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

Abstract

AimsInternational criteria for the diagnosis of ocular sarcoidosis (OS) was established by the first International Workshop on Ocular Sarcoidosis (IWOS) and validations studies revealed certain limitations of the criteria. To overcome the limitations, revised IWOS criteria was established in an international meeting. This manuscript was aimed at reporting the revised IWOS criteria.MethodsA consensus workshop was carried out to discuss and revise the IWOS criteria. The workshop was held on 27 April 2017, in Nusa Dua, Bali, Indonesia. Prior to the workshop, a questionnaire proposing revised criteria and consisting of one item for differential diagnosis, seven items for ocular clinical signs, 10 items for systemic investigations and three categories of diagnostic criteria was circulated to 30 uveitis specialists. Questionnaire items with over 75% support were taken as consensus agreement; items with below 50% support were taken as consensus disagreement and items with 50%–75% support were discussed at the workshop. Of the latter items, those supported by two-thirds majority in the workshop were taken as consensus agreement.ResultsThe survey and subsequent workshop reached consensus agreements of the revised criteria for the diagnosis of OS as follows: (1) other causes of granulomatous uveitis must be ruled out; (2) seven intraocular clinical signs suggestive of OS; (3) eight results of systemic investigations in suspected OS and (4) three categories of diagnostic criteria depending on biopsy results and combination of intraocular signs and results of systemic investigations.ConclusionsRevised IWOS criteria were proposed by a consensus workshop.

Ocular manifestations during sarcoidosis: about 13 cases. Experience of the Military Hospital Mohamed V of Rabat

“…The majority were women (77%). The median free interval between the diagnosis of sarcoidosis and the first ophthalmologic sign was six years [2][3][4][5][6][7][8][9][10]. The median follow-up of the patients was 36 months (between 7 and 130 months).…”

“…According to the data of the literature, the The 2017 Revised criteria of the International Workshop on Ocular Sarcoidosis (IWOS) for the diagnosis of ocular sarcoidosis [8] identifies seven clinical intraocular signs and five ancillary tests suggestive of ocular sarcoidosis. In our serie, two of the seven clinical signs were found frequently: mutton fat keratic precipitates and/or iris nodules (Koeppe/Bussaca) in 56% of the cases and bilateralil inflammation in 92% of cases).…”

Ocular Sarcoidosis

“…(2) seven intraocular clinical signs suggestive of ocular sarcoidosis; (3) eight laboratory tests in suspected ocular sarcoidosis; and (4) three categories of diagnostic criteria depending on biopsy results and combination of intraocular signs and results of systemic investigations (►Table 4). 99 The most substantial changes were the addition of four systemic investigations: (1) lymphopenia; (2) CD4 alveolar lymphocytosis; (3) parenchymal lung changes consistent with sarcoidosis; and (4) abnormal label uptake on 67Ga scintigraphy or 18-fluorodeoxyglucose positron-emission tomography (18-FDG PET).…”

“…101 However, measurement of the vitreous CD4 þ/CD8þ ratio is a challenging investigation and the majority of panel members involved in the revision of the IWOS criteria considered this test to be too invasive to be performed solely for the purpose of diagnosing ocular sarcoidosis. 99 Several recent reports have demonstrated the value of 18-FDG PET to detect occult sites of the disease. It may help determine an accessible site for a biopsy, particularly in difficult cases, such as cardiac or neurological involvements • Probable ocular sarcoidosis: diagnosis not supported by biopsy and bilateral hilar lymphadenopathy absent, but three intraocular signs and two systemic investigations selected from two to eight are present and has become the preferred nuclear imaging technique to assess sarcoidosis activity.…”

“…159 In the literature before 2000, 57 cases had been published, of which 35 involved the optic nerve itself, 9 were compressions due to intracerebral lesions, 6 were due to hydrocephalus, and 6 were due to the uveitis complications. 158 Involvement of the optic nerve in sarcoidosis may arise through a variety of mechanisms: inflammation of the nerve itself, [160][161][162][163] compression or infiltration by an inflammatory mass adjacent to the anterior visual pathway (e.g., the orbital apex or pituitary gland), 163,164 a secondary involvement through ischemic complications of retinal and choroidal inflammation and glaucoma, 7, 66,70,71,99,165 granulomas within the optic disc, 161,164 meningeal inflammation within the optic nerve sheath leading to an optic perineuritis, 166 and finally as a consequence of hydrocephalus. 167 The disorder is more common in women and proportionately in the African and Caribbean ethnic groups.…”