title: Marked improvement in autoimmune pulmonary alveolar proteinosis with severe hypoxemia in a patient treated with ambroxol: a case report.
abstract: BACKGROUND
  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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Respiratory Medicine, Shinko Hospital, 1-4-47, Wakihama-cho, Chuo-ku, Kobe, 651-0072, Japan. nao.oda7221@gmail.com.
authors.forename: Nao
authors.initials: N
authors.lastname: Oda
authors.affiliation: Department of Respiratory Medicine, Shinko Hospital, 1-4-47, Wakihama-cho, Chuo-ku, Kobe, 651-0072, Japan. koji.tamai@gmail.com.
authors.forename: Koji
authors.initials: K
authors.lastname: Tamai
authors.affiliation: Department of Respiratory Medicine, Shinko Hospital, 1-4-47, Wakihama-cho, Chuo-ku, Kobe, 651-0072, Japan. ysuzuki1957@gmail.com.
authors.forename: Yujiro
authors.initials: Y
authors.lastname: Suzuki
authors.affiliation: Department of Respiratory Medicine, Shinko Hospital, 1-4-47, Wakihama-cho, Chuo-ku, Kobe, 651-0072, Japan. haru.yoshi815@gmail.com.
authors.forename: Harukazu
authors.initials: H
authors.lastname: Yoshimatsu
authors.affiliation: Department of Respiratory Medicine, Shinko Hospital, 1-4-47, Wakihama-cho, Chuo-ku, Kobe, 651-0072, Japan. hiromtok@kuhp.kyoto-u.ac.jp.
authors.forename: Hirofumi
authors.initials: H
authors.lastname: Matsuoka
authors.affiliation: Department of Respiratory Medicine, Shinko Hospital, 1-4-47, Wakihama-cho, Chuo-ku, Kobe, 651-0072, Japan. matsumotoyusuke1982@gmail.com.
authors.forename: Yusuke
authors.initials: Y
authors.lastname: Matsumoto
authors.affiliation: Department of Respiratory Medicine, Shinko Hospital, 1-4-47, Wakihama-cho, Chuo-ku, Kobe, 651-0072, Japan. nobuhiko8242002@yahoo.co.jp.
authors.forename: Nobuhiko
authors.initials: N
authors.lastname: Okada
mesh_terms: D000368:Aged; D000551:Ambroxol / Q000627:therapeutic use*; D001327:Autoimmune Diseases / Q000188:drug therapy*; D005100:Expectorants / Q000627:therapeutic use*; D005260:Female; D016178:Granulocyte-Macrophage Colony-Stimulating Factor / Q000008:administration & dosage; D006801:Humans; D000860:Hypoxia / Q000188:drug therapy; D008171:Lung Diseases / Q000000981:diagnostic imaging; D011649:Pulmonary Alveolar Proteinosis / Q000188:drug therapy*; D014057:Tomography, X-Ray Computed
chemical_list: D005100:Expectorants; D000551:Ambroxol; D016178:Granulocyte-Macrophage Colony-Stimulating Factor