, it should be included among the list of possible causes because prompt diagnosis and treatment may result in good visual recovery. Key words: autoimmune disease, exudative retinal detachment, giant nodular scleritis, inflammation, vitritis.
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Fig. 3. Patient depicted in Case 3. A. B-scan ultrasound showing thickening of the posterior sclera with juxtascleral fluid (white arrowheads). B. T1-weighted magnetic resonance imaging with fat suppression and gadolinium enhancement. Axial section through the left orbit with enhancement seen along the posterior sclera, lateral rectus muscle, and optic nerve sheath.
References 1. Rahman A, Isenberg DA. Systemic lupus erythematosus. N Engl J Med 2008;358:929–939. 2. Johnson AE, Gordon C, Palmer RG, Bacon PA. The prevalence and incidence of systemic lupus erythematosus in Birmingham, England. Relationship to ethnicity and country of birth. Arthritis Rheum 1995;38:551–558. 3. D’Cruz DP, Khamashta MA, Hughes GR. Systemic lupus erythematosus. Lancet 2007;369:587–596. 4. Read RW. Clinical mini-review: systemic lupus erythematosus and the eye. Ocul Immunol Inflamm 2004;12:87–99. 5. Frith P, Burge SM, Millard PR, Wojnarowska F. External ocular findings in lupus erythematosus: a clinical and immunopathological study. Br J Ophthalmol 1990;74:163–167.
6. Heiligenhaus A, Dutt JE, Foster CS. Histology and immunopathology of systemic lupus erythematosus affecting the conjunctiva. Eye 1996;10:425–432. 7. Read RW, Weiss AH, Sherry DD. Episcleritis in childhood. Ophthalmology 1999;106:2377–2379. 8. Jabs DA, Mudun A, Dunn JP, Marsh MJ. Episcleritis and scleritis: clinical features and treatment results. Am J Ophthalmol 2000;130:469–476. 9. Watson PG, Hayreh SS. Scleritis and episcleritis. Br J Ophthalmol 1976;60:163–191. 10. Adan CB, Trevisani VF, Vasconcellos M, de Freitas D, de Souza LB, Mannis M. Bilateral deep keratitis caused by systemic lupus erythematosus. Cornea 2004;23: 207–209. 11. Jabs DA, Fine SL, Hochberg MC, Newman SA, Heiner GG, Stevens MB. Severe retinal vaso-occlusive disease in systemic lupus erythematous. Arch Ophthalmol 1986;104: 558–563. 12. Au A, O’Day J. Review of severe vaso-occlusive retinopathy in systemic lupus erythematosus and the antiphospholipid syndrome: associations, visual outcomes, complications and treatment. Clin Exp Ophthalmol 2004; 32:87–100. 13. Shein J, Shukla D, Reddy S, Yannuzzi LA, Cunningham ET Jr. Macular infaction as a presenting sign of systemic lupus erythematosus. Retinal Cases 2:55–60. 14. Nguyen QD, Uy HS, Akpek EK, Harper SL, Zacks DN, Foster CS. Choroidopathy of systemic lupus erythematosus. Lupus 2000;9:288–298. 15. Cunningham ET Jr, Alfred PR, Irvine AR. Central serous chorioretinopathy in patients with systemic lupus erythematosus. Ophthalmology 1996;103:2081–2090. 16. Jabs DA, Hanneken AM, Schachat AP, Fine SL. Choroidopathy in systemic lupus erythematosus. Arch Ophthalmol 1988; 106:230–234. 17. Jabs DA, Miller NR, Newman SA, Johnson MA, Stevens MB. Optic neuropathy in systemic lupus erythematosus. Arch Ophthalmol 1986;104:564–568. 18. Bettman JW Jr, Daroff RB, Sanders MD, Joyt WF. Papilledema and asymptomatic intracranial hypertension in systemic lupus erythematosus. A fluorescein angiographic study of resolving papilledema. Arch Ophthalmol 1968;80: 189–193. 19. McCluskey PJ, Watson PG, Lightman S, Haybittle J, Restori M, Branley M. Posterior scleritis: clinical features, systemic associations, and outcome in a large series of patients. Ophthalmology 1999;106:2380–2386. 20. Sainz de la Maza M, Foster CS, Jabbur NS. Scleritis associated with systemic vasculitic diseases. Ophthalmology 1995;102: 687–692. 21. Shields JA, Augsburger JJ, Brown GC, Stephens RF. The differential diagnosis of posterior uveal melanoma. Ophthalmology 1980;87:518–522. 22. Arevalo JF, Shields CL, Shields JA. Giant nodular posterior scleritis simulating choroidal melanoma and birdshot retinochoroidopathy. Ophthalmic Surg Lasers Imaging 2003;34: 403–405. 23. Brod RD, Saul RF. Nodular posterior scleritis. Arch Ophthalmol 1990;108:1170–1171. 24. Finger PT, Perry HD, Packer S, Erdey RA, Weisman GD, Sibony PA. Posterior scleritis as an intraocular tumour. Br J Ophthalmol 1990;74:121–122. 25. Gedde SJ, Augsburger JJ. Posterior scleritis as a fundus mass. Ophthalmic Surg 1994;25:119–121.
331
POSTERIOR SCLERITIS AND LUPUS 26. Gupta A, Bansal RK, Bambery P. Posterior scleritis related fundal mass in a patient with rheumatoid arthritis. Scand J Rheumatol 1992;21:254–256. 27. Pe´rez-Campagne E, Guex-Crosier Y, Schalenbourg A, Uffer S, Zografos L. [Giant nodular posterior scleritis compatible with ocular sarcoidosis simulating choroidal melanoma]. Arch Soc Esp Oftalmol 2007;82:563–566. 28. Shukla D, Kim R. Giant nodular posterior scleritis simulating choroidal melanoma. Indian J Ophthalmol 2006;54:120–122. 29. Wang JK, Lai PC, Yang CH. Subretinal mass as a presenting sign of posterior scleritis: a case report. Kaohsiung J Med Sci 2003;19:522–525. 30. Demirci H, Shields CL, Honavar SG, Shields JA, Bardenstein DS. Long-term follow-up of giant nodular posterior scleritis
31.
32.
33.
34.
simulating choroidal melanoma. Arch Ophthalmol 2000;118: 1290–1292. Accuracy of diagnosis of choroidal melanomas in the Collaborative Ocular Melanoma Study. COMS report no. 1. Arch Ophthalmol 1990;108:1268–1273. Feldon SE, Sigelman J, Albert DM, Smith TR. Clinical manifestations of brawny scleritis. Am J Ophthalmol 1978;85: 781–787. Osman Saatci A, Saatci I, Kocak N, Durak I. Magnetic resonance imaging characteristics of posterior scleritis mimicking choroidal mass. Eur J Radiol 2001;39:88–91. Chaques VJ, Lam S, Tessler HH, Mafee MF. Computed tomography and magnetic resonance imaging in the diagnosis of posterior scleritis. Ann Ophthalmol 1993;25: 89–94.
