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Exudative retinal detachment in relapsing polychondritis
Exudative Retinal Detachment in Relapsing Polychondritis Case Report and Literature Review Neelakshi Bhagat, MD, Ronald L. Green, MD, Steven E. Feldon, MD, Jennifer I. Lim, MD Objective: To report the atypical ocular symptoms (arterialized conjunctival vessels, exudative retinal detachment) that can be the presentating manifestations of relapsing polychondritis. Design: Observational case report and literature review. Methods: A complete ocular and systemic evaluation was performed on a patient with relapsing polychondritis and exudative retinal detachment. Main Outcome Measures: Retinal, choroidal, and scleral findings. Results: A 73-year-old man with relapsing polychondritis presented with a unilateral large bullous exudative retinal detachment and marked choroidal and scleral thickening bilaterally. Conclusions: Ophthalmologists should consider relapsing polychondritis in the differential diagnosis of exudative retinal detachment. A combination of echographic and laboratory findings can assist in the accurate diagnosis of this rare condition. Ophthalmology 2001;108:1156 –1159 © 2001 by the American Academy of Ophthalmology. Relapsing polychondritis, a rare systemic autoimmune disorder, was first described in 1923 by Jaksch-Wartenhorst.1 Several systems— ophthalmologic, otorhinolaryngeal, respiratory, muskolo-skeletal, renal, cardiovascular, and dermatologic— can be involved, not necessarily simultaneously, making the diagnosis difficult.2 The prognosis is poor if the diagnosis is delayed. The disease can be fatal if laryngotracheal collapse or renal failure ensues. Ocular involvement (corneoscleral perforation, retinal vasculitis, or optic neuritis) can lead to blindness. Ocular symptoms are a major component of the disease, being present in 60% to 71% of patients with relapsing polychondritis.2,3 Ocular involvement can be the initial manifestation of this disease in up to 19% of patients.2 Episcleritis was found to be the most common ocular finding (39%) in the large series of 112 patients reported by Isaak et al.2 Other manifestations were scleritis (14%), iritis (9%), retinopathy (9%), muscle paresis (5%), optic neuritis (5%), and peripheral corneal thinning (4%). Lid edema,2 ptosis,4 conjunctival salmon patch,5 and vitritis4 have been reported also. Originally received: June 5, 2000. Accepted: February 12, 2001. Manuscript no. 200332. From the Doheny Eye Institute and the Keck School of Medicine, University of Southern California, Los Angeles, California. Supported in part by the National Institutes of Health, Bethesda, Maryland (grant no.: EY03040) and Research to Prevent Blindness, Inc., New York, New York. The authors have no proprietary interest in any of the materials used in this study. Reprint requests to Jennifer I. Lim, MD, Doheny Eye Institute 4703, 1450 San Pablo Street, Los Angeles, CA 90033.
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© 2001 by the American Academy of Ophthalmology Published by Elsevier Science Inc.
We report an interesting case of unilateral exudative retinal detachment in a patient with associated choroidal thickening who concurrently sought treatment from an ear, nose, and throat specialist for swollen, tender ears. On incisional biopsy of the pinna, he was diagnosed as having relapsing polychondritis.
Case Report A 73-year-old healthy, Hispanic man was referred to the Retina Service at Doheny Eye Institute for evaluation of a retinal detachment in his left eye. He reported a 2-month history of gradually worsening superior visual field defect. His symptoms were worse in the morning and improved as the day progressed. On presentation, he reported acute loss of vision in the left eye for 2 days. Prior ocular history included evaluation by the neuro-ophthalmology service 4 months previously. At that time, he had bilateral arterialized conjunctival vessels, ptosis, diplopia, ocular hypertension, and decreased vision in the right eye. The results of a fundus examination were noted to be normal. A low-grade carotid– cavernous fistula was suspected. However, bilateral carotid and vertebral arteriography failed to demonstrate any evidence of an arteriovenous fistula. No intracranial aneurysm or unusual venous drainage pattern was visualized. Soon thereafter, the conjunctival injection, ptosis, and diplopia resolved, and we concurred that a small dural fistula had closed spontaneously. During our examination, he reported no medical problems other than tender, swollen ears for 2 months. On examination by an otolaryngologist 1 week previously, a biopsy of the left pinna was performed. Prednisolone 40 mg/day for 1 week was prescribed, Other medications included acetazolamide (Diamox) 250 mg/day, latanoprost (Xalatan; Pharmacia & Upjohn, Kalamazoo, MI), brimonidine 0.2% (Alphagan, Irvine, CA), and dorzolamide hydrochloride-timolol maleate (Cosopt, West Point, PA) eye drops for ocular hypertension. Family history was noncontributory. ISSN 0161-6420/01/$–see front matter PII S0161-6420(01)00568-1
Bhagat et al 䡠 Exudative Retinal Detachment in Relapsing Polychondritis
Figure 1. A color fundus photo of the left eye shows an exudative retinal detachment. The arrow is pointing to the more bullous area of the exudative retinal detachment.
Ocular examination revealed visual acuities of 20/40 in the right eye and finger counting at 4 feet in the left eye. There was a trace afferent pupillary defect in the left eye. Hertel measurements were 18 mm in each eye with a base of 100 mm. Confrontational visual field was normal for the right eye, but marked constriction of superior visual field was noted in the left eye. Results of slit-lamp examination of the right eye were normal except for mild nuclear sclerotic lens changes. The left eye slit-lamp examination revealed a slightly shallow anterior chamber with mild nuclear sclerotic lenticular changes. Intraocular pressures were 18 mmHg in the right eye and 19 mmHg in the left eye. Fundus examination of the right eye revealed a pink and healthy optic nerve with a cup-to-disc ratio of 0.3. The posterior pole and the periphery were noted to be normal. The left eye fundus revealed a bullous, exudative, inferior retinal detachment that covered the posterior pole and the optic nerve. Shallow 360° peripheral choroidals were noted. On subsequent examination 5 days later, visual acuity in the left eye had improved to 3/200. The exudative retinal detachment had improved slightly (Fig 1). The optic nerve could be seen and was found to be normal. The right eye, however, had new fundus findings. The choroid in the posterior pole appeared edematous, giving an unusual orange color to the posterior pole (Fig 2). Shallow peripheral choroidals were noted.
Figure 2. A front color fundus photograph of the right eye shows an edematous-appearing choroid (arrow).
Figure 3. A late-phase fluorescein angiogram showing multiple pinpoint leaks at the level of the retinal pigment epithelium.
Fluorescein angiography showed normal choroidal filling, but also showed early and late pinpoint leaks at the level of the retinal pigment epithelium in the posterior pole (Figs 3 and 4) and the midperiphery of both eyes. Ultrasonography revealed normal-sized globes with moderately thickened sclera with extremely thickened choroids in both eyes, greater in the left eye than the right (Figs 5 and 6). The choroidal thickening appeared to be medium reflective using the standardized A-scan. There was no T-sign. Peripheral shallow choroidals were noted bilaterally. The left eye also revealed a large inferior exudative retinal detachment.
Figure 4. A fluorescein angiogram of the right eye showing multiple areas of hyperfluorescence in the posterior pole region.
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Ophthalmology Volume 108, Number 6, June 2001
Discussion
Figure 5. A B-scan ultrasound of the left eye demonstrates thickening of the choroidal layer (double arrowhead). Note the presence of subretinal fluid (arrow).
The differential diagnosis included reactive benign lymphoid hyperplasia, metastatic carcinoma, uveal effusion syndrome, scleritis, Harada’s disease, lymphoma, or an infiltrative process. The exudative retinal detachment could have been the result of an idiosyncratic response to steroids. Laboratory work-up revealed a low hemoglobin level, and normal liver function tests, prostate-specific antigen level, chest radiograph, and computerized tomography scan of the abdomen. The erythrocyte sedimentation rate was elevated at 85 mm/hour. The pathology report of the biopsy of the pinna revealed a diagnosis of relapsing polychondritis. The enzyme-linked immunosorbent assay results for collagen II antibody were, however, negative. The serous retinal detachment of the left eye decreased with improvement of the visual acuity within 5 days of presentation. The ears showed decreased swelling and tenderness. The improvement was most likely the result of the steroid pulse treatment. The scleritis and choroidal thickening with exudative response were attributed to the underlying relapsing polychondritis. The ptosis may have been a manifestation of relapsing polychondritis, as well.
Figure 6. An ultrasound examination of the right eye similarly shows choroidal thickening (double arrowhead) and the presence of less subretinal fluid (arrow).
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Relapsing polychondritis is an idiopathic autoimmune disease in which autoantibodies to type II collagen and cellmediated immunity to cartilage components are found. It usually manifests between 40 and 60 years of age and has no gender predilection.2 In most patients, mild anemia and elevated erythrocyte sedimentation rates are demonstrated.2,6 The most common ocular manifestations are episcleritis and scleritis— diffuse, nodular, or necrotizing, mostly anterior and unilateral. Unilateral exudative retinal detachment has been described in only two patients.6,7 Anderson7 reported unilateral exudative retinopathy in a 21-year-old male with relapsing polychondritis that initially resembled early Coats’ disease but progressed to bullous serous retinal detachment. Brink et al,6 in 1994, described a 70-year-old woman with unilateral episcleritis, scleritis, and exudative retinal detachment at presentation that resolved with the use of prednisone 40 mg/day for 1 month.6 Magargal et al3 described a 46-year-old woman with relapsing polychondritis with bilateral sclerouveitis and localized macular detachments at presentation. These resolved with 80 mg/day of prednisone for 1 month. We describe an unusual initial presentation of the ocular manifestation mimicking dural fistula. Four months later, the patient sought treatment for unilateral exudative bullous retinal detachments with marked bilateral choroidal and scleral thickening on ultrasonography. No clinical evidence of scleritis was noted. Although the pathologic analysis of the pinna revealed findings consistent with relapsing polychondritis, the enzyme-linked immunosorbent assay failed to detect collagen II antibodies. The enzyme-linked immunosorbent assay was sent for analysis 3 weeks after the pulse treatment with steroids, when the ocular and aural symptoms were resolving. As usually noted in patients with relapsing chondritis, the erythrocyte sedimentation rate was elevated and mild anemia was present. Because type II collagen is found in both cartilage and sclera, immune complexes may play a role in the pathogenesis of scleritis in relapsing polychondritis. Anderson7 noted granulomatous inflammation of the sclera and choroid with nonspecific conjunctival vasculitis in a patient with relapsing polychondritis. Hoang-Xuan et al4 reported the presence of immunoglobulin and complement deposition in the vessel walls. Immune complex infiltration within the choroid may give rise to marked choroidal thickening. The choroidal detachments may be associated with exudative retinal detachments, as seen in our patient. Ocular symptoms can occur as the presenting manifestation of the disease in almost one fifth of the patients2 with relapsing polychondritis. It is important that relapsing polychondritis be included in the differential diagnosis of exudative retinal detachment and nonspecific history of red eye. The prognosis is poor if the diagnosis is delayed. The 5-year survival rate is 74%.2,8 Mortality results from pneumonia, systemic vasculitis, aneurysms, valve disorders, renal failure, or complications from treatment.2,8 Treatment guidelines for relapsing polychondritis are not well defined. Nonsteroidal antiinflammatory drugs (indomethacin)2,4 and corticosteroid therapy (prednisone 1 mg/kg
Bhagat et al 䡠 Exudative Retinal Detachment in Relapsing Polychondritis daily)2,4 may be sufficient to treat mild ocular inflammation resulting from relapsing polychondritis. Immunosuppressants and cytotoxic agents may be needed to treat scleritis. The eight patients with scleritis and relapsing polychondritis who were being treated with systemic steroids, additional drugs (cyclophosphamide, dapsone, azathioprine, methotrexate) were needed to control the inflammation in the series by Hoang-Xuan et al.4 Cyclophosphamide may be the first drug of choice in the presence of necrotizing scleritis. Azathioprine may be added for failures and for patients with nodular scleritis.4
References 1. Jaksch-Wartenhorst R. Polychondropathia. Wien Arch Inn Med 1923;6:93–100. 2. Isaak BL, Liesegang TJ, Michet CJ Jr. Ocular and systemic
3. 4.
5.
6.
7.
8.
findings in relapsing polychondritis. Ophthalmology 1986;93: 681–9. Magargal LE, Donoso LA, Goldberg RE, et al. Ocular manifestations of relapsing polychondritis. Retina 1981;1:96 –9. Hoang-Xuan T, Foster CS, Rice BA. Scleritis in relapsing polychondritis. Response to therapy. Ophthalmology 1990;97: 892– 8. Tucker SM, Linberg JV, Doshi HM. Relapsing polychondritis, another cause for a “salmon patch.” Ann Ophthalmol 1993; 25:389 –91. Brink H, Rademakers J, Verbeek A, et al. Ocular manifestations of relapsing polychondritis. Three case histories. Doc Ophthalmol 1994;87:159 – 66. Anderson B Sr. Ocular lesions in relapsing polychondritis and other rheumatoid syndromes. The Edward Jackson Memorial Lecture. Am J Ophthalmol 1967;64:35–50. Michet CJ Jr, McKenna CH, Luthra HS, O’Fallon WM. Relapsing polychondritis. Survival and predictive role of early disease manifestations. Ann Intern Med 1986;104:74 – 8.
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© 2001 by the American Academy of Ophthalmology Published by Elsevier Science Inc.
We report an interesting case of unilateral exudative retinal detachment in a patient with associated choroidal thickening who concurrently sought treatment from an ear, nose, and throat specialist for swollen, tender ears. On incisional biopsy of the pinna, he was diagnosed as having relapsing polychondritis.
Case Report A 73-year-old healthy, Hispanic man was referred to the Retina Service at Doheny Eye Institute for evaluation of a retinal detachment in his left eye. He reported a 2-month history of gradually worsening superior visual field defect. His symptoms were worse in the morning and improved as the day progressed. On presentation, he reported acute loss of vision in the left eye for 2 days. Prior ocular history included evaluation by the neuro-ophthalmology service 4 months previously. At that time, he had bilateral arterialized conjunctival vessels, ptosis, diplopia, ocular hypertension, and decreased vision in the right eye. The results of a fundus examination were noted to be normal. A low-grade carotid– cavernous fistula was suspected. However, bilateral carotid and vertebral arteriography failed to demonstrate any evidence of an arteriovenous fistula. No intracranial aneurysm or unusual venous drainage pattern was visualized. Soon thereafter, the conjunctival injection, ptosis, and diplopia resolved, and we concurred that a small dural fistula had closed spontaneously. During our examination, he reported no medical problems other than tender, swollen ears for 2 months. On examination by an otolaryngologist 1 week previously, a biopsy of the left pinna was performed. Prednisolone 40 mg/day for 1 week was prescribed, Other medications included acetazolamide (Diamox) 250 mg/day, latanoprost (Xalatan; Pharmacia & Upjohn, Kalamazoo, MI), brimonidine 0.2% (Alphagan, Irvine, CA), and dorzolamide hydrochloride-timolol maleate (Cosopt, West Point, PA) eye drops for ocular hypertension. Family history was noncontributory. ISSN 0161-6420/01/$–see front matter PII S0161-6420(01)00568-1
Bhagat et al 䡠 Exudative Retinal Detachment in Relapsing Polychondritis
Figure 1. A color fundus photo of the left eye shows an exudative retinal detachment. The arrow is pointing to the more bullous area of the exudative retinal detachment.
Ocular examination revealed visual acuities of 20/40 in the right eye and finger counting at 4 feet in the left eye. There was a trace afferent pupillary defect in the left eye. Hertel measurements were 18 mm in each eye with a base of 100 mm. Confrontational visual field was normal for the right eye, but marked constriction of superior visual field was noted in the left eye. Results of slit-lamp examination of the right eye were normal except for mild nuclear sclerotic lens changes. The left eye slit-lamp examination revealed a slightly shallow anterior chamber with mild nuclear sclerotic lenticular changes. Intraocular pressures were 18 mmHg in the right eye and 19 mmHg in the left eye. Fundus examination of the right eye revealed a pink and healthy optic nerve with a cup-to-disc ratio of 0.3. The posterior pole and the periphery were noted to be normal. The left eye fundus revealed a bullous, exudative, inferior retinal detachment that covered the posterior pole and the optic nerve. Shallow 360° peripheral choroidals were noted. On subsequent examination 5 days later, visual acuity in the left eye had improved to 3/200. The exudative retinal detachment had improved slightly (Fig 1). The optic nerve could be seen and was found to be normal. The right eye, however, had new fundus findings. The choroid in the posterior pole appeared edematous, giving an unusual orange color to the posterior pole (Fig 2). Shallow peripheral choroidals were noted.
Figure 2. A front color fundus photograph of the right eye shows an edematous-appearing choroid (arrow).
Figure 3. A late-phase fluorescein angiogram showing multiple pinpoint leaks at the level of the retinal pigment epithelium.
Fluorescein angiography showed normal choroidal filling, but also showed early and late pinpoint leaks at the level of the retinal pigment epithelium in the posterior pole (Figs 3 and 4) and the midperiphery of both eyes. Ultrasonography revealed normal-sized globes with moderately thickened sclera with extremely thickened choroids in both eyes, greater in the left eye than the right (Figs 5 and 6). The choroidal thickening appeared to be medium reflective using the standardized A-scan. There was no T-sign. Peripheral shallow choroidals were noted bilaterally. The left eye also revealed a large inferior exudative retinal detachment.
Figure 4. A fluorescein angiogram of the right eye showing multiple areas of hyperfluorescence in the posterior pole region.
1157
Ophthalmology Volume 108, Number 6, June 2001
Discussion
Figure 5. A B-scan ultrasound of the left eye demonstrates thickening of the choroidal layer (double arrowhead). Note the presence of subretinal fluid (arrow).
The differential diagnosis included reactive benign lymphoid hyperplasia, metastatic carcinoma, uveal effusion syndrome, scleritis, Harada’s disease, lymphoma, or an infiltrative process. The exudative retinal detachment could have been the result of an idiosyncratic response to steroids. Laboratory work-up revealed a low hemoglobin level, and normal liver function tests, prostate-specific antigen level, chest radiograph, and computerized tomography scan of the abdomen. The erythrocyte sedimentation rate was elevated at 85 mm/hour. The pathology report of the biopsy of the pinna revealed a diagnosis of relapsing polychondritis. The enzyme-linked immunosorbent assay results for collagen II antibody were, however, negative. The serous retinal detachment of the left eye decreased with improvement of the visual acuity within 5 days of presentation. The ears showed decreased swelling and tenderness. The improvement was most likely the result of the steroid pulse treatment. The scleritis and choroidal thickening with exudative response were attributed to the underlying relapsing polychondritis. The ptosis may have been a manifestation of relapsing polychondritis, as well.
Figure 6. An ultrasound examination of the right eye similarly shows choroidal thickening (double arrowhead) and the presence of less subretinal fluid (arrow).
1158
Relapsing polychondritis is an idiopathic autoimmune disease in which autoantibodies to type II collagen and cellmediated immunity to cartilage components are found. It usually manifests between 40 and 60 years of age and has no gender predilection.2 In most patients, mild anemia and elevated erythrocyte sedimentation rates are demonstrated.2,6 The most common ocular manifestations are episcleritis and scleritis— diffuse, nodular, or necrotizing, mostly anterior and unilateral. Unilateral exudative retinal detachment has been described in only two patients.6,7 Anderson7 reported unilateral exudative retinopathy in a 21-year-old male with relapsing polychondritis that initially resembled early Coats’ disease but progressed to bullous serous retinal detachment. Brink et al,6 in 1994, described a 70-year-old woman with unilateral episcleritis, scleritis, and exudative retinal detachment at presentation that resolved with the use of prednisone 40 mg/day for 1 month.6 Magargal et al3 described a 46-year-old woman with relapsing polychondritis with bilateral sclerouveitis and localized macular detachments at presentation. These resolved with 80 mg/day of prednisone for 1 month. We describe an unusual initial presentation of the ocular manifestation mimicking dural fistula. Four months later, the patient sought treatment for unilateral exudative bullous retinal detachments with marked bilateral choroidal and scleral thickening on ultrasonography. No clinical evidence of scleritis was noted. Although the pathologic analysis of the pinna revealed findings consistent with relapsing polychondritis, the enzyme-linked immunosorbent assay failed to detect collagen II antibodies. The enzyme-linked immunosorbent assay was sent for analysis 3 weeks after the pulse treatment with steroids, when the ocular and aural symptoms were resolving. As usually noted in patients with relapsing chondritis, the erythrocyte sedimentation rate was elevated and mild anemia was present. Because type II collagen is found in both cartilage and sclera, immune complexes may play a role in the pathogenesis of scleritis in relapsing polychondritis. Anderson7 noted granulomatous inflammation of the sclera and choroid with nonspecific conjunctival vasculitis in a patient with relapsing polychondritis. Hoang-Xuan et al4 reported the presence of immunoglobulin and complement deposition in the vessel walls. Immune complex infiltration within the choroid may give rise to marked choroidal thickening. The choroidal detachments may be associated with exudative retinal detachments, as seen in our patient. Ocular symptoms can occur as the presenting manifestation of the disease in almost one fifth of the patients2 with relapsing polychondritis. It is important that relapsing polychondritis be included in the differential diagnosis of exudative retinal detachment and nonspecific history of red eye. The prognosis is poor if the diagnosis is delayed. The 5-year survival rate is 74%.2,8 Mortality results from pneumonia, systemic vasculitis, aneurysms, valve disorders, renal failure, or complications from treatment.2,8 Treatment guidelines for relapsing polychondritis are not well defined. Nonsteroidal antiinflammatory drugs (indomethacin)2,4 and corticosteroid therapy (prednisone 1 mg/kg
Bhagat et al 䡠 Exudative Retinal Detachment in Relapsing Polychondritis daily)2,4 may be sufficient to treat mild ocular inflammation resulting from relapsing polychondritis. Immunosuppressants and cytotoxic agents may be needed to treat scleritis. The eight patients with scleritis and relapsing polychondritis who were being treated with systemic steroids, additional drugs (cyclophosphamide, dapsone, azathioprine, methotrexate) were needed to control the inflammation in the series by Hoang-Xuan et al.4 Cyclophosphamide may be the first drug of choice in the presence of necrotizing scleritis. Azathioprine may be added for failures and for patients with nodular scleritis.4
References 1. Jaksch-Wartenhorst R. Polychondropathia. Wien Arch Inn Med 1923;6:93–100. 2. Isaak BL, Liesegang TJ, Michet CJ Jr. Ocular and systemic
3. 4.
5.
6.
7.
8.
findings in relapsing polychondritis. Ophthalmology 1986;93: 681–9. Magargal LE, Donoso LA, Goldberg RE, et al. Ocular manifestations of relapsing polychondritis. Retina 1981;1:96 –9. Hoang-Xuan T, Foster CS, Rice BA. Scleritis in relapsing polychondritis. Response to therapy. Ophthalmology 1990;97: 892– 8. Tucker SM, Linberg JV, Doshi HM. Relapsing polychondritis, another cause for a “salmon patch.” Ann Ophthalmol 1993; 25:389 –91. Brink H, Rademakers J, Verbeek A, et al. Ocular manifestations of relapsing polychondritis. Three case histories. Doc Ophthalmol 1994;87:159 – 66. Anderson B Sr. Ocular lesions in relapsing polychondritis and other rheumatoid syndromes. The Edward Jackson Memorial Lecture. Am J Ophthalmol 1967;64:35–50. Michet CJ Jr, McKenna CH, Luthra HS, O’Fallon WM. Relapsing polychondritis. Survival and predictive role of early disease manifestations. Ann Intern Med 1986;104:74 – 8.
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