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HMS Homeostasis I > Restrictive Lung Disease > Flashcards

Restrictive Lung Disease Flashcards

1
Q

Functions of type II pneumocytes

A

Synthesis of surfactant, alveolar epithelial repair, and ion and fluid transport

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2
Q

Microatelectasis

A

Collapse of alveoli on a microscopic level

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3
Q

Four surfactant-associated proteins

A

SP-A, SP-B, SP-C, SP-D

SP-A and SP-D are opsonins that function in lung defense.

SP-B and SP-C are particularly crucial for surfactant function.

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4
Q

When type I cells are damaged, the reparative process involves . . .

A

. . . hyperplasia of the type II cells and eventual differentiation into type I-like cells.

Normally, this results in some hyperplastic type II cells undergoing apoptosis, while the remainder transdifferentiate into thin, delicate, type I cells. If this process is defective, it may instead result in idiopathic pulmonary fibrosis.

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5
Q

Unlike the alveolar epithelial cells, which are ____, junctions between capillary endothelial cells permit ____.

A

Unlike the alveolar epithelial cells, which are quite impermeable under normal circumstances, junctions between capillary endothelial cells permit passage of small-molecular-weight proteins.

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6
Q

Major components of lung insterstitial spaces

A

Collagen, elastin, proteoglycans, a variety of macromolecules involved with cell-cell and cell-matrix interactions, some nerve endings, and some fibroblast-like cells. Also some lymphocytes.

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7
Q

‘Insterstitial’ in interstitial lung disease

A

The interstitium formally refers only to the region of the alveolar wall exclusive of and separating the alveolar epithelial and capillary endothelial cells.

However, interstitial lung diseases affect all components of the alveolar wall: epithelial cells, endothelial cells, and cellular and noncellular components of the interstitium. In addition, the disease process often extends into the alveolar spaces and therefore is not limited to the alveolar wall.

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8
Q

The idiopathic interstitial pneumonias represent . . .

A

The idiopathic interstitial pneumonias represent a subgroup of disorders within the broader category of diffuse parenchymal lung disease/interstitial lung disease.

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9
Q

Two components of most diffuse parenchymal lung diseases

A
  1. Inflammatory
  2. Fibrotic
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10
Q

Hypersensitivity pneumonitis

A

Granulomatous inflammation induced by hypersensitivity against a particular substance. Common cause of lung granulomas, along with sarcoidosis.

Many potential agents have been identified (including possibly vitamin E acetate)

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11
Q

Idiopathic interstitial pneumonias

A

Display variable amounts of nonspecific inflammation and fibrosis, and they lack granulomas or specific pathologic features characteristic of other previously well-defined diseases. Sometimes come along with a rheumatic disease, like scleroderma.

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12
Q

Almost any diffuse parenchymal lung disease, given enough time, will . . .

A

. . . lose any distinctive features of prior interstitial inflammation or alveolitis and just look like extensive fibrosis, sometimes with cysts. Even granulomas will disappear.

Thus, we say they all converge at end-stage diffuse parenchymal lung disease

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13
Q

Genes that predispose to diffuse parenchymal lung disease

A
  • MUC5B
  • hTERT
  • SP-C

Loss of MUC5B would remove the lung’s shield to particulate damage, loss of hTERT makes it less possible to regenerate tissue, and loss of SP-C makes surfactant dysfunctional.

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14
Q

Pathogenetic features of diffuse parenchymal lung disease

A
  1. Initiation (by antigens or toxins)
  2. Propagation (through the inflammatory response)
  3. Fibrosis
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15
Q

Functional consequences of fibrosis

A
  1. Decreased compliance
  2. Decrease in lung volumes
  3. Loss of alveolar-capillary surface area and impaired diffusing capacity
  4. Isolated abnormalities in small airway function
  5. Disturbances in gas exchange (usually hypoxemia w/o CO2 retention)
  6. Pulmonary hypertension
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16
Q

Patients with diffuse parenchymal lung disease tend to breathe . . .

A

Patients with diffuse parenchymal lung disease tend to breathe with smaller tidal volumes but increased respiratory frequency

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17
Q

Decreases in TLC, FRC, and RV in restrictive lung diseases are . . .

A

Decreases in TLC, FRC, and RV in restrictive lung diseases are direct consequences of the change in lung compliance.

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18
Q

Mechanism of reduced diffusing capacity in diffuse parenchymal lung disease

A

It’s actually NOT membrane thickening, which most people expect.

Rather, the processes of inflammation and fibrosis destroy a portion of the alveolar-capillary interface and reduce the surface area available for gas exchange. You have less functional alveolar space per alveolus.

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19
Q

Abnormalities in Small Airways Function in diffuse parenchymal lung disease

A

Large airways usually function normally, and so FEV1/FVC is usually normal or even increased.

However, inflammation and fibrosis in the peribronchiolar regions often narrow the lumen of the small airways or bronchioles, increaseing resistance to flow in these smaller channels (detectable on FEF25%-75%)

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20
Q

Mechanism of hypoxia in restrictive lung disease

A

Hypoxemia without CO2 retention, and in fact hypocapnia typically is present.

V̇/Q̇ mismatch is major contributor.

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21
Q

Pulmonary hypertension is common in severe diffuse parenchymal lung disease, resulting from ___

A

Pulmonary hypertension is common in severe diffuse parenchymal lung disease, resulting from hypoxemia and obliteration of small pulmonary vessels.

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22
Q

Patients with diffuse parenchymal lung disease most commonly have ___ as the presenting symptom

A

Patients with diffuse parenchymal lung disease most commonly have dyspnea as the presenting symptom

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23
Q

Clinical features of diffuse parenchymal lung disease

A
  • Dyspnea
  • Cough, usually nonproductive
  • Dry inspiratory crackles on auscultation, most prominent at lung bases
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24
Q

Radiographic picture of diffuse parenchymal involvement

A

Interstitial pattern, with enhanced lung-markings

Possible retracted lungs due to changed FRC and TLC.

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25
Q

Diagnostic distinction between the different types of diffuse parenchymal lung disease often requires ___.

A

Diagnostic distinction between the different types of diffuse parenchymal lung disease often requires investigation at the microscopic or histologic level (biopsy or bronchoalveolar lavage)

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26
Q

___ is rarely a feature of diffuse parenchymal lung disease, and if it appears is often indicative of a complicating process (like infection)

A

Hypercapnia is rarely a feature of diffuse parenchymal lung disease, and if it appears is often indicative of a complicating process (like infection)

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27
Q

Conceptual framework for idiopathic pulmonary fibrosis

A
  1. Injury to alveolar epithelial cells by unknown agent
  2. Defective type II pneumocyte hyperplasia and differentiation response
  3. PDGF/TGFb-driven fibroblast activation
  4. Fibrosis
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28
Q

TERC gene

A

Telomerase RNA component

The RNA transcript that telomerase uses as a guide RNA! Disruption of this gene can, of course, lead to telomere mismanagement

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29
Q

Clinical picture for idiopathic pulmonary fibrosis

A
  • 50-70 yr old on first presentation
  • Insidious onset
  • Dyspnea chief compaint
  • Dry crackles on examination
  • Clubbing of digits
  • Interstitial pattern on CXR
  • Honeycombing on CT
    • ANA test
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30
Q
A

Honeycomb CT scan

Typical for idiopathic pulmonary fibrosis. Indicative of extensive, irreversible lung fibrosis.

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31
Q

Treatments for idiopathic pulmonary fibrosis

A

Nintedanib - tyrosine kinase inhibitor that blocks downstream signaling of several fibrogenic growth factors, including PDGF, FGF, and VEGF

Anti-TGFb

Lung transplant

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32
Q

Sarcoid is characterized by ____

A

Sarcoid is characterized by naked granulomas

33
Q

In addition to the lungs proper, sarcoid frequently effects . . .

A

. . . hilar and mediastinal draining lymph nodes.

34
Q

Proposed pathogenesis of sarcoid

A
35
Q

T lymphocytes in sarcoidosis nonspecifically . . .

A

T lymphocytes in sarcoidosis nonspecifically activate B lymphocytes, leading to production of a variety of immunoglobulins and the common finding of polyclonal hypergammaglobulinemia.

36
Q

In addition to granulomas in the lung parenchyma or intrathoracic lymph nodes, an ___ often occurs in sarcoidosis.

A

In addition to granulomas in the lung parenchyma or intrathoracic lymph nodes, an alveolitis often occurs in sarcoidosis.

The alveolitis is composed of mononuclear cells, including macrophages and lymphocytes

37
Q

CXRs in sarcoidosis

A
  1. Enlargement of lymph nodes, most commonly bilateral hilar lymphadenopathy
  2. Parenchymal lung disease, interstitial pattern
38
Q

Clinical picture for sarcoidosis

A
  • Chief concern dyspnea or nonproductive cough, or referred due to incidental CXR findings
  • No inspiratory crackles on auscultation
  • Extra-pulmonary involvement (especially eyes, skin, cardiac, peripheral lymph nodes, neurologic)
  • May present with Löfgren syndrome
  • Hypergammaglobulinemia
  • Increased vitamin D activation by granuloma macrophages resulting in hypercalcinuria or hypercalcemia
39
Q

Löfgren syndrome

A
  • Acute onset
  • Bilateral hilar lymphadenopathy
  • Erythema nodosum (painful red nodules) typically on the anterior surface of the lower legs
  • Fever
  • Lower extremity arthralgias

For whatever reason, has an excellent prognosis, with 80% spontaneous remission

40
Q

Patients with sarcoid often display inappropriate ___.

A

Patients with sarcoid often display inappropriate anergy.

This is particularly true regarding any immune response requiring intact delayed-type hypersensitivity.

41
Q

___ on an otherwise healthy individual is extremely suggestive of sarcoidosis.

A

Bilateral hilar lymphadenopathy on an otherwise healthy individual is extremely suggestive of sarcoidosis.

42
Q

___ is a definitive diagnostic tecnhique for sarcoid.

A

Transbronchial lung biopsy showing naked granulomas is a definitive diagnostic tecnhique for sarcoid.

43
Q

Weird things that granuloma macrophages make in sarcoid

A
  • Active vitamin D (leading to hypercalcemia)
  • Angiotensin converting enzyme (possibly hypertension)
44
Q

Many sarcoidosis patients do not require ___

A

Many sarcoidosis patients do not require treatment.

The disease often improves or disappears spontaneously.

On the other hand, some patients have refractory disease, and are treated with heavy-duty drugs like methotrexate, leflunomide, or chronic corticosteroids.

45
Q

The less efficient an individual’s breathing, the more ___ is wasted on ___.

A

The less efficient an individual’s breathing, the more ventilation is wasted on dead space.

46
Q

____ are a hallmark histopathologic feature of IPF

A

Fibroblastic foci (clusters of proliferating fibroblasts indicating active collagen deposition) are a hallmark histopathologic feature of IPF

47
Q

Characteristic chest CT features of IPF in include ___

A

Characteristic chest CT features of IPF in include peripheral, basilar reticular opacities with architectural distortion including honeycomb changes and traction bronchiectasis.

48
Q
A

Fibroblastic focus

Active fibroblasts synthesizing matrix

Strongly suggestive of IPF

49
Q

Clubbing of nails is usually a sign of . . .

A

. . . some sort of hypoxic disease. Could be pulmonary, cardiovascular, anemia, etc.

But, if it is congenital, it’s probably normal. Ask about ‘recent changes.’

50
Q

Interstitial inflammation is located. . .

A

. . . beneath the endothelium, but not past the basement membrane

51
Q

Neutrophils on lung biopsy suggest ___.

A

Neutrophils on lung biopsy suggest an active infection.

52
Q

Inflammatory cells within veins and arteries on biopsy suggest. . .

A

. . . vasculitis

53
Q

Sub-pleural restriction

A

Honeycombing/fibrosis of the distal or pleural margin

Common in early idiopathic pulmonary fibrosis

54
Q

For idiopathic pulmonary fibrosis, decrease in oxygenation on exercise is thought to be due to. . .

A

. . . exacerbation of V/Q mismatch rather than a diffusion problem

55
Q

“Honeycombing” phenomenon is due to fibrosis of the. . .

A

. . . interlobular septae

56
Q

You can think of IPF as a kind of ___ of the lung.

A

You can think of IPF as a kind of keloid scar of the lung.

57
Q

___ are elevated in IPF patients and may play a role in pathogenesis.

A

IL-25, ILC2s, and IL-13 are elevated in IPF patients and may play a role in pathogenesis.

IL-25 drives ILC2 proliferation and activates ILC2s. ILC2s secrete IL-13, and IL-13 drives fibrosis in fibroblasts and redifferentiated smooth muscle.

58
Q

When you see bilateral hilar lymphadenopathy, your first thought should be sarcoidosis. Your second thought should be ___.

A

When you see bilateral hilar lymphadenopathy, your first thought should be sarcoidosis. Your second thought should be lymphoma

59
Q

If you are concerned someone has sarcoidosis, you should order ___.

A

If you are concerned someone has sarcoidosis, you should order an ECG.

If sarcoid affects the cardiac conduction system, usually the AV node, it would be fatal, and it is important to rule this out. You would be looking for an longer PR interval, greater than 0.2 seconds (1 big box)

60
Q

Lung biopsies are usually taken ___.

A

Lung biopsies are usually taken blind!

So if you get a negative result, it does not rule out pathology

For this reason, you usually do multiple biopsies. It is very easy to miss patchy pathologies like sarcoidosis.

61
Q

Sarcoid granulomas tend to be ____ within the lung.

A

Sarcoid granulomas tend to be peribronchial/periateriolar within the lung.

62
Q

Tactile fremitus evaluation

A

When doing tactile fremitus, the vibration will be increased when over a fluid-filled parenchyma (pulmonary edema), but decreased when over a fluid-filled pleura (pleural effusion).

63
Q

Peribronchual cuffing

A

Circle outlining a dark space on CXR. Representative of fluid surrounding an airway.

64
Q

Kerley B lines

A

Appear in periphery of lung along the pleural line, indicative of pulmonary edema.

65
Q

Luspateracept

A

Anti-TGFb

66
Q

Albuterol is no good for ___.

A

Albuterol is no good for any lung disease without bronchospasm.

67
Q

Helium Dilution Test

A

Seal the nose airway, hook up to a patient’s lungs, and let helium diffuse. The concentration change tells you how much volume it was diluted in, and thus the volume of the lungs.

Note that this does not work well for regions of the lung with high time constants, and so may underestimate.

68
Q

DLCO/VA

A

DLCO over alveolar volume

69
Q

Diagnosing restrictive lung disease with spirometry

A

YOU CAN’T!!!

Spirometry can only suggest restrictive pathology, but you have to get lung volumes to make it definitive (TLC). Really bad obstructive disease will also have reduced FVC due to flow limitation, especially with emphysema. But of course, if you check the lung volumes on these emphysema patients, their TLCs will be elevated. This form of pathology is called pseudorestrictive

70
Q

Types of chest wall disease

A
  • Obesity
  • Kyphoscoliosis
  • Ankylosing spodylitis
  • Scleroderma (tight skin)
  • Skin graft vs host disease
  • Thoracic burn and fibrosis
71
Q

Characteristics of chest wall disease

A
  • Asymmetrically decreased lung volumes
    • vvv TLC, vv FRC, v RV
  • Rapid, shallow breathing
  • DLCO normal when corrected for alveolar volume (DLCO/VA)
  • Viariable increased PCO2, but decreased PO2
72
Q

Neuromuscular lung disease

A

The only disease with really substantially decreased MIP / MEP (some reduction of MEP with emphysema)

73
Q

Hemidiaphragmatic paralysis

A

vvv MIP

  • MEP

One sided diaphragm elevtaion on CXR at full inspiration

Worse PFTs on supine spirometry

74
Q

For diagnosing diseases affecting the diaphragm, ___ is often a useful diagnostic technique

A

For diagnosing diseases affecting the diaphragm, supine spirometry is often a useful diagnostic technique

75
Q

Diseases that can cause combined obstructive and restrictive pathology

A
  • Sarcoidosis
  • Pulmonary hypersensitivity
76
Q

Granuloma differential

A
  • Tuberculosis (not always caseating, beware)
  • Sarcoid
  • Hypersensitivity pneumonitis
  • Beryllium disease
  • White lung (silica)
77
Q

Naked granulomas according to a pathologist

A

Just have fewer lymphocytes, but not none. Claims that IFNg is still required for maintenance of naked granulomas.

78
Q

Asteroid bodies

A

Debris within a giant cell

HMS Homeostasis I (62 decks)

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