Isolated Pulmonary Vasculitis

A 41-year-old woman presented with cough and heart murmur for a half-month. She has hyperthyroidism but is well controlled. The results of laboratory tests, including kidney and liver functions, electrolytes, and general urine, were normal. The erythrocyte sedimentation rate (32 mm/h; normal value, <20 mm/h) and C-reactive protein (35 mg/L; normal range, 0–20 mg/L) were high; hemoglobin (122 g/L; normal value, 110–160 g/L), leukocytosis (white blood cells 8.8×109/L; normal value, 4.8–10.8×109/L), and thrombocytosis (299×109/L; normal value, 100–300×109/L) were normal. Initial blood tests showed that troponin I at 0.44 ng/mL (normal value, 0–0.16 ng/mL), B-type natriuretic peptide at 1630 pg/mL (normal value, 0–400 pg/mL), and creatine kinase at 6.6 ng/mL (normal value, 0–4.0 ng/mL) were elevated. Immunology testing revealed negative antinuclear antibody lineage.Chest x-ray film demonstrated clear lung fields but an enlarged heart. ECG showed sinus tachycardia, right axis, and signs of right atrium and right ventricle overload.Transthoracic echocardiography demonstrated a giant right atrium (56 mm) and right ventricle (57 mm) (Figure 1A and online-only Data Supplement Movie I). The left ventricular size (42 mm) and systolic function (ejection fraction=70%) were normal. The intimal was significantly thickened in the distal pulmonary artery (PA) and branches (Figure 1B). Color Doppler flow imaging showed a turbulence color signal in the left PA and no flow signals within the right PA (Figure 1C, and online-only Data Supplement Movie II); mild tricuspid valve regurgitation with systolic pulmonary artery pressure was estimated at 95 mm Hg (Figure 1D).Figure 1. A , Transthoracic echocardiographic 4-chamber view showed marked enlarged right atrium and ventricle. B , Parasternal short-axis view showed significant thickening of the intimal in left and …