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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
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

Respiratory-AC (28 decks)

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