Baker/Parks: Restrictive Lung Disease

Question 1

Characterized by reduced expansion of lung parenchyma and reduced total lung capacity
Presents with dyspnea, tachypnea, end-inspiratory crackles, cyanosis
Can progress on to 2o Pulm HTN, right heart failure/cor pulmonale

Answer 1

restrictive lung disease

Question 2

What does restrictive lung disease do to FVC? TLC? DLCO? FEV1? FEV1/FVC?

Answer 2

all decrease, but FVC is reduced moreso than FEV1, so FEV1/FVC will usu increase

Question 3

5 types of restrictive lung disease?

Answer 3

fibrosing
granulomatous
eosinophilic
smoking related
other

Question 4

What is happening pathologically in restrictive lung disease?

Answer 4

basically something injures the lung
the lung reacts with inflammation, wound healing and fibrosis
fibrosis leads to amputation of distal airways
distal airways get dilated and look like a honeycomb

Question 5

Due to chronic progressive fibrosis which amputates distal airways

Answer 5

honeycomb lung

**seen in idiopathic pulmonary fibrosis

Question 6

a clinico-pathologic syndrome involving fibrosis of the lung interstitium; a clinical picture (dyspnea + cough) + X-ray and imagine changes (fibrosis on lung CT) + pathologic changes (honeycomb lung).

Answer 6

idiopathic pulmonary fibrosis

Question 7

What are the pathological changes in idiopathic pulmonary fibrosis referred to as?

Answer 7

usual interstitial pneumonia

**this pattern also seen in other disease, such as connective tissue diseases and hypersensitivity pneumonia

Question 8

What might you see grossly in idiopathic pulmonary fibrosis?

Answer 8

diffuse fibrosis

cystic spaces with fibrosis = honeycombing

Question 9

What might you see histologically with idiopathic pulmonary fibrosis?

Answer 9

normal areas alternating with abnormal areas of varying stage
dense subpleural fibrosis

Question 10

What is the new hypothesis for the pathogenesis of idiopathic pulmonary fibrosis?

Answer 10

thought to be due to cyclical lung injury, leading to unusual wound healing and fibrosis

**might be due to TGF-beta from injured pneumocytes inducing fibrosis

Question 11

What are some factors that can increase your risk of developing idiopathic pulmonary fibrosis?

Answer 11

smoking
exposure to metal fumes or wood dust
genetic predisposition (factors that affect pneumocytes)
age >50

Question 12

What is the treatment for IPF?

Answer 12

steroids or immune suppressants

lung transplant**

Question 13

So how does idiopathic pulmonary fibrosis present?

Answer 13

insidious onset with dry cough and dyspnea
onset at age 40-70
hypoxemia and clubbing later on
gradual deterioration

Question 14

Diffuse ILD of unknown etiology (like IPF) but…

Biopsies fail to show diagnostic features of any of the other well-characterized ILDs

Answer 14

nonspecific interstitial pneumonia

Question 15

What are the two subtypes of nonspecific interstitial pneumonia?

Answer 15

cellular: mild to moderate chronic interstitial inflammation, uniform or patchy, occurs in younger and has better outcome
fibrosing: more common, diffuse or patchy interstitial fibrosis, no honeycombing, older population, worse outcome

Question 16

How does nonspecific interstitial pneumonia present? What age group is it seen in?

Answer 16

dyspnea and cough for several months

46-55 yo

Question 17

presents with cough and dyspnea
patchy airspace opacities
long term changes of fibrinous exudate

Answer 17

cryptogenic organizing pneumonia

Question 18

What will you see morphologically in cryptogenic organizing pneumonia?

Answer 18

Masson bodies: polyploid plugs of loose organizing connective tissue
no temporal heterogeneity, interstitial fibrosis, or honeycomb lung
underlying lung architecture normal

Question 19

What can cause cryptogenic organizing pneumonia?

Answer 19

secondary to infections or inflammatory injury
viral or bacterial pneumonia
inhaled toxins
drugs
CT disease
GVHD

Question 20

Pulmonary involvement can occur in these three connective tissue disorders

Answer 20

Rheumatoid arthritis
Systemic sclerosis
SLE

Question 21

Diseases induced by inhalation of organic and inorganic particulates, chemical fumes and vapors.

Answer 21

pneumoconioses

Question 22

Which particles, large or small, are the most dangerous when inhaled?

Answer 22

small particles, sized 1-5 micrometers - these are the perfect size to settle in to the distal airways
also, these particles can move into tissues/fluids and reach toxic levels

**larger particles stay within the lung parenchyma and cause fibrosing collagenous pneumoconioses

Question 23

Which particles are more likely to move into tissues/fluids and cause acute lung injury?

Which particles are more likely to stay in the lung parenchyma and cause fibrosing collagenous pneumoconioses?

Answer 23

small particles

vs

large particles

Question 24

What happens to particles of dust or other small molecules when they are inhaled?

Answer 24

they are ingested by macrophages in the alveoli; the macrophages release mediators which lead to continual cell injury and interstitial fibrosis

Question 25

When particles enter the alveoli after inhalation, (blank) ingest the particles. This causes release of mediators and (blank), which cause continual cell injury. Continual cell injury leads to (blank) proliferation and (blank) deposition.

Answer 25

macrophages; ROS; fibroblast; collagen

Question 26

This is another chemical which can be inhaled, leading to markedly thickened visceral pleura on the lung, and severe interstitial fibrosis

Answer 26

asbestos

Question 27

How can smoking agitate pneumoconcioses (chronic exposure to small particles)?

Answer 27

smoking reduces mucociliary clearance so if there is less clearance, there are more particles retained, which worsens the effect of the inhaled dust or particles **the effect of smoking is particularly magnified in asbestos inhalation

Question 28

List 3 types of inhalation exposure pneumoconioses

Answer 28

Coal workers pneumoconiosis silicosis asbestos

Question 29

Causes a spectrum of disease, from antrocosis (black lung), to simple, to complex disease; usually minimal to no decrease in lung function; no increased risk of cancer or TB

Answer 29

Coal Workers' pneumoconiosis

Question 30

Most prevalent occupational disease in the world Foundry work, sandblasting, mining, stone cutting Slowly progressive from decades of exposure Nodular, fibrosing lesions; increased susceptibility to TB and possibly carcinogenic

Answer 30

Silicosis

Question 31

Abundant lipoproteinaceous material in alveoli | Identical to alveolar proteinosis

Answer 31

acute silicosis

Question 32

What is the most common offender in silicosis?

Answer 32

quartz * *pure quartz is the worst * * causes tiny nodules that eventually coalesce into hard collagenous scars

Question 33

What will you see on chest Xray with silicosis?

Answer 33

upper zone nodules | egg shell calcification in hilar lymph nodes

Question 34

Asbestos exposure affects these two portions of the lung

Answer 34

pleura: pleural effusions, fibrous plaques, diffuse pleural fibrosis, mesothelioma pulmonary parenchyma: lung carcinoma, interstitial fibrosis

Question 35

What are the two forms of asbestos? Which is more common? Which is more likely to cause pathology? Which form is associated with mesothelioma? Which form gets stuck deeper in the lung?

Answer 35

serpentine (curly) and amphibole (needle-like) most common: serpentine (curly) more likely to cause pathology: needle-like associated with mesothelioma: needle-like able to go deep: needle-like

Question 36

Does smoking on top of asbestos exposure increase risk of mesothelioma? Does it increase the risk of lung carcinoma?

Answer 36

no change in mesothelioma risk, but does increase risk of lung carcinoma bc asbestos fibers can absorb toxic chemicals in smoke

Question 37

What will you see in asbestosis?

Answer 37

diffuse pulmonary interstitial fibrosis asbestos bodies (macrophages absorb asbestos fibers) eventual honeycombing similar to UIP

Question 38

What areas of the lung are affected first in asbestosis?

Answer 38

lower lung | subpleura areas

Question 39

How does asbestosis present clinically?

Answer 39

dyspnea on exertion (later dyspnea at rest) productive cough symptoms rare if less than 10 yrs of exposure, more common if greater than 20 yrs exposure

Question 40

What can happen to the pleura with asbestos exposure?

Answer 40

plaque formation! common over domes of diaphragm, but can cover anterior and posterolateral parietal pleura pleural effusion mesothelioma

Question 41

What are the two granulomatous restrictive lung diseases?

Answer 41

sarcoidosis | hypersensitivity pneumonitis

Question 42

``` Systemic Disease of unknown cause Noncaseating granulomas in many tissues and organs Female >> male Blacks >>>>> whites (10x more frequent) Rare in Asian populations ```

Answer 42

sarcoidosis

Question 43

What is a distinguishing feature in sarcoidosis?

Answer 43

noncaseating granulomas in many organs

Question 44

What are 3 likely things that could cause sarcoidosis?

Answer 44

disordered immune regulation (cell mediated response due to an unknown antigen) genetic predisposition environmental exposure (a microbe)

Question 45

Because the granulomas surround the airways in sarcoidosis, what can be done to make the diagnosis of sarcoid?

Answer 45

transbronchial biopsy

Question 46

How will sarcoidosis present on X ray?

Answer 46

hilar lymph nodes affected bilaterally **big hilar lymph nodes in the center of the X-ray - can't miss em!

Question 47

How do people with sarcoidosis present?

Answer 47

fever, cough, dyspnea hours after exposure to an inhaled organic antigen other symptoms: chest pain coughing up blood fever, fatigue, weight loss, anorexia, night sweats

Question 48

What happens to calcium levels in sarcoidosis?

Answer 48

hypercalcemia

Question 49

T/F: In sarcoidosis, virtually no organ is spared!

Answer 49

True **lung, lymph nodes, spleen, liver, bone marrow, skin, etc etc

Question 50

``` Immune mediated, mostly interstitial lung disorders caused by abnormal sensitivity/reactivity to an inhaled organic antigen Many syndromes - named by association Farmer’s lung Pigeon breeder’s lung Humidifier/Air-conditioner lung ```

Answer 50

hypersensitivity pneumonitis

Question 51

Unlike asthma, hypersensitivity involves the (blank)

Answer 51

alveoli

Question 52

What are some morphological features of hypersensitivity pneumonitis?

Answer 52

``` Centered around bronchioles Interstitial pneumonitis Lymphocytes, plasma cells, macrophages Noncaseating granulomas Interstitial fibrosis, honeycombing, obliterative bronchiolitis (late) ```

Question 53

What should be done to resolve hypersensitivity pneumonitis?

Answer 53

early recognition and removal of causative agent!! **if not removed, can lead to serious chronic fibrotic disease

Question 54

Infiltration of eosinophils in the lungs

Answer 54

pulmonary eosinophilia

Question 55

Focal areas of consolidation with lymps and eosinophils Fever, night sweats, dyspnea Responds well to steroids

Answer 55

chronic eosinophilic pneumonia

Question 56

What can cause secondary eosinophilia?

Answer 56

``` infection drug rxn asthma vasculitis aspergillosis ```

Question 57

Accumulation of acellular surfactant in the intra-alveolar and bronchiolar spaces Three types Acquired Congenital Fatal, typically noted in the newborn that develops rapidly progressive respiratory distress. 3-6 mo w/o tranplant Secondary Uncommon. Due to malignancies, immunodeficiencies, silicosis…etc.

Answer 57

pulmonary alveolar proteinosis

Question 58

Of the three types of pulmonary alveolar proteinosis, which is the most common? What is it likely caused by? How do you treat it?

Answer 58

acquired form makes up 90% of cases; likely due to antibody to granulocyte-macrophage colony stimulating factor; whole lung lavage is the standard treatment