2.2.1 Restrictive Pulmonary Diseases

Question 1

What is the treatment for sarcoidosis?

Answer 1

STEROIDS

Question 2

What are the lower set of arrows pointing at?

Answer 2

Langerhans cells

Question 3

What are some possible pulmonary or extrapulmonary causes of restrictive lung dz?

Answer 3

Effective loss of lung tissue

Decrease in lung’s ability to expand (compliance)

Decrease in gas exchange

Question 4

What does she got, doc?

Answer 4

Hypersensitivity pneumonitis

Question 5

What are the lungs’ responses to the different severities of injury (minor, moderate, severe)?

Answer 5

Minor: emphysema

Moderate: interstitial fibrosis

Sever: scar

Question 6

What are some industries that could expose an individual to abestos?

Answer 6

mining, milling, and fabrication

Question 7

Rare dz characterized by bilateral patchy asymmetric pulmonary opacification on imaging

Answer 7

Pulmonary alveolar proteinosis

Question 8

Characteristic histopathologic lesions are polypoid plugs of loose organizing connective tissue within small airways, alveolar ducts, and the surrounding alveoli

Answer 8

Cryptogenic organizing pneumonia (COP), formerly known as bronchiolitis obliterans-organizing pneumonia (BOOP)

Question 9

What are some syndromes that are classified under hypersensitivity pneumonitis?

Answer 9

Bird fancier’s dz (bird stuff)

Farmer’s lung (moldy hay)

Bagassosis (moldy sugar cane)

Humidifer lung (thermophilic bacteria)

Question 10

What are some common physiological manifestations (3) of restrictive pulmonary dz?

Answer 10

Reduced CO diffusion capacity

Reduced lung volume

Reduced compliance

Question 11

What are interstitial pneumonais?

Answer 11

Group of inflammatory and fibrosing disorders affecting the alveolar septae and other supporting structures of the lung rather than the airspaces.

Question 12

What might these be?

Answer 12

Noncaseating granulomas of sarcoidosis

Question 13

What are two types of granulomatous interstitial lung dz’s?

Answer 13

Sarcoidosis and hypersensitivity pneumonitis

Question 14

What are the two arrows pointing at?

Answer 14

Silica particles

Question 15

Name that conditioin.

Answer 15

Respiratory Bronchitis or Desquamative interstitial pneumonia

Question 16

What is the typical histiologic pattern of IPF?

Answer 16

Usual interstitial pneumonia (UIP)

Question 17

Interstitial lung dz w/ proliferation of Langerhans cells resulting in nodules.

Answer 17

Pulmonary Langerhans cell histiocytosis/eosinophilic granuloma

Question 18

What is a classic characteristic of langerhan’s histiocytosis?

Answer 18

Birbecks’s granules and pneumothorax

Question 19

What are some components of the pathogenesis of sarcoidosis?

Answer 19

Cell-mediated immune response to unknown Ag

CD4 Th

Increased TNF

HLA-A1 and HLA-B8 (genetic factors)

Environment

Question 20

How does asbestos affect the risk for lung cancer? abestos and a smoker?

Answer 20

asbestos: 5 fold increase

asbestos + smoker: 55 fold increase

Question 21

Insidious onset (slow, progessive), 55-75 years at presentation, dyspnea and dry cough, subsequent hypoxemia, cyanosis, and clubbing.

These are all chacterisitics of which restrictive pulmonary dz?

Answer 21

Idiopathic pulmonary fibrosis (IPF)

Question 22

What is the median survival time of IPF patients?

Answer 22

3 years

Question 23

What are two characterisitics of the histological pattern usual interstitial pneumonia?

Answer 23

Spatially and temporally heterogenous

Subpleural and interlobular septal distribution

Question 24

What are the the race and gender specificities of sarcoidosis?

Answer 24

Women > men

AA > European descent

Question 25

Non-neoplastic reaction of the lung to inhaled mineral dust, organic particles, or chemical vapors

Answer 25

Pneumonconiosis

Question 26

Which two conditions fall into the other category of interstitial lung dz's?

Answer 26

Pulmonary alveolar proteinosis and Langerhan's cell histiocytosis/eosinophilc granuloma

Question 27

What are the arrows pointing at?

Answer 27

Fibroblastic foci

Question 28

A multisystem dz that leads to the formation of noncaseating granulomas.

Answer 28

Sarcoidosis

Question 29

Name that condition.

Answer 29

Sarcoidosis

Question 30

What are the five categories of interstitial lung dz?

Answer 30

Fibrosing Granulomatous Eosinophilic Smoking-related Other

Question 31

Answer 31

Cryptogenic Organizing Pneumonia

Question 32

Restrictive pulmonary dz is characterized by reduced TLC. If the cause of this dz is pulmonary, this is referred to as what type of dz?

Answer 32

Interstitial lung dz

Question 33

Describe the clinical symptoms that are associated with the physiological changes in restrictive pulmonary dz.

Answer 33

Question 34

Name some conditions that are classified as fibrosing interstitial lung dz's?

Answer 34

**Idiopathic pulmonary fibrosis (UIP)** Non-specific interstitial pneumonia (NSIP) **Cryptogenic Organizing Pneumonia (COP or BOOP)** Drug Connective Tissue Dz **Pneumoconiosis**

Question 35

What are some conditions that result in extrapulmonary restriction of the lungs?

Answer 35

Obesity, Pleural dz, Kyphoscoliosis, Neuromuscular dz

Question 36

What are three examples of pneumoconiosis?

Answer 36

Asbestosis, Silicosis, Coal Workers Pneumoconiosis

Question 37

What is the suspected lag b/t asbestos exposure and the development of mesothelioma?

Answer 37

20 yr lag

Question 38

What results on a PFT would lead you to believe a patient was suffering from a restrictive lung dz?

Answer 38

Decreased TLC Normal FEV1/FVC (both proportionally reduced, if anything this might be increased)

Question 39

What might this patient have?

Answer 39

IPF

Question 40

Spectrum of immunologically mediated, predominatly interstitial, lung disorders caused by intense, often prolonged exposure to inhaled organic Ag

Answer 40

Hypersensitivity pneumonitis