Título

POSTERIOR UVEITIS WITH RETINAL VASCULITIS SECONDARY TO BEHÇET DISEASE: A CASE REPORT

Introdução

Behçet disease (BD) is a systemic vasculitis that affects a large number of organs with a variable spectrum of clinical set. It is a polygenic autoinflammatory disease, of which the etiology is still unclear.
Geographical variation in prevalence and disease expression in addition to the presence of several clinical subsets, account for the difficulty in developing a universal classification criteria. It is known that eye involvement is a relevant factor for diagnosis of Behçet disease.

Métodos

Here we report a case of posterior uveitis with retinal vasculitis secondary to Behçet disease evaluated with multimodal retinal exams - Fundus Retinography, Angiofluoresceinography and Optic Coherence Tomography (OCT) before and after systemic and ocular treatment.

Resultados

31-years-old woman with sudden vision loss in both eyes for one day. At first evaluation, the best-corrected visual acuity (BCVA) was 20/100 in the right eye (OD) and 20/60 in the left eye (OS). Slit-lamp examination was normal. Fundoscopy showed tortuous vessels, macular whitish lesions with retinal hemorrhages in both eyes. Fluorescein angiography demonstrated macular occlusive vasculitis with retinal vein sheathing. Cystoid macular edema was showed on the optic coherence tomography. At medical record review, she reported episodes of oral painful ulcers during the last year. In biochemical examinations, there were no increases in inflammatory rate, angiotensin-converting enzyme or complement levels. Autoantibodies and serological tests for infectious diseases were negative. HLA-B51 was positive in this patient. Based on these findings, this case was diagnosed as posterior uveitis and retinal vasculitis associated with Behcet disease. The patient was treated with pulse IV methylprednisolone therapy followed by oral prednisone plus an injection of dexamethasone implant in the OD. After that, patient was given infliximab, according to the 2018 European League Against Rheumatism recommendations for the management of Behçet disease. BCVA one month after treatment was 20/200 in the OD and 20/20 in the OS and remains stable. There was a resolution of vasculitis and macular edema in both eyes, however, visual acuity in the right eye did not improve possibly due to foveal ischemia.

Conclusões

Therefore, our case highlights the need for early diagnosis and management in ocular Behçet, especially with posterior uveitis, in order to prevent severe and permanent visual damage.

Palavras Chave

Behçet disease; posterior uveitis; retinal vasculitis

Arquivos