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


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

In This Article

Definition of Eye Disease

The definition of ocular disease depends on the patient's presentation. As shown in Table 1, certain criteria have been proposed to support the diagnosis of definite or probable ocular sarcoidosis in a patient with known sarcoidosis.[5]

On the other hand, the patient may present with ocular findings compatible with sarcoidosis but no symptoms from extraocular disease. In this situation, several groups have tried to identify features that support ocular sarcoidosis, including roentgenographic findings on high-resolution computed tomography (HRCT) or cellular elements in the bronchoalveolar lavage (BAL).[6] However, the gold standard for definitive diagnosis remains biopsy confirmation.[7] Figure 1 summarizes the findings of a workshop charged with establishing criteria for ocular sarcoidosis.[8] As can be seen only those patients with a positive biopsy were classified with definite sarcoidosis. The authors also point out that other causes of granulomatous disease, including tuberculosis, must be excluded.

Figure 1.

Proposed criteria for the level of certainty for the diagnosis of ocular sarcoidosis.[8] The criteria were proposed for those with known systemic disease and possible ocular disease, as well as for those who present with only ocular disease and possible sarcoidosis. Other known causes of granulomatous disease, such as tuberculosis, must also be ruled out.

The figure also defines three other subgroups of patients: presumed, probable, and possible. These criteria were developed for patients with either ocular disease only or ocular disease plus known systemic disease. Table 2 lists the features defined by this group as supportive of ocular sarcoidosis. These include ocular findings as well as systemic findings. Not all of the ocular findings are specific. For example, shaped peripheral anterior synechiae can be due to multiple causes and are not that specific for sarcoidosis. On the other hand, iris nodules are highly suggestive of sarcoidosis. Major defining features include the presence or absence of bilateral hilar adenopathy and the performance of a biopsy. Patients with a biopsy that is negative for granulomas can still have possible ocular sarcoidosis. Both probable and possible sarcoidosis rely on the presence of multiple ocular and clinical features. This approach has been supported by one retrospective case-controlled study of Japanese patients.[9]