From: Ocular sarcoidosis in adults and children: update on clinical manifestation and diagnosis
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 |