RESUMO
A 51-year-old man with rheumatoid arthritis and diabetes mellitus presented with new onset left-sided hemiparesis, left-sided neglect, and left-sided incongruous, denser inferiorly, homonymous hemianopsia. Magnetic resonance image of the brain showed prominent swelling of the right frontal, parietal, and occipital lobes greater than on the left with significant change in the fluid-attenuated inversion recovery signal in the gray matter. Perinuclear antineutrophil cytoplasmic antibodies titers were elevated, and skin biopsy demonstrating leukocytoclastic vasculitis. He showed marked clinical and radiographic improvement in association with recovery of vascular abnormalities after the initiation of prednisone, pulse therapy with methylprednisolone, and methotrexate. Clinicians should be aware of the possibility of neuro-ophthalmic manifestations of rheumatoid arthritis, including rheumatoid arthritis-related vasculitis causing homonymous hemianopsia.
Assuntos
Artrite Reumatoide/diagnóstico , Hemianopsia/diagnóstico , Vasculite do Sistema Nervoso Central/diagnóstico , Vasculite Leucocitoclástica Cutânea/diagnóstico , Anticorpos Anticitoplasma de Neutrófilos/sangue , Artrite Reumatoide/tratamento farmacológico , Artrite Reumatoide/imunologia , Diabetes Mellitus/diagnóstico , Diagnóstico Diferencial , Quimioterapia Combinada , Glucocorticoides/uso terapêutico , Hemianopsia/tratamento farmacológico , Hemianopsia/imunologia , Humanos , Imunossupressores/uso terapêutico , Imageamento por Ressonância Magnética , Masculino , Metotrexato/uso terapêutico , Metilprednisolona/uso terapêutico , Pessoa de Meia-Idade , Prednisona/uso terapêutico , Pulsoterapia , Vasculite do Sistema Nervoso Central/tratamento farmacológico , Vasculite do Sistema Nervoso Central/imunologia , Vasculite Leucocitoclástica Cutânea/tratamento farmacológico , Vasculite Leucocitoclástica Cutânea/imunologia , Testes de Campo Visual , Campos VisuaisRESUMO
A 12-year-old girl presented with a sudden decrease in her right visual acuity and homonymous hemianopsia. An angiography of the retinal arteries demonstrated recanalized occlusion of the right retinal artery. Cerebral angiography showed bilateral internal carotid artery stenosis associated with the development of collateral circulation. Laboratory evaluations revealed dual antineutrophil cytoplasmic antibodies (ANCA) positivity [anti-proteinase (anti-PR3) ANCA and anti-myeloperoxidase (anti-MPO) ANCA], anticardiolipin (aCL) antibodies, and low titers of antinuclear antibodies (ANA). There was no evidence of active systemic lupus erythematosus (SLE), ANCA-related vasculitis, or other risk factors for cerebral occlusion, such as antiphospholipid syndrome (APS). Dual positivity for both cytoplasmic (c-ANCA) and perinuclear (p-ANCA) antineutrophil antibodies has been found previously in a small number of reports, but to our knowledge, this case represents the first case of moyamoya disease associated with dual ANCA positivity.