Acute Posterior Multifocal Placoid Epitheliopathy and Thyroiditis

Pigment

Harold N. Jacklin, MD \s=b\ A young woman developed the characteristic clinical and fluorescein angiographic findings of acute posterior multifocal placoid retinal pigment epitheliopathy while being treated for acute thyroiditis. Widespread involvement of the cornea, uveal tract, as well as the optic nerve and retina indicate a generalized ocular Inflammation in the clinical course of this patient. The relationship between the subacute thyroiditis and acute posterior multifocal placoid retinal pigment epitheliopathy may indicate an underlying viral cause of both disorders.

(Arch Ophthalmol 95:995-997, 1977)

In

1968, Gass described acute poste-

rior multifocal placoid pigment epitheliopathy.1 Three young women developed rapid bilateral visual loss due to multiple, placoid, flat, yellow-

white lesions believed to be located in the pigment epithelium. Fluorescein angiography demonstrated initial screening hypofluorescence in the involved areas, which later showed hyperfluorescence. In the later phase of the disease the placoid lesions left scars at the level of the pigment epithelium. With spontaneous resolution, visual acuity fortunately improved to near normal despite residual scattered areas of pigmentation. Subsequent cases of acute posterior multifocal placoid pigment epitheliopathy have been reported in both males and females of various ages. Iritis, episcleritis, vasculitis, and papillitis have been occasionally reported in conjunction with the choroidal and retinal lesions. The cause still remains undetermined in this syndrome. The association with various preceding Accepted for publication Nov 13, 1976. Dr Jacklin is in private practice in Greensboro, NC. Read in part before the Atlantic Section

meeting of the Association for Research in Vision and Ophthalmology, Durham, NC, Nov 15, 1975. Reprint requests to 1014 N Elm St, Greensboro, NC 27401 (Dr Jacklin).

viral infections, mycobacterial infections, and erythema nodosum and cerebral vasculitis has been reported.-'1" The use of oral contraceptives, hormones, or both, in several of the female patients in these cases has been noted. The following case describes acute posterior multifocal placoid pigment epitheliopathy in a young woman in association with thyroiditis. Papillitis, uveitis, and corneal infiltration were noted during the prolonged clinical course of this patient. REPORT OF A CASE A 20-year-old woman was seen in June 1975 with complaints of bilateral blurred vision of one month's duration. In February 1976, the patient began taking oral contraceptive tablets. In April the patient was seen by her internist for a sore throat and was found to have tenderness and enlargement of the thyroid gland due to acute thyroiditis. The patient was given .(Hi gm of thyroid and methylprednisolone. The patient quickly became asymptomatic, but after discontinuing the medication without advice, the condition recurred. Thyroid studies showed abnormally low values and the patient was started again on a regimen of systemic steroids and thyroid. In May she began noticing the onset of constant light sensation and blurring of vision in both eyes. When seen in June, results of an ophthalmologic examination disclosed a visual acuity of 20/200 in the right eye and 20/100 in the left with best correction. External ocular, motility, slit-lamp, and tonometry examination findings were within normal limits. Fundus examination showed sharply demarcated, gray, placoid circumscribed lesions in the posterior retina and choroid, particularly in the macular area (Fig 1 and 2). Scattered pigmentation was located adjacent to the margins of several of the areas. The vitreous was clear. Fluorescein angiography confirmed the presence of a widespread hypofluorescence in the early venous phase and late hyperfluorescence of both eyes (Fig 3 through 6). The patient was given prednisone again, 15 mg, four times a day for her eye condition, in

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conjunction with her previous thyroid and birth control tablets. In July, however, the patient began to note aching of both eyes

movement and further deterioration of vision. Examination showed severe elevation of the optic nerve of the left eye with blurred margins and venous congestion (Fig 7). The retinal placoid lesions were fading and additional lacey scattered pigmentation could be seen in the posterior retina. Visual acuity was finger counting at four feet in the right eye and 20/100 in the left eye. Visual fields showed enlargement of both blind spots. The birth control tablets were discontinued and the systemic prednisone tapered due to weight gain and

on

cushingoid facial changes. The patient was admitted in September for hospital evaluation of possible optic neuritis or papillitis. All medications had been discontinued except the thyroid. The patient began to note resolution of her eye pain on movement and an improvement in vision. Photophobia and irritation with redness was noted in the right eye suddenly in late October. Slit-lamp examination revealed ciliary injection, corneal edema, and limbal corneal thinning with early corneal neovascularization of the right eye (Fig 8). The anterior chamber was clear in the right eye, although one or two keratic precipitates could be seen on the corneal endothelium of the left eye. Topical steroids were applied frequently to the involved right eye. Within a week the

Fig 1.—Multifocal placoid lesions in retinal pigment epithelium and choroid (right eye).

Fig 2.—Multifocal placoid lesions in retinal pigment epithelium and choroid (left

Fig 3.—Fluorescein angiogram hypofluorescence (right eye).

Fig 5.—Fluorescein angiogram hyperfluorescence (right eye).

Fig 6.—Fluorescein angiogram hyperfluorescence (left eye).

eye).

with late

Fig 8—Corneal and limbal vascularization inflammation (right eye).

with

early

with late

Fig 9.—Multifocal chorioretinal scarring (right eye) one year later.

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Fig 4.—Fluorescein angiogram hypofluorescence (left eye).

Fig 7.—Optic

nerve

with

early

papillitis (left eye).

Fig 10.—Multifocal chorioretinal scarring

(left eye)

one

year later.

pain and redness cleared in the right eye. In June 1976, a year after the initial

¡nllammation, right eye and

vision was 20/200 in the 20/30 in the left. Corneal vascularization remained at the limbus in the right eye. Fundus examination showed multifocal choroidal scarring in both eyes. The optic discs were sharp with slight pallor. No thyroid enlargement or tenderness was present (Fig 9 and 10). The following laboratory determinations were performed: complete blood cell count; corrected crythrocyte sedimentation rate; white blood cell differential; automated chemical analyses; CSF analysis; toxoplasmosis; and histoplasmosis serum liters. All were normal. Thyroid function tests were abnormally low: T4/T„ 2.3 ug/100 ml; T:„ 235 ug/100 ml; T,/T, index, 0.54. Antithyroglobulin antibodies were negative. Indired lluoresccnt antithyroid microsomal antibodies were positive. The serum was then titered, using an indirect hemagglutination antithyroid microsomal antibody procedure at a dilution of 1:6,400. Chest roentgenogram, brain scan, and EEG were

normal.

COMMENT

The retinal pigment epithelium is thought to be the primary site of

disease in drusen, fundus flavimaculat-us, and acute retinal pigment epithelitis. Retinal pigment epithelium disease

secondarily

affects the

course

of

the disease in presumed histoplasmosis, senile macular degeneration, and

hereditary retinal dispharmacologie agents such as quinolines and pheno-

some of the

Various

eases.'71"

thiazines

are

also known to affect the

retinal pigment epithelium.'" The site of inflammation in acute multifocal placoid pigment epitheliopathy is speculative but felt to be in the retinal pigment epithelium or choriocapillaris.' ••'•'" Bird and Hamilton and Hayreh felt that the disease represented an occlusive disorder of the terminal choroidal arterioles with infarction and degeneration of the overlying retinal pigment epithelium." The presence of associated optic nerve inflammation, uveitis, keratitis, episcleritis, and retinal vasculitis may implicate a more widespread vascular inflammation within the eye. Holt and others have also described multifocal cerebral vasculitis in conjunction with the typical eye findings of acute multifocal retinal pigment epitheliopathy.'" Papillitis or optic neuritis has been described in four cases of acute multifocal placoid pigment epitheliopathy."" Blurring of the disc margins, hyperemia, edema, superficial hemorrhage, and distention with spontaneous venous pulsations were noted. Relative afferent pupillary defects were seen in two of the reported cases. Fluorescein angiography showed the deep plexus of vessels on the disc surface to be greatly dilated with fluorescein leakage. Hayreh has described optic disc vasculitis of undetermined cause occurring in young individuals as a result of involvement of the short ciliary vessels in the loose prelamallar '

region of the optic disc." The papillitis or optic disc vasculitis in acute multifocal pigment epitheliopathy may

indicate similar location of vascular involvement. Episcleritis and keratitis, as described in the present case, may also indicate involvement in the anterior segment blood vessel supply. To my knowledge, thyroiditis has not been previously reported in association with acute multifocal placoid retinal pigment epitheliopathy. Thyroiditis is an acute or subacute or chronic generalized self-limiting nonbacterial inflammation of the thyroid gland.-1 Complete recovery of normal thyroid function usually takes place during a period of weeks or months. The cause of this condition is not known, although the clinical features suggest a viral infection as in acute multifocal placoid pigment epitheliopathy.-' A mild transient increase in circulating antithyroid antibodies is common, as is demonstrated in this case. Treatment with aspirin, thyroid hormones, and cortical steroids is symptomatically effective but does not seem to alter the clinical or histologie progress of the disease. The reported case followed the clinical description and course of subacute thyroiditis. The relationship between the concurrent inflammation in the thyroid gland and acute multifocal

placoid retinal pigment epitheliopathy is uncertain but may indicate in both disorders.

a

viral

cause

References 1. Gass D: Acute posterior multifocal placoid pigment epitheliopathy. Arch Ophthalmol 80:177\x=req-\

185, 2.

1968.

Schlaegel

Essentials of Uveitis. Boston,

P:

Brown & Co, 1969, p 103. Little,Maumenee entities in uveitis: An A: 3.

Clinical

approach to the study of intraocular inflammation. Am Ophthalmol 69:1-27, 1970. J

4. Van Buskirk

E, Lessell S, Friedman E: Pigment epitheliopathy and erythema nodosum.

Arch Ophthalmol 85:369-372,

1971. 5. Bird A, Hamilton A: Placoid pigment epitheliopathy presenting with bilateral serous retinal detachment. Br Ophthalmol 56:881-886, J

1972. 6. Kirkman T, Ffytche T, Sanders M: Placoid

Pigment epitheliopathy with retinal vasculitis and papillitis. Br Ophthalmol 56:875-880, 1972. J

7. Deutman

A, Oosterhuis J, Boen-Tan T, et al:

Acute posterior multifocal placoid pigment

epitheliopathy or chorioepitheliopathy: Pigment J 1972.

capillaritis. Br Ophthalmol 56:863-874, 8. Ryan S, Maumenee A: Acute posterior multifocal placoid pigment epitheliopathy. Am J Ophthalmol 74:1066-1074, 1972.

9. Fitzpatrick R, Robertson D: Acute posterior multifocal placoid pigment epitheliopathy. Arch

89:373-376, 1973. Ophthalmol 10. Annesley W, Tomer T, Shields J: Multifocal

placoid pigment epitheliopathy.

Am Ophthalmol 76:511-518, 1973. 11. Brown M, Ebert A, Landas G: Pigment epitheliopathy in patient with mycobacterial infection. J Pediatr Ophthalmol 10:278-281, J

1973. 12. Fishman G, Rabb F, Kaplan J: Acute posterior multifocal placoid pigment epitheliopathy. Arch Ophthalmol 92:173-177, 1974. 13. Savino P, Weinbert R, Yassin J, et al: Diverse manifestations of acute posterior multifocal placoid pigment epitheliopathy. Am Ophthalmol 77:659-662, 1974. 14. Lewis R, Martonyi C: Acute posterior multifocal placoid pigment epitheliopathy: A J

recurrence.

15. Azar

Arch Ophthalmol 93:235-238, 1975. P, Gohd R, Waltman D, et al: Acute

posterior multifocal placoid pigment epitheliopathy associated with an adenovirus type V infection. Am J Ophthalmol 80:1003-1005, 1975. 16. Holt W, Regan C, Trempe C: Acute poste-

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rior multifocal placoid pigment epitheliopathy. Am Ophthalmol 81:403-411, 1976. 17. Marmor M: The retinal pigment epithelium Clin 15:131\x=req-\ and ocular disease. Int 146, 1975. 18. Krill A, Deutman A: Acute retinal pigment epithelitis. Am J Ophthalmol 74:193-205, 1972. 19. Friedman M: Bilateral recurrent acute retinal pigment epithelitis. Am Ophthalmol J

Ophthalmol

J

79:567-570, 1975.

20. Zinn K, Greenseid D: Toxocology of the retinal pigment epithelium. Int Ophthalmol Clin

15:147-158, 1975. 21. Hayreh S: Segmental

nature of the choroidal vasculature. Br J Ophthalmol 59:631-648, 1975. 22. Hayreh S: Optic disc vasculitis. Br J Ophthalmol 56:652-670, 1972. 23. Colcock B: Thyroiditis, in Sedgwick C (ed): Surgery of the Thyroid Gland. Philadelphia, WB Saunders Co, 1974, chap 9. 24. Greene J: Subacute thyroiditis. Am Med 51:97-108, 1971. J

Acute posterior multifocal placoid pigment epitheliopathy and thyroiditis.

Acute Posterior Multifocal Placoid Epitheliopathy and Thyroiditis Pigment Harold N. Jacklin, MD \s=b\ A young woman developed the characteristic cli...
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