Female Predominant Uveitides Without Systemic Involvement
Birdshot chorioretinopathy (BCR) is a rare bilateral posterior uveitis. It is more common in women (58% of a cohort of 1157 BCR patients were female). It has strong association with the HLA-A29 and tends to affect those of Northern European ancestry and those in middle age (average, 48 to 53 y). BCR patients usually produce pathogenic T-cell subsets that are associated with IL-17 cytokines.
The white dot syndromes are a group of rare posterior uveitic diseases. Most of these conditions described below are more common in women. Multiple evanescent white dot syndrome (MEWDS) classically presents as numerous small discrete white lesions at the posterior pole and mid-periphery in young myopic women between the ages of 20 to 45. Of the reported 77 MEWDS patients, 74% were female. MEWDS is thought to have a viral etiology and usually resolves spontaneously. Multifocal choroiditis with pan-uveitis (MFC) differs from MEWDS in that patients are more likely to have irreversible visual impairment. In this cohort, 75% of 406 MFC patients were female. MFC patients usually present with a bilateral vitritis along with punched-out chorioretinal scars with pigmented borders at the posterior poles and peripheries. MFC tends to be a chronic disorder with a poor visual prognosis due to CNV. The cause is unknown. Punctate inner choroidopathy (PIC) is also similar to MEWDS and is similarly associated with young myopic women. In the cohort, 85% of 471 PIC patients were female. Similar to MFC, CNV can occur in PIC leading to vision loss. Acute zonal occult outer retinopathy (AZOOR) is the last of the white dot syndromes that is more common in women. In the cohort, 79% of 150 AZOOR patients were female. AZOOR classically presents in a young woman with photopsia. There is rapid loss of large areas of outer retinal function, but with minimal fundus changes. The cause is unknown and no effective treatment has been found. Further, in all the white dot syndromes, no sex differences in disease severity or symptoms have been noted.