Images in Clinical Medicine
Pulmonary Alveolar Proteinosis
N Engl J Med 2002; 347:2133December 26, 2002
- Article
Figure 1 A 26-year-old man presented with nonproductive cough and exertional dyspnea. Physical examination disclosed acrocyanosis. Chest radiography showed patchy alveolar and interstitial disease in a perihilar “batwing” distribution (Panel A). High-resolution computed tomography of the chest (Panel B) revealed extensive bilateral air-space disease (arrows) and a “crazy paving” appearance within the affected air spaces (arrowheads). Pulse oximetry revealed oxyhemoglobin desaturation to 79 percent when the patient was walking. Pulmonary-function testing demonstrated a moderate restrictive ventilatory defect and severe reduction in lung volumes and diffusion capacity. Fiberoptic bronchoscopy with transbronchial lung biopsies showed alveolar filling with amorphous, granular eosinophilic material and preserved alveolar septal architecture (Panel C, hematoxylin and eosin, ×200). This pathological finding is diagnostic of pulmonary alveolar proteinosis. The patient underwent bilateral sequential whole-lung lavage, which resulted in resolution of his symptoms and improvement in pulmonary function and radiographic findings.
Selim M. Arcasoy, M.D.
Columbia University College of Physicians and Surgeons, New York, NY 10032Paul N. Lanken, M.D.
University of Pennsylvania School of Medicine, Philadelphia, PA 19104- Citing Articles (3)
Citing Articles
1
Isham Huizar, Mani S Kavuru. (2009) Alveolar proteinosis syndrome: pathogenesis, diagnosis, and management. Current Opinion in Pulmonary Medicine 15:5, 491-498
CrossRef2
Shih-Chuan Tsai, Tsung-Huai Kuo, Chien-Long Kuo, Hua-Tzu Huang, Wen-Pei Lin, Khee-Siang Chan, Hurng-Sheng Wu. (2004) Gallium Scanning in Pulmonary Alveolar Proteinosis. Clinical Nuclear Medicine 29:10, 667-669
CrossRef3
Jeffrey J. Presneill, Koh Nakata, Yoshikazu Inoue, John F. Seymour. (2004) Pulmonary alveolar proteinosis. Clinics in Chest Medicine 25:3, 593-613
CrossRef
