Abstract
Poliangiite granulomatosa e uma doenca sistemica que afeta qualquer orgao, com predilecao pelo trato respiratorio superior, pulmoes e rins. Este artigo tem como objetivo relatar um caso atipico de uma paciente com esclerite nodular como manifestacao inicial da poliangiite granulomatosa (Wegener), mimetizando um quadro de tuberculose. A paciente apresentou hiperemia ocular e baixa acuidade visual progressiva por 1,5 anos, seguido por dor ocular por dois meses. A paciente possuia nodulos subpleurais com densidade de partes moles, linfonodomegalia em janela aorto-pulmonar e espessamento pleural bilateral discreto, negativo para bacilos alcool-acido resistentes (BAAR). O diagnostico histologico revelou uma vasculite granulomatosa sugestiva de vasculite nao infecciosa (poliangiite granulomatosa). Foi iniciada pulsoterapia com ciclofosfamida.
Highlights
Granulomatosis with polyangiitis (GPA), formerly known as Wegener’s granulomatosis[1], is a systemic disease of unknown aetiology that can affect several organs, especially the upper respiratory tract, lungs, and kidneys
The disease’s association with antineutrophil cytoplasmic antibody (ANCA), and a positive biopsy of affected tissues are important for diagnosis
GPA is a disease of unknown aetiology and was first described in 1937
Summary
Granulomatosis with polyangiitis (GPA), formerly known as Wegener’s granulomatosis[1], is a systemic disease of unknown aetiology that can affect several organs, especially the upper respiratory tract, lungs, and kidneys. It is characterised by granulomatous inflammation, tissue necrosis, and various degrees of small and medium calibre vasculitis 2. Ocular involvement is observed in 50-60% of cases, and it persists throughout the patient’s life in up to 87% of cases. This can be the sole manifestation of the disease in 8-16% of patients[3]. Treatment with cytotoxic and immunosuppressive agents has significantly improved the prognosis of GPA, which used to have a high mortality rate before the advent of these therapies[3,4]
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