IWOS Criteria for the Diagnosis of Ocular Sarcoidosis (OS)

Sarcoidosis is a systemic inflammatory disorder with unknown aetiology, affecting many organs including the eye and characterised by non-caseating granulomas.

Revised International Workshop on Ocular Sarcoidosis (IWOS) criteria for the diagnosis of ocular sarcoidosis (OS)

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 angiotensin-converting enzyme (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.

 

 

References:

  1. Mochizuki M, Smith JR, Takase H, Kaburaki T, Acharya NR, Rao NA; International Workshop on Ocular Sarcoidosis Study Group. Revised criteria of International Workshop on Ocular Sarcoidosis (IWOS) for the diagnosis of ocular sarcoidosis. Br J Ophthalmol. 2019 Feb 23. [Medline]
  2. Yang SJ, Salek S, Rosenbaum JT. Ocular sarcoidosis: new diagnostic modalities and treatment. Curr Opin Pulm Med. 2017 Sep;23(5):458-467. [Medline]

Created Jul 25, 2019.