Path: Restrictive Lung Disease

Question 1

What does a honeycomb lung appearance signify?

Answer 1

End Stage lung disease

Question 2

Histologic pattern seen in Idiopathic Pulmonary Fibrosis (IPF).

Answer 2

Usual Interstitial Penumonia

Question 3

What cell types undergo hyperplasia in IPF?

Answer 3

Type II pneumocytes

Fibroblasts

Question 4

Tx for IPF

Answer 4

Lung Transplant

Question 5

How do IPF and Nonspecific Interstitial Pneumonia appear different on biopsybased on fibrosing pattern?

Answer 5

IPF has temporal heterogeneity
-you will see both early and late signs of collagen and fibrosis deposition along with areas of normal lung tissue in the same biopsy.

Question 6

Tx for nonspecific Interstitial Pneumonia

Answer 6

Steroids

Question 7

Tx for Cryptogenic Organizing Pneumonia (Bronchiolitis obliterans organizing pneumonia: or BOOP)

Answer 7

Oral Steroids

Question 8

Characteristic microscopic finding indicating BOOP.

Answer 8

Masson Bodies: polypoid plugs of loose organizing connective tissue

Question 9

General cause of Pneumoconioses

Answer 9

Inhalation of mineral dust

Question 10

What 2 conditions can result from chronic simple coal workers pneumoconiosis?

Answer 10

1. Centrilobular Emphysema
   - if dilation of adjacent alveoli occurs
2. Progressive Massive Fibrosis

Question 11

Does coal workers pneumoconiosis (CWP) increase risk of lung cancer?

Answer 11

No

Question 12

Who is at risk of developing Silicosis (type of pneumoconiosis)?

Answer 12

Foundry workers
Sandblasters
Stone Cutters

Question 13

What is the pathogenesis of Silicosis?

Answer 13

Inhalation of the particles causes macrophages to release mediators
-TNF, IL, free radicals, cytokines, stimulate fibroblasts

Question 14

How does silicosis progression differ from CWP?

Answer 14

Progression occurs even if the exposure is removed in Silicosis.

Question 15

What is viewed on microscopy in lung tissue with silicosis?

Answer 15

Birefringent needle like crystals piercing the tissue under polarized light

Question 16

Name the 2 types of asbestos that can cause asbestosis.

Answer 16

1. Serpentine: curly, flexible

2. Amphiboles: straight, stiff

Question 17

Why does smoking increase risk of asbestosis?

Answer 17

The silica crystals (asbestos) that cause asbestosis are removed by the mucociliary elevator which does not function in smokers

Question 18

What gross finding can be seen on lung tissue in a patient that had asbestosis.

Answer 18

Pleural Plaques

Question 19

What is Caplan Syndrome?

Answer 19

Rheumatoid Arthritis patients that are exposed to mining dusts that develop fibrosis in the lungs. Granulomatous inflammation occurs.

Question 20

How is Sarcoidosis diagnosed?

Answer 20

Dx of exclusion. Non-Caseating granulomas are present and all other diseases are ruled out.

Question 21

Treatment for sarcoidosis.

Answer 21

Steroids

-it’s thought to be an autoimmune rxn or a malfunction of the immune system

Question 22

What are Schaumann Bodies?

Answer 22

Name for the non-Caseating granulomas in sarcoidosis.

same for asteroid bodies, the names just describe the pattern seen in microscopy

Question 23

What is the cause for Hypersensitivity Pneumonitis?

Answer 23

Intense, prolonged exposure to inhaled organic dusts and occupational antigens, fungi, animal proteins etc.

Question 24

Cause of Tropical Pulmonary Eosinophilia.

Answer 24

Wuchereria bancrofti

Question 25

Best Tx for Desquamative Interstitial Pneumonia.

Answer 25

Stop Smoking

-disease process reverses

Question 26

Best Tx for Respiratory Bronchiolitis-Associated Interstitial Lung Disease.

Answer 26

Stop Smoking

-disease process reverses

Question 27

Pathogenesis of Acquired Alveolar Proteinosis

Answer 27

Autoimmune

-Anti-granulocyte-macrophage colony stimulating factor antibody

Question 28

Tx for congenital Alveolar Proteinosis.

Answer 28

Lung transplant

-gene mutation doesn’t allow proper lung development