RESUMO
Behçet's disease (BD) is a multisystem disorder of unknown etiology. Renal involvement in Behçet's disease is not frequent. Pauciimmune necrotizing and crescentic glomerulonephritis is associated with antineutrophil cytoplasmic antibodies (ANCA) which are believed to activate neutrophils and cause vasculitis, especially if there is a concurrent synergistic inflammatory process. In this case report, we describe a patient diagnosed with BD who also developed pauciimmune necrotizing and crescentic glomerulonephritis. We also compared this case with similar previous cases reported in the literature. The aim of this study is to add information regarding ANCA, pauciimmune glomerulonephritis and Behçet's disease.
Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/complicações , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/metabolismo , Síndrome de Behçet/complicações , Síndrome de Behçet/metabolismo , Adulto , Anticorpos Anticitoplasma de Neutrófilos/metabolismo , Humanos , Imunoglobulinas/metabolismo , MasculinoAssuntos
Antígenos CD/genética , Síndrome de Behçet/genética , Polimorfismo Genético/genética , Adolescente , Adulto , Idoso , Síndrome de Behçet/patologia , Antígeno CTLA-4 , Estudos de Casos e Controles , Proliferação de Células , Feminino , Humanos , Modelos Logísticos , Masculino , Pessoa de Meia-Idade , Linfócitos T/patologia , Adulto JovemRESUMO
We report a case of a 47-year-old woman with Wegener's granulomatosis complicated by central diabetes insipidus. The patient had initially seronegative polyarthritis which mostly responded well to methotrexate and steroid therapy. Eight months later the patient suffered from polyuria and polydipsia. There were no abnormalities of the anterior pituitary hormones. MR images showed only loss of brightness of the posterior pituitary. Extensive evaluation of the patient revealed the presence of ANCA, in c-ANCA pattern and also PR3 positivity. Three months later findings of glomerulonephritis, as suggested by an active urine sediment and gradual proteinuria, and, finally, asymptomatic pulmonary nodules completed the clinical picture of Wegener's disease within 1 year. Renal biopsy showed crescent formation in two glomeruli, consistent with ANCA-related glomerulonephritis which showed pauci-immün depositions by direct immunofluorescence. Diabetes insipidus symptoms mostly regressed; renal and pulmonary findings completely disappeared with glucocorticoid and pulse cyclophosphamide treatment. These findings show that diabetes insipidus may rarely develop early in the disease process and ANCA positivity was directly indicative of Wegener's granulomatosis before the classic clinical signs of the disease.