Abstract
Purpose: :
Primary stromal choroiditis includes entities for which inflammation springs in the choroidal stroma such as Vogt-Koyanagi-Harada (VKH) disease and birdshot chorioretinitis (BC) and are therefore best analyzed with ICGA. Although both entities have common disease mechanisms, the disease profiles differ and the aim of this work was to point out in a retrospective study the major differences in inflammatory behavior, disease management and outcomes using ICGA, fluorescein angiography (FA) and optical coherence tomography (OCT).
Methods: :
Both VKH and BC patients were managed in a standard way when presenting and followed in our centre. Acute VKH was treated with high dose intravenous corticosteroids(CS) followed by oral CS and benefited from ICGA-guided follow-up (FU) with addition of immunosuppressive agents (ISA) at the 1st ICGA-detected recurrence during CS taper. BC was treated with dual CS and ISA in case of functional loss. Inflammatory profiles were recorded, including FA&ICGA signs as well as OCT findings in both groups. Duration and type of therapy as well as functional and morphological evolution was noted.
Results: :
9 VKH(1M/8F) and 18 BC patients(5M/13F) having had standard care were included in the study. Mean age at entry was 33±10 for VKH and 49±11 for BC patients. For VKH patients disease pattern consisted of hyperacute inflammation with severe retinal (FA) and choroidal (ICGA) lesions, the 1st being the consequence of the severe underlying choroiditis. CS alone was sufficient in 2/9 cases and CS with ISA was necessary in 7/9, mean therapy duration was 30±34 months and the number of healed cases was 6/9 with a therapy-free FU of 32±22 months. Secondary spill-over retinal inflammation regressed rapidly (23±18 days) followed by choroidal lesions (resolution of hypofluorescent dark dots/HDDs in 22±18 months in 6/9 cases). In BC, onset was insidious with dual independent retinal and choroidal inflammatory disease. Combined CS and ISA was necessary in 16/18 cases with a mean therapy duration of 9±4 years and ongoing therapy in all cases at the end of FU. Response to therapy was good in the choroid with resolution of HDDs but retinal disease which, unlike in VKH, is not secondary to choroiditis, could at best be stabilized with slow evolution towards atrophy as shown by SOCT (310±43µm at last FU versus 395±62µm at entry) .
Conclusions: :
Albeit inflammation is severe & hyperacute in VKH, it responds well to therapy as the choroid is the only source of inflammation with retinal disease only secondary to massive choroiditis. In BC, choroiditis is responding to therapy, but independently developing retinitis is more resistant to therapy and is responsible for prolonged therapy and substantial disease morbidity.
Keywords: chorioretinitis • imaging/image analysis: clinical • inflammation