P2 Case C Answers

With limited information, a resident asks your expert pulmonary opinion about a confusing patient on his medicine service. Quickly, he summarizes the patient as a 47 yo man who presented with dry cough, exertional dyspnea, late inspiratory crackles in lower lung fields, and whose Pulmonary Function Tests showed all volumes and capacities equally decreased. The only other pertinent history known is that he was treated 10 years ago for an unknown cancer, in which he is in remission. When the patient was asked what she was treated with, all she knows that it was an antibiotic-type of chemotherapy, though the names evades him.

1. Your clinical gestalt takes you to this pathologic image. Describe what you see and what the etiology of this condition.

Cor pulmonale. Note the increased ventricular volume and hypertrophied muscle of the right ventricle (arrow). The patient had pulmonary hypertension.

2.  Unfortunately, the patient dies and the resulting pathology images follows. What do you see in each?

"This is an example of pulmonary fibrosis. Some cases of restrictive lung disease have no known etiology--this is, they are known as idiopathic pulmonary fibrosis. The alveolitis that produces fibroblast proliferation and collagen deposition is progressive over time." -Webpath

Honey Comb Lung: Alveolar fibrosis leads to proximal dilatation of the small airways. Indicates an end stage process.  

3. Describe the pathogenesis in 1-2 sentences.

Repeated cycles of alveolitis are triggered by an unknown agent. (TGF-Beta release from injured pneumocytes induces fibrosis)

4. The patient was previously treated with Chemotherapy. Of the drugs below, which one is not implicated in interstitial fibrosis. 

a. Nitrosourea

b. Cyclophosphomide

c. Amiodarone

d. GM-CSF

e.  Bleomycin/Busulfan

f. Methotrexate