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 (the norm is 95 percent). Moist rales were heard on auscultation in both lungs. The patient was urgently intubated upon admission; a chest X-ray showed dense periradicular opacities in both lungs, arranged in a “bat wing” pattern. A CT scan also showed well-defined opacities reaching 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. The tests showed severe anemia (hemoglobin 53 grams per liter) and a creatinine level of 610 (with a norm of 62-115) micromoles per liter, 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 clinical presentation and examination data, diffuse alveolar hemorrhage was suspected in the patient. It was confirmed by repeated bronchoalveolar lavage, which yielded aliquots of fluid with a progressive increase in blood content. To clarify the diagnosis, the man underwent an analysis for antineutrophil cytoplasmic antibodies (ANCA) and a kidney biopsy. They allowed us to identify ANCA-associated vasculitis, a rare heterogeneous autoimmune disease characterized by necrotizing inflammation of small vessels and a variety of clinical manifestations (most often from the lungs and kidneys). The patient was prescribed glucocorticoids, cyclophosphamide, plasma exchange and hemodialysis in a continuous mode, but despite the treatment, he died 23 days after admission.
