Limited Antineutrophil Cytoplasmic Antibody-Associated Vasculitis Presenting With Diffuse Alveolar Hemorrhage: A Case Report and Literature Review.

ABSTRACT

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) presents a significant medical challenge, with an annual incidence of 2.4 to 4.1 cases per 100,000 individuals and a prevalence of 29.6-54.6 cases per 100,000 individuals in the US. Diffuse alveolar hemorrhage (DAH) is a rare but grave complication of AAV, often associated with a mortality rate as high as 58.3%. This case study highlights a 52-year-old male patient who presented with hemoptysis and was diagnosed with DAH on bronchoscopy, subsequently testing positive for ANCA-PR3. Prompt intervention, including pulse steroids, rituximab, and plasmapheresis, along with the novel FDA-approved drug avacopan, led to significant improvement within four weeks. Early recognition and aggressive management are crucial in mitigating the life-threatening consequences of DAH in AAV, emphasizing the importance of bronchoscopy and advanced therapeutic modalities. This case underscores the potential efficacy of avacopan in managing ANCA vasculitis post-acute phase, offering hope for improved outcomes in this challenging condition.