Uveitis in Turner's syndrome

Abstract

Background. Turner's syndrome is a chromosomal abnormality where phenotypic females have either a missing X chromosome or a structural aberration of the X chromosome. Several ocular diseases have been associated with Turner's syndrome in the past, including one case only of proven iridocyclitis.

Methods. In this study we report the clinical findings of three females with Turner's syndrome and uveitis followed up for a mean period of 12.6 months. Two were observed in childhood or adolescence (10 and 16 years old).

Results. All the patients showed iridocyclitis, in one case complicated by the onset of papilledema and cystoid macular edema. Associated systemic diseases (psoriasis and juvenile seronegative arthritis) were diagnosed in two cases. The third patient showed positive antinuclear antibody and HLA-DR11, without any clinical or radiologic signs of arthritis. The iridocyclitis tended to become chronic with time, and this may be partially due to the endocrinologic contraindications to the use of systemic steroids. Nevertheless, the final visual acuity was ≥8/10 in all three cases.

Conclusions. Iridocyclitis should be included in the list of ocular manifestations in Turner's syndrome. It may tend to become chronic and may be found especially in those patients presenting other associated autoimmune systemic disease.