Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Pulmonology (CP) > 15 - Interstitial lung diseases > Flashcards

15 - Interstitial lung diseases Flashcards

1
Q

Define interstitial lung disease

A

Lung parenchymal disorders with common clinical, radiological, physical and pathologic features (hallmark= involvement of the space between alveoli)

RESTRICTIVE (not obstructive) diseases

**hard to distinguish from one another, a lot of overlap

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What are the contents of the interstitium?

A

Between alveoli:

  • type I pneumocytes
  • type II pneumocytes
  • capillaries (capillary endothelium)
  • interstitial cells
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What is the underlying pathogenesis of interstitial lung disease?

A

“Alveolitis”; damage to pneumocytes and endothelial cells leads to leukocyte release of cytokines which mediate/stimulate interstitial fibrosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What are the consequences of fibrosis in interstitial lung disease?

A
  • decreased lung compliance
  • decreased lung expansion during inspiration
  • increased elastic recoil (a non compliant lung wants to always be in its original resting state and will have more recoil back to that state)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

At what point in the pathogenesis is interstitial lung disease irreversible?

A

Fibrosis of the lung is irreversible

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What are 5 main clinical symptoms seen in interstitial lung disease?

A
  1. dry cough
  2. dyspnea
  3. late inspiratory crackles, bibasilar
  4. cor pulmonal (pulmonary HTN -> right heart failure)
  5. bilateral reticulonodular infiltrates on chest xray
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What are 5 categories of chronic interstitial lung disease?

A
  1. fibrotic
  2. granulomatous
  3. eosinophilic
  4. smoking-related
  5. “other” (e.g. pulmonary alveolar proteinosis)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What are 6 possible causes of fibrotic chronic interstitial lung disease?

A
  1. Usual interstitial pneumonia (idiopathic pulmonary fibrosis)
  2. nonspecific interstitial pneumonia (NSIP)
  3. cryptogenic organizing pneumonia (COP)
  4. collagen vascular diseases
  5. pneumoconiosis
  6. drug reactions
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What are 2 possible causes of granulomatous chronic interstitial lung disease?

A
  1. sarcoidosis
  2. hypersensitivity pneumonitis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What are 3 known causes of interstitial lung disease?

A
  1. occupational and environmental inhalants
    *pneumoconiosis= mineral dusts
    *hypersensitivity pneumonitis= organic dusts
  2. drugs/toxins (chemo, antibiotics)
  3. infections (CMV, TB, mycoplasma)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What are 3 interstitial lung diseases with unknown causes?

A
  1. idiopathic pulmonary fibrosis
  2. sarcoidosis
  3. collagen vascular disorders/vasculitis (RA, SLE, Wegener’s, Goodpasture’s)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Define pneumoconioses

A

Umbrella term for several non-neoplastic lung diseases that appear in response to inhalation of dusts (coal, silica, asbestos)

**25% of chronic interstitial lung disease cases are pneumoconioses

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What are the 4 main factors that affect the development of pneumoconioses?

A
  1. amount of dust retained in the lung parenchyma and airways
  2. size, shape, and buoyancy of particles
  3. particle solubility and physiochemical reactivity
  4. possible additional effects/other irritants (e.g. smoking)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What are the 2 main types of pneumoconioses?

A
  1. fibrogenic
  • “coal workers’ pneumoconiosis”= carbon dust
  • silicosis= crystalline silica dust
  • asbestosis= asbestos fibers
  1. non-fibrogenic (e.g. from barium/tin)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Describe Coal Workers Pneumoconiosis (CWP)

A
  • anthracotic (carbon) pigment from
    • coal mines
    • urban centers
    • tobacco smoke
  • anthracotic pigment in interstitial compartment and lymph nodes ​
    • simple CWP
    • complicated CWP
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Describe simple CWP

A
  • fibrous opacities < 1cm
  • usually in upper lobes
  • characterized by coal dust deposits adjacent to respiratory bronchioles
17
Q

Describe complicated CWP

A
  • fibrous opacities > 1 cm (with or without central necrosis)
  • massive fibrosis= crippling lung disease (aka black lung disease)
  • complications=
    • cor pulmonale
    • caplan syndrome (CWP with rhematoid nodules)
  • NO increased incidence of TB/cancer
18
Q

What is the most common occupational disease worldwide?

A

Silicosis:

  • from crystalline silicon dioxide (quartz/sand)
  • quartz activates alveolar macrophages after engulfment, leading to cytokine release and fibrogenesis
19
Q

Describe silicosis

A
  • nodular opacities with concentric layers of collagen
  • can see polarizable quartz particles
  • “egg shell” calcifications in hilar lymph nodes
  • complications=
    • cor pulmonale
    • caplan syndrome
    • increased risk for TB and cancer
20
Q

Describe asbestos-related disease

A
  • deposition in respiratory bronchioles, alveolar ducts, and alveoli
  • ferruginous bodies= macrophages phagocytose asbestos fibers and coat them with ferritin (iron + protein)
21
Q

What are some possible outcomes (6) from asbestos-related disease?

A
  • benign pleural plaques
  • diffuse interstitial fibrosis (from repeat exposure)
  • bronchogenic carcinoma (additional risk with smoking)
    *20 years after exposure
  • mesothelioma (not associated with smoking)
    ​*25-40 years after exposure
  • cor pulmonale
  • Caplan syndrome
  • NO increased risk for TB
22
Q

What is shown in these photos?

A

Asbestos bodies

23
Q

Describe berylliosis

A
  • granulomatous inflammation
  • complications=
    • cor pulmonale
    • lung cancer
  • beryllium is used in the nuclear and airspace industry
24
Q

Describe hypersensitivity pneumonitis

A
  • inhaled antigen producing granulomatous interstitial pneumonitis
  • type III hypersensitivity reaction
    • first exposure= IgG in serum
    • second exposure= Ab + inhaled antigen -> immune complexes -> interstitial inflammation
    • chronic exposure= type IV hypersensitivity (granuloma formation)
25
Q

What are 3 important types of hypersensitivity pneumonitis?

A
  1. farmer’s lung (from moldy hay)
  2. silo fillers’ disease (inhalation of gases from plant material; nitrogen oxides)
  3. byssinosis (from cotton/linen/hemp; textile factory workers “monday fever” since symptoms subside over the weekend when not being exposed)
26
Q

What are some indications for hypersensitivity pneumonitis?

A
  • interstitial and alveolar infiltrates of:
    • inflammatory cells
    • peri-bronchiolar accentuation
    • ill-defined granulomas (NOT necrotizing; how you distinguish from TB which has necrotic center)
  • lung biopsy may be needed to diagnose
27
Q

Describe sarcoidosis and its prevalence

A
  • multisystem granulomatous disease
  • comprises 25% of chronic interstitial lung disease cases
  • AA:white ratio 10:1
  • M:F ratio 1:2
  • 70% < 40 years
  • classic example= young african american female non-smoker
28
Q

What is the etiology of sarcoidosis?

A
  • unknown but it’s a disorder of immune regulation
    • unkown antigen interacts with CD4 T cells ->
    • cytokine release ->
    • monocytes/histiocytes recruited ->
    • non-necrotizing granuloma formation
  • diagnosis of exclusion
    • look at exposures to rule out hypersensitivity pneumonitis
    • necrotizing granuloma= TB
29
Q

What tissues are commonly involved in sarcoidosis?

A
  • lung (most common)
  • lymph nodes
  • spleen
  • liver (granulomatous hepatitis)
  • skin (erythema nodosum)
  • eyes (uveitis)
30
Q

What are some lab/radiological findings of sarcoidosis?

A
  • increased ACE levels
  • hypercalcemia
  • polyclonal gammopathy
  • cutaneous anergy (lack of response to skin antigens because of CD4 T cell consumption)
  • bilateral hilar adenopathy and reticulonodular shadows on chest xray
31
Q

Describe idiopathic pulmonary fibrosis

A
  • 15% of chronic interstitial lung disease cases
  • M > F, 40-70 years
  • broad encompassing term for a number of types of idiopathic interstitial pneumonias
  • nonspecific symptoms (dyspnea, fever, fatigue, cough)
32
Q

What is the pathophysiology of idiopathic pulmonary fibrosis?

A
  • repeated injury to the lung causes alveolitis ->
  • cytokine release causes interstitial fibrosis ->
  • interstitial fibrosis -> irregular dilation of adjacent airways resulting in honeycomb lung
33
Q

What is the clinical course of idiopathic pulmonary fibrosis?

A

Progressive disease- end stage lung/cor pulmonale

Mean survival= 3 years

34
Q

What are 3 causes of collagen vascular disease?

A

collagen vascular disease= 10% of interstitial lung disease cases

  1. SLE (lung disease in 50% of patients)
    *common manifestation= pleural effusion
  2. Rheumatoid arthritis
    *Caplan syndrome, pleural effusions
  3. Scleroderma
    *systemic sclerosis and pulmomary vascular hypertrophy
35
Q

Describe 2 pulmonary hemorrhage syndromes

A
  1. Goodpasture syndrome
    *autoantibodies to collagen IV -> destruction of basement membrane in kidney and lung alveoli
    *necrosis with intra-alveolar hemorrhage
  2. Granulmatosis with polyangiitis (Wegener’s)
    *necrotizing granulomas and vasculitis

Pulmonology (CP) (23 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions