Pulmonary involvement in Anti‐Neutrophil Cytoplasmic Antibody Associated Vasculitis: A single centre case series

Respirology Case Reports(2022)

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摘要
Anti-Neutrophil Cytoplasmic Antibody associated Vasculitides (AAV) comprise a rare group of disorders in which respiratory tract involvement is variable and often severe. The rarity and heterogeneity of AAV makes this a challenging condition to diagnose and manage. In this single-centre case series of 44 patients with AAV-associated respiratory disease, we provide an overview of disease manifestations, management aspects and treatment outcomes. Data from this case series highlight the real-world diagnostic and therapeutic challenges of the AAV respiratory disease spectrum; including uncertainties in the management of fibrosing interstitial lung disease, tracheobronchial stenosis and diffuse alveolar haemorrhage.
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关键词
antineutrophil cytoplasmic antibody, cyclophosphamide, rituximab, vasculitis
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