Suclinical signs persistance in Vogt-Koyanagi-Harada disease (VKHD) patients treated with early high-dose corticosteroids and immunosuppressive therapy (IMT)

Abstract

Purpose : To evaluate the impact of early IMT over subclinical inflammation in VKHD, besides corticosteroid-sparing effect.

Methods : VKHD patients in the acute phase were prospectively included, with a minimum 12-m follow-up. They were treated with methylprednisolone pulsetherapy followed by oral prednisone (1mg/Kg/d with slow tapering) and early IMT (within 2 m). Azathioprine was used as first choice drug; mofetil mycophenolate was used if there was clinical intolerance, toxicity and/or drug resistance. Follow-up protocol included every 3-m clinical and imaging exams [indocyanine green and fluorescein angiographies and enhanced depth imaging optical coherence tomography] performed with Spectralis HRA+OCT. Full-field electroretinogram (ffERG) was carried out at inclusion, 6 and 12 m. Eyes were categorized in 2 different groups based on scotopic ffERG parameters: fluctuation ≥ 30% (worsening group) or stable visual function group. Subclinical signs were defined as: optic disc or perivascular leakage; dark dots and/or subfoveal choroidal thickness increase. Clinical signs were determined as: cells in anterior chamber; choroidal neovascularization (CNV) and/or macular edema. Changes in treatment were based on presence of clinical signs and/or ≥ 30% ERG parameters worsening in 2 consecutive exams. Descriptive statistics, Fisher exact and Mann-Whitney tests were used to analyze data. This study was approved by Institutional Ethics Committee and followed the Helsinki declaration.

Results : Eleven women were included, with median age at diagnosis of 37 yo and median time from symptoms till treatment of 23 d (range, 8-67 d). During follow-up, 4 eyes (18.2%) were defined as ffERG worsening group and 18 eyes (81.8%) as ffERG stable group. Both groups presented clinical and subclinical signs of inflammation in a similar proportion. However, marked pleocytosis (p=0.042) and choroidal folds (p=0.002) were more frequent on ffERG worsening group. There was no CNV during 12-m follow-up. Other results are shown in Table 1.

Conclusions : In this limited sample of patients, subclinical inflammation signs persisted, despite optimal early IMT. Worsening ERG group had indicators of a more severe disease from the onset.

This is an abstract that was submitted for the 2018 ARVO Annual Meeting, held in Honolulu, Hawaii, April 29 - May 3, 2018.

 

Table 1 – Scotopic full-field electroretinogram parameters-based groups of patients with Vogt-Koyanagi-Harada disease and their clinical characteristics