Autoimmune optic neuropathy as the first manifestation of systemic lupus erythematosus

Abstract

We described a 35 years old female patient with bilateral visual loss and pain on eye movement, mild papillary edema in acute phase, arcuate scotoma and complementary test positive for antinuclear antibodies that did not respond to corticosteroid therapy. The lack of clinical criteria for systemic lupus erythematosus (SLE) didn't prevent the institution of the specific treatment with corticosteroids and azathioprine. After seven months the diagnosis was made after a skin manifestation of the disease. This case shows the value of the ocular complaints in systemic diseases. And how the ophthalmologic exam can help the clinician elaborating a diagnosis. It is also very important for ophthalmologists and rheumatologists due to the fact that it calls the attention to another diagnostic hypothesis in patients with nonspecific optic neuritis, even with inconclusive laboratory tests. Maybe some ocular findings deserve to be included to the diagnostic criteria already established for SLE.Keywords: Optic neuritis/etiology; Optic nerve diseases/etiology; Lupus erythematosus, systemic; Collagen diseases; Case reports RESUMO Descrevemos caso de um paciente de 35 anos do sexo feminino, com perda visual bilateral associada à dor à movimentação ocular, edema papilar moderado na fase aguda, escotoma arqueado e exame complementar positivo para anticorpos antinucleares, que não responderam à terapia com corticosteróides. A falta de critérios clínicos para o lúpus eritematoso sistêmico (LES) não impediu a instituição do tratamento específico com corticosteróides e azatioprina. Depois de sete meses, o diagnóstico foi feito após uma manifestação da doença de pele. Este caso mostra o valor das queixas oculares em doenças sistêmicas e como o exame oftalmológico pode ajudar o clínico na elaboração de um diagnóstico. Também é muito importante para oftalmologistas e reumatologistas, devido ao fato de que chama a atenção para outra hipótese diagnóstica em pacientes com neurite óptica não-específica, mesmo com os testes laboratoriais conclusivos. Talvez alguns achados oculares merecem ser incluídos com os critérios de diagnóstico já estabelecido para o LES.Descritores: Neurite óptica/etiologia; Doenças do nervo óptico/etiologia; Lupus eritematoso sistêmico; Doenças do colágeno; Relatos de casos 1

Systemic Lupus Erythematosus Presenting as Optic Neuropathy: A Case Report

“…A recently reported case by Amigo et al described superior arcuate defects in the visual field and the unique feature was that SLE was diagnosed seven months after the first manifestation of the disease as optic neuropathy. In our case, inferior arcuate defects in the visual fields could be appreciated [12].…”

“…The first case of SLE manifesting as optic neuropathy was reported in 1978 by Cinfro and Frenkel [7]. Fourteen more cases have been reported since the publication of the first case of SLE presenting as optic neuropathy [7-12]. A review of 13 cases by Fridge M et al reported that in 31% of cases, the diagnosis of SLE was preceded by optic neuropathy.…”

Neuropatia óptica dominante associada à hipoacusia e apresentação tardia

“…The differential diagnosis of the condition includes other diseases that cause optic atrophy, including Leber hereditary optic neuropathy, Leigh syndrome, Costeff optic atrophy syndrome, normal tension glaucoma, and nutritional deficiencies (16,17) .…”