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Respiratory-AC > 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

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2
Q

What are the contents of the interstitium?

A

Between alveoli:

  • type I pneumocytes
  • type II pneumocytes
  • capillaries (capillary endothelium)
  • interstitial cells
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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

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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)
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5
Q

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

A

Fibrosis of the lung is irreversible

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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
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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)
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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
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9
Q

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

A
  1. sarcoidosis
  2. hypersensitivity pneumonitis
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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)
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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)
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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

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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)
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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)
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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
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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
What are 3 important types of hypersensitivity pneumonitis?
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
What are some indications for hypersensitivity pneumonitis?
* 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
Describe sarcoidosis and its prevalence
* 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
What is the etiology of sarcoidosis?
* 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
What tissues are commonly involved in sarcoidosis?
* lung (most common) * lymph nodes * spleen * liver (granulomatous hepatitis) * skin (erythema nodosum) * eyes (uveitis)
30
What are some lab/radiological findings of sarcoidosis?
* 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
Describe idiopathic pulmonary fibrosis
* 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
What is the pathophysiology of idiopathic pulmonary fibrosis?
* repeated injury to the lung causes alveolitis -\> * cytokine release causes interstitial fibrosis -\> * interstitial fibrosis -\> irregular dilation of adjacent airways resulting in **honeycomb lung**
33
What is the clinical course of idiopathic pulmonary fibrosis?
Progressive disease- end stage lung/cor pulmonale Mean survival= 3 years
34
What are 3 causes of collagen vascular disease?
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
Describe 2 pulmonary hemorrhage syndromes
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

Respiratory-AC (28 decks)

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