• Alveolar proteinosis (Nejm 2003;349:2527)

Pathophys:Surfactant substance accumulates in alveoli due to auto-antibodies vs granulocyte macrophage colony-stimuating factor, hence impairing granulocyte function (Nejm 2007;356:527)

M > F, median age onset = 39 yr

Sx:Insidious onset DOE and cough

Si:Rales (50%)

Death from restrictive disease, though some spontaneous remissions

Lab: Mild incr LDH

Xray:

• Asbestosis: lower lobe predominant; fine streak pattern; pleural effusions (21%) (Ann IM 1971;74:178); increased incidence of mesothelioma and carcinoma
• Berryliosis: acute or chronic for yr after exposure; looks like sarcoid (Ann IM 1988;108:687); a hypersensitivity pneumonitis (Nejm 1989;320:1103)
• Chronic occupational pneumonitis (Occupational "Asthma" (Byssinosis, Mill Fever, etc.)) and allergic pneumonitis/alveolitis
• Cystic fibrosis (Cystic Fibrosis (Mucoviscidosis)) when end stage
• Eosinophilic granuloma (Langerhan's Cell Histiocytosis (Histiocytosis X), Letterer-Siwe Disease, Hand-Schüller-Christian Syndrome, and Eosinophilic Granuloma)
• Idiopathic pulmonary fibrosis (Interstitial Pneumonias (Idiopathic Pulmonary Fibrosis, Hamman-Rich Syndrome)
• Lymphatic carcinomatosis: alveolar cell, breast
• Pulmonary thesaurosis (Nejm 1974;290:660): seen in hairdressers. Patchy infiltrates. Clears when avoid hair sprays. Nodes look like sarcoid. Lung looks like idiopathic pulmonary fibrosis
• Sarcoid (Sarcoidosis)
• Silicosis: eggshell-calcified nodes, progressive massive fibrosis “bat wings”
• Talcosis: increased density of pattern
• Tuberculosis (Tuberculosis)
• Tungsten carbide maker’s exposure: rx’able by stopping dust exposure? from cobalt involved in process (Ann IM 1971;75:709)

Rx: Whole-lung bronchoaveolar lavage