Ocular Sarcoidosis

Abstract

Sarcoidosis is one of the leading causes of inflammatory eye disease. Any part of the eye and its adnexal tissues can be involved. Uveitis and optic neuropathy are the main manifestations, which may require systemic treatment. Two groups of patients with sarcoid uveitis can be distinguished: one of either sex and any ethnicity in which ophthalmological findings are various and another group of elderly Caucasian women with mostly chronic posterior uveitis. Clinically isolated uveitis revealing sarcoidosis remains a strictly ocular condition in a large majority of cases. Although it can be a serious condition involving functional prognosis, early recognition in addition to a growing therapeutic arsenal (including intravitreal implant) has improved the visual prognosis of the disease in recent years. Systemic corticosteroids are indicated when uveitis does not respond to topical corticosteroids or when there is bilateral posterior involvement, especially macular edema. In up to 30% of the cases that require an unacceptable dosage of corticosteroids to maintain remission, additional immunosuppression is used, especially methotrexate. As with other forms of severe noninfectious uveitis, monoclonal antibodies against tumor necrosis factor-α have been used. However, only very rarely does sarcoid uveitis fail to respond to combined corticosteroids and methotrexate therapy, a situation that should suggest either poor adherence or another granulomatous disease. Optic neuropathy often affects women of African and Caribbean origins. Some authors recommend that patients should be treated with high-dose of corticosteroids and concurrent immunosuppression from the onset of this manifestation, which is associated with a poorer outcome.

Sarcoidosis: A Clinical Overview from Symptoms to Diagnosis

“…Heerfordt's syndrome can be rarely associated with cranial nerve involvement, particularly affecting the trigeminal nerve [96] along with visceral involvement [97]. Sarcoid uveitis is associated with a favorable visual outcome, since most patients experience mild or no visual impairment [98,99]. However, 2.4 to 10% of patients with sarcoid uveitis develop severe visual impairment (defined as best-corrected visual acuity <20/200 in at least one eye) [90,92,98,100] with cystoid macular edema being the main cause of visual loss [98,101,102].…”

“…Agrawal et al recently performed a systematic review and meta-analysis which yielded, for sarcoidosis, a pooled overall diagnostic yield of 93% for EBUS-intranodal forceps biopsy vs. 58% for EBUS-transbronchial needle biopsy (p < 0.00001) [202]. Figure 8 provides an algorithm based on recent ATS guidelines for the diagnosis of sarcoidosis and that incorporates the value of minor salivary gland biopsy (MSGB) and 18 F-FDG PET in sarcoidosis diagnosis [27,36,99]. Two studies in uveitis pa-tients showed that granulomas were only found on MSGBs in patients with elevated ACE or with a compatible chest CT [203,204].…”

Extrapulmonary sarcoidosis with a focus on cardiac, nervous system, and ocular involvement

“…Ocular sarcoidosis (OS) is a common extrapulmonary manifestation. The incidence of ocular involvement is high (12–89%) among sarcoidosis patients [ [56] , [57] , [58] ]. If left untreated, OS can lead to irreversible damage, including partial or complete loss of vision [59] .…”

“…Symptoms of OS are the presenting complaints in (20–40%) of sarcoidosis patients [59] . The incidence and presentation vary across age, gender, ethnicity and geographic location [ 56 , 58 , 59 ]. The highest incidence rates are in women, blacks, and Japanese [ 56 – 58 ].…”

“…The incidence and presentation vary across age, gender, ethnicity and geographic location [ 56 , 58 , 59 ]. The highest incidence rates are in women, blacks, and Japanese [ 56 – 58 ]. The US-based ACCESS study cited ocular involvement in 12% of 736 cases of sarcoidosis, while ocular involvement was documented in (51–68%) of sarcoidosis cases in Japan [ 56 , 57 ].…”