Retinitis pigmentosa (RP) is an inherited
retinal degeneration that affects predominantly peripheral visual fields.
Macular edema may cause additional central visual acuity decrease.
Fluorescein angiography and/or optical coherence tomography detect the presence of
macular edema in 10-20% of RP patients.
Macular edema can manifest at any stage of the disease and may be unilateral or bilateral. In X-linked forms,
macular edema is very rare. The origin of
macular edema in RP patients still remains poorly understood. The possible pathophysiological role of
autoantibodies has been suggested (
retinal,
carbonic anhydrase, and
enolase antibodies).
Drug therapy is the primary treatment for
macular edema in patients with R P. Systemic
carbonic anhydrase inhibitors, such as oral
acetazolamide or topical
dorzolamide, still are the mainstay of initial
therapy. If
cystoid macular edema is refractory to
acetazolamide, intravitreal
corticosteroid injections could be administered. Intravitreal anti-
vascular endothelial growth factor therapy has also been used in cases of
macular edema persistence after oral
acetazolamide therapy, though with uncertain results.
Vitrectomy can also be proposed, but its role is not clear yet. Autoimmune retinopathies (AIRs) are a group of
rare diseases characterized by acute or subacute progressive vision loss and are thought to be mediated by
autoantibodies specific to
retinal antigens. The AIRs encompass
paraneoplastic syndromes, such as
cancer-associated retinopathy and
melanoma-associated retinopathy, and a larger group of AIRs that have similar clinical and immunological findings but without underlying
malignancy. These diseases may also be complicated by
macular edema.