Interstitial Lung Disease

Question 1

Only method that can distinguish the 4 categories of Diffuse Parenchymal Lung Disease.

Answer 1

Lung Biopsy

• surgical is needed b/c a large sample required
• bronchoscopic not sufficient

Question 2

Most common type of Idiopathic Interstitial Pneumonia

Answer 2

Idiopathic Pulmonary Fibrosis (IPF)

Usual Interstitial Pneumonia (UIP) pattern on Bx

Question 3

Main culprit in starting the cascade of tissue damage in Interstitial Lung Disease.

Answer 3

Macrophages

Question 4

Which 2 types of Idiopathic Interstitial Pneumonia can be reversed simply by quitting smoking?

Answer 4

Desquamative Interstitial Pneumonia

Respiratory Bronchiolitis IP

Question 5

What other conditions can show a UIP pattern on biopsy?

Answer 5

Collagen Vascular Disease
Asbestosis
Drug Induced lung disease
Occupational or Environmental disorders

Question 6

What is the prognosis for a pt diagnosed with Idiopathic Pulmonary Fibrosis?

Answer 6

Death within 3-8 years

-cough becomes debilitating, cyanosis and cor pulmonale occur in later stages

Question 7

Diagnostic test of choice for IPF.

Answer 7

High Resolution CT scan (HRCT)

Question 8

How does IPF appear on CT?

Answer 8

Bilateral Peripheral opacities in the base of the lung

Question 9

What is the only treatment proven to increase survival in patients with IPF?

Answer 9

Lung Transplant

Question 10

How are Desquamative IP and Respiratory Bronchiolitis IP differentiated on CT?

Answer 10

DIP shows a more diffuse infiltrate in the tiny air spaces

RBIP shows a sharp well-defined infiltrate in the respiratory bronchioles

Question 11

What is Sarcoidosis?

Answer 11

A syndrome involving abnormal collections of chronic inflammatory cells (granulomas) that can form as nodules in multiple organs, mainly in the lungs and CNS.

Question 12

Best way to Dx sarcoidosis.

Answer 12

CT

-it’s often asymptomatic and found incidentally

Question 13

What are the 5 stages of Sarcoidosis on Radiology?

Answer 13

Stage 0: Normal
Stage 1: Bilateral Hilar Lymphadenopathy
Stage 2: BHL and parenchymal infiltrates
Stage 3: Parenchymal Infiltrates without BHL
Stage 4: End Stage Fibrotic Lung Disease

Question 14

What laboratory levels will be abnormal in patients with sarcoidosis?

Answer 14

Hypercalcemia w/ hypercaliuria

High ACE levels

Question 15

How is sarcoidosis treated?

Answer 15

Since it is a granuloma inflammatory process, corticosteroids work very well to suppress the immune system.

Only serious cases need to be treated since many patients are asymptomatic

Question 16

What is Hypersensitivity pneumonitis and what causes it?

Answer 16

Immune induced, non-IgE mediated, inflammatory lung disease. (originally case was called Farmer’s Lung)

Caused by repeated exposure to an organic causative agent (dusts, allergens)

Question 17

What is the Pathogenesis of Hypersensitivity Pneumonitis?

Answer 17

Mostly Type III hypersensitivy with Immune complex deposition in the parenchyma.

Some cases show Type IV T-cell mediated response leading to parenchymal fibrosis.

Question 18

Best treatment for Hypersensitivity Pneumonitis.

Answer 18

Prevention and Avoidance of the offending agent.

Question 19

What is Cryptogenic Organizing Pneumonia?

Answer 19

AKA: Bronchiolitis Obliterans with or w/o Organizing Pneumonia (BOOP)

It is granulation tissue that obstructs small bronchioles and extends into the distal alveolar ducts causing cough, fever, dyspnea. Mimics pneumonia.