We report the case of a 51-year-old woman who presented with bilateral progressive deterioration in vision after taking chloroquine for severe rheumatoid arthritis for 10 years. She was found to have a bull's eye pattern of depigmentation in the macula of both eyes. Despite cessation of chloroquine, her vision did not improve. The clinical presentation of chloroquine retinopathy is discussed, along with the importance of scheduled eye examination for individuals taking chloroquine or hydroxychloroquine.published_or_final_versio

Lai, WW

Lam, DSC

Hong Kong Medical Journal

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Title Chloroquine-induced bull's eye maculopathyAuthor(s) Lai, WW; Lam, DSCCitation Hong Kong Medical Journal, 2005, v. 11 n. 1, p. 55-57Issued Date 2005URL http://hdl.handle.net/10722/137049Rights Creative Commons: Attribution 3.0 Hong Kong LicenseHong Kong Med J Vol 11 No 1 February 2005     55CASE REPORTKey words:Arthritis, rheumatoid;Chloroquine;Macula lutea;Retinal diseases ! !"#$%   !"Hong Kong Med J 2005;11:55-7Chloroquine-induced bull’s eyemaculopathy○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ ○ !"#$%&'()*Department of Ophthalmology and VisualSciences, The Chinese University of HongKong, Prince of Wales Hospital, Shatin,Hong KongWW Lai, MDDSC Lam, FRCS (Edin), FHKAM (Ophthalmology)Correspondence to: Dr WW Lai(e-mail: wicolai@cuhk.edu.hk)WW Lai  DSC Lam  We report the case of a 51-year-old woman who presented withbilateral progressive deterioration in vision after taking chloroquinefor severe rheumatoid arthritis for 10 years. She was found to havea bull’s eye pattern of depigmentation in the macula of both eyes.Despite cessation of chloroquine, her vision did not improve. Theclinical presentation of chloroquine retinopathy is discussed, alongwith the importance of scheduled eye examination for individualstaking chloroquine or hydroxychloroquine. !"#$ RN !"#$%&'()*+,-. /01 NM !"#$%&'()*+,-./0123"456789:; !"#$%&'()*+,-./012!"345&67895 !"#$%&'()*'(+,-./012345678IntroductionChloroquine is prescribed as an antimalarial, as well as a treatment forinflammatory conditions including rheumatoid arthritis and systemiclupus erythematosus. It has been recognised that prolonged use ofchloroquine may result in degeneration of the retinal pigment epitheliumand the neurosensory retina, causing a ‘bull’s eye’ pattern of depigmenta-tion of the macula, and subsequent central visual loss.1,2 We report a caseof bull’s eye maculopathy developed as a result of chronic use ofchloroquine.Case reportA 51-year-old woman from Mainland China presented with a historyof progressive deterioration in vision in both eyes for 6 months. Hermedical history included severe rheumatoid arthritis, for which shehad received chloroquine therapy for approximately 10 years. Therewas no record of the cumulative dosage given. On examination, hervisual acuity was 20/100 in both eyes. Slit lamp examination waswithin normal limits in both eyes. Dilated fundus examination revealeda ring of depigmentation of the retinal pigment epithelium in themacula (Fig). The remainder of the fundus examination was normal.Fluorescein angiography (Fig) was then performed, which showed abull’s eye pattern of granular hyperfluorescence, corresponding towindow defects in both eyes. A diagnosis of chloroquine-induced bull’seye maculopathy was made. Following discussion with the patient’sphysician, chloroquine treatment was discontinued. Azathioprine wassubstituted as a treatment for her rheumatoid arthritis and she respondedwell. On follow-up examination 3 months later, the woman’s visionremained unchanged.Lai and Lam56      Hong Kong Med J Vol 11 No 1 February 2005DiscussionChloroquine, and to a lesser extent its analogue,hydroxychloroquine, have been used for the treatmentof malaria for many years. More recently, they havealso been used in the treatment of systemic lupuserythematosus, rheumatoid arthritis, and otherinflammatory and dermatologic conditions. Prolongeduse of these agents may result in toxicity to the retina.The earliest sign of toxicity is paracentral visualfield loss.1-4 This may occur in advance of anyophthalmoscopic findings. However, many patientsmay be asymptomatic in the early stages.1-3 As thecondition worsens, alterations in the macula occur,characterised by atrophy of the retinal pigmentepithelium in the form of a bull’s eye.1-3 A small,central island of the fovea may be spared initially.The ring of depigmentation may progressively enlargeand affect the foveola, at which time central visionmay be affected. The bull’s eye pattern of depigmen-tation may be best appreciated on fluoresceinangiography.The exact mechanism of chloroquine toxicity is notfully understood. Reversal of visual field loss has beendocumented. However, most cases in which paracen-tral scotomas have developed or in which bull’s eyemaculopathy has become evident have not shown anysignificant recovery of vision.5Most individuals who develop evidence of toxicityhave received a cumulative chloroquine dose of 300 g,or a daily dose greater than 3 mg/kg,3,4 or a daily doseof hydroxychloroquine greater than 6.5 mg/kg.6 It mustbe highlighted that although irreversible damage to theretina may occur with these medications, the incidenceis very low. During the early stages of use of thesemedications, toxicity is rare. Most patients whodevelop loss of vision have used the medication formore than 5 years. The incidence of toxicity increaseswith higher doses and longer duration of use.7-10Individuals who are taking chloroquine orhydroxychloroquine should undergo periodic andcomprehensive eye examinations. The aim of theFig. The bull’s eye pattern of depigmentation associated with chloroquine maculopathy is evident on fundusphotographs and highlighted by fluorescein angiography: photographs of the (a) right eye and (b) left eye; andangiograms of the (c) right eye and (d) left eye(a) (b)(c) (d)Hong Kong Med J Vol 11 No 1 February 2005      57Chloroquine-induced bull’s eye maculopathyexamination is to allow early identification and mini-misation of toxicity. It must be emphasised that thereis no established criteria for diagnosing toxicity be-fore a stage where permanent visual loss develops.9The examination should include testing of best-corrected visual acuity, as well as slit lamp and di-lated fundus examinations. Baseline central visual fieldtesting using the Amsler grid (Keeler, Windsor, UnitedKingdom) or the automated Humphrey visual fieldanalyser (Humphrey, Dublin, United States) shouldalso be performed. Optional testing, such as assess-ment of colour vision, fundus photography, fluoresceinangiography, and multifocal electroretinography maybe considered depending on the presentation.The frequency of eye examinations is based on therelative risk of developing retinopathy. If a baselineophthalmologic examination is normal in someonewho has just started treatment with chloroquine orhydroxychloroquine, screening during the next 5 yearscan be done at the same frequency as that recom-mended by the American Academy of Ophthalmologyfor regular eye examinations (Table10).Individuals at high risk of developing retinopathyinclude: those taking more than 3 mg/kg/d ofchloroquine,  or  more than 6.5 mg/kg/d ofhydroxychloroquine; those who have used themedication for more than 5 years; individuals withobese body habitus; those with liver or renal disease(because these drugs are dependent on both hepaticand renal clearance); those with pre-existing retinaldisease; and those older than 60 years.9 These high-risk individuals should undergo annual and compre-hensive eye examinations. They should be asked toperform regular Amsler grid testing of their centralvisual field at home and to return promptly for a com-plete eye examination should there be any changesnoted on the examination.To date, there is no medical treatment for chloro-quine and hydroxychloroquine toxicity other thancessation of the medication. Prior to discontinuing thedrug, consideration must be given to the managementof the underlying systemic condition. Discussionwith the rheumatologist or other physician managingtreatment is important, because the cessation of thedrug may lead to worsening of the systemic disease.Individuals with possible signs of toxicity may befollowed closely (eg every 3 months). Those withdefinite toxicity may have to discontinue the medica-tion immediately. It must be noted that vision loss maycontinue for several months after discontinuing thedrug. Early detection is important because theoretically,some degree of visual recovery is possible at the veryearly stage of functional loss after cessation of themedication.References1. Bernstein H, Zvaifler N, Rubin M, Mansour AM. Theocular deposition of chloroquine. Invest Ophthalmol 1963;2:384-92.2. Henkind P, Rothfield NF. Ocular abnormalities in patientstreated with synthetic antimalarial drugs. N Engl J Med1963;269:433-9.3. Elman A, Gullberg R, Nilsson E, Rendahl I, WachtmeisterL. Chloroquine retinopathy in patients with rheumatoidarthritis. Scand J Rheumatol 1976;5:161-6.4. Cruess AF, Schachat AP, Nicholl J, Augsburger JJ. Chloro-quine retinopathy. Is fluorescein angiography necessary?Ophthalmology 1985;92:1127-9.5. Easterbrook M. Long-term course of antimalarial maculopathyafter cessation of the treatment. Can J Ophthalmol 1992;27:237-9.6. Tobin DR, Krohel G, Rynes RI. Hydroxychloroquine.Seven-year experience. Arch Ophthalmol 1982;100:81-3.7. Mavrikakis M, Papazoglou S, Sfikakis PP, Vaiopoulos G,Rougas K. Retinal toxicity in long term hydroxychloroquinetreatment. Ann Rheum Dis 1996;55:187-9.8. Levy GD, Munz SJ, Paschal J, Cohen HB, Pince KJ, PetersonT. Incidence of hydroxychloroquine retinopathy in 1207patients in a large multicenter outpatient practice. ArthritisRheum 1997;40:1482-6.9. Marmor MF, Carr RE, Easterbrook M, Farjo AA, MielerWF; American Academy of Ophthalmology. Recommenda-tions on screening for chloroquine and hydroxychloroquineretinopathy: a report by the American Academy ofOphthalmology. Ophthalmology 2002;109:1377-82.10. American Academy of Ophthalmology. Comprehensiveadult medical eye evaluation, Preferred Practice Pattern.San Francisco: American Academy of Ophthalmology;2000.)sraey(egA ycneuqerfnoitanimaxE92-0293-0346-0456tnemtaertgnirudecnotsaeltAtnemtaertgnirudeciwttsaeltAsraey4-2yrevEsraey2-1yrevETable. Frequency of comprehensive eye examinationrecommended by the American Academy ofOphthalmology according to age10≥

Chloroquine-induced bull's eye maculopathy

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Chloroquine-induced bull's eye maculopathy

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