Marked improvement in autoimmune pulmonary alveolar proteinosis with severe hypoxemia in a patient treated with ambroxol: a case report

• Published in: Journal of medical case reports Vol. 9; no. 1; p. 100
• Main Authors: Oda, Nao, Tamai, Koji, Suzuki, Yujiro, Yoshimatsu, Harukazu, Matsuoka, Hirofumi, Matsumoto, Yusuke, Okada, Nobuhiko
• Format: Journal Article
• Language: English
• Published: England BioMed Central Ltd 06.05.2015; BioMed Central
• Subjects: Aged; Ambroxol - therapeutic use; Autoimmune Diseases - drug therapy; Blood lipids; Care and treatment; Case Report; Case studies; CT imaging; Diagnosis; Expectorants - therapeutic use; Female; Granulocyte-Macrophage Colony-Stimulating Factor - administration & dosage; Health aspects; Humans; Hypoxia; Hypoxia - drug therapy; Lung Diseases - diagnostic imaging; Macrophages; Membrane lipids; Patient outcomes; Pulmonary alveolar proteinosis; Pulmonary Alveolar Proteinosis - drug therapy; Risk factors; Surface active agents; Tomography, X-Ray Computed
• ISSN: 1752-1947; 1752-1947
• DOI: 10.1186/s13256-015-0588-6

Pulmonary alveolar proteinosis is characterized by accumulation of surfactant and phospholipids in the pulmonary alveoli. Whole lung lavage is considered the first-line therapy, which requires special techniques. To the best of our knowledge, there have only been limited reports that have demonstrated the effectiveness of ambroxol on a mild case of pulmonary alveolar proteinosis. A 72-year-old Japanese woman presented to our hospital with a one-year history of productive cough and progressive dyspnea. Her chest computed tomography scan showed a bilateral crazy-paving pattern in both of her lungs. She was diagnosed with autoimmune pulmonary alveolar proteinosis based on bronchoalveolar lavage findings and the presence of serum anti-granulocyte macrophage colony-stimulating factor antibodies. She was severely hypoxemic, so we recommended whole lung lavage or inhaled granulocyte macrophage colony-stimulating factor treatment, which she refused. We initiated treatment with ambroxol and her symptoms markedly improved. Although whole lung lavage is the first-line therapy for pulmonary alveolar proteinosis, oral ambroxol could be an alternative treatment option, even in patients with severe respiratory compromise.