Wegener's Granulomatosis with Tracheal Involvement and Severe Pulmonary Hemorrhage

格納肉芽腫（Wegener's Granulomatosis）是一種壞死型肉芽腫性血管炎，病變主要在小動脈、靜脈及毛細血管，侵犯的器官包含上、下呼吸道，腎臟，眼睛和皮膚。如侵犯氣管，會在氣管內膜形成疤痕組織造成呼吸道狹窄，輕者成阻塞性肺炎，重者則導致呼吸衰竭。合併呼吸道侵犯的韋格納肉芽腫較少併發嚴重肺出血，可是一旦發生，則可能導致立即地呼吸困難和呼吸衰竭。本文報告一例韋格納肉芽腫合併氣管侵犯及嚴重肺出血個案。

關鍵字

韋格納肉芽腫；肺出血

並列摘要

Wegener's granulomatosis (WG) is a systemic autoimmune vasculitis of small-to-mediumsized vessels. It mainly involves the upper and the lower respiratory tracts, the kidneys, skin, and eyes. It can occur at any age and affects both sexes equally. Diffuse pulmonary hemorrhage is an unusual manifestation. Once this occurs, the respiratory condition can deteriorate rapidly, leading to respiratory failure. Early diagnosis and early use of immunosuppressive agents are the main means of managing it. We describe a case of newly diagnosed WG that presented with tracheal involvement and pulmonary hemorrhage. In spite of standard combination therapy with cyclophosphamide and pulse methylprednisolone, the patient still passed away due to severe pulmonary hemorrhage leading to hypoxia and multi-organ failure.

並列關鍵字

Wegener's granulomatosis ； pulmonary hemorrhage

國際替代計量

Wegener's Granulomatosis with Tracheal Involvement and Severe Pulmonary Hemorrhage

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