Robert P. Baughman, M.D.; Elyse E. Lower, M.D., Ph.D.; Adam H. Kaufman, M.D.

Disclosures

Semin Respir Crit Care Med. 2010;31(4):452-462. 

In This Article

Ocular Manifestations of Sarcoidosis

As listed in Table 3, ocular involvement in sarcoidosis encompasses a wide range of clinical manifestations[1] and several organ systems, including the eye itself, the vasculature of the eye, the optic nerve, the extraocular muscles, the bones that comprise the orbit itself, lacrimal glands, and the skin surrounding the eyes. Because involvement of any of these areas can affect the vision and/or operation of the eyes, the general definition of ocular disease encompasses findings related to any of these structures.

Uveitis

Uveitis is the most common manifestation of ocular sarcoidosis.[10] The uvea is divided into three areas based on the source of blood flow.[22] The anterior and posterior uvea possess different blood supplies with the intermediate area representing a watershed between the two areas. As listed in Table 4, the signs and symptoms vary among the three parts of the uvea.

Anterior uveitis occurs in 20 to 70% of patients,[2,22,23] and the typical presentation is an acute iritis or iridocyclitis.[24] Although many patients experience severe pain and photophobia, over a third of patients may have no ocular symptoms.[14] Therefore, it is recommended that all patients with sarcoidosis undergo annual ophthalmologic examination regardless of symptoms.[7] Among the physical findings of acute anterior uveitis are the mutton fat or granulomatous keratic precipitates, which are highly suggestive of sarcoidosis.[8]

Chronic anterior uveitis is associated with iris nodules.[1] Although Busacca nodules are true granulomatous lesions found on the iris, a Koeppe nodule is a granulomatous lesion found only on the pupillary margin. It may become the nidus for posterior synechiae. Chronic anterior uveitis can lead to band keratopathy, glaucoma, and cataract formation. Because corticosteroids can also cause cataracts and glaucoma, the clinician may not be able to determine whether the disease or the treatment caused the problem initially. However, corticosteroids can worsen both conditions.

Intermediate uveitis, a common manifestation of chronic ocular sarcoidosis, involves inflammation occurring in the vitreous, pars plana, or peripheral retina.[1,25] Diagnosis requires a detailed eye examination of the periphery of the retina. However, the ophthalmologist may be rewarded for the effort with the finding of a string of pearls or snowballs. Because intermediate uveitis can also be found in multiple sclerosis or be idiopathic,[22,25] this finding is only suggestive of sarcoidosis.[8]

Some series report posterior uveitis in over 20% of ocular sarcoidosis cases.[24,26] The most common manifestation is periphlebitis associated with segmental cuffing, sheathing, and perivenous infiltrates, referred to as candlewax drippings.[27] Although these findings are highly suggestive of ocular sarcoidosis, these lesions may be subclinical and only identified with fluorescein angiography.[27] Capillary disease can lead to ischemia and subsequent neovascularization and vitreous hemorrhage.[28,29] Choroidal granulomas can also be observed, and with resolution, they can lead to areas of hypopigmentation or scarring.

Cystoid macular edema can be caused by chronic inflammation of anterior, intermediate, or posterior uveitis.[27] This form of the disease may be particularly refractory to conventional antiinflammatory therapy,[30] and it can play a decisive role in visual outcome of patients with chronic uveitis.[31]

Optic Neuropathy

Optic neuropathy is a relatively rare complication of sarcoidosis, it may lead to rapid loss of vision.[1,32] It involves only one of the many cranial nerves that can be affected by sarcoidosis.[33,34] Optic nerve involvement can be associated with papillitis, papilledema, and even granulomas on the head of the optic head.[32] As depicted in Fig. 4, paleness of the optic disk is the most common finding. Optic atrophy may occur if the patient does not respond to therapy. Optic neuropathy requires systemic therapy, and patients often require steroid-sparing alternatives. The anti-tumor necrosis factor (TNF) agent infliximab has been successfully prescribed for the treatment of some cases of refractory disease.[35,36] However, infliximab has also been associated with subsequent development of optic neuritis.[37,38]

Figure 4.

Optic nerve of the left eye (OS) compared with normal appearance of the right eye (OD).

Orbital Disease

Ocular sarcoidosis can also affect the adnexa and the orbit.[15] Patients often experience multiple affected areas (Fig. 3) with the disease more commonly reported in older patients.[15] In one study, adnexal disease was more common in African American women.[10]

Orbital disease can lead to eye entrapment and associated diplopia. Although lacrimal disease is common,[10] some patients may develop a mass without lacrimal involvement.[39] Figure 5 demonstrates an orbital mass medial to the optic nerve. In this patient, the mass led to nasolacrimal duct obstruction and associated dacryocystitis. Although a granulomatous mass is one possible cause of dacrocystitis,[40] most cases of dacryocystitis are due to nongranulomatous processes. In two large studies of over 500 patients designed to evaluate the role of biopsy for dacryocystitis, less than 1% of patients were diagnosed with sarcoidosis.[41,42] In our experience, sarcoidosis patients with dacryocystitis usually have evidence for other organ involvement, including skin, sinus, and chest disease. We usually aggressively treat these patients for sinus sarcoidosis as part of their treatment for dacrocystitis.[43]

Figure 5.

The mass on the medial aspect of the left eye (circled) was biopsied and found to be noncaseating granulomas. The patient had sinus symptoms including recurrent infections and bleeding. She also experienced an associated dacryocystitis.

The lacrimal gland is the most common adnexal area of involvement.[15] Lacrimal gland disease can appear prior to other manifestations of the disease,[44] and it may lead to subsequent sicca syndrome.

Miscellaneous Conditions

Miscellaneous conditions include scleritis, glaucoma, and cataracts. Glaucoma and cataracts are usually associated with uveitis. As noted, they can also be a complication of the disease treatment with corticosteroids.

Scleritis has been reported in less than 3% of ocular sarcoidosis patients.[23] In contrast, it is a more common manifestation of Wegener granulomatosis.[45] Although scleral involvement is often an asymptomatic nodule, the presence of a scleral nodule can lead to a biopsy, which may confirm the diagnosis of sarcoidosis.[22] Scleral and conjunctival nodules are more readily appreciated by the use of a confocal microscope.[46]

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