Table 1.

Revised International Workshop on ocular sarcoidosis (IWOS) criteria for the diagnosis of ocular sarcoidosis (OS)⁷, published in 2019

I. Other causes of granulomatous uveitis must be ruled out

II. Intraocular clinical signs suggestive of OS

1. Mutton-fat keratic precipitates (large and small) and/or iris nodules at pupillary margin (Koeppe) or in stroma (Busacca)

2. Trabecular meshwork nodules and/or tent-shaped peripheral anterior synechia

3. Snowballs/string of pearls vitreous opacities

4. Multiple chorioretinal peripheral lesions (active and atrophic)

5. Nodular and/or segmental periphlebitis (± candle wax drippings) and/or macroaneurysm in an inflamed eye

6. Optic disc nodule(s)/granuloma(s) and/or solitary choroidal nodule

7. Bilaterality (assessed by ophthalmological examination including ocular imaging showing subclinical inflammation)

III. Systemic investigation results in suspected OS

1. Bilateral hilar lymphadenopathy (BHL) by chest X-ray and/or chest computed CT scan

2. Negative tuberculin test or interferon-gamma releasing assays

3. Elevated serum ACE

4. Elevated serum lysozyme

5. Elevated CD4/CD8 ratio (> 3.5) in bronchoalveolar lavage fluid

6. Abnormal accumulation of gallium-67 scintigraphy or 18F-fluorodeoxyglucose positron emission tomography imaging

7. Lymphopenia

8. Parenchymal lung changes consistent with sarcoidosis, as determined by pulmonologists or radiologists

IV. Diagnostic criteria

Definite OS: diagnosis supported by biopsy with compatible uveitis

Presumed OS: diagnosis not supported by biopsy, but BHL present with two intraocular signs Probable OS: diagnosis not supported by biopsy and BHL absent,but three intraocular signs and two systemic investigations selected from two to eight are present

Reproduced from Br J Ophthalmol., Mochizuki M, et al., 103(10):1418–22, copyright 2019 with permission from BMJ Publishing Group Ltd