Diffuse Alveolar Hemorrhage in ANCA-Associated Vasculitis
Irene S. Lee
N Engl J Med 2025;393:72
DOI: 10.1056/NEJMicm2416576
A 57-year-old man with hypertension presented to the emergency department with a 2-week history of worsening shortness of breath. His oxygen saturation was 68% while he was breathing ambient air. He was confused and unable to speak. Physical examination was notable for diffuse crackles in both lungs. Tracheal intubation was performed. A chest radiograph showed dense, perihilar airspace opacities in both lungs in a “batwing” distribution (Panel A). Computed tomography of the chest also showed well-demarcated airspace opacities that spared the subpleural regions. A “crazy-paving pattern” (ground-glass opacities with superimposed septal thickening) was seen in the right upper lobe (Panel B, asterisk). Laboratory studies were notable for a hemoglobin level of 5.3 g per deciliter (reference range, 14.0 to 18.0) and a creatinine level of 6.9 mg per deciliter (610 μmol per liter; reference range, 0.7 to 1.3 mg per deciliter [62 to 115 μmol per liter]). Urinalysis revealed substantial hematuria and proteinuria. Given the patient’s clinical presentation and radiographic findings, diffuse alveolar hemorrhage was suspected and was subsequently confirmed by serial bronchoalveolar lavage, which produced progressively bloodier aliquots of lavage fluid (Panel C). On the basis of this finding, antineutrophil cytoplasmic antibody (ANCA) titers, and a renal biopsy, a final diagnosis of ANCA-associated vasculitis was later made. Despite treatment with glucocorticoids, cyclophosphamide, and plasma exchange as well as renal-replacement therapy, the patient died 23 days after admission.
