Título / Title

HLA-A29 POSITIVE BIRDSHOT CHORIORETINOPATHY IN A BRAZILIAN PATIENT

Introdução / Purpose

Birdshot chorioretinopathy (BSCR) is a rare cause of uveitis, with cases ranging from 4,3% of uveitis patients in Europe to 0,1% in other populations, and with a prevalence of less than 1 per 100,000 (0.1-0.6/100,000). It is predominantly seen in middle-aged (mean of 53 years) and females (54,1%), and most prevalent in caucasian populations, being most commonly diagnosed in people of Northern European ancestry. The reports of cases in Latino-Hispanic, African American and Japanese people are rare. This poster has the purpose to present a typical birdshot case in a Brazilian patient, with HLA-A29 positivity.

Material e Método / Methods

We report a case of a 71-year-old female patient, Brazilian with German ancestry, with a history of progressive low vision for several years undiagnosed. We present her clinical and complementary exams with typical Birdshot Chorioretinopathy findings.

Resultados / Results

She showed classic late BSCR findings, with disc pallor, diffuse vascular attenuation, areas of small oval chorioretinal atrophy in mid periphery and also macular atrophy. Fluorescein Angiography revealed window defect hyper-fluorescence at posterior pole and macula, late staining along the vessels and deep leaking points at choroidal level with multifocal distribution in all 4 quadrants mid periphery. Optical Coherence Tomography showed diffuse severe RPE and outer retinal layers atrophy in the macular area in both eyes. After excluding infectious causes, HLA-A29 was requested and it was positive, confirming the diagnosis.

Discussão e Conclusões / Conclusion

In the presence of a suggestive clinical condition, even if the patient is not caucasian, Birdshot Chorioretinopathy is a possible diagnosis. Other causes must be excluded, such as syphillis, tuberculosis and sarcoidosis, and HLA- A29 must be requested to confirm the diagnosis. Considering the strong genetic association of BSCR and the miscegenation of world population, it is also important to question the patients ancestry, as it may reinforce the diagnosis such as this presented report.

Palavras Chave

Birdshot, HLA-A29, Chorioretinopathy

Area

CLINICAL RETINA