Bod: Respiratory: Restrictive Lung Disease

Front 1

Permeability edema is not associated with what?

Back 1

Cardiac dysfunction

Front 2

What are the signs and symptoms of the ARDS syndrome?

Back 2

1. Dyspnea
2. Progressive hypoxemia
3. Bilateral radiographic lung infiltrates.
4. Decreased pulmonary complicance.

Front 3

What is one histiological feature that is often seen in ARDS? (hint: membranes)

Back 3

Hyaline membranes plastered against alveolae. Fluid plasma protienacious components.

Front 4

What are the three main causes of ARDS?

Back 4

1. Sepsis
2. Trauma
3. Shock

Front 5

What are the three stages of ARDS/DAD?

Back 5

1. Exudative
2. Proliferative
3. Fibrotic

Front 6

Explain the exudative phase of DAD.

Back 6

Edema and hemorrhage.

- First 4-7 days
- Lungs are heavy (1000 g)
- Visceral pleura is red-blue and focally hemorrhagic
- Eosinophilic HYALINE MEMBRANES
- Interstitial and alveolar edema

Front 7

Explain the proliferative phase of DAD.

Back 7

Organization and repair.

- 1-3 weeks
- lungs weigh more than 2000 grams
- Lungs are rigid and pale red.
- HYPERPLASTIC TYPE 2 CELLS (quite reactive)

Front 8

Normal weight of lungs?

Back 8

Combined weight of about 800 grams. Right is heavier than left.

Front 9

Explain the fibrotic phase of DAD.

Back 9

End-stage fibrosis.

- 3-4 weeks
- Collagen in alveolar walls WIDENS ALVEOLAR SEPTA
- Fibrotic microcysts

Front 10

What are the three main histologic changes for the three phases of DAD?

Back 10

Exudative - hyaline membranes
Proliferative - type II hyperplaisia
Fibrotic - fibrosis

Front 11

Is DAD idiopathic or non-idiopathic?

Back 11

Acute and non-idiopathic.

Front 12

What does BOOP stand for? What are other names for BOOP?

Back 12

BOOP = Brochiolitis Obliterans Organizing Pneumonia.

Other names:
- Cryptogenic organizing pneumonia.
- Organizing pneumonia-like lesion
- Unresolved/chronic pneumonia

Front 13

What are the major histologic changes of BOOP?

Back 13

1. Patchy (not diffuse) brochiolitis obliterans with organizing pneumonia. Bronicholitis obliterans is manifested as intraluminal polyps containing hisitocytes, lymphocytes, plasma cells (no giant cells/granulomas).

2. Interstitial mononuclear cell infiltrate, variable in density.

3. ABSENCE of honeycombing or extensive interstitial fibrosis.

Front 14

What does UIP stand for ? Is it idiopathic?

What are some synonyms for UIP?

Back 14

Usual initerstitial pneumonia. UIP is chronic and usually idiopathic (may occur in the setting of SLE or rheumatoid disease).

UIP is also known as:
- IPF (Interstitial Pulmonary Fibrosis)
- Fibrosing alveolitis
- Chronic interstitial pneumonia

Front 15

UIP demographics?

Back 15

50-60
Male predominance

Front 16

UIP findings with diagnostic testing.

Back 16

Interstitial markings on X-ray (especially in the lower lobes). Can also see loss of lung volume.

Decreased TLC, VC

Diffusing capacity is reduced.

Hypoxemia

Front 17

UIP is the same as what?

Back 17

UIP = IPF (idiopathic pulmonary fibrosis)

"The pathologist's 'UIP' is what the clinician refers to as interstitial pulmonary fibrosis or idiopathic pulmonary fibrosis (IPF)."

Front 18

Presentation of UIP is often seen in what stage?

Back 18

End stage fibrosis.

Front 19

What PFTs are seen with UIP?

Back 19

Decreased TLC and VC

Front 20

What do ABGs show with UIP?

Back 20

Hypoxemia; low pCO2 due to hyperventilation secondary to lung "stiffness" and decreased arterial oxygen ???

Front 21

UIP: gross pathology

Back 21

Outer surface: looks "cirrhotic" and has a "hobnailed" appearance.

Cut surface: microcysts with intervening fibrosis = HONEYCOMB PATTERN. May also see some emphysema present.

Front 22

Honeycomb pattern

Back 22

UIP

Front 23

What is a major histopathologic feature of the fibrotic stage of UIP?

Back 23

Fibroblastic foci.

Front 24

Is emphysema a restrictive or obstructive disease?

Back 24

Obstructive.

Front 25

Stages of UIP.

Back 25

Stages are similar to that of DAD

1. Acute (almost never seen): Edema, hyaline membranes

2. Proliferative (also rarely observed): Interstitial intralveolar fibrosis, proliferation of type II pneumocytes --> cuboidalization of the epithelium, hyaline membranes still present.

3. Fibrotic (best described phase): HONEYCOMB. Architectural disorganization. Air spaces are of irregular shape with thickened, edematous, fibrotic walls, microcysts. FIBROBLASTIC FOCI. Prolferation of the smooth muscle in the interstitium.

Front 26

UIP is more prominent at what part of the lung?

Back 26

At the periphery. (patchiness, spatial disuniformity)

Front 27

UIP: prognosis

Back 27

The more fibroblastic foci, the worse the prognosis.

Survival from time the symptoms are first reported is often less than 5 years. Women tend to survive longer than men.

Front 28

Explain hypersensitivity pneumonitis

Back 28

Repeated inhalation of dusts --> allergic response in lung parenchyma --> formation of non-caseating granulomas and inflammation.

Litlle known about gross pathology.

Front 29

Diagnostic triad of HSP (actually 4)

Back 29

1. Bronchiolitis
2. Chronic interstitial inflammation/pneumonitis
3. Loose or incomplete granulomas (not as tight as those of sarcoidosis), seen in about 70% of biopsies.

(4. Foreign-body material is also seen in about 60% of biopsies)

Front 30

What is the single most common feature of the microscopic pathology of HSP?

Back 30

Interstital infiltrate consisting primarily of lymphocytes and plasma cells (with occasional neutrophils and eosinophils).

Front 31

What is the prognosis of HSP?

Back 31

Good. Advancement to advanced pulmonary disease does occur but is rare.

Front 32

What is the most characteristic finding of sarcoidosis?

Back 32

Bilateral hilar lymphadenopathy

Front 33

Sarcoidosis affects what ages?

Back 33

20-40

Front 34

Sarcoidosis: describe granulomas

Back 34

Non-caseating. Minimal fibrinoid necrosis can be seen at times.

Front 35

Sarcoidosis: PFTs

Back 35

Reduction in lung volumes.
Diffusing capacity is reduced.

Front 36

Sarcoidosis: pathology of granulomas

Back 36

Granulomas involve wall of bronchioles and follow the path of the pulmonary lymphatics.

Front 37

Sarcoidosis: diagnosis

Back 37

Diagnosis of exclusion. Need to rule out:
- TB: Use AFB (Zielh-Nielsen) stain.
- Fungal infection: GMS (Gomori-methenamine Silver) stain.
- Beryllium exposure.

Front 38

What is the prototype of acute interstitial/restrictive lung disease?

Back 38

ARDS/DAD

Front 39

What is the prototype of a chronic interstitial/restricitive disease?

Back 39

UIP

Front 40

What do these abbreviations stand for?

IPF:
HSP:
AIP:
COP:

Back 40

IPF: Interstitial Pulmonary Fibrosis
HSP: Hypersensitivity Pneumonitis
AIP: Acute Interstitial Pneumonia
COP: Cryptogenic Organizing Pneumonia

Front 41

What is the most important cause of noncardiogenic pulmonary edema?

Back 41

Increases permeability of the alveolar capillary membrane.

Front 42

c/c DAD and AIP

Back 42

AIP is exactly the same as DAD except that the etiology is unknown.

Most DAD is caused by sepsis, trauma, or shock.

DAD and AIP are the same except for the cause.

Front 43

Of those diagnosed with ARDS, what percentage have abnormal pulmonary function tests 1 year later?

Back 43

Approx 2/3

Front 44

80-85% of patients with BOOP report being responsive to what?

Back 44

Steroids.

Front 45

Is BOOP idiopathic?

Back 45

Yes, most cases are idiopathic.

Front 46

In most cases, which predominates, the bronchiolitis obliterans or the organizing pneumonia?

Back 46

The organizing pneumonia.

Front 47

What term may eventually replace BOOP?

Back 47

COP (Cryptogenic Organizing Pneumonia)

Front 48

Onset of UIP?

Back 48

Can be rapidly progressive, but is more commonly slow and insidious.

Front 49

What is the most common symptom of UIP? Common physical findings?

Back 49

Insidious increase in dyspnea.

Rapid shallow respirations.
Fine rales over the lower lobes.

Front 50

UIP: treatment

Back 50

Corticosteroids
Anti-inflammatory drugs
Anti-fibrotic cytokines
Lung transplantation

Front 51

UIP: risks during later stages

Back 51

Lung cancer
Superimposed pneumonias.

Front 52

What is sarcoidosis?

Back 52

"A multisystem granulomatous disorder"

Front 53

Sarcoidosis: symptoms

Back 53

Usually asymptomatic, but can present with cough and increasing dyspnea.

Front 54

Sarcoidosis: gross presentation at end-stage

Back 54

Honeycomb lung

Front 55

(review)

What are the three major groups of extra-pulmonary restrictive lung disease? What are the types of lung disease within each group?

Back 55

EXTRA-PULMONARY RESTRICTIVE LUNG DISEASE

1. Extra-parenchymal lung disease.
A. Impaired muscle function
a. Spinal cord
- AML
- Polio
b. Motor neurons
- Peripheral neuropathies
- Guillain-Barre Syndrome
c. Neuromuscular junction
- Botulism
- Myasthenia Gravis
- Organophosphate poison
d. Muscle
- Dystrophies
- Myopathies

B. Decreased chest wall compliance
a. Skin
- Scleroderma
- Severe burns
- Obesity
b. Bone
- Severe kyphoscoliosis
- Ankylosing spondylitis
- Thoracoplasty
c. Pleura
- Fibrothorax

C. Other
- Pneumothorax
- Pleural effusions
- Complete obstruction of a bronchus.

Front 56

How do impaired chest wall muscles affects PFTs?

Back 56

- Lowers TLC and VC.
- FEV1/FVC is normal (typical restrictive pattern).
- RV is fairly normal.
- Lung parenchyma is normal, so diffusing capacity is usually normal.
- The maximum inspiratory pressure (MIP) will be REDUCED in patients with impaired muscle function.

Front 57

Extreme obesity can be thought of as what type of lung disease?

Back 57

A restrictive lung disease. The compliance of the chest wall is decreased.

Front 58

What type of respiratory presentation is seen with Guillian-Barre syndrome?

Back 58

Often acute respiratory failure.

Front 59

What type of respiratory presentation is seen with kyphoscoliosis?

Back 59

Chronic - respiratory failure develops over the course of years.

Growth of the pulmonary capillary bed may be diminished, therefore decreasing the diffusing capacity.

Front 60

What is cor pulmonale?

Back 60

When there is a heart problem caused by an initial lung problem.

Front 61

What are the primary symptoms of restrictive lung disease?

Back 61

Dyspnea and reduced exercise tolerance.

Front 62

Acute interstitial lung disease usually presents as what?

Back 62

ARDS with DAD + acute edema formation.

Front 63

What is the list of diseases that can cause acute interstitial restrictive lung disease?

Back 63

ACUTE INTERSTITIAL LUNG DISEASE

1. Circulatory causes
- Pulmonary edema (cardiogenic, non-cardiogenic [ARDS])
- Fat emboli
- Hemorrhage (trauma and other)

2. Infections
- Measle virus pneumonia
- Pneumocystis pneumonia
- Miliary tuberculosis
- Invasive aspergillus pneumonia

3. Drug reactions
- Nitrofurantoin

4. Allergic reactions
- Eosinophilic pneumonias (some)

5. Toxins
- Chlorine gas
- Nitrogen dioxide (silo-filler's disease)

6. Collagen vascular disease
- Acute systemic lupus erythematosis

Front 64

What type of lung defect category can DAD be classified as?

Back 64

A restrictive defect. Can also be thought of as a pulmonary edema syndrome.

Front 65

If someone came to you with decreased gas exchange and a horrible CXR, what are some of the things that you would want to make sure that you covered in your history?

Back 65

Main things that will help you narrow your search are:
A. Fever?
B. Left ventricular failure?

More minor:
1. History of major trauma? (ARDS, fat emboli)

2. Fever? (Infections: measles pneu, Pneumocytosis pneu, miliary TB, invasive aspergillus pneu)

3. Inhalation of gasses? (Chlorine gas, nitrogen-dioxide [silo-filler's lung], smoke inhalation)

4. Drug allergies? (nitrofurantoin)

Front 66

What is chronic restrictive interstitial lung disease in a nutshell?

Back 66

Some sort of inflammatory process that led to the deposition of collagen in the interstitium.

Note that deposition of amyloid protein or malignant cells can also cause restrictive interstitial lung disease.

Front 67

When presented with a patient with chronic restrictive lung symptoms and increased interstitial markings on CXR, what things do you make sure to include in your history taking?

Back 67

1. History of inhaling inorganic materials at work? (asbestosis, silicosis)

2. Inhalation of organic dusts at home that incite an allergic reaction? (hypersensitivity pneumonitis)

3. Meds? (methotrexate, bleomycin)

4. IV drug use?

5. Exposure to high concentrations of O2 over prolonged periods of mechanical ventolation?

6. History of collagen vascular disease? (scleroderma, SLE, RA)

Often need a lung biopsy

Front 68

Upper lobe diffuse, finely nodular increased interstitial markings make you think of what?

Back 68

Silicosis

Front 69

Calcified pleural plaques make you think of what?

Back 69

Asbestosis

Front 70

Bilateral enlargement of the hilar nodes + increased interstitial markings make you think of what?

Back 70

Sarcoidosis

Front 71

What is the list of chronic restrictive interstital lung disease?

Back 71

1. Circulatory causes
- LV failure (long-standing)
- Mitral stenosis
- Post ARDS (not common)

2. Infections
- Post-viral

3. Physical agents
- Chronic aspiration
- Radiation

4. Drugs
- Bleomycin
- Methotrexate
- Amiodarone

5. Inorganic dusts
- Asbestosis
- Silicosis
- Beryllium

6. Organic dusts
- Hypersensitivity pneumonitis

7. Collagen vascular disease
- Scleroderma
- SLE
- RA

8. Tumors
- Lymphatic spread of malignant cells
- Alveolar carcinoma
- Lymphoma

9. Idiopathic with granulomas
- Sarcoidosis
- Wegeners' granulomatosis
- Lymphatoid granulomatosis

10. Idiopathic without granulomas
- Idiopathic Pulmonary Fibrosis (IPF)
- Desquamate interstitial pneumonitis
- Amyloid deposition
- Lymphatoid interstital pneumonitis
- Alveolar proteinosis

Front 72

What is kind of strange about the FEV1/FVC ratio with chronic restrictive interstitial lung disease?

Back 72

The FEV1/FVC ratio is actually INCREASED in some cases because the noncompliant interstitium tends to "tether" the airways open.

Front 73

In chronic restrictive interstitial lung disease, how does the lung diffusion capacity usually present?

Back 73

It is usually decreased.

Front 74

IPF: etiology

Back 74

Unknown

Front 75

IPF: physical findings (2)

Back 75

1. Bibasalar crackles
2. Digital clubbing

Front 76

IPF: PFTs

Back 76

1. Decreased TLC, RV, VC
2. FEV1/FVC ratio is INcreased.
3. Diffusing capacity is decreased

Front 77

IPF: ABGs

Back 77

1. Hypoxemia
2. Hypocapnea (due to hyperventilation in response to lung stiffness and decreased PaO2)

Front 78

IPF: what does lung biopsy show?

Back 78

Usual Interstitial Pneumonitis (UIP)

Front 79

IPF: prognosis

Back 79

Median survival from time symptoms are first reported are generally less than 5 years.

Women survive longer than men

Those with pulmonary hypertension (30-40%) have the worst outcomes.

Front 80

IPF: treatment

Back 80

Corticosteroids/anti-inflammatory drugs

Front 81

IPF: cancer risk?

Back 81

Increased.

Front 82

IPF: as the disease progresses, it goes from ________ respiratory failure to ________ respiratory failure.

Back 82

As the disease progresses, it goes from HYPOXIC respiratory failure to HYPERCAPNIC respiratory failure.

Front 83

Sarcoidosis: may see an obstructive defect when what occurs?

Back 83

Occlusion of an airway by a granuloma.

Front 84

Sarcoidosis: diffusing capacity

Back 84

Reduced.

Front 85

Non-caseating granulomas in the setting of chronic restrictive interstitial lung disease makes you think of what?

Back 85

Sarcoidosis, HSP

Front 86

Active sarcoidosis: treatment

Back 86

Corticosteroids