Interstitial Lung Disease

Question 1

Interstitial Lung Disease

Answer 1

Heterogeneous group of diseases characterized by acute, subacute, or chronic infiltration of the interstitium of the lungs by fluid, cells, and connective tissue components.

Question 2

Two most common interstitial lung disease

Answer 2

Idiopathic Pulmonary Fibrosis

Idiopathic nonspecific interstitial pneumonia

Question 3

Pulmonary function tests associated with interstitial lung disease?

Answer 3

Restrictive physiology and impaired gas exchange. Decrease FVC and TLC, preserved FEV/FVC ratio, decreased DLCO, increased A-a gradient.

Question 4

Some classic radiographic manifestations of ILD

Answer 4

Traction bronchiectasis, reticular densities, honeycombing, cysts.

Inflammatory process will have ground glass opacities and consolidation

Question 5

Interstitial Pulmonary Fibrosis Histopathology pattern?

Answer 5

Usual interstitial pneumonia.

Question 6

Median survival for IPF?

Answer 6

2.8 years. 90% mortality in the ICU

Question 7

Clinical features of IPF?

Answer 7

Subacute or chronic illness, insidious onset of dyspnea. Cough, rales, restrictive PFTs. Decreased DLCO decreased A-a gradient.

Question 8

Radiology of Idiopathic pulmonary fibrosis?

Answer 8

Densities at the bottom of the lungs. Begins near the plura and moves inwards.

Question 9

Usual interstitial pneumonia

Answer 9

Histopathologic pattern of idiopathic pulmonary fibrosis.

Question 10

Fibroblast focus

Answer 10

Confirms UIP histopathology.

Question 11

Pathogenesis of IPF

Answer 11

Cell death and impaired reepithelialization. This causes basement membrane damage, leading to the release of growth factors and other products of epithelial cell injury. These growth factors impair repithelialization and cause proliferation of myofibroblasts that do not undergo apoptosis. These fibroblasts cause collagen and matrix remdeling. Finally vascular remodeling too.

Question 12

Genetics of IPF?

Answer 12

25% of patients with IPF have family members with interstitial lung disease. Autosomal dominant pattern of inheritance.

Question 13

Factors associated with decreased survival of IPF

Answer 13

Pulmonary hypertension, low DLCO, decrease in PFTs in 6 months. Fibroblast foci too

Question 14

Two agents used in IPF therapy

Answer 14

Pirfenidone

Nintedanib

Question 15

Pirfenidone

Answer 15

Blocks pro-fibrotic cytokines TGFB and PDGF

Question 16

Nintedanib

Answer 16

Triple tyrosine kinase inhibitor (VEGF, fibroblastGF, PDGF)

Question 17

Acute Interstitial Pneumonia

Answer 17

Catastrophic acute respiratory failure with DAD on pathology. Mimics ARDS with a very high mortality

Question 18

Two smoking related interstital lung disease

Answer 18

Respiratory bronchiolitis interstitial lung disease, desquamative interstitial pneumonia

Question 19

Nonspecific interstitial pneumonia

Answer 19

Better prognosis than usual interstitial pneumonia, associated with connective tissue disorders and drug use. Can be treated

Question 20

Cryptogenic organizing pneumonia

Answer 20

Best prognosis of all, associated with connective tissue disorders and drugs.

Question 21

Some connective tissue diseases

Answer 21

Systemic sclerosis (scleroderma)
Rheumatoid Arthritis
SLE
Dermatomyositis
Polymyositis
Sjogren's Syndrome

Question 22

Connective tissue disorder effect on parenchyma of lung

Answer 22

Inflammation, fibrosis, bleeding

Question 23

CTD effect on airways

Answer 23

Bronchitis, bronchiolitis,bronchiectasis

Question 24

Which CTD causes pleural effusions?

Answer 24

SLE

Question 25

Can CTD’s cause UIP?

Answer 25

Yes

Question 26

What CTDs are associated with nonspecific interstitial pneumonia

Answer 26

Scleroderma, polymyositis/dermatomyositis.

Fair prognosis

Question 27

What drug can cause interstitial lung disease? How to treat?

Answer 27

Nitrofurantoin (which is used to treat UTIs), stopping drug can clear lungs

Question 28

Sarcoidosis

Answer 28

Forms noncaseating granulomas result of an immunological response to an antigenic trigger. Affects multiple organs, lungs in >80%. Diagnosis of exclusion. Infections/noninfectious etiologies must be ruled out.

Question 29

Staging of Sarcoid

Answer 29

0- normal
1-bilateral hilar lymphadenopathy
2 - bilateral hilar lymphadenopathy with bilateral infiltrates
3- just infiltrates
4- pulmonary fibrosis