IS “LUPUS CHOROIDOPATHY” SECONDARY TO CORTICOSTEROIDINDUCED CENTRAL SEROUS CHORIORETINOPATHY? Stephen R. Smith, MD, Lee M. Jampol, MD
Patients with lupus erythematosus may develop serous retinal detachments secondary to lupus choroidopathy. In some patients this choroidopathy is actually a manifestation of central serous chorioretinopathy from systemic corticosteroids used to treat the lupus. Decreasing or stopping the steroids is the treatment of choice.
From Northwestern University, Feinberg School of Medicine, Department of Ophthalmology, Chicago, Illinois.
Case Report A 32-year-old woman had a 4-day history of a dramatic decrease in vision of the right eye. The referring diagnosis was “lupus choroiditis.” The patient had an 11-year history of SLE and 4 months previously developed peripheral edema. Kidney biopsy showed rapidly progressive glomerulonephritis. Treatment was started with intravenous methylprednisolone (2 doses of 500 mg) and cyclophosphamide. Medication at the time of discharge from the hospital was prednisone (60 mg/day). When seen in the eye clinic 3 months later, her medications included prednisone (30 mg/day) and cyclophosphamide (1,700 mg monthly). Visual acuity was 20/400 in the right eye and 20/25 in the left eye. She identified zero of eight Ishihara plates on the right side and eight of eight plates on the left side. Intraocular pressures and results of external examination and anterior chamber examination were normal. Ophthalmoscopy of the right eye revealed a normal optic nerve head and vessels. Two areas of serous neurosensory detachment with extension under the fovea were seen (Fig. 1A). The extrafoveal portions of the detachments were filled with white debris thought to be fibrin. Ophthalmoscopy of the left eye revealed two small retinal pigment epithelium (RPE) detachments inferior and temporal to the fovea. Fluorescein angiography of the right eye (Fig. 1B) revealed two spots of focal leakage corresponding to the two extrafoveal serous elevations with filling of the neurosensory detachments in late-phase views. Underlying each area of neurosensory detachment, a small pigment epithelial detachment could be seen with the leakage occurring at the edge of the RPE detachment in one area and the center of the RPE detachment in the other. The left eye had focal dye accumulation in the two areas corresponding to the pigment epithelial detachments. Our diagnosis was corticosteroid-induced CSC. In consultation with the rheumatologist, we tapered the patient’s dose of prednisone over the next several days from 30 mg to 17.5 mg. Two weeks later, the serous elevation in the fovea had largely resolved, but an inferior peripheral retinal detachment was noted. Vision had not improved. Angiography showed decreased but continued leakage. Because partially tapering the prednisone dose did not result in complete resolution, focal thermal green laser was applied to the leaking spots in the right eye. One week after focal laser application, visual acuity had improved to 20/50; there was complete
S
ystemic lupus erythematosus (SLE) is an autoimmune disease characterized by deposition of immune complexes in internal organs including the vascular system. Many of the manifestations of SLE are a result of tissue damage secondary to this vasculitis.1 Retinal findings include cotton-wool spots and retinal hemorrhages related to vasculitis or retinal, disk, or choroidal vasoocclusion related to thrombosis from anticardiolipin antibodies. Another reported ocular complication is multifocal serous retinal detachments, which has been postulated as a choroidopathy secondary to systemic lupus.2 For the purpose of this discussion, we will term this entity “lupus choroidopathy.” We describe a case of serous retinal detachments in a patient with SLE and nephritis. On the basis of the fundus appearance and clinical course, we believe that the chorioretinal findings of lupus choroidopathy in this patient were secondary to central serous chorioretinopathy (CSC) from systemically administered corticosteroids. The presentation and course of this patient lead us to the conclusion that lupus choroidopathy as described by Jabs et al2 is often a presentation of corticosteroid-induced CSC, rather than choroidal vasculitis or thrombosis. Supported in part by an unrestricted grant from Research to Prevent Blindness, New York, NY. The authors have no proprietary interest to report. Reprint requests: Lee M. Jampol, Northwestern University, Feinberg School of Medicine, Department of Ophthalmology, 645 North Michigan Avenue, Suite 440, Chicago, IL 60611; e-mail: [email protected]
7
RETINAL CASES & BRIEF REPORTSℜ
8
●
2007
●
VOLUME 1
●
NUMBER 1
Fig. 1. A, Ophthalmoscopy demonstrates two areas of serous detachment with fibrin exudates. Serous fluid extends under the fovea. B, Late-phase findings of fluorescein angiography. Two major areas of leakage are seen.
flattening of the serous elevations with markedly decreased fibrin under the retina. The fovea was dry with some scattered yellow flecks. Fluorescein angiography showed no leakage in the areas treated with laser. At a follow-up visit 1 month later, visual acuity of the right eye had improved to 20/30. Retinal examination revealed complete resolution of serous and RPE detachments of the right eye (Fig. 2, A and B). Yellow fleck deposits were visible in the area of the former fluid pockets. The left eye had a small persistent pigment epithelial detachment temporal to the fovea with resolution of the pigment epithelial detachment inferior to the fovea.
Discussion The entity called “lupus choroidopathy,” as reported by Jabs et al2, consists of serous detachments of the neurosensory retina and the RPE. Although uncommon, it has occasionally been reported since 1988.2– 4 Our diagnosis for the present patient was CSC secondary to corticosteroid use. Our case closely resembles previously reported cases of lupus choroidopathy. Our patient developed visually significant
serous retinal detachments after her SLE was controlled with corticosteroids and immune modulation. The appearance and timing of her complaints indicate a diagnosis of CSC mediated by corticosteroids rather than activation of her SLE. Our management included a decrease in her prednisone dose. She had considerable resolution of fluid in the fovea, but fluorescein angiography revealed some continued leakage under the superotemporal and inferotemporal serous detachments. We applied laser to the areas of leakage, with prompt successful resolution of the serous detachments. There has been previous success reported in treating lupus choroidopathy with reduction of corticosteroid dose, as we did.3 Successful laser treatment was previously reported in a case of lupus choroidopathy by Shimura et al.4 In that case, laser was applied in an eye 2 weeks after increasing dosages of cyclophosphamide and systemic steroid to control SLE. The in-
Fig. 2. Findings after tapering the corticosteroid dose and application of focal thermal green laser. A, Serous detachments are gone. Most of the fibrin has resorbed. Some fine white deposits remain. B, Fluorescein angiography shows absence of leakage.
9
CORTICOSTEROIDS AND LUPUS CHOROIDOPATHY
creased immune suppression was followed by worsening visual acuity and increasing area of serous detachment in the left eye. After application of thermal laser, the patient had regression of the serous detachments and improvement of visual acuity. Corticosteroids may cause neurosensory detachments or RPE detachments in patients with or without lupus.5 In reviewing the reported findings of cases of lupus choroidopathy presented by Jabs et al,2 it is clear that some are attributable to corticosteroid use. These cases were described before the widespread recognition of corticosteroids as a cause of CSC. Case 1 described by Jabs et al was a woman with visual symptoms beginning after initiation of corticosteroid treatment. Figure 1 of their report revealed multiple serous detachments containing fibrin exudates. These findings are very similar to those for our patient. The patient’s systemic disease required continued corticosteroid treatment. She had no resolution of her ophthalmic problems before dying of cardiopulmonary failure. Case 2 described by Jabs et al was a patient with visual complaints beginning after initiation of corticosteroid therapy for SLE. Again, the findings displayed in their Figure 2 are similar to those for our patient. Visual acuity improved over time with control of the disease. In contrast, lupus choroidopathy in Cases 4 and 6 described by Jabs et al presented before corticosteroid therapy, and these patients had definite improvement after beginning corticosteroid treatment. These cases probably represent choroidal vasculitis. Cases 3 and 5 described by Jabs et al did not have enough information to allow for assessment of lupus choroidopathy versus CSC. Additional reported cases3,4 support corticosteroids as the cause of serous detachments in the setting of SLE. Together with our case, these lead us to believe that some cases of lupus choroidopathy are corticoste-
roid-induced CSC. However, other presentations of lupus choroidopathy appear to represent immune-mediated choroidal vasculitis. Timing of visual symptoms may provide a clue in distinguishing between these two possibilities. In cases where visual symptoms precede induction of immune modulation, corticosteroids may be of benefit. In patients who develop symptoms after beginning corticosteroid treatment, the physician should consider tapering the corticosteroid dose if the systemic disease findings allow. Adjunctive use of laser photocoagulation may be beneficial in treating CSC when corticosteroids must be maintained to control SLE. Treatment of CSC in the setting of SLE entails cooperation between the ophthalmologist and the physician managing the SLE in balancing the ocular and systemic manifestations of the disease. Key words: central serous chorioretinopathy, chorioretinopathy, choroidopathy, choroiditis, corticosteroids, lupus erythematosus, vasculitis. References 1. 2.
3.
4.
5.
Mills JA. Systemic lupus erythematosus. N Engl J Med 1994; 330:1871–1878. Jabs DA, Hanneken AM, Schachat AP, Fine SL. Choroidopathy in systemic lupus erythematosus. Arch Ophthalmol 1988; 106:230–234. Khng CG, Yap EY, Au-Eong KG, et al. Central serous retinopathy complicating systemic lupus erythematosus: a case series. Clinical & Experimental Ophthalmology 2000;28:309– 313. Shimura M, Tatehana Y, Yasuda K, et al. Choroiditis in systemic lupus erythematosus: systemic steroid therapy and focal laser treatment. Jpn J Ophthalmol 2003;47:312–315. Jampol LM, Weinreb R, Yannuzzi LA. Involvement of corticosteroids and catecholamines in the pathogenesis of central serous chorioretinopathy: a rationale for new treatment strategies. Ophthalmology 2002;109:1765–1766.
Patients with lupus erythematosus may develop serous retinal detachments secondary to lupus choroidopathy. In some patients this choroidopathy is actually a manifestation of central serous chorioretinopathy from systemic corticosteroids used to treat the lupus. Decreasing or stopping the steroids is the treatment of choice.
From Northwestern University, Feinberg School of Medicine, Department of Ophthalmology, Chicago, Illinois.
Case Report A 32-year-old woman had a 4-day history of a dramatic decrease in vision of the right eye. The referring diagnosis was “lupus choroiditis.” The patient had an 11-year history of SLE and 4 months previously developed peripheral edema. Kidney biopsy showed rapidly progressive glomerulonephritis. Treatment was started with intravenous methylprednisolone (2 doses of 500 mg) and cyclophosphamide. Medication at the time of discharge from the hospital was prednisone (60 mg/day). When seen in the eye clinic 3 months later, her medications included prednisone (30 mg/day) and cyclophosphamide (1,700 mg monthly). Visual acuity was 20/400 in the right eye and 20/25 in the left eye. She identified zero of eight Ishihara plates on the right side and eight of eight plates on the left side. Intraocular pressures and results of external examination and anterior chamber examination were normal. Ophthalmoscopy of the right eye revealed a normal optic nerve head and vessels. Two areas of serous neurosensory detachment with extension under the fovea were seen (Fig. 1A). The extrafoveal portions of the detachments were filled with white debris thought to be fibrin. Ophthalmoscopy of the left eye revealed two small retinal pigment epithelium (RPE) detachments inferior and temporal to the fovea. Fluorescein angiography of the right eye (Fig. 1B) revealed two spots of focal leakage corresponding to the two extrafoveal serous elevations with filling of the neurosensory detachments in late-phase views. Underlying each area of neurosensory detachment, a small pigment epithelial detachment could be seen with the leakage occurring at the edge of the RPE detachment in one area and the center of the RPE detachment in the other. The left eye had focal dye accumulation in the two areas corresponding to the pigment epithelial detachments. Our diagnosis was corticosteroid-induced CSC. In consultation with the rheumatologist, we tapered the patient’s dose of prednisone over the next several days from 30 mg to 17.5 mg. Two weeks later, the serous elevation in the fovea had largely resolved, but an inferior peripheral retinal detachment was noted. Vision had not improved. Angiography showed decreased but continued leakage. Because partially tapering the prednisone dose did not result in complete resolution, focal thermal green laser was applied to the leaking spots in the right eye. One week after focal laser application, visual acuity had improved to 20/50; there was complete
S
ystemic lupus erythematosus (SLE) is an autoimmune disease characterized by deposition of immune complexes in internal organs including the vascular system. Many of the manifestations of SLE are a result of tissue damage secondary to this vasculitis.1 Retinal findings include cotton-wool spots and retinal hemorrhages related to vasculitis or retinal, disk, or choroidal vasoocclusion related to thrombosis from anticardiolipin antibodies. Another reported ocular complication is multifocal serous retinal detachments, which has been postulated as a choroidopathy secondary to systemic lupus.2 For the purpose of this discussion, we will term this entity “lupus choroidopathy.” We describe a case of serous retinal detachments in a patient with SLE and nephritis. On the basis of the fundus appearance and clinical course, we believe that the chorioretinal findings of lupus choroidopathy in this patient were secondary to central serous chorioretinopathy (CSC) from systemically administered corticosteroids. The presentation and course of this patient lead us to the conclusion that lupus choroidopathy as described by Jabs et al2 is often a presentation of corticosteroid-induced CSC, rather than choroidal vasculitis or thrombosis. Supported in part by an unrestricted grant from Research to Prevent Blindness, New York, NY. The authors have no proprietary interest to report. Reprint requests: Lee M. Jampol, Northwestern University, Feinberg School of Medicine, Department of Ophthalmology, 645 North Michigan Avenue, Suite 440, Chicago, IL 60611; e-mail: [email protected]
7
RETINAL CASES & BRIEF REPORTSℜ
8
●
2007
●
VOLUME 1
●
NUMBER 1
Fig. 1. A, Ophthalmoscopy demonstrates two areas of serous detachment with fibrin exudates. Serous fluid extends under the fovea. B, Late-phase findings of fluorescein angiography. Two major areas of leakage are seen.
flattening of the serous elevations with markedly decreased fibrin under the retina. The fovea was dry with some scattered yellow flecks. Fluorescein angiography showed no leakage in the areas treated with laser. At a follow-up visit 1 month later, visual acuity of the right eye had improved to 20/30. Retinal examination revealed complete resolution of serous and RPE detachments of the right eye (Fig. 2, A and B). Yellow fleck deposits were visible in the area of the former fluid pockets. The left eye had a small persistent pigment epithelial detachment temporal to the fovea with resolution of the pigment epithelial detachment inferior to the fovea.
Discussion The entity called “lupus choroidopathy,” as reported by Jabs et al2, consists of serous detachments of the neurosensory retina and the RPE. Although uncommon, it has occasionally been reported since 1988.2– 4 Our diagnosis for the present patient was CSC secondary to corticosteroid use. Our case closely resembles previously reported cases of lupus choroidopathy. Our patient developed visually significant
serous retinal detachments after her SLE was controlled with corticosteroids and immune modulation. The appearance and timing of her complaints indicate a diagnosis of CSC mediated by corticosteroids rather than activation of her SLE. Our management included a decrease in her prednisone dose. She had considerable resolution of fluid in the fovea, but fluorescein angiography revealed some continued leakage under the superotemporal and inferotemporal serous detachments. We applied laser to the areas of leakage, with prompt successful resolution of the serous detachments. There has been previous success reported in treating lupus choroidopathy with reduction of corticosteroid dose, as we did.3 Successful laser treatment was previously reported in a case of lupus choroidopathy by Shimura et al.4 In that case, laser was applied in an eye 2 weeks after increasing dosages of cyclophosphamide and systemic steroid to control SLE. The in-
Fig. 2. Findings after tapering the corticosteroid dose and application of focal thermal green laser. A, Serous detachments are gone. Most of the fibrin has resorbed. Some fine white deposits remain. B, Fluorescein angiography shows absence of leakage.
9
CORTICOSTEROIDS AND LUPUS CHOROIDOPATHY
creased immune suppression was followed by worsening visual acuity and increasing area of serous detachment in the left eye. After application of thermal laser, the patient had regression of the serous detachments and improvement of visual acuity. Corticosteroids may cause neurosensory detachments or RPE detachments in patients with or without lupus.5 In reviewing the reported findings of cases of lupus choroidopathy presented by Jabs et al,2 it is clear that some are attributable to corticosteroid use. These cases were described before the widespread recognition of corticosteroids as a cause of CSC. Case 1 described by Jabs et al was a woman with visual symptoms beginning after initiation of corticosteroid treatment. Figure 1 of their report revealed multiple serous detachments containing fibrin exudates. These findings are very similar to those for our patient. The patient’s systemic disease required continued corticosteroid treatment. She had no resolution of her ophthalmic problems before dying of cardiopulmonary failure. Case 2 described by Jabs et al was a patient with visual complaints beginning after initiation of corticosteroid therapy for SLE. Again, the findings displayed in their Figure 2 are similar to those for our patient. Visual acuity improved over time with control of the disease. In contrast, lupus choroidopathy in Cases 4 and 6 described by Jabs et al presented before corticosteroid therapy, and these patients had definite improvement after beginning corticosteroid treatment. These cases probably represent choroidal vasculitis. Cases 3 and 5 described by Jabs et al did not have enough information to allow for assessment of lupus choroidopathy versus CSC. Additional reported cases3,4 support corticosteroids as the cause of serous detachments in the setting of SLE. Together with our case, these lead us to believe that some cases of lupus choroidopathy are corticoste-
roid-induced CSC. However, other presentations of lupus choroidopathy appear to represent immune-mediated choroidal vasculitis. Timing of visual symptoms may provide a clue in distinguishing between these two possibilities. In cases where visual symptoms precede induction of immune modulation, corticosteroids may be of benefit. In patients who develop symptoms after beginning corticosteroid treatment, the physician should consider tapering the corticosteroid dose if the systemic disease findings allow. Adjunctive use of laser photocoagulation may be beneficial in treating CSC when corticosteroids must be maintained to control SLE. Treatment of CSC in the setting of SLE entails cooperation between the ophthalmologist and the physician managing the SLE in balancing the ocular and systemic manifestations of the disease. Key words: central serous chorioretinopathy, chorioretinopathy, choroidopathy, choroiditis, corticosteroids, lupus erythematosus, vasculitis. References 1. 2.
3.
4.
5.
Mills JA. Systemic lupus erythematosus. N Engl J Med 1994; 330:1871–1878. Jabs DA, Hanneken AM, Schachat AP, Fine SL. Choroidopathy in systemic lupus erythematosus. Arch Ophthalmol 1988; 106:230–234. Khng CG, Yap EY, Au-Eong KG, et al. Central serous retinopathy complicating systemic lupus erythematosus: a case series. Clinical & Experimental Ophthalmology 2000;28:309– 313. Shimura M, Tatehana Y, Yasuda K, et al. Choroiditis in systemic lupus erythematosus: systemic steroid therapy and focal laser treatment. Jpn J Ophthalmol 2003;47:312–315. Jampol LM, Weinreb R, Yannuzzi LA. Involvement of corticosteroids and catecholamines in the pathogenesis of central serous chorioretinopathy: a rationale for new treatment strategies. Ophthalmology 2002;109:1765–1766.
