Idiopathic Pulmonary Fibrosis

Question 1

Pulmonary function test findings in Idiopathic pulmonary fibrosis

Answer 1

• restrictive pattern
• reduced DLCO
• arterial hypoxemia that is exaggerated or elicited by exercise

Question 2

Histologic hallmark and chief diagnostic criterion of UIP

Answer 2

Heterogenous apperance at low magnification with alternating areas of normal lung, interstitial inflammation, foci of proliferating fibroblasts, dense collagen fibrosis and honeycomb changes

• most severely affected : peripheral subpleural parenchyma

Question 3

Drug that appears to improve cough in usual interstitial pneumonia (UIP)

Answer 3

Thalidomide

Question 4

Definition of Exacerbation in IPF

Answer 4

• worsening of dyspnea within a few days to four weeks
• newly developing diffuse ground-glass abnormality and/or consolidation superimposed on a background reticular or honeycomb pattern consistent with the UIP pattern
• worsening hypoxemia
• absence of infectious pneumonia, heart failure and sepsis

Question 5

Rate of exacerbations of UIP

Answer 5

10-57%

Question 6

Histologic pattern during episodes of exacerbation of UIP

Answer 6

Diffuse alveolar hemorrhage (atypical hyperplastic type II pneumocytes) on the background of UIP

Question 7

Management of UIP in Exacerbation

Answer 7

No therapy found to be effective
- should be referred early for lung transplant because of the unpredictability of disease progression
- MV often required but usually not successful
(Mortality 75%)

Question 8

HRCT findings on IPF

Answer 8

Patchy, predominantly basilar, subpleural reticular opacities often associated with traction bronchiectasis and honeycombing

Question 9

In IPF patients, treatment with this 3-drug regimen has been shown to increase the risks for hospitalization and death

Answer 9

Prednisone
Azathioprine
NAC or warfarin (in IPF px who lacked other indications for anticoagulation)