Restrictive Pulmonary Diseases

Question 1

This is the inflammation and fibrosis of the pulmonary connective tissue over time.

Answer 1

Chronic interstitial (restrictive) dieases

Question 2

What are the 4 classic Sx for restrictive diseases?

Answer 2

Dyspnea
Tachypnea
End-inspiratory crackles
Cyanosis

Question 3

The diffusing capacity for which molecule is decreased in restrictive conditions?

Answer 3

CO

Question 4

Why do we give 2 shits about the diffusion of CO? What does it tell us?

Answer 4

The Diffusing Capacity of the Lungs for CO (DLCO) is a measurement of the ability of the lungs to transfer gas across the alveoli. in restrictive diseases, it’s ↓

Question 5

What are the 3 things on CXR for restrictive diseases?

Answer 5

b/l infiltrates of small nodules, irregular lines, or ground-glass shadows

Question 6

What is the end result of restrictive diseases? (think <3)

Answer 6

Pulmonary HTN –> RHF

cor pulmonale

Question 7

This is the fribrosis of the lung interstitum from cyclical lung injury of epithelial activation from an unkown agent,

Answer 7

Idiopathic Pulmonary Fibrosis (IPF)

Question 8

Which mediator is responsible for the the cyclical healing and fibrosis in IPF?

Answer 8

TGF-beta

Question 9

What is the morphology of the pleural surface in IPF?

Answer 9

Cobble stone appearance from scarring

Question 10

What is the morphology of the alveolar walls in IPF?

Answer 10

sooooper doooooper THICK

Question 11

What is the morphology of the interstitum in IPF?

Answer 11

patchy interstitial fibrosis –> honeycomb fibrosis

Question 12

What are the main Sx for IPF?

Answer 12

Gradual increasing dyspnea, dry cough

Question 13

True or False: in nonspecific interstitial PNA, there is diffuse patchy interstitial fibrosis with fibroblastic foci and honeycombing, which makes it hard to distinguish from IPF.

Answer 13

FALSE. THere is no fibroblastic foci or honeycombing.

Question 14

What causes nonspecific interstitial PNA?

Answer 14

nobody knows…

Question 15

This is the disease of unknown origin where there is cough, dyspnea, subpleural/peribronchial patchy areas of consolidation with polypoid plugs of loose organizing tissue.

Answer 15

Cryptogenic Organizing PNA

Question 16

What is the Tx for IPF?

Answer 16

Transplant

Question 17

Thse are non-neoplastic lung reactions to chronic inhalation of mineral dusts that are fibrogenic.

Answer 17

Pneumoconioses

Question 18

Which cells eats the small particles and triggers fibrosis in Pneumoconioses?

Answer 18

Macrophages

Question 19

True or False: the smaller the particle, the more rapid the toxic levels achieved, and the faster the acute lung injury.

Answer 19

True

Question 20

Which substance causes coal workers pneumoconiosis (CWP)?

Answer 20

Carbon

Question 21

This is a disease of CWP where the inhaled carbon piugment is engulfed by macrophages which then accumulate in the lungs connective tissue along with the lymphatics.

Answer 21

Athracosis

Question 22

Does a mild or severe exposure of carbon cause anthracocis in CWP?

Answer 22

Mild

Question 23

This is the type of CWP where there are coal macules (carbon-laden macrophages) and somewhat larger coal nodules in the upper lobes and middle lung.

Answer 23

Simple CWP

Question 24

This is the form of CWP where there is multiple intensely blackened scars of dense collagne and pigment that is often necrotic.

Answer 24

Complicated CWP

Question 25

What is the high-yield disease association with CWP?

Answer 25

Rheumatoid Arthritis | causes Caplan syndrome

Question 26

What % of CWP pts get progressive massive fibroisis, leading to increasing pulmonary dysfxn, pulmonary HTN, and cor pulmonale?

Answer 26

< 10%

Question 27

What is the susbtance inhaled to cause silicosis pneumoconiosis?

Answer 27

Silica dust

Question 28

Who is at risk for silicosis?

Answer 28

Miners and sandblasters

Question 29

What is the morpholgy and location for silicosis in the lung?

Answer 29

fibrotic nodules in the upper lobes

Question 30

Inhalation of silica causes it to interact with epithelial cell and macrophages, leading to the release of what?

Answer 30

mediators (like TNF)

Question 31

These are thin sheets of calcification in the lymph nodes in silicosis

Answer 31

Eggshell calcification

Question 32

The nodules in silicosis are hylaninized collagen surrounded by a capsule of what?

Answer 32

Of more codensed collagen collagen on collagen... a collagen sandwhich.

Question 33

What are the classic Sx to silicosis?

Answer 33

shortness of breath, slow to kill, progressive even if pt is no longer exposed.

Question 34

What is the pt susceptible to in silicosis?

Answer 34

TB UPPER LUNG = SILICOSIS AND TB. I can't wait for this to be a test question. It will. OMG it will.

Question 35

*Additional Pathoma* This is the penumoconiosis in miners/aerospace workers that has noncaseating granulomas, hilar lymph nodes, and organs involved.

Answer 35

Berylliosis

Question 36

*Additional Pathoma* Berylliosis increases the risk for what?

Answer 36

Lung CA

Question 37

This is the pneumoconiosis of construction workers/plumers/shipyard workers where there is fibrosis of the lungs and pleura.

Answer 37

Asbestosis

Question 38

What is the most common form of asbestos, serpentine or amphibole?

Answer 38

Serpentine

Question 39

Where does serpentine asbestos impact?

Answer 39

URT

Question 40

Why is amphibole asbestos more rare but more pathogenic?

Answer 40

Dur to its aerodynamic properties (like a spear), causes mesothelioma

Question 41

Both forms of asbestos are fibrogenic and increase the risk of CA by doing what?

Answer 41

Generates free radicles

Question 42

There is a big correlation between lung carcinoma and what habit of asbestos workers?

Answer 42

Smoking

Question 43

Thse are golden brown fusiform or beaded rods with a translucent center with Fe.

Answer 43

Asbestos body

Question 44

What is the mrophology of the lung in asbestosis?

Answer 44

Diffuse interstitial fibrosis with multiple asbestos bodies.

Question 45

So there is fibrosis of what area in asbestosis, lung or pleura?

Answer 45

TRICK QUESTION. Both.

Question 46

So there is cancer development in which area from asbestosis, lung or pleura?

Answer 46

TRICK QUESTION AGAIN. Both.

Question 47

What is the common Sx for asbestosis?

Answer 47

Progressive dyspnea with productive cough 10-20 years after exposure.

Question 48

What shows up on CXR for asbestosis?

Answer 48

irregular linear densitis (esp lower lobes), possible pleural effusion

Question 49

Which cancer is pretty unique to asbestosis, carcinoma or mesothelioma?

Answer 49

Mesothelioma

Question 50

Which cancer is more COMMON in asbestosis, carcinoma or mesothelioma?

Answer 50

Carcinoma

Question 51

What are the 2 common drugs to cause IPF?

Answer 51

Bleomycin and Amiodarone

Question 52

What therapy can induce IPF from direct damage?

Answer 52

Radiation

Question 53

Methotrexate and Nitrofurantoin can cause what problems in the lung?

Answer 53

hypersensitivity pneumonitis

Question 54

This is the form of radiation pneumonitis where there is fever, dyspnea, pleural effusion and radiologic infiltrates 1-6mo after fractionated irradiation.

Answer 54

Acute radiation pneumonitis

Question 55

Chronic radiation pneumonitis is the consequence of repair of the injured endothelial/epithelial cells and severe atypia of which lung cells?

Answer 55

Type II cells and fibroblasts

Question 56

This is a systemic disease of unknown cause characterized by noncaseating granulomas in many tissues and organs.

Answer 56

Sarcoidosis

Question 57

What 2 things do u see on CXR for sarcoidosis?

Answer 57

bilateral hilar lymphadenopathy or lung involvement

Question 58

Who is at risk for sarcoidosis?

Answer 58

Black women from the south east

Question 59

What type of immune response causes sarcoidosis?

Answer 59

CD4+ Th activation to unknown Ag

Question 60

What is the morphology of the noncaseating granulomas for sarcoidosis?

Answer 60

aggregte of lightly clustered EPITHELIOID cells with Shaumann bodies and stellate inclusions (ASTEROID BODIES) in giant cells.

Question 61

What are the Sx to eye involvement in sarcoidosis?

Answer 61

Uveitis

Question 62

What are the Sx to lymph node involvement in sarcoidosis?

Answer 62

Enlarged, calcified, hilar and mediastinal nodes involved

Question 63

What are the Sx to bone marrow involvement in sarcoidosis?

Answer 63

small circumscribed areas of bone resporption within the marrow cavity and diffuse reticulated pattern throughout the cavity.

Question 64

Dry eyes and mouth mimic which syndrome in sarcoidosis?

Answer 64

Sjoren syndrome

Question 65

What are the Sx to skin involvement in sarcoidosis?

Answer 65

subQ nodules Erythmateous plaques Flat scaly slightly reddened lesions

Question 66

What are the Sx of onset in sarcoidosis?

Answer 66

Respiratory abnormalitits (SOB, cough, chest pain, hemoptysis)

Question 67

What 2 things are increased on blood exams for sarcoidosis?

Answer 67

1. ACE | 2. Ca++ (hypercalcemia from 1a-hydroxylase activity of granulomas)

Question 68

Most patients recover w/o Tx for sarcoidosis, but if they need help, what is the class of drugs u can give?

Answer 68

Steroids

Question 69

This is a spectrum of immunologically mediated, interstitial lung disorders caused by intense, prolonged exposure to inhaled organic Ag's.

Answer 69

Hypersensitivity penumonitis

Question 70

What type of hypersensitivity Rxn is Hypersensitivity penumonitis?

Answer 70

Type IV | T-cell mediated

Question 71

What 4 things show up on the lungs in Hypersensitivity penumonitis?

Answer 71

1. Noncaseating granulomas 2. Interstitial pneumonitis 3. Interstitial Fibrosis/honeycombing/obliterative bronchiolitis 4. Intra-alveolar infiltrate

Question 72

What are the Sx to Hypersensitivity penumonitis?

Answer 72

Fever, cough, dyspnea after exposure, gets better after removing exposure.

Question 73

You can get interstitial fibrosis after what time in Hypersensitivity penumonitis?

Answer 73

Chronic exposure to allergen

Question 74

Which WBC is elevated in Hypersensitivity penumonitis?

Answer 74

Eosinophils | it's a hypersensitivity rxn

Question 75

This is the infiltration of eosinophils in the lungs from high levels of alveolar levels of attractants, like IL-5.

Answer 75

Pulmonary eosinophilia

Question 76

What causes acute eosinophilic PNA with respiratory failure?

Answer 76

Unknown

Question 77

Simple pulmonary eosinophilia (Leoffler syndrome) shows what in the lungs?

Answer 77

Transient pulmonary lesions

Question 78

Infections with microfilarie can cause what type of pulmonary eosinophilia?

Answer 78

Tropical eosinophilia

Question 79

Other infections, hypersensitivity pneumonitis, or drug allergies can trigger which type of pulmonary eosinophilia?

Answer 79

secondary eosinophilia

Question 80

This is the form of pulmonary eosinophilia where there are focal areas of cellular consolidation (lymphocytes and eosinophils) of the lung substance distributed chiefly in the periphery of the lung fields.

Answer 80

Idiopathic chronic eosinophilic PNA

Question 81

Desquamative interstitial pneumonia (DIP) and respiratory bronchiolitis-associated interstitial lung disease (RBAILD) are associated with what?

Answer 81

Smoking

Question 82

DIP has large collections of which cells tpye in airspaces>

Answer 82

macrophages

Question 83

What are in the macrophages in DIP?

Answer 83

dark brown (Fe) pigment | called smokers macrophages

Question 84

What happens to the alveolar septa in DIP?

Answer 84

It thickens from inflammatory rxns

Question 85

What are the Sx to DIP?

Answer 85

suddent onset dyspnea and dry cough, clubbing

Question 86

What is the 100% effective Tx for DIP?

Answer 86

STOP SMOKING U IDIOT | steroids help tooooo

Question 87

RBAILD has the presence of pigmented intraluminal macrophages where in the lung?

Answer 87

Respiratory bronchioles

Question 88

What are the manifestations of RBAILD?

Answer 88

severe pulmonary Sx, abnrml pulm fxn, imaging abnormalitis, centriolobular emphysema

Question 89

How do u improve the clinical course of RBAILD?

Answer 89

STOP. SMOKING. STOP IT. STOP.

Question 90

This is where there is bilateral patchy asymmateric pulmonary opacification and accumulation of acellular surfactant in intraalveolar and bronchiolar spaces.

Answer 90

Pulmonary Alveolar Proteinosis (PAP)

Question 91

Are 90% of PAP cases acquired or genetic?

Answer 91

Acquired (autoimmune disorder)

Question 92

What are the Sx to congential PAP?

Answer 92

immediate-onset neonatal respiratory distress, fatal within 3-6mo w/o a transplant

Question 93

What is the morphology of the alveoli in PAP?

Answer 93

Homogenous, granular precipitate, large consolidation of the lungs.

Question 94

What test is + for the alveolar precipitate in PAP?

Answer 94

PAS+ | PAS for PAP

Question 95

What is coughed up in adults with PAP?

Answer 95

large CHUNKS of gelatinous material

Question 96

Is sarcoidosis related to smoking?

Answer 96

No

Question 97

What are the 0-4 stages to classify sarcoidosis on CXR?

Answer 97

``` Stage 0- normal CXR Stage 1- hilar adenopathy Stage 2- hilar adenopathy w/ pulmonary infiltrates Stage 3- infiltrates w/o adenopathy Stage 4- fibrotic lung disease ```

Question 98

Which elecrolyte is elevated in sarcoidosis?

Answer 98

Ca++

Question 99

What is the first line therapy for sarcoidosis?

Answer 99

Steroids

Question 100

What enzyme is elevated in sarcoidosis?

Answer 100

ACE

Question 101

Which pts are at risk for lymphangioleiomyomatosis?

Answer 101

Women of childbearing age

Question 102

What are the Sx to lymphangioleiomyomatosis?

Answer 102

Recurrent pneumothoraces, chylous pleural effusions, diffuse infiltrates with hypoxemia, and airflow obstruction

Question 103

What are scattererd throughout the lungs in lymphangioleiomyomatosis?

Answer 103

Well-defined cysts

Question 104

Which hormone worsens the disease in lymphangioleiomyomatosis?

Answer 104

Estrogen

Question 105

What is the Tx for chronic hypersensitivity pneumonitis?

Answer 105

Corticosteroids

Question 106

WHAT ORGANISM IS PATIENTS WITH SILICOSIS AT RISK FOR?

Answer 106

MYCOBACTERIUM TB OMG LEARN THIS.

Question 107

What are the 4 causes of ARDS?

Answer 107

Sepsis PNA Pancreatitis Trauma