Sir,

We would like to comment on the letter from Dweck et al. First, the presentations in this Fabry case was not typical of a true uveitis because it lacked some common features of a uveitis, such as injection of ocular surface, kerato precipitates of posterior corneal surface, or posterior synechia of iris, despite the anterior chamber cells were very prominent. We believe the change of vascular permeability contributed to the prominent cells in both anterior and posterior chamber and thus consider this case a uveitis-like picture rather than a true uveitis. Second, we agree with the speculation that the occurrence of uveitis in the Fabry case was a coincidence. However, because the clinical condition markedly improved after enzyme replacement therapy, this coincidence was not very likely.

Based on the experience of many vitreoretinal doctors on the use of corticosteroids in diabetic macular oedema,1, 2 we believe the use of steroid in this case was similar to that in diabetic retinopathy: a vascular leakage-decreasing effect rather than a true anti-inflammatory effect. This explained why the effect of steroid was temporary and the enzyme replacement therapy had more long-term effect.

We believe the combined therapy of steroid and enzyme replacement therapy would be a reasonable approach to cases with Fabry disease or Gaucher disease.