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Flashcards in Interstitial Lung Disease Deck (19):

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

Lung Biopsy
-surgical is needed b/c a large sample required
-bronchoscopic not sufficient


Most common type of Idiopathic Interstitial Pneumonia

Idiopathic Pulmonary Fibrosis (IPF)

Usual Interstitial Pneumonia (UIP) pattern on Bx


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



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

Desquamative Interstitial Pneumonia
Respiratory Bronchiolitis IP


What other conditions can show a UIP pattern on biopsy?

Collagen Vascular Disease
Drug Induced lung disease
Occupational or Environmental disorders


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

Death within 3-8 years
-cough becomes debilitating, cyanosis and cor pulmonale occur in later stages


Diagnostic test of choice for IPF.

High Resolution CT scan (HRCT)


How does IPF appear on CT?

Bilateral Peripheral opacities in the base of the lung


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

Lung Transplant


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

DIP shows a more diffuse infiltrate in the tiny air spaces

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


What is Sarcoidosis?

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


Best way to Dx sarcoidosis.

-it's often asymptomatic and found incidentally


What are the 5 stages of Sarcoidosis on Radiology?

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


What laboratory levels will be abnormal in patients with sarcoidosis?

Hypercalcemia w/ hypercaliuria
High ACE levels


How is sarcoidosis treated?

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


What is Hypersensitivity pneumonitis and what causes it?

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)


What is the Pathogenesis of Hypersensitivity Pneumonitis?

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

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


Best treatment for Hypersensitivity Pneumonitis.

Prevention and Avoidance of the offending agent.


What is Cryptogenic Organizing Pneumonia?

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.