Sarcoid Uveitis: An Intriguing Challenger

Abstract

The purpose of our work is to describe the actual knowledge concerning etiopathogenesis, clinical manifestations, diagnostic procedures, complications and therapy of ocular sarcoidosis (OS). The study is based on a recent literature review and on the experience of our tertiary referral center. Data were retrospectively analyzed from the electronic medical records of 235 patients (461 eyes) suffering from a biopsy-proven ocular sarcoidosis. Middle-aged females presenting bilateral ocular involvement are mainly affected; eye involvement at onset is present in one-third of subjects. Uveitis subtype presentation ranges widely among different studies: panuveitis and multiple chorioretinal granulomas, retinal segmental vasculitis, intermediate uveitis and vitreitis, anterior uveitis with granulomatous mutton-fat keratic precipitates, iris nodules, and synechiae are the main ocular features. The most important complications are cataract, glaucoma, cystoid macular edema (CME), and epiretinal membrane. Therapy is based on the disease localization and the severity of systemic or ocular involvement. Local, intravitreal, or systemic steroids are the mainstay of treatment; refractory or partially responsive disease has to be treated with conventional and biologic immunosuppressants. In conclusion, we summarize the current knowledge and assessment of ophthalmological inflammatory manifestations (mainly uveitis) of OS, which permit an early diagnostic assay and a prompt treatment.

Insights into the diagnosis and management of sarcoid uveitis: A review

“…42,43 Posterior uveitis can be associated with neurosarcoidosis. 34 Uveitis is traditionally thought to be granulomatous, however, this pattern occurs in only about half of all cases. 44 The clinical features of sarcoid uveitis are shown in Table 3.…”

“…Retinal artery macroaneurysms 5. Occlusive retinal vasculitis is uncommon 34 6. Optic nerve granulomas (Figure 3C) and solitary choroidal granulomas (Figure 3D) are rare but highly suggestive of sarcoidosis 50 7.…”

“…iCNVM are often located near or at the edge of choroidal granulomas. 34,77 They may be difficult to detect on fluorescein angiography (FA) due to similar leakage patterns of both iCNMV and choroidal lesions: early hypofluorescence with lake leakage. Additionally, extensive retinochoroidal involvement in posterior uveitis can further make the detection of iCNVM challenging by FA.…”

“…Periphlebitis is usually located at the equator and periphery. 34 The presence of nodular or segmental periphlebitis, and/or macroaneurysms is highly suggestive of sarcoidosis. 64 Perifoveal capillary arcade disruption, perifoveal areas of capillary hypoperfusion/non-perfusion and perifoveal capillary abnormalities (capillary dilation, microaneurysms, shunting vessels) can all be observed on FA in ocular sarcoid and these changes have been shown to negatively correlate with best corrected visual acuity (BCVA).…”

Characteristics of Cellular Infiltration into Posterior Vitreous in Eyes with Uveitis on the Classification Basis Assessed Using Optical Coherence Tomography

“…Granulomatous inflammation is a well-defined chronic or subacute inflammatory process, in which the activated macrophages play the main role and transform into epithelial cells. 6 In contrast, Behçet’s disease was a representative disease of nongranulomatous uveitis. The main infiltrating cells in nongranulomatous inflammation are all kinds of leucocytes: polymorphonuclear neutrophils, eosinophils, basophils, lymphocytes, and macrophages.…”