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MS2 Respiratory > 9/16- Pathology of Interstitial Lung Diseases > Flashcards

9/16- Pathology of Interstitial Lung Diseases Flashcards

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1
Q

What are some of the shared clinical and radiological manifestations of ILDs?

A

Symptoms:

  • Progressive dyspnea
  • Tachypnea
  • Persistent, non-productive cough

Diffuse abnormalities in lung mechanics and gas transfer (restrictive pattern, PFTs)

Imaging (HRCT):

  • Nodules
  • Irregular lines
  • Ground-glass opacities
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2
Q

The pulmonary interstitium is derived from what embryologic feature?

A

Splanchnic mesenchyme

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3
Q

What is the composition of the pulmonary interstitium? Function?

A

Supporting framework of the lung

  • Fine elastic fibers
  • Few fibroblasts
  • Inflammatory cells
  • Lymphovascular spaces
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4
Q

What are the ILD pattern-based categories?

What are the ILDs within each?

A

Fibrotic

  • UIP
  • NSIP (also cellular component)
  • Pneumoconiosis (macrophage nodules in silicosis)

Granulomatous

  • Sarcoidosis
  • HP (also cellular component)

Alveolar filling

  • COP
  • DIP
  • RB-ILD
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5
Q

Again, which ILDs are in the fibrotic pattern?

A
  • UIP
  • NSIP
  • Pneumoconioses
  • Asbestosis
  • Silicosis
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6
Q

What is the definition of usual interstitial pneumonia?

A
  • Subpleural/interlobular septal location
  • Patchy, temporally heterogenous interstitial fibrosis
  • Dense fibrotic scars
  • Fibroblastic foci

Honeycombing (end stage lung disease)

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7
Q

What may cause usual interstitial pneumonia?

A
  • Drug toxicity
  • Collagen vascular diseases
  • Unknown

(idiopathic UIP=idiopathic pulmonary fibrosis, IPF)

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8
Q

What is seen here?

A

HRCT of UIP (usual interstitial pneumonia)

  • Subpleural disease with honeycomb cysts
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9
Q

What is seen here?

A

Gross image of IUP

  • Cobble-stone pleural surface due to scarring
  • Subpleural process, patchy
  • Primarily lower lobes
  • Fibrosis, honeycomb
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10
Q

What are the major pathologic findings of UIP?

A
  • Patchy interstitial fibrosis, remodeling
  • Fibrosis subpleural, paraseptal and temporally heterogenous
  • Dense fibrosis with remodeling/honeycombing
  • Fibroblastic foci
  • Chronic interstitial inflammation, mild-moderate
  • Honeycombing
  • Inflamed cystic spaces filled with mucin
  • Lined by metaplastic bronchial cells
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11
Q

What is seen here?

A

Microscopic findings in UIP

  • Patchy fibrosis
  • Subpleural distribution
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12
Q

What is seen here?

A

Microscopic findings in UIP

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13
Q

What is seen here?

A

Microscopic findings in UIP

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14
Q

What is honeycombing?

A

End stage lung disease

  • Dense collagenous scars result in lung remodeling
  • Collapse of alveolar walls
  • Formation of cystic spaces lined by epithelial cells, filled with mucus and inflammatory cells
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15
Q

What is nonspecific interstitial pneumonia?

A
  • Histology does not conform to other ILD’s
  • Temporally uniform
  • Cellular (inflammatory) pattern
  • Fibrosing pattern
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16
Q

What causes nonspecific interstitial pneumonia?

A
  • Most idiopathic
  • Connective tissue diseases
  • Occupational and environmental exposures
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17
Q

What are the major pathologic findings in NIP?

A

2 patterns, prognostically different

Cellular pattern

  • Mild to moderate interstitial lymphoplasmacytic infiltrate (uniform distribution)
  • Infiltrate involves alveolar interstitium
  • Type II pneumocyte hyperplasia
  • Lung architecture preserved

Fibrosing pattern

  • Temporally uniform, diffuse (NO fibroblastic foci)
  • Lung architecture preserved (NO honeycombing)
  • Can see some chronic inflammatory cells
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18
Q

What is seen here?

A

NSIP: cellular pattern

  • Infiltrate on left
  • Normal lung on right
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19
Q

What is seen here?

A

NSIP: cellular pattern

  • Interstitial infiltrate
  • Architecture preserved
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20
Q

What is seen here?

A

NSIP: cellular pattern

  • Lymphoplasmacytic infiltrate
  • Type II pneumocyte hyperplasia
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21
Q

What is seen here?

A

NSIP: fibrosing pattern

  • Temporally uniform fibrosis
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22
Q

What is Pneumoconiosis?

A

Non-neoplastic lung reaction to inhalation of dust particles, chemical fumes, and vapors (occupational lung disease)

2 forms:

  • Asbestos-related diseases
  • Silicosis
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23
Q

What is asbestos?

A

Hydrated silicate fibers

  • Amphiboles: straight fibers; association with mesothelioma
  • Chrysotile: serpentine/curved fibers: primary form used in industry
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24
Q

What are some asbestos-related diseases?

A
  • Asbestosis: bilateral diffuse interstitial fibrosis
  • Fibrous pleural plaques
  • Pleural effusions
  • Mesothelioma: cancer of mesothelium (1000x’s compared to non-exposed)
  • Lung cancer: 55 fold increase in lung ca (Asb + smoking) vs 5 fold increase (Asb alone)
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25
What are some features of asbestosis (broadly)?
- Bilateral diffuse interstitial fibrosis caused by inhalation of asbestos fibers - Pattern of fibrosis is indistinguishable from other forms of ILD’s (need to see asbestos bodies) - Primarily involves lower lobes
26
What are gross features of asbestosis?
- Subpleural and parenchymal fibrosis +/- honeycomb change (lower lobe disease)
27
What are microscopic features of asbestosis?
Patchy interstitial and subpleural fibrosis (fibers stimulate release of mediators leading to repeated cycles of inflammation/fibrosis) - Starts around respiratory bronchioles and alveolar ducts, proceeds distally - Fibrosis similar to UIP Asbestos bodies (asbestos fiber + iron-protein coat
28
What is seen here?
Gross asbestosis - Honeycomb changes and fibrosis seen in lower lobes
29
What is seen here?
Microscopic Asbestosis - Thickening of pleura - Fibrosis (?) Looks a lot like UIP
30
What is seen here?
Asbestosis - Interstitial fibrosis - Asbestos bodies!
31
What is seen here?
Pleural plaques - Parietal pleura and domes of diaphragm
32
What is silicosis? What causes it?
- Most prevalent chronic occupational lung disease worldwide - Long exposure, slowly progressing nodular/fibrosing pneumoconiosis Caused by: inhalation of crystalline silicon dioxide (silica) - Sandblasting, coal mining, stone masonry, ceramic/pottery manufacture, etc
33
What is silica? What form causes silicosis?
Has amorphous and crystalline forms - **Crystalline** is more fibrogenic vs amorphous - Quartz, most abundant form of crystalline silica and therefore most frequently implicated in silicosis - Silica causes activation of macrophage mediators including fibrogenic cytokines
34
What are features of early and late lesions in silicosis? Other pathogenic features?
- **Early lesions**: dust-filled macrophages, lymphatic/bronchovascular distribution - **Later lesions**: silicotic nodules (lamellar fibrosis with birefringent silica particles) - Uniform fibrosis begins around bronchioles, can get honeycombing - **Upper lobe** disease
35
What is seen here?
Silicosis **- Early:** black nodules (upper lobe disease) **- Late:** dense nodules and parenchymal fibrosis
36
What is seen here?
Silicosis - Early lesions (dust filled macules) - Peribronchiole/lymphovascular routes
37
What is seen here?
Silicosis - Silicotic collagenous nodules - Birefringent silica particles
38
What are granulomatous ILDs?
- HP - Sarcoidosis
39
What is hypersensitivity penumonitis (HP)?
- Immune mediated ILD; prolonged exposure to inhaled organic antigens (AKA extrinsic allergic alveolitis) - Interstitial inflammatory and granulomatous reaction - Inhale dust with antigens from bacterial spores, fungi or animal proteins _Examples:_ - Farmer’s lung - Bird fancier’s lung - Humidifier/air conditioner lung - Maple bark stripper’s disease
40
What are microscopic findings in HP? (acute and subacute/chronic findings)
**Acute phase:** Neutrophils in alveoli and respiratory bronchioles (last 1-2 days) **Subacute/chronic:** - Interstitial lymphoplasmacytic infiltrate, begins around bronchioles (~100%) - Ill-defined, random, non-caseating granulomas (~67%) - Patchy organizing pneumonia (60%) (granulation type tissue in alveolar spaces) \* Can see fibrosis in late stage disease; can mimic UIP
41
What is seen here?
Hypersensitivity pneumonitis (HP) - Bronchiolocentric lymphoplasmacytic infiltrate (seen in 100%)
42
What is seen here?
Hypersensitivity pneumonitis (HP) - Giant cells; ill-defined granulomas (seen in 67%)
43
What is seen here?
Hypersensitivity pneumonitis (HP) - Organizing pneumonia (60%)
44
What is sarcoidosis? Pathologic findings?
- Systemic disease of unknown cause - Non-caseating granulomas involving lungs, lymph nodes, liver, spleen, bone marrow, skin, eyes, etc * Hilar lymph nodes or lung involvement (90%) - Disordered immune reaction in genetically predispose individuals Pathologic findings: - Multiple nodules; pleura, interlobular septa, bronchovascular structures - Well-formed granulomas * Epithelioid histiocytes * Multinucleated giant cells * Chronic inflammatory cells * Usually no necrosis (noncaseating) * Lung architecture preserved Still need to rule out infection
45
What is seen here?
Sarcoidosis - Well formed granulomas (much more so than HP) - Coalescing of nodules along bronchovascular channels
46
What is seen here?
Sarcoidosis - Coalescing of nodules along interlobular septum (left) and bronchovascular structure (right)
47
What is seen here?
Sarcoidosis - Well defined granulomas - No necrosis - Multinucleated giant cells, histiocytes, various inflammatory cells
48
What is seen here?
Top left: **Asteroid bodies** Top right and bottom: **Schaumann bodies** (70%) NOT specific for sarcoidosis
49
What are ILDs included in alveolar filling category?
- COP _Smoking-related ILDs:_ - RB-ILD - DIP
50
What is cryptogenic organizing pneumonia (COP)? Pathologic findings?
- Granulation tissue in distal airspaces - OP associated many conditions: infections, collagen vascular diseases, HP, inhalation injuries, XRT - COP = idiopathic OP (AKA BOOP, (bronchiolitis obliterans organizing pneumonia) _Pathologic findings:_ - Intraluminal plugs of granulation tissue (Masson bodies) in distal airways (bronchioles, alveolar ducts, alveoli) - Patchy distribution; temporally homogenous - Lung architecture preserved, NO honeycombing - Mild chronic interstitial inflammation
51
What causes cryptogenic organizing pneumonia (COP)?
Idiopathic
52
What is seen here?
COP - Patchy foci of nodular consolidation
53
What is seen here?
COP - Granulation tissue in airways - Overall lung architecture preserved
54
What is seen here?
Cryptogenic Organizing Pneumonia (COP) - "Bronchiolitis obliterans"- granulation tissue looks to be obliterating alveoli (this entity also seen in pts with chronic rejection)
55
What is seen here?
COP - Organizing granulation tissue within alveolar space
56
What is seen here?
COP - Myxoid appearance: fibroblasts, histiocytes, chronic inflammatory cells
57
What is respiratory bronchiolitis? Microscopic findings?
- Exuberant form of RB (clinical/radiologic findings of diffuse “interstitial” LD) - Primarily small airway disease (bronchioles/adjacent alveolar ducts, alveoli) - Virtually all patients current or past heavy smokers (at least 30 pack-year) _Microscopic findings:_ - Finely pigmented (dusty brown) macrophages in resp. bronchioles and adjacent alveolar ducts and alveoli - Bronchiole wall may have chronic inflammation and fibrosis - Metaplastic bronchiolar epithelium involves alveolar ducts \* Extensive RB + clinical and X-ray of ILD = RB-ILD
58
What is seen here?
Respiratory bronchiolitis (RB) - Nodular collections of macrophages in bronchioles and surrounding alveoli
59
What is seen here?
Respiratory bronchiolitis (RB) - Collection of macrophages in interstitium and adjacent alveolar tissue
60
What is seen here?
Respiratory bronchiolitis (RB) - Very fine, dusty brown pigment - Overall lung architecture preserved
61
What is seen here?
Respiratory bronchiolitis (RB): BAL specimen - Left: fine smoker's pigment - Right: hemosiderin (bulkier pigment)
62
What is seen here?
Respiratory bronchiolitis (RB) - Left: peribronchiolar fibrosis - Right: metaplastic bornchiole epithelium
63
What is Desquamative interstitial pneumonia (DIP)? Major pathologic findings?
- Desquamative – “desquamated” cells (believed to be pneumocytes) - Exclusively in current or former smokers - Numerous alveolar macrophages - Uniform fibrosis & chronic inflammation within interstitium - NO honeycombing _Pathologic features:_ - Diffuse involvement of lung parenchyma - Large accumulation of alveolar macrophages - Uniform fibrotic thickening of alveolar septa (mild-moderate) - Chronic interstitial inflammation (mild) - NO honeycombing, fibroblast foci, organizing pneumonia
64
What is seen here?
Desquamative interstitial pneumonia (DIP) - Some type II pneumocyte hyperplasia - Chronic inflammation
65
What is seen here?
Desquamative interstitial pneumonia (DIP) - Some fibrosis - Chronic inflammation - Macrophages within alveolar spaces
66
Summary of ILDs
Want to separate UIP from other ILDs - Heterogeneous, FF, honeycombing = UIP - Cellular and uniform fibrous patterns = NSIP - Uniform, luminal granulation tissue = COP - Hyaline membranes + interstital organization = DAD - Pigment macrophages + smoking Hx = RB-ILD/DIP - Granulomas + interstitial chronic infiltrate = HP - Noncaseating granulomas + hilar adenopathy = Sarcoidosis - Asbestos bodies + lower lobe disease = Asbestosis - Nodules/birefringent particles + upper lobe disease = Silicosis

MS2 Respiratory (26 decks)

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