Pulmonary alveolar proteinosis - a crazy presentation of dyspnea

• Main Authors: Inge Raadal Skov, Elisabeth Bendstrup, Jesper Rømhild Davidsen
• Format: Article
• Language: English
• Published: Taylor & Francis Group 2019-01-01
• Series: European Clinical Respiratory Journal
• Subjects: pap; pulmonary alveolar proteinosis; gm-csf; granulocyte-macrophage colony-stimulating factor; hrct; crazy paving; wll; whole lung lavage; rare lung disease
• Online Access: http://dx.doi.org/10.1080/20018525.2018.1552065

This case report demonstrates 44-year old man, presenting with recurring clinical pneumonias during a period of over 1 year. The patient was clinically affected with, i.a., weight loss, finger clubbing and severely reduced diffusion capacity. Repetitive chest x-rays showed bilateral and consolidating infiltrates, and a high-resolution computed tomography of the thorax (HRCT) exposed ground glass opacities superimposed on a reticular pattern as the so-called ‘crazy paving’ pattern. A bronchoscopic alveolar lavage revealed alveolar proteinaceous material compatible with the diagnosis pulmonary alveolar proteinosis (PAP). PAP is a rare syndrome where surfactant is accumulated in the alveoli, causing respiratory disease in typically young to middle-aged patients with male predominance. Both symptoms and prognosis are variable, and range from spontaneous remission to terminal respiratory failure. The standard treatment is whole lung lavage, where surfactant is mechanically rinsed from the lungs. The lack of specific clinical symptoms makes it easy to overlook the diagnosis, as supported by this case report. It serves as a reminder, that the findings of a crazy paving pattern on HRCT in young adults should alert of this rare disease, and advises on the further examinations required to make the diagnosis.