Interstitial Lung Disease

Question 1

What is Pulmonary Fibrosis?

Answer 1

thickening of the interstitial septum of the lung between the arteriolar space and alveolus interfering with gas exchange in both directions

Question 2

What are commonly associated caused of Pulmonary Fibrosis?

Answer 2

Idiopathic interstitial pulmonary fibrosis
Radiation
Bleomycin, Busulfan
Amiodarone, Methysergide
Nitrofurantoin, Methotrexate

Question 3

How does pulmonary fibrosis present?

Answer 3

Dyspnea worse on exertion
Fine rales or crackles
Loud P2 heart sound
Clubbing of the fingers

Question 4

What is Coal worker’s Pneumoconiosis associated with?

Answer 4

Coal

Question 5

What is Silicosis associated with?

Answer 5

Sandblasting
Rock mining
Tunneling

Question 6

What is Asbestosis associated with?

Answer 6

Shipyard workers
pipe fitting
insulators

Question 7

What is Byssinosis associated with?

Answer 7

Cotton

Question 8

What is Berylliosis associated with?

Answer 8

Electronic manufacturing

Question 9

What is Bagassosis associated with?

Answer 9

Moldy sugar cane

Question 10

What is the best initial test for Pulmonary Fibrosis?

Answer 10

CXR

Question 11

What is the most accurate test for Pulmonary Fibrosis?

Answer 11

Lung Biopsy showing granulomas in Berylliosis

Question 12

What is seen on PFTs in Pulmonary Fibrosis?

Answer 12

Restrictive pattern

DLCO decreased in proportion to severity of thickening

Question 13

What are the treatment options for Interstitial lung diseases?

Answer 13

Prednisone for WBC or inflammatory infiltrate
Azathioprine long-term if response to steroids
Pirfenidoen: antifibrotic agent inhibits collagen synthesis
Nintedanib: TK-inhibitor blocks fibrogenic growth factors and inhibits fibroblasts

Question 14

What is the Mechanism of Pirfenidone?

Answer 14

Antifibrotic: inhibits collagen synthesis

Question 15

What is the Mechanism oF Nintedanib?

Answer 15

Tyrosine-Kinase Inhibitor: blocks fibrogenic growth factors and inhibits fibroblasts

Question 16

What is Sarcoidosis?

Answer 16

Idiopathic inflammatory disorder predominately of the lungs most commonly in African American Women

Question 17

How does Sarcoidosis commonly present?

Answer 17

Young African American Women with SOB on exertion and occasional fine rales without wheezing

Question 18

What is seen on CXR for sarcoidosis?

Answer 18

Hilar Lymphadenopathy

Question 19

What are other common symptoms of sarcoidosis?

Answer 19

Erythema Nodosum
Parotid gland enlargement
Facial Palsy
Heart block and restrictive cardiomyopathy
CNS involvement
Uveitis and Iritis

Question 20

What is seen on CBC in Sarcoidosis?

Answer 20

Polycythemia from chronic hypoxia

Question 21

What is the most accurate test for Sarcoidosis?

Answer 21

Right heart or Swans-Ganz Catheter

Question 22

What are the treatment options for Sarcoidosis?

Answer 22

Prostacyclin Analogues (PA vasodilators)

Endothelin Antagonist

PDE-I

cGMP stimulators

CCB

Oxygen to slow progression

Lung transplant

Question 23

What Prostcyclin analogues are commonly used in sarcoidosis?

Answer 23

Epoprostenol
Treprostinil
Iloprost
Beraprost
Selexipag

Question 24

What Endothelin Anatagonists are commonly used for Sarcoidosis?

Answer 24

Bosentan
Ambrisentan
Macitentan

Question 25

What Phosphodiesterase Ihibitors (PDE-I) are commonly used in Sarcoidosis?

Answer 25

Sildenafil Tadalafil

Question 26

What cGMP stimulator is commonly used in Sarcoidosis?

Answer 26

Riociguat

Question 27

What is the most accurate test for BOOP/COP: Bronchiolitis Obliterans Organizing Pneumonia, Cryptogenic Organizing Pneumonia?

Answer 27

Lung biopsy

Question 28

What is the treatment for BOOP/COP?

Answer 28

Glucocorticoids

Question 29

How do BOOP/COP present?

Answer 29

patchy process with proliferation of granulation tissue in small airways and ducts causing cough, dyspnea, fever, malaise and weight loss