Pathology of Interstitial Lung Disease

Question 1

What are types of Restrictive lung diseases?

Answer 1

External:
-deformed chest wall
-pleural space filled with fluid
Inside Lung
-Edema, interstitial and in air spaces
-cells in interstitium, inflammation, tumor, granuloma, fibrosis
-acellular material: Amyloid, collagen

Question 2

What is Chronic Diffuse intersititial Disease?

Answer 2

Reduced compliance(stiff lungs) => Dyspnea => Hypoxia

Question 3

What is the end-stage result to Interstitial Lung Disease?

Answer 3

Honeycomb lung

Question 4

How are Acute lung injury and ARDS related?

Answer 4

ARDS is worse version of ALI

Diffuse alveolar damage seen in both

Question 5

What are causes of Direct lung injuries?

Answer 5

Infection and Aspir.(common)

O2 Toxicity

Question 6

What are causes of InDirect lung injuries?

Answer 6

Shock and Sepsis (common)
Inhaled Toxins, Drugs
Transfusion related (TRALI)

Question 7

What are the Charicteristics of ALI/ARDS?

Answer 7

Bilateral Infiltrates on CXR

Pulm Capillary Wedge Pressure

Question 8

What chemicals are produced that activate neutrophils in Early ALI and ARDS?

Answer 8

TNF and IL-8, IL-1
Leukotrienes
MIF
PAF
Proteases

Question 9

What is the difference between ARDS in Adults and Neonates?

Answer 9

Adults: Diffuse Damage to alveolar capillary walls
Neo: Def in Surfactant

Question 10

What is Tx of ARDS?

Answer 10

Inhalation of Nitric Oxide

Question 11

What is the Morphology of ARDS?

Answer 11

Lungs Heavy, firm, red, and stiff
Hyaline Membrane
Inc type II pneumocytes

Question 12

What is TRALI?

Answer 12

Transfusion related Acute Lung Injury caused by anti-HLA or anti-HNA

Question 13

What are the categories of Chronic ILD?

Answer 13

Fibrosing: UIP, NSIP, COP, CV, pneumoconiosis: associated with drugs/radiation
Granulomatous: Sarcoidosis, HSPneu
Eosinophilic
Smoking Related: DIP, Resp Bronchilitis

Question 14

What is the end stage of Diffuse Interstitial Disease?

Answer 14

End Stage Fibrotic Lung(honeycomb)

Question 15

What leads to Fibroblstic Foci in IPF?

Answer 15

Abnormal Wound healing leads to fibroblastic/myelofibroblastic proliferation

Question 16

What chemical decreases Length of Telomeres in Epithelial cells of the lung?

Answer 16

TGF-B1 reduces activity of telomerase

Inhibits Caveolin in Fibroblasts

Question 17

What are the charicteristics of early and late IPF?

Answer 17

Early: Alveolitis; Leukocyte infiltration, TII pneumocyte proliferation#### FIBROBLASTIC FOCI
TEMPORAL HETEROGENEITY
Late: Dense Fibrosis => collapse of alveolar walls, formation of cystic spaces

Question 18

What is the only intersitial lung disease that is not responsive to steroids?

Answer 18

UIP(IPF) Need transplant!

Question 19

What is the pulmonary involvement in collagen vascular disorders?

Answer 19

Reumatoid Arthritis
SCLERODERMA
SLE

Question 20

What is Pneumoconiosis?

Answer 20

Non-neoplastic lung rxn to inhalation of irritants in the workplace

• Coal Dust
• Silica
• Asbestos

Question 21

What is the Key factor in severity of Pneumoconiosis?

Answer 21

Capacity of Inhaled dusts to stimulate fibrosis