Table 1

Items of a question-based survey and results of survey responses of the sixth IWOS

ItemsAgreeDisagree%Agreement
I. Other causes of granulomatous uveitis must be ruled out24483
II. Intraocular clinical signs suggestive of OS
 1. Mutton-fat keratic precipitates and/or iris nodules (Koeppe or Busacca)300100
 2. Trabecular meshwork nodules and/or tent-shaped peripheral anterior synechia28293
 3. Snowballs/string of pearls vitreous opacities28293
 4.Multiple chorioretinal peripheral lesions (active and atrophic)29197
 5. Periphlebitis and/or macroaneurysm in an inflamed eye27390
 6. Opticdisc nodule(s)/granuloma(s) and/or choroidal nodule29197
 7. Bilaterality (clinically or by imaging)20871
III. Systemic investigations in suspected OS
 1. BHL by chest X-ray or chest computed CT scan300100
 2. Negative tuberculin test or IGRA27393
 3. Elevated serum ACE28293
 4. Elevated serum lysozyme23777
 5. Elevated serum soluble IL-2 receptor20969
 6. Elevated serum calcium191163
 7. Elevated serum gamma-globulin141647
 8. Elevated serum KL-6141548
 9. Elevated CD4/CD8 ratio (>3.5) in BAL fluid26487
 10. Abnormal 67-gallium scintigraphy or 18F-FDG PET27390
IV. Diagnostic criteria
 Definite OS: diagnosis supported by biopsy with compatible uveitis300100
 Presumed OS: diagnosis not supported by biopsy, but BHL present with two intraocular signs24680
 Probable OS: diagnosis not supported by biopsy and BHL absent, but three intraocular signs and two systemic investigations selected from two to eight are present201067
  • BAL, bronchoalveolar lavage; BHL, bilateral hilar lymphadenopathy; CD, cluster of differentiation; FDG PET, fluorodeoxyglucose positron emission tomography; IGRA, interferon-gamma releasing assays; IL, interleukin; IWOS, International Workshop on Ocular Sarcoidosis; KL, Krebs von den Lungen; OS, ocular sarcoidosis.