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

9/16- Pathology of Interstitial Lung Diseases Flashcards

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
Q

What are some features of asbestosis (broadly)?

A
  • 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
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26
Q

What are gross features of asbestosis?

A
  • Subpleural and parenchymal fibrosis

+/- honeycomb change (lower lobe disease)

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27
Q

What are microscopic features of asbestosis?

A

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
Q

What is seen here?

A

Gross asbestosis

  • Honeycomb changes and fibrosis seen in lower lobes
29
Q

What is seen here?

A

Microscopic Asbestosis

  • Thickening of pleura
  • Fibrosis (?)

Looks a lot like UIP

30
Q

What is seen here?

A

Asbestosis

  • Interstitial fibrosis
  • Asbestos bodies!
31
Q

What is seen here?

A

Pleural plaques

  • Parietal pleura and domes of diaphragm
32
Q

What is silicosis? What causes it?

A
  • 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
Q

What is silica? What form causes silicosis?

A

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
Q

What are features of early and late lesions in silicosis?

Other pathogenic features?

A
  • 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
Q

What is seen here?

A

Silicosis

- Early: black nodules (upper lobe disease)

- Late: dense nodules and parenchymal fibrosis

36
Q

What is seen here?

A

Silicosis

  • Early lesions (dust filled macules)
  • Peribronchiole/lymphovascular routes
37
Q

What is seen here?

A

Silicosis

  • Silicotic collagenous nodules
  • Birefringent silica particles
38
Q

What are granulomatous ILDs?

A
  • HP
  • Sarcoidosis
39
Q

What is hypersensitivity penumonitis (HP)?

A
  • 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
Q

What are microscopic findings in HP? (acute and subacute/chronic findings)

A

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
Q

What is seen here?

A

Hypersensitivity pneumonitis (HP)

  • Bronchiolocentric lymphoplasmacytic infiltrate (seen in 100%)
42
Q

What is seen here?

A

Hypersensitivity pneumonitis (HP)

  • Giant cells; ill-defined granulomas (seen in 67%)
43
Q

What is seen here?

A

Hypersensitivity pneumonitis (HP)

  • Organizing pneumonia (60%)
44
Q

What is sarcoidosis? Pathologic findings?

A
  • 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
Q

What is seen here?

A

Sarcoidosis

  • Well formed granulomas (much more so than HP)
  • Coalescing of nodules along bronchovascular channels
46
Q

What is seen here?

A

Sarcoidosis

  • Coalescing of nodules along interlobular septum (left) and bronchovascular structure (right)
47
Q

What is seen here?

A

Sarcoidosis

  • Well defined granulomas
  • No necrosis
  • Multinucleated giant cells, histiocytes, various inflammatory cells
48
Q

What is seen here?

A

Top left: Asteroid bodies

Top right and bottom: Schaumann bodies (70%)

NOT specific for sarcoidosis

49
Q

What are ILDs included in alveolar filling category?

A
  • COP

Smoking-related ILDs:

  • RB-ILD
  • DIP
50
Q

What is cryptogenic organizing pneumonia (COP)?

Pathologic findings?

A
  • 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
Q

What causes cryptogenic organizing pneumonia (COP)?

A

Idiopathic

52
Q

What is seen here?

A

COP

  • Patchy foci of nodular consolidation
53
Q

What is seen here?

A

COP

  • Granulation tissue in airways
  • Overall lung architecture preserved
54
Q

What is seen here?

A

Cryptogenic Organizing Pneumonia (COP)

  • “Bronchiolitis obliterans”- granulation tissue looks to be obliterating alveoli (this entity also seen in pts with chronic rejection)
55
Q

What is seen here?

A

COP

  • Organizing granulation tissue within alveolar space
56
Q

What is seen here?

A

COP

  • Myxoid appearance: fibroblasts, histiocytes, chronic inflammatory cells
57
Q

What is respiratory bronchiolitis?

Microscopic findings?

A
  • 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
Q

What is seen here?

A

Respiratory bronchiolitis (RB)

  • Nodular collections of macrophages in bronchioles and surrounding alveoli
59
Q

What is seen here?

A

Respiratory bronchiolitis (RB)

  • Collection of macrophages in interstitium and adjacent alveolar tissue
60
Q

What is seen here?

A

Respiratory bronchiolitis (RB)

  • Very fine, dusty brown pigment
  • Overall lung architecture preserved
61
Q

What is seen here?

A

Respiratory bronchiolitis (RB): BAL specimen

  • Left: fine smoker’s pigment
  • Right: hemosiderin (bulkier pigment)
62
Q

What is seen here?

A

Respiratory bronchiolitis (RB)

  • Left: peribronchiolar fibrosis
  • Right: metaplastic bornchiole epithelium
63
Q

What is Desquamative interstitial pneumonia (DIP)?

Major pathologic findings?

A
  • 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
Q

What is seen here?

A

Desquamative interstitial pneumonia (DIP)

  • Some type II pneumocyte hyperplasia
  • Chronic inflammation
65
Q

What is seen here?

A

Desquamative interstitial pneumonia (DIP)

  • Some fibrosis
  • Chronic inflammation
  • Macrophages within alveolar spaces
66
Q

Summary of ILDs

A

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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