A 57-year-old man with hypertension and a two-week history of progressive dyspnea was admitted to the emergency department of an American hospital. His oxygen saturation was 68 percent (normal: 95 percent). Auscultation revealed moist crackles in both lungs. Upon admission, the patient was urgently intubated. A chest X-ray revealed dense hilar opacities in both lungs, arranged in a "bat-wing" pattern. A CT scan also revealed well-defined opacities extending into the subpleural areas, and a "cobblestone" sign (ground-glass opacities superimposed on thickened alveolar septa) was observed in the upper lobe of the right lung. Test results revealed severe anemia (hemoglobin 53 grams per liter) and a creatinine level of 610 micromoles per liter (normal range: 62–115), as well as hematuria and proteinuria. Dr. Irene Lee of Baylor College of Medicine described the case in The New England Journal of Medicine.
Based on the patient's clinical presentation and examination data, diffuse alveolar hemorrhage was suspected. This was confirmed by repeated bronchoalveolar lavage, which yielded fluid aliquots with a progressive increase in blood content. To confirm the diagnosis, the man underwent antineutrophil cytoplasmic antibody (ANCA) testing and a kidney biopsy. These tests revealed ANCA-associated vasculitis, a rare heterogeneous autoimmune disease characterized by necrotizing inflammation of small vessels and a variety of clinical manifestations (most commonly involving the lungs and kidneys). The patient was prescribed glucocorticoids, cyclophosphamide, plasma exchange, and continuous hemodialysis. However, despite this treatment, he died 23 days after admission.
