2.2.1 Restrictive Pulmonary Diseases

Question 1

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

Answer 1

Decreased TLC

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

Question 2

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

Answer 2

Effective loss of lung tissue

Decrease in lung’s ability to expand (compliance)

Decrease in gas exchange

Question 3

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 3

Interstitial lung dz

Question 4

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

Answer 4

Obesity, Pleural dz, Kyphoscoliosis, Neuromuscular dz

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 interstitial pneumonais?

Answer 6

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

Question 7

What are the five categories of interstitial lung dz?

Answer 7

Fibrosing

Granulomatous

Eosinophilic

Smoking-related

Other

Question 8

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

Answer 8

Idiopathic pulmonary fibrosis (UIP)

Non-specific interstitial pneumonia (NSIP)

Cryptogenic Organizing Pneumonia (COP or BOOP)

Drug

Connective Tissue Dz

Pneumoconiosis

Question 9

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

Answer 9

Sarcoidosis and hypersensitivity pneumonitis

Question 10

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

Answer 10

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

Question 11

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

Answer 11

Reduced CO diffusion capacity

Reduced lung volume

Reduced compliance

Question 12

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

Answer 12

Question 13

What is the typical histiologic pattern of IPF?

Answer 13

Usual interstitial pneumonia (UIP)

Question 14

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 14

Idiopathic pulmonary fibrosis (IPF)

Question 15

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

Answer 15

Spatially and temporally heterogenous

Subpleural and interlobular septal distribution

Question 16

What are the arrows pointing at?

Answer 16

Fibroblastic foci

Question 17

What might this patient have?

Answer 17

IPF

Question 18

What is the median survival time of IPF patients?

Answer 18

3 years

Question 19

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

Answer 19

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

Question 20

Answer 20

Cryptogenic Organizing Pneumonia

Question 21

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

Answer 21

Pneumonconiosis

Question 22

What are three examples of pneumoconiosis?

Answer 22

Asbestosis, Silicosis, Coal Workers Pneumoconiosis

Question 23

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

Answer 23

mining, milling, and fabrication

Question 24

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

Answer 24

asbestos: 5 fold increase

asbestos + smoker: 55 fold increase

Question 25

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

Answer 25

20 yr lag

Question 26

What are the two arrows pointing at?

Answer 26

Silica particles

Question 27

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

Answer 27

Sarcoidosis

Question 28

What are some components of the pathogenesis of sarcoidosis?

Answer 28

Cell-mediated immune response to unknown Ag

CD4 Th

Increased TNF

HLA-A1 and HLA-B8 (genetic factors)

Environment

Question 29

Name that condition.

Answer 29

Sarcoidosis

Question 30

What might these be?

Answer 30

Noncaseating granulomas of sarcoidosis

Question 31

What are the the race and gender specificities of sarcoidosis?

Answer 31

Women > men

AA > European descent

Question 32

What is the treatment for sarcoidosis?

Answer 32

STEROIDS

Question 33

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

Answer 33

Hypersensitivity pneumonitis

Question 34

What does she got, doc?

Answer 34

Hypersensitivity pneumonitis

Question 35

What are some syndromes that are classified under hypersensitivity pneumonitis?

Answer 35

Bird fancier’s dz (bird stuff)

Farmer’s lung (moldy hay)

Bagassosis (moldy sugar cane)

Humidifer lung (thermophilic bacteria)

Question 36

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

Answer 36

Pulmonary Langerhans cell histiocytosis/eosinophilic granuloma

Question 37

What are the lower set of arrows pointing at?

Answer 37

Langerhans cells

Question 38

Rare dz characterized by bilateral patchy asymmetric pulmonary opacification on imaging

Answer 38

Pulmonary alveolar proteinosis

Question 39

Name that conditioin.

Answer 39

Respiratory Bronchitis or Desquamative interstitial pneumonia