Nephrol Dial Transplant
August 2024
Background: Pulmonary haemorrhage with hypoxia caused by anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) has a high early mortality. Avacopan, an oral C5a receptor antagonist, is an approved treatment for AAV, but patients with pulmonary haemorrhage requiring invasive pulmonary ventilation support were excluded from the Avacopan for the Treatment of ANCA-Associated Vasculitis (ADVOCATE) Trial.
Methods: A retrospective, observational, multicentre case series of AAV patients with hypoxic pulmonary haemorrhage, requiring oxygen support or mechanical ventilation, who received avacopan.
Giant cell arteritis (GCA), also known as granulomatous arteritis is a systemic vasculitis mainly affecting extra cranial branches of carotid arteries. It can rarely affect other vascular beds causing thoracic aorta aneurysm, dissection and rarely cause myocardial infarction through coronary arteritis. It can cause considerable diagnostic dilemma due to varied clinical presentations.
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