Vogt-Koyanagi-Harada disease (VKHD) after systemic treatment discontinuation

Abstract

Purpose : To describe the clinical course of VKHD after systemic treatment discontinuation

Methods : Retrospective study with 11 patients (22 eyes) with VKHD after systemic treatment discontinuation for at least 12mo. All patients were followed from acute disease onset with systematic clinical and imaging evaluation (indocyanine green and fluorescein angiographies and enhanced depth imaging optical coherence tomography (Spectralis HRA+OCT)). All patients were treated with methylprednisolone pulse therapy followed by oral prednisone (1mg/kg/day) with slow tapering; 5 patients also received non-steroidal immunosuppressive therapy. Presence/fluctuation of clinical sign of inflammation (anterior chamber cells (ACC)); optic disc (OD) or perivascular leakage, dark dots (DD), subfoveal choroidal thickness (CT) increase ≥ 30% (subclinical inflammation); and complications were analyzed during treatment period (TP) and post-treatment period (PTP). Criteria for treatment discontinuation were no anterior uveitis relapse, stable subclinical inflammatory signs and stable full-field electroretinogram (ffERG) parameters for at least 12mo. Binary ocular data were analyzed by descriptive statistics and generalized estimated equations. This study was approved by the Institutional Ethics Committee and followed the Helsinki declaration

Results : Mean disease duration at TP and PTP was 37.6±15.8mo (range12-59mo) and 33.6±21.3mo (range 12-87mo), respectively. At the last TP visit, visual acuity (VA) was 0.1±0.2logMAR; no eyes had ACC; DD were present in all eyes (mean score 5.3±0.9), while OD leakage was observed in 2 eyes (1 patient). During PTP, 1 patient (2 eyes) had transitional ACC, but none had systemic treatment restarted. All evaluated parameters (events/year and at last visit) improved in PTP, with statistical significance only for DD score fluctuation (p=0.004). ffERG parameters remained stable on PTP despite some subclinical inflammatory signs were still present. Table 1 describes TP and PTP data.

Conclusions : This is a pioneer description of VKHD course after systemic treatment discontinuation. After a systematic follow-up, criteria used for treatment discontinuation seems adequate. Subclinical inflammation tends to ameliorate during PTP. Further studies with longer follow-up are needed to better understand subclinical inflammation signs in VKHD after treatment discontinuation and to detect risk factors for late recurrence.

This is a 2021 ARVO Annual Meeting abstract.

 

Clinical data