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Block 9 Week 3 Meg > Baker/Parks: Restrictive Lung Disease > Flashcards

Baker/Parks: Restrictive Lung Disease Flashcards

1
Q

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

A

restrictive lung disease

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2
Q

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

A

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

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3
Q

5 types of restrictive lung disease?

A 
fibrosing
granulomatous
eosinophilic
smoking related
other
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4
Q

What is happening pathologically in restrictive lung disease?

A

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

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5
Q

Due to chronic progressive fibrosis which amputates distal airways

A

honeycomb lung

**seen in idiopathic pulmonary fibrosis

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6
Q

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).

A

idiopathic pulmonary fibrosis

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7
Q

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

A

usual interstitial pneumonia

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

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8
Q

What might you see grossly in idiopathic pulmonary fibrosis?

A

diffuse fibrosis

cystic spaces with fibrosis = honeycombing

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9
Q

What might you see histologically with idiopathic pulmonary fibrosis?

A

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

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10
Q

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

A

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

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11
Q

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

A

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

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12
Q

What is the treatment for IPF?

A

steroids or immune suppressants

lung transplant**

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13
Q

So how does idiopathic pulmonary fibrosis present?

A

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

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14
Q

Diffuse ILD of unknown etiology (like IPF) but…

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

A

nonspecific interstitial pneumonia

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15
Q

What are the two subtypes of nonspecific interstitial pneumonia?

A

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

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16
Q

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

A

dyspnea and cough for several months

46-55 yo

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17
Q

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

A

cryptogenic organizing pneumonia

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18
Q

What will you see morphologically in cryptogenic organizing pneumonia?

A

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

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19
Q

What can cause cryptogenic organizing pneumonia?

A 
secondary to infections or inflammatory injury
viral or bacterial pneumonia
inhaled toxins
drugs
CT disease
GVHD
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20
Q

Pulmonary involvement can occur in these three connective tissue disorders

A

Rheumatoid arthritis
Systemic sclerosis
SLE

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21
Q

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

A

pneumoconioses

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22
Q

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

A

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

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23
Q

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?

A

small particles

vs

large particles

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24
Q

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

A

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

25
Q

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.

A

macrophages; ROS; fibroblast; collagen

26
Q

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

A

asbestos

27
Q

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

A

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

28
Q

List 3 types of inhalation exposure pneumoconioses

A

Coal workers pneumoconiosis
silicosis
asbestos

29
Q

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

A

Coal Workers’ pneumoconiosis

30
Q

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

A

Silicosis

31
Q

Abundant lipoproteinaceous material in alveoli

Identical to alveolar proteinosis

A

acute silicosis

32
Q

What is the most common offender in silicosis?

A

quartz

  • *pure quartz is the worst
    • causes tiny nodules that eventually coalesce into hard collagenous scars
33
Q

What will you see on chest Xray with silicosis?

A

upper zone nodules

egg shell calcification in hilar lymph nodes

34
Q

Asbestos exposure affects these two portions of the lung

A

pleura: pleural effusions, fibrous plaques, diffuse pleural fibrosis, mesothelioma

pulmonary parenchyma: lung carcinoma, interstitial fibrosis

35
Q

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?

A

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

36
Q

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

A

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

37
Q

What will you see in asbestosis?

A

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

38
Q

What areas of the lung are affected first in asbestosis?

A

lower lung

subpleura areas

39
Q

How does asbestosis present clinically?

A

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

40
Q

What can happen to the pleura with asbestos exposure?

A

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

pleural effusion

mesothelioma

41
Q

What are the two granulomatous restrictive lung diseases?

A

sarcoidosis

hypersensitivity pneumonitis

42
Q 
Systemic Disease of unknown cause
Noncaseating granulomas in many tissues and organs
Female >> male
Blacks >>>>> whites (10x more frequent)
Rare in Asian populations
A

sarcoidosis

43
Q

What is a distinguishing feature in sarcoidosis?

A

noncaseating granulomas in many organs

44
Q

What are 3 likely things that could cause sarcoidosis?

A

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

45
Q

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

A

transbronchial biopsy

46
Q

How will sarcoidosis present on X ray?

A

hilar lymph nodes affected bilaterally

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

47
Q

How do people with sarcoidosis present?

A

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

48
Q

What happens to calcium levels in sarcoidosis?

A

hypercalcemia

49
Q

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

A

True

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

50
Q 
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
A

hypersensitivity pneumonitis

51
Q

Unlike asthma, hypersensitivity involves the (blank)

A

alveoli

52
Q

What are some morphological features of hypersensitivity pneumonitis?

A 
Centered around bronchioles
Interstitial pneumonitis
Lymphocytes, plasma cells, macrophages
Noncaseating granulomas
Interstitial fibrosis, honeycombing, obliterative bronchiolitis (late)
53
Q

What should be done to resolve hypersensitivity pneumonitis?

A

early recognition and removal of causative agent!!

**if not removed, can lead to serious chronic fibrotic disease

54
Q

Infiltration of eosinophils in the lungs

A

pulmonary eosinophilia

55
Q

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

A

chronic eosinophilic pneumonia

56
Q

What can cause secondary eosinophilia?

A 
infection
drug rxn
asthma
vasculitis
aspergillosis
57
Q

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.

A

pulmonary alveolar proteinosis

58
Q

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

A

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

Block 9 Week 3 Meg (10 decks)

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