Successful treatment of an overlapping choriocapillaritis between multifocal choroiditis and acute zonal occult outer retinopathy (AZOOR) with adalimumab (Humira™)

To report a case of overlapping choriocapillaritis that initially presented as multifocal choroiditis (MFC) but later showed features compatible with acute zonal occult outer retinopathy (AZOOR) resistant to standard immunosuppression that responded only to adalimumad therapy. A 41-year-old patient presented with multiple small, discrete yellow-whitish spots in both eyes, compatible with MFC. A few weeks later, despite treatment with sub-Tenon and systemic corticosteroids, a choroidal neovascularization occurred in the right eye. The patient was treated with intravitreal anti-vascular endothelial growth factor. After 2 months, reduced visual acuity, photopsia and visual field defect in the left eye occurred. Spectral domain optical coherence tomography revealed photoreceptor outer segment defects common to all choriocapillaritis. The additional finding of an annular scotoma and a 360° ring on indocyanine green angiography led us to make the diagnosis of presumed AZOOR. Despite the combination of several immunosuppressive agents leading to temporary control of the disease, the patient experienced a further worsening. At that point, adalimumab was introduced, which led to an obvious improvement. This case supports the hypothesis that two different entities of the so-called AZOOR complex can be possible in the same eye, even asynchronously. In our case, anti-tumor necrosis factor alpha monoclonal antibody therapy represented a valid treatment option in a patient unresponsive to traditional immunosuppressive treatments.