RESUMO
Immunoglobulin G4-related disease (IgG4-RD) can involve nearly any organ system, including the central and peripheral nervous systems. IgG4 antibodies are not known to play a primary etiologic role in disease. IgG4-RD must be distinguished from a growing number of immune-mediated conditions in which IgG4 autoantibodies contribute directly to pathophysiology. The most common neurologic features of IgG4-RD result from disease in the orbits, pachymeninges, and substance of the pituitary gland and stalk, as well as a perineuropathy that can involve peripheral or cranial nerves. Disease affecting the brain parenchyma is rare but reported.
Assuntos
Doenças Autoimunes do Sistema Nervoso/diagnóstico , Doenças Autoimunes do Sistema Nervoso/etiologia , Imunoglobulina G , Doenças Autoimunes do Sistema Nervoso/terapia , Glucocorticoides/uso terapêutico , HumanosRESUMO
Orbital tumor lesions in pediatric population encompass a wide range of pathological processes, which are very different in etiology and prognosis. They usually require an emergency in histological diagnosis because of their quick growth. Beside malignant and benign tumors, the inflammatory pseudo-tumors group included dysimmune orbital involvement secondary to systemic vasculitis such as granulomatosis with polyangiitis (ex-Wegener's granulomatosis). We report the case of a seven-year-old girl suffering from a severe primitive ANCA vasculitis, revealed by an orbital mass.
Assuntos
Granulomatose com Poliangiite/diagnóstico , Pseudotumor Orbitário/etiologia , Anticorpos Anticitoplasma de Neutrófilos/sangue , Asma/complicações , Bronquite/complicações , Criança , Progressão da Doença , Feminino , Glomerulonefrite Membranoproliferativa/etiologia , Granulomatose com Poliangiite/complicações , Granulomatose com Poliangiite/imunologia , Humanos , Laringoestenose/etiologia , Pseudotumor Orbitário/patologiaRESUMO
A doença de Still do adulto é uma desordem inflamatória rara, caracterizada por picos febris diários, rash evanescente, artrite e envolvimento de múltiplos órgãos. Manifestações oculares têm sido descritas associadas às manifestações clínicas clássicas da doença. Será descrito um caso de pseudotumor orbitário, condição clínica incomum, em uma paciente com diagnóstico de doença de Still do adulto.
Adult-onset Still's Disease is a rare systemic inflammatory disorder characterized by quotidian spiking fevers, evanescent rash, arthritis, and multiorgan involvement. Ocular manifestations have been reported associated with the classical clinical findings of this disease. We describe a case of orbital pseudo tumor, an uncommon clinical condition, in a patient with diagnosis of adult-onset Still's Disease.
