Restrcitive Part 3: Smoking Related Diseases Flashcards
What are the three diseases she wants us to know that are smoking related restrictive diseases?
Desquamative Interstitial Pneumonia, Respiratory bronchiolitis-ILD, and Langerhans Cell Histiocytosis
How do we characterize DSIP?
Large collections of macrophages in the alveolar spaces in current or former smokers
What is the classic histo feature of DSIP?
Accumulation of macrophages in the alveolar spaces with dusty brown pigment in their cytoplasm called smokers’ macrophages
What is the prognosis for DSIP, when do patients usually present, and what are two treatments?
Good prognosis.
4th and 5th decade
Stop smoking and corticosteroids.
How do we characterize RB-ILD?
Chronic inflammation and peribronchiolar fibrosis.
Classic histo feature of RB-ILD?
Smoker’s macrophages in respiratory bronchioles, alveolar ducts and peribronchiolar spaces.
What does she say is an important histo feature of RB-ILD?
Peribronchiolar metaplasia.
How do we best characterize Langerhans Cell Histiocytosis?
Langerhans cells and eosinophils collect and eventually causes scarring that damages alveoli which results in cysts.
What is a big time complication of the cysts?
They can rupture and cause pneumothorax
4 histo features of LCH?
Langerhans cells, eosinophils, cysts and scarring
Because of the involvement of Langerhans cells, what three things are positive?
Positive for s100, CD1a, and CD207.
What is the typical patient presenting with LCH?
Young adult smokers
How do we best characterize Pulmonary Alveolar Proteinosis?
Accumulation of surfactant in the alveolar and bronchiolar spaces due to defects in GM-CSF or pulmonary macrophage dysfunction.
What is the most common form of PAP and what is the mechanism?
Autoimmune. Circulating antibodies against GM-CSF.
What is the histo feature of PAP?
Alveoli contain surfactant proteins
