Mycophenolate mofetil combined with systemic corticosteroids prevents progression to chronic recurrent inflammation and development of ‘sunset glow fundus’ in initial‐onset acute uveitis associated with Vogt–Koyanagi–Harada disease

Abstract

Use of MMF as first-line therapy combined with systemic corticosteroids in patients with initial-onset acute VKH disease prevents progression to chronic recurrent granulomatous inflammation and development of 'sunset glow fundus'.

Initial-onset acute and chronic recurrent stages are two distinctive courses of Vogt-Koyanagi-Harada disease

“…However, this classification did not consider time from the onset of symptoms, and specific findings observed at certain time point from the symptoms presentation and outcomes related to the stage of VKH disease were not introduced [ 13 , 16 ]. Moreover, as an attempt to introduce a categorization of VKH patients in clinical studies, different intervals of time have been used [ 5 , 14 , 17 – 20 ]. Another major shortcoming of these revised criteria is the fact that highly sensitive diagnostic modalities, such as indocyanine green angiography (ICGA) [ 21 ], measurement of choroidal thickness using enhanced depth imaging optical coherence tomography (EDI-OCT) [ 22 ] or swept source optical coherence tomography (SS-OCT) [ 23 ], and high-frequency ultrasound biomicroscopy (UBM) for in vivo visualization of the anterior segment [ 24 ], especially crucial for early identification of the inflammatory process, diagnosis of atypical cases and follow up of the disease, are missing.…”

“…However, up till now there has been no agreement neither in the terminology nor in the development of clear and explicit definitions for the different VKH courses described here. In this regard, patients with an early course of the condition at diagnosis have been classified as having an early [ 15 ], a new onset-acute [ 19 ], an acute-resolved [ 31 ], an acute VKH disease [ 18 ] or an initial-onset acute VKH disease [ 8 , 17 ]. On the other hand, subjects with late presentation have been referred to as subacute [ 2 , 34 ], chronic [ 17 , 26 , 35 ] or late [ 7 , 15 , 16 ].…”

“…This distinction was based in Chee et al findings, who observed that an early treatment (within 2 weeks from onset of symptoms) of the disease with high doses of corticosteroids, led more frequently to complete resolution and fewer complications than the administration of low or even high but late (between 2 and 4 weeks from onset of symptoms) doses of oral corticosteroids [ 36 ]. Others authors have considered a longer interval of time between symptoms onset and diagnosis: 1 month [ 14 , 17 ], 2 months [ 19 ] and 3 months [ 25 ]. Da Silva et al classified VKH disease as early when symptoms of the disease were present for less than 4 weeks.…”

“…This classification was in agreement with their findings of classic uveitic phase manifestations only in the early VKH group [ 14 ]. Abu el Asrar et al [ 17 ] considered those cases of VKH presenting with granulomatous choroiditis, exudative retinal detachment, and disc hyperaemia and swelling, as initial-onset acute disease, in accordance to the findings of Yang et al [ 5 ] They observed that those patients who fulfilled this category presented to their consultation in a range of time from the onset of symptoms between 1 to 30 days [ 17 ]. Nakayama et al excluded from their study those patients presenting with sunset glow fundus, or those who presented to their consultation with more than 2 months from the onset of VKH disease symptoms [ 19 ].…”

“…Recently, Abu El-Asrar analyzed their VKH disease cases. As previously reported, all patients were treated with mycophenolate mofetil in addition to systemic corticosteroids as first line therapy [ 17 ]. Patients were divided patients into three groups based on the clinical pattern at presentation: patients with initial-onset acute uveitis without AS inflammation, patients with initial-onset acute uveitis with AS inflammation and those with chronic recurrent uveitis.…”