To describe the long-term outcomes of patients with limited Vogt-Koyanagi Harada syndrome (VKH), characterized by steroid-responsive serous retinal detachments without other signs of intraocular inflammation.
Retrospective case series.
Consecutive patients from the Royal Victorian Eye and Ear Hospital with acute bilateral serous retinal detachments without anterior chamber inflammation, with no previous ocular history or trauma, and with negative screening for vasculitis and other systemic autoimmune disease were included. Serous retinal detachment was confirmed on optical coherence tomography (OCT) and fundus fluorescein angiography. Visual acuity, treatment regimen, complications, and duration of follow-up were recorded.
Nine patients (6 male, median age 29 years [interquartile range (IQR) 27–36 years]) from January 2010 through May 2014 were studied. Median presenting logMAR visual acuity (VA) was 0.48 (IQR −0.1 to 3.0). Six patients were initially commenced on intravenous methylprednisolone for 3 days, followed by oral prednisolone. All received a course of tapering oral prednisolone (1 mg/kg prior to taper). Median duration of treatment was 9 weeks (IQR 8–14 weeks). Median time to complete resolution of subretinal fluid on OCT was 3.7 weeks (range, 2–12 weeks), with a corresponding improvement in VA (median logMAR 0.00; range, −0.08 to 0.04). There was no recurrence after a mean follow-up of 145.2 weeks (95% confidence interval 72.5–217.9).
In contrast to typical VKH, patients with limited VKH in our series appear to have good outcomes with systemic corticosteroid treatment. They have a marked improvement of VA and no episodes of relapse after cessation of corticosteroid treatment.
Vogt-Koyanagi-Harada syndrome (VKH) is a multisystem autoimmune disorder characterized by bilateral granulomatous uveitis with exudative retinal detachments that is often associated with neurologic and cutaneous manifestations. Despite being a single disease entity, patients do not always manifest all possible signs of VKH and have very different clinical manifestations depending on the stage at which they present. The revised diagnostic criteria reflect this understanding, subdividing VKH into either complete, incomplete, or probable groups. In contrast to “complete” and “incomplete” VKH, “probable” VKH refers to patients with isolated ocular disease with no other systemic involvement, previously described as Harada disease. This is in reference to Harada’s initial description of a posterior uveitis with exudative retinal detachment, although cerebrospinal fluid pleocytosis was also evident.
The prognosis for VKH is variable, dependent on early recognition and treatment, as well as median duration of disease. Complications include cataract, glaucoma, choroidal neovascular membranes, and subretinal fibrosis, with visual acuity at last follow-up ranging from worse than logMAR 1.0 to logMAR 0.3 or better. However, not all current knowledge on its prognosis is based on the revised diagnostic criteria established in 2001, and to date no studies have described the clinical outcomes for each separate subgroup of VKH (“complete,” “incomplete,” and “probable”).
In this study, using the revised diagnostic criteria for VKH, we analyzed data from 9 consecutive patients with probable VKH syndrome, characterized by serous retinal detachments without other signs of intraocular inflammation, and describe their response to treatment and long-term outcomes.
This was an observational case series that adhered to the tenets of the Declaration of Helsinki and was conducted with the approval of the Royal Victoria Eye and Ear Hospital Human Research and Ethics Committee.
All patients with probable VKH disease who were seen by the Ocular Immunology Clinic at the Royal Victorian Eye and Ear Hospital, Melbourne, Australia, between January 1, 2010 and May 31, 2014 were included in this study. Criteria from the first international workshop on Vogt-Koyanagi-Harada disease were used to establish the diagnosis of VKH disease. This included the following: (1) no history of penetrating ocular trauma or surgery preceding the initial onset of uveitis; (2) no clinical or laboratory evidence suggestive of other ocular disease entities; (3) bilateral ocular involvement (focal areas of subretinal fluid, or bullous serous retinal detachments). Patients who had signs of anterior uveitis were excluded from this study.
Other possible causes for the patient’s uveitis were excluded on investigations performed at the clinicians’ discretion (all of whom were subspecialists in ocular inflammatory disease). At a minimum, these included a chest radiograph examination, treponemal serology, quantiferon-gold, and angiotensin-converting enzyme levels to rule out other systemic diseases and infectious causes of uveitis.
Alternative diagnoses such as posterior scleritis or uveal effusion syndrome were ruled out on clinical grounds. Posterior scleritis was excluded through the absence of localized ocular pain, lack of scleral injection, and the absence of a T sign on B-scan ultrasound. Our cohort also did not have features of uveal effusion syndrome, which is characterized by choroidal effusions, shifting subretinal fluid, and a lack of response to corticosteroids—none of which were seen in our patients.
Patients were identified from a database that was established in 2010. Information on all patients who had been evaluated and treated for probable VKH disease was collected retrospectively from clinical charts. Clinical information at each visit included patient demographics, all current medications, visual acuity, slit-lamp examination, the grade of inflammation in the anterior chamber and in the vitreous, and the presence of structural complications such as cystoid macular edema (CME). The results of diagnostic testing, including optical coherence tomography (OCT), were also collected. Data on the use of corticosteroids, which included the dose and route of administration, and the use of other immunosuppressive drugs were also collected at each visit. In addition, patients who had not been reviewed in clinic for over 1 year were contacted via mail or phone to ask about ocular symptoms and whether they had continued other follow-up with other medical professionals, such as their general practitioner (GP) and/or private ophthalmologists. With patients’ consent, additional data were collected from their GP and private ophthalmologists. Data were entered into a password-protected standardized Microsoft Excel data entry form.
Main Outcome Measures
The development of vision loss and of structural ocular complications was assessed in each patient at each visit. Vision outcomes included loss of visual acuity to logMAR 0.4 or worse, and to logMAR 1.0 or worse. Visual acuity was measured at each visit with a Snellen visual acuity chart. Refractions were performed when deemed necessary by the examining ophthalmologist. Vision outcomes were assessed for all affected eyes. The incidence of ocular complications in those eyes that were free of ocular complication at presentation also was assessed. The development of CME was defined as the presence of macular thickening with cystoid spaces that was seen on clinical examination, the presence of a petalloid pattern of leakage at the macula on fundus fluorescein angiogram, or the presence of cystoid spaces on OCT.
Subretinal neovascularization was diagnosed by clinical examination and confirmed on fluorescein angiography. Epiretinal membrane formation, subretinal fibrosis, and exudative retinal detachment were diagnosed by funduscopic examination.
Ocular coherence tomography data were performed using 2 separate machines, dependent on availability (Cirrus-HT OCT; Zeiss, California, USA, or Spectralis ICT; Heidelberg Engineering, Heidelberg, Germany). Data collected included the presence of subretinal fluid and/or septae, central macular thickness, and choroidal thickness (for Spectralis scans).
Variables were summarized as median and interquartile range (IQR) or as mean (95% confidence interval [CI]) if normally distributed. Any trends over time were assessed visually with graphs.
All analyses were undertaken using Stata IC 12.1 for Windows (StataCorp LP, College Station, Texas, USA).