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

Journal of Ophthalmic Inflammation and Infection

Table 2 The criteria for sarcoidosis-associated uveitis by Standardization of Uveitis Nomenclature (SUN) Working Group³³, published in 2021

1. Compatible uveitic picture, either
a. Anterior uveitis OR
b. Intermediate or anterior/intermediate uveitis OR
c. Posterior uveitis with either choroiditis (paucifocal choroidal nodule(s) or multifocal choroiditis) OR
d. Panuveitis with choroiditis or retinal vascular sheathing or retinal vascular occlusion
AND
2. Evidence of sarcoidosis, either
a. Tissue biopsy demonstrating non-caseating granulomata OR
b. Bilateral hilar adenopathy on chest imaging
Exclusions
1. Positive serology for syphilis using a treponemal test
2. Evidence of infection with Mycobacterium tuberculosis^a either
a. Histologically- or microbiologically-confirmed infection with M. tuberculosis^bOR
b. Positive interferon-Ɣ release assay (IGRA)^c OR
c. Positive tuberculin skin test^d

Reprinted from Am J Ophthalmol. 228, Standardization of Uveitis Nomenclature Working G. Classification Criteria for Sarcoidosis-Associated Uveitis. p: 220–30., Copyright 2021, with permission from Elsevier
^aRoutine exclusion of tuberculosis is not required in areas where tuberculosis is non-endemic but should be performed in areas where tuberculosis is endemic or in tuberculosis-exposed patients. With evidence of latent tuberculosis in a patient with a uveitic syndrome compatible with either sarcoidosis or tubercular uveitis and bilateral hilar adenopathy, the classification as sarcoid uveitis can be made only with biopsy confirmation of sarcoidosis (and therefore exclusion of tuberculosis)
^bE.g. biopsy, fluorochrome stain, culture, or polymerase chain reaction based assay
^cE.g. Quantiferon-gold or T-spot
^dE.g. Purified protein derivative (PPD) skin test; a positive result should be > 10 mm induration