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S3B1 Pathology > Chronic Diffuse Interstitial Lung disease > Flashcards

Chronic Diffuse Interstitial Lung disease Flashcards

1
Q

All chronic interstitial lung diseases are characterized by what two key factors?

A

inflammation & fibrosis

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2
Q

What are the classic 5 presentations of restrictive lung disease?

A
  1. Dyspnea
  2. tachypnea
  3. End-inspiratory crackles
  4. eventual cyanosis
  5. no wheezing or other signs of airway obstruction
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3
Q

What radiological findings would you expect to see in an individual with interstitial lung disease?

A

nodules

irregular lines

ground glass shadows

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4
Q

What are the potential sequelae associated with interstitial lung disease?

A
  • secondary pulmonary hypertension & right sided heart failure
  • scarring & gross destruction of lungs
    • “honeycomb” lung
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5
Q

Describe what is shown in the images on the right & lett.

A
  • Left: normal lung histology
  • Right: Usual Interstitial Pneumonia (Idiopathic Pulmonary Fibrosis)
    • patchy disease
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6
Q

What is the clinical presentation of Idiopathic Pulmonary Fibrosis?

This disease belongs in what larger Category?

Most commonly affected demographic?

A
  • Insidious onset
    • slowly progressive shortness of breath
    • dry cough
    • no fever
    • inspiratory crackles
    • clubbing in fingers
    • eventual respiratory failure & death in 3-5 yrs
  • Demographics
    • 50 -70 yrs
    • male
  • Fibrosing Disease
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7
Q

What is the difference between idiopathic pulmonary fibrosis (IPF) and usual interstitial pneumonia (UIP)?

A

IPF is clinical syndrom

UIP is a histologic pattern

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8
Q

Does idiopathic pulmonary fibrosis respond to corticosteroids?

A

no

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9
Q

What pathology is shown in the provided image?

A

Hypertrophic osteoarthropathy

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10
Q

What pathology is shown in the provided image?

Describe how you identified this.

A

Usual Interstitial Pneumonia

lung parenchyma with thickened alveolar walls (arrows) surrounded by areas of normal lung

the fibrotic alveolar walls have scattered smal lymphocytes & occasional plasma cells

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11
Q

What pathology is shown in the provided image?

Describe its characteristics.

A

Usual Interstitial Pneumonia

Patchy interstitial fibrosis (right)

mild/moderate chronic inflammation with fibrotic areas

Temporal heterogeneity of lesions (lesions of different ages & varying stages of development) - pale/blue/pink areas are younger lesions (more fibroblasts) - pinker areas means more collagen, which means older lesions

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12
Q

Is the provided image an example of an eary or older lesion found with usual interstitial pneumonia?

How can you tell this is usual interstitial pneumonia?

A

Early

Fibroblastic focus (black open arrow) shows young collagen admixed with spindled fibroblasts

fibroblast pugs are in the wall

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13
Q

What pathology & stage is shown int the provided images?

A

Usual Interstitial Pneumonia - Late Stage

  • Left
    • massive multicystic changes due to “honeybombing” of lung at periphery
  • Right
    • honeybomcing is characterized by dilated air spaces at the lung periphery
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14
Q

What is the most common cause of death for patients diagnosed with usual interstitial pneumonia?

A
  • Death: respiratory failure
  • ~ 5 years
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15
Q

Does Usual Interstitial Pneumonia respond to corticosteroids?

A

no

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16
Q

What samples are needed to make the diagnosis of usual interstitial pneumonia if clinical & radiographic information alone is not enough?

A

lung biopsy (VATS or open lung biopsy)

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17
Q

What demogrpahics are most commonly affected by nonspecific interstitial pneumonia?

A
  • 25-60 yrs
  • female
  • seen with collagen-vascular disease
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18
Q

Does nonspecific interstitial pneumonia respond to corticosteroids?

A

yes, dramatic response

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19
Q

What pathology is shown in the provided images?

A

temporarily “uniform”

  • Left
    • small lyphoid aggregates seen at lower power
  • Right
    • associated with interstitial widening & some interstitial fibrosis
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20
Q

What is the cause of Cryptogenic Organizing Pneumonia?

Symptoms?

Presentation?

A
  • Idiopathic primary or secondary to other conditions
  • Symptoms
    • cough
    • dyspnea
    • fever
    • malaise
    • flu-like symptoms
  • Presentation
    • mild inflammation
    • may involve any part of either or both lungs
    • normal lung architecture
    • NO interstitial fibrosis or honeycomb lung
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21
Q

Doescryptogenic organizing pneumonia respond to corticosteroids?

A

most patients respond to corticosteroids

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22
Q

What pathology is shown in the provided images?

A

Cryptogenic Organizing Pneumonia (COP/BOOP)

  • Left
    • patchy organization
    • loose granulation tissue in terminal airways & alveoli
  • Right
    • mild interstitial pneumonia & patchy organization within airways
23
Q

What is pneumoconioses?

What 6 factors affect pathogenesis?

A

chronic lung diseases related to inhalation of dust particles or chemicals

  1. amoutn of paticles/fumes
  2. size, shape, buoyancy of particles
  3. solubility & cytotoxicity
  4. interaction with fibroblasts, macrophages
  5. activation of inflammasome
  6. other irritants
24
Q

What pathology is shown in the provided images?

How can you tell?

A
  • Left: normal lung, thin section
    • scattered minimal accumulations of anthracotic pigment & intact parenchyma
  • Right
    • simple Coal Worker’s Pneumoconiosis, thin section
    • multiple small, circumscribed black nodules, mainly in the upper lung
25
Q

What pathology is shown in the provided image?

A

Complicated Coal Workers’ Pneumoconiosis

thin section, large black irregular fibrotic lesion has destroyed the perihilar lung parenchyma

26
Q

What pathology is shown in the provided image?

A

Simple Coal Workers’ Pneumoconiosis

Coal dust macule has focal interstitial pigment deposition

27
Q

What pathology is shown in the provided image?

A

Complicated Coal Workers’ Pneumoconiosis

progressive massive fibrosis with haphazardly arranged collagen bundles intespersed with carbon pigment

28
Q

What is the most prevalent chronic occupational disease in the world?

A

silicosis

29
Q

What is the cause of silicosis?

What demographics are most commonly affected?

It can lead to what other complications?

A
  • Caused by inhalation of cryatalline silicon dioxide (silica)
  • Demographics
    • mining & quary work
    • repair or demolition of concrete structure
    • sandblasting
    • sone carvers
  • Complications
    • disease progresses even if exposure ends
    • increased susceptibilty to tuberculosis
    • increase risk for carcinoma
30
Q

Describe the pathogenesis of silicosis

A
  • Silica gets inhaled & activate the inflammasome
    • activation of protein complex that recognizes products of dead cells, microbes & particulate matter
    • induces scretion of biologically active interleukin 1
  • Eventually lead to massive fibrosis
31
Q

What pathology is shown in the provided images?

A

Silicosis

  • Left: scarring has contracted the upper lobed into a small dark mass (arrow)
    • note the dense pleural thickening
  • Right: Thin section
    • typical well-demarcated, round, whorled nodules in the upper lobe
32
Q

What pathology is shown in the provided images?

A

Silicosis

  • Right: several colescent collagenous silicotic nodules
  • Left: Silica crystals, polarized light microscopy
    • bright white crystals of varying size
33
Q

What are the Asbestos-related Diseases?

A
  1. Localized fibrous plaques
  2. Recurrent pleural effusions
  3. Parenchymal interstitial fibrosis
  4. Lung carcinoma
  5. Mesotheliomas
  6. Carcinomas of larynx, ovary, colon & others
34
Q

Identify the 3 main forms of Asbestos shown in the provided image.

Describe the characteristics of each.

A
  1. Crystotile
    • flexible, curved
  2. Amosite
    • straight, stiff, brittle
  3. Crocidolite
    • thin, extremely sharp
35
Q

What pathology is shown in the provided image?

Identify the defining characteristics

A

Asbestosis

  • Left: lower lobe with patchy fibrosis (pale) & visceral pleura is thickened (arrowhead)
  • Right: advanced case with honeycombing (arrowhead) and lower lobe fibrosis
36
Q

What pathology is shown in the provided image?

A

Asbestosis

the lung parenchyma is extensively replaced by dense fibroconnective tissue (black curved arrow), leaving only focal areas of uninvolved lung parenchyma (black straight arrows)

37
Q

What pathology is shown in the provided images?

A

Asbestosis

  • Left
    • peribronchiolar fibrosis accompanied by asbestos bodies (arrows)
  • Right
    • numerous asbestos bodies within a fibrotic alveolar septum

Asbestos bodies: rods w/ knobs at either end (they are coated in iron)

38
Q

What pathology defining feature is shown in the provided images?

A

asbestos bodies

  • (left) iron-stain - beaded morphology and deep blue color
  • (right) papanicolaou stain - clear fibrous core coated by iron-containing material
39
Q

What pathology is shown in the provided image?

How do you know?

What kind of symptoms would you expect to see in someone with this pathology?

A

Asbestos-related Fibrous Peural Plaques

  • Left: dense fibrous tissue w/ minimal cellular reaction
  • Right: large, discrete fibrocalcific plaques on the pleural surface of the diaphragm
    • tan/yellow material is plaque (collagen)

Typically asymptomatic

40
Q

What is sarcoidosis?

What are the most commonly affected demographics?

A
  • Sarcoidosis
    • non-necrotizing granulomas in multiple organs, most commonly lungs
    • bronchocentric, perivascular distribution
  • Demographics
    • young adults
    • female
    • African American
41
Q

What procedure is performed to diagnose sarcoidosis?

A

trans-bronchial biopsy

do NOT need open lung biopsy

42
Q

What is the triad found in patients with symptomatic sarcoidosis?

A

Lofgren syndrom

  1. fever
  2. erythema nodosum
  3. bilateral hilar lymph adenopathy

Saroidosis is a diagnosis of exclusion

43
Q

What pathology is shown in the provided image?

A

Sarcoidosis

non-necrotizing granulomas

can have giant cells within them

44
Q

Identify the histological features shown it the provided images.

These features are typically present in what pathology?

A

Multinucleated giant cells, with distinctive cytoplasmic inclusions

  • Upper left: Asteroid body
    • star-like body within the giant cell
  • Upper right: Schaumann body
  • Lower left: Schuamann bodies
  • Lower right: Schaumann bodies, polarized light
45
Q

What is hypersensitivity pneumonitis?

What is it caused by what 3 main conditions?

A
  • Immune-mediated
    • can be acute or chronic
  • Due to prolonged exposure to inhaled organic antigens
    • proteins from bird feathers & feces
    • thermophilic bacteria in hay
    • thermophilic bacera in heated water
46
Q

What complication can result from chronic hypersensitivity pneumonitis?

A

end stage fibrosis

47
Q

What pathology is shown in the provided images?

A

Hypersensitivity Pneumonitis

  • Left: interstitial “granulomas” are poorly formed & may be missed (arrow)
  • Right: Giant cells within the granulomas may draw attention to their presence (arrows)
48
Q

What pathology is shown in the provided image?

A

Desquamative Interstitial Pneumonia

49
Q

What Desquamative Interstitial Pneumonia?

Cause?

Symptoms?

A
  • Large numbers of macrophages accumulate within alveolar spaces – only mild thickening of alveolar walls
  • Associated wth injury from smoking
  • Symptoms
    • subacute onset dyspnea, cough
50
Q

What is Pulmonary Langerhans Cell Histiocytosis?

Describe its pathogenesis.

What types of lesions would you see?

A
  • Focal collections of Langerhands cells +/- eosinophils
  • Scarring
    • airway destruction and alveolar damage
      • irregular cystic spaces
  • Lesions:
    • stellate & nodular
51
Q

What pathology is shown in the provided images?

A

Langerhans Cell Histiocytosis

  • Left:
    • Stellate shaped area at periphery of lung with sparse infiltrate
  • Right:
    • Nodular lesion composed of sheets of Langerhans cells, adjacent inflammation
52
Q

What pathology is indicated by the cells shown in the following images?

A

Langerhans Cell Histiocytosis

  • Left:
    • Langerhans cell histiocytes
    • Oval to spindle cells with nuclear convolution and nuclear grooves (black curved arrow)
  • Right:
    • Birbeck granules, seen only on EM
    • zipper-like structures that are pathognomonic for Langerhans Cell Histiocytosis
53
Q

Waht is Pulmonary Alveolar Proteinosis?

Types?

Symptoms?

A
  • Alveoli filled with surfactant proteinaceous material
    • caused by defects in GM-CSF or pulmonary macrophages
  • Types
    • autoimmune, secondary, hereditary
  • Symptoms
    • dyspnea, chest pain, fever, & cough
    • heavy congested lungs bilaterally
54
Q

What pathology is shown in the provided images?

A
  • Left:
    • proteinaceous material (curved arrow)
    • fresh blood (open arrow)
    • inflammation (straight arrow)
  • Right
    • complete filling of alveolar spaces by granular, acellular proteinaceous material

S3B1 Pathology (7 decks)

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