A Ocular Sarcoidosis

A ny structure of the eye may be involved in sarcoido- sis. The frequency of ophthalmic manifestations varies, depending on the selection of the material. In four, large histologically confirmed studies in the United States and Europe totaling 1440 sarcoidosis patients, the fre- quency of ocular involvement averaged 35%, with the range from 28 to 50%.

The low incidence of ocular sar- coidosis in some series may have resulted from the inclu- sion of only patients with symptomatic lesions, because ocular sarcoidosis may remain asymptomatic and only be discovered by routine slit-lamp examination.

Geographic and racial differences in the incidence of ocular sarcoidosis have been reported. In blacks, the eye involvement is more frequent and serious than in whites.

COMMON MANIFESTATIONS

Similar to the skin, granulomas on the eye can readily be recognized and satisfactory monitored.

Uveitis is the most common eye lesion in sarcoidosis (14–33%)

and is represented by acute/subacute uveitis, chronic uveitis, and posterior uveitis (choroidoretinitis).

Conjunctival involvement is the second most common ocular finding in ocular sarcoidosis. Phlyctenular or non- specific conjunctivitis occurs in (3–25%).

Keratocon- junctivitis sicca is seen in approximately 5% of all sarcoidosis patients.

Asymptomatic lacrimal dysfunc- tion producing dry eye is frequent, as Schremer’s test is positive in these patients.

Acute/subacute uveitis–iridocyclitis occurs suddenly, with watering and redness of eyes, cloudy vision, and photophobia. These patients may have other manifesta- tions of early sarcoidosis including erythema nodosum,

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CHAPTER 9

Ocular Sarcoidosis

FIGURE 9.1 Subacute uveitis. Note the circumcorneal ciliary congestion, the irregular pupils, and the characteristic “mutton fat”(candle spots), keratic precipitates in the anterior chamber.

(Courtesy of Dr. Anka Stanojevic-Paovic, Professor of Ophthal- mology, Clinical Center, Belgrade, Serbia.)

FIGURE 9.2 Acute nodular iritis.

62 Atlas of Sarcoidosis

and bilateral hilar adenopathy. Acute iridocyclitis tends to clear spontaneously. The prognosis is good.

Chronic sarcoid uveitis is rare in Caucasians, as are years long active retinal or choroidal granulomas. The repeating episodes of chronic uveal inflammation and treatment with corticosteroids lead to serious complica- tions, such as cataract, glaucoma, and loss of vision in approximately 25% of patients.

DIAGNOSIS ^3,4

Typical eye lesions in ocular sarcoidosis include granulo- matous uveitis, conjunctival follicles, and lacrimal gland

enlargement. They are associated with bilateral hilar adenopathy, erythema nodosum, peripheral lym- phadenopathy, and depression of delayed-type hypersen- sitivity. In the absence of clinical features of sarcoidosis, conjunctival and lacrimal biopsies are required for a positive diagnosis. Angiotensin converting enzyme (ACE) activity in tears supports the diagnosis.

REFERENCES

1. Angi M, De Caro G, Bergamo L, et al. low prevalence of uveitis in Italian sarcoidosis patients. Sarcoidosis 1991;8:

181–182.

2. James D, Angi M. Ocular sarcoidosis. In: James D, ed.

Sarcoidosis and Other Granulomatous Disorders, vol 73.

New York: Marcel Dekker, 1994:275–284.

3. Karma A. Ocular sarcoidosis. Sem Resp Med 1992;13:

425–431.

4. Chumbley L, Kearns T. Retinopathy of sarcoidosis. Am J Opthal 1972;73:123–131.

5. Nichols C, Eagle R, Yanoff M, et al. Conjunctival biopsy as an aid in the evaluation of the patient with suspected sar- coidosis. Ophthalmology 1980;87:287–291.

6. Mihailovic-Vucinic V. Sarkoidoza. Infohome, Belgrad, Yugoslavia, 2000.

7. Khan F, Wessley Z, Chazin S, et al. Conjunctival biopsy in sarcoidosis. Ann Ophthalmol 1977;9:671–676.

FIGURE 9.3 A nodular lesion in the fornix of the left eye. FIGURE 9.4 Noncaseating granuloma of the eye. The biopsy sample taken from the granulomatous tissue of the lid resulted in a diagnosis of long-lasting sarcoid granuloma with chronic sarcoidosis.

FIGURE 9.5 Biopsy sample representing old granuloma with

hyalinisation.