Birdshot retinochoroiditis in Brazil: a multicenter review of 40 patients

Abstract

Background Birdshot retinochoroiditis (BRC) is a rare and chronic bilateral uveitis mostly found in Caucasians. As few data are available about the clinical course of BRC in Hispanic patients, we aimed to report the clinical findings and the evolution of BRC in Brazilian patients. Methods This retrospective cohort multicenter nationwide study was performed by analyzing the records of patients with BRC diagnoses from Brazilian ophthalmological centers from April 1995 to May 2020. Results Forty patients (80 eyes) with a diagnosis of BRC were evaluated. The mean age was 53 years, and there was no sex predominance. All tested patients (34/40) were positive for HLA-A29. The diagnosis of BRC was made following the Levinson et al. criteria, and all ancillary tests were performed to exclude differential diagnoses. Clinical signs and symptoms, such as complications and treatment, were described. Conclusions BRC evolution in Brazilian patients seems to have some peculiarities that diverge from the published literature available about Caucasians, as AS inflammation is higher in this population.

Long-Term Outcomes of Birdshot Chorioretinopathy Treated with Corticosteroids: A Case Reports

“…Contrary to what has been found by several recent study [ 27 , 28 ], we found a statistically significant improvement in BCVA before/after treatment, overall and in the two groups of interest. It should be noted, however, that although the mean initial BCVA did not differ between these two groups, there was a different distribution of patients between subgroups: our results suggest a greater visual improvement for patients with severely impaired initial BCVA (<20/40 Snellen, Log MAR 0.30) in the methylprednisolone group, supporting our practice of administering corticosteroids loading doses to these patients.…”

“…Furthermore, nearly a fifth of patients were experiencing a relapse or had not reach control at the end of follow up. In our experience and as demonstrated in many studies [ 31 , 33 ], BSCR is a condition requiring prolonged treatment with a very slow tapering in corticosteroid therapy, sometimes with the long-term maintenance of a low dose of cortisone if well tolerated (>30 months according to Maleki et al [ 28 ]). As relapses occurred in nearly a third of patients per year, a delay of three months does not allow us to consider a patient in remission, thereby explaining the major difference in results between the study issued by Crowell et al and ours.…”

Coriorretinopatia de Birdshot: relato de caso e perspectivas sobre o tratamento

“…Descrita inicialmente por Ryan e Maumenee em 1980, a coriorretinopatia de Birdshot (BCR) é uma uveíte posterior crônica, não infecciosa, bilateral, caracterizada por múltiplos pontos hipopigmentados coroidianos. (1)(2)(3) É uma doença rara, que acomete de 0,1 a 0,6 indivíduo para cada 100 mil habitantes, predominando em mulheres de meia-idade (média de 50 anos), caucasianas e com forte associação ao HLA-A29 (90 a 95% dos pacientes com BCR são positivos). (2,4) O paciente pode queixar-se de moscas volantes, fotopsias, visão borrada, perda de campo visual periférico, discromatopsia e nictalopia, muitas vezes com acuidade visual (AV) de 1,0.…”

“…(4) Na angiofluoresceinografia (AFG), as lesões são hipofluorescentes, mas podem ser hiperfluorescentes em estágios crônicos (por defeito em janela). (1) Geralmente, não são bem delimitadas, sendo mais definidas com a indocianina verde (ICV) como lesões focais hipofluorescentes. O exame de tomografia de coerência óptica (OCT) é capaz de demonstrar as lesões coroidianas, além de edema macular e alteração na zona elipsoide.…”

“…(2,6) A perda visual ocorre devido a edema macular ou dano retiniano com perda de fotorreceptores, além de complicações associadas, como catarata, glaucoma, neovascularização de coroide, atrofia óptica e maculopatia em celofane. (1,2,4,5) Tratamento imunossupressor precoce é recomendado, utilizando corticoide sistêmico associado a outras drogas imunossupressoras, porém, em casos com má resposta clínica, pode ser necessário uso de agentes biológicos, como imunoglobulina intravenosa, adalimumabe, dentre outros. (4,7) A seguir, relata-se um caso de paciente com BCR em ajuste de terapia imunossupressora, que evoluiu com bom controle após uso de imunobiológico.…”