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Respiratory Medicine > Pathology slide set 3 > Flashcards

Pathology slide set 3 Flashcards

1
Q

What are the 3 hypersensitivity pneumonitis discussed

A
  • Farmer’s lung
  • Pigeon breeder’s lung or bird fancier’s disease
  • Humidifier or air-conditioner lunger
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2
Q

What does a BAL show in hypersensitivity pneumonitis

A
  • increased proinflammatory cytokines (IL-8 and MIP-1alpha)

- increased CD4 and CD8 T lymphocytes

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3
Q

Which sufactant dysfunction disorder is autosomal dominant?

A

surfactant protein C

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4
Q

besides lung involvement, what are the next most common organs involved in sarcoidosis

A

eyes and skin

Then spleen

then liver

then bone marrow (pharyngeal bones of hand and feet)

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5
Q

bilateral sarcoidosis of parotid, submaxillary, and sublingual glands

A

the uveoparotid involvement: MIKULICZ syndrome

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6
Q

the pathogensis of pneumonconioses depends on what 4 things

A
  • amount of dust retained
  • size, shape, and buoyancy of particles
  • particle solubility
  • additional effects of other irritants
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7
Q

as silicosis progresses, what happens to the nodules

A

become hard collagenous scars

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8
Q

what type of particles tend to persist in lung parenchyma for years and evoke fibrosing collagenous pneumoconioses

A

larger insoluble particles such as silicosis

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9
Q

What are the environmental factors that cause injury in idiopathic pulmonary fibrosis

A
  • tobacco smoke
  • occupational irritants
  • viruses
  • persistent gastric reflux
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10
Q

extensive scarring in advanced restrictive disease leads to what gross finding

A

honeycomb lung

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11
Q

What is the most innocuous coal induced pulmonary lesion in coal miners lung

A

Anthrocosis

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12
Q

epidemiology for Pulmonary Langerhans cells histiocytosis

A

young smokers

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13
Q

What do patients with desquamative interstitial pneumonia present with

A
  • cough
  • dyspnea
  • clubbing

over weeks or months

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14
Q

if an infarction occurs with a PE then what can be concluded about the patient

A

cardiovascular compromise

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15
Q

small lamellar bodies with electron dense cores are diagnostic of what/

A

ABCA3 mutation

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16
Q

Describe the granulomas in sarcoidosis

A

small, noncaseating often with multinucleated giant cells

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17
Q

CD4/CD8 ratio in sarcoidosis

A

5:1 to 15:1 suggesting pathogenic involvement of CD4 helper T cells

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18
Q

What cytotoxic cancer therapy drugs causes pulmonary damage and fibrosis

A

bleomycin

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19
Q

what is usually the first manifestation of asbestos

A

dyspnea on exertion

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20
Q

HLAs involved in Goodpasture

A

-HLA-DRB1*1501 and 1502

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21
Q

general pulmonary function changes in patients with restrictive lung disease

A

reductions in

  • diffusing capacity
  • lung volume
  • lung compliance
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22
Q

What cell function is impaired in sarcoidosis

A

dendritic

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23
Q

Adult patients with PAP present how?

A

cough and abundant sputum that is gelatinous

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24
Q

What is the only tissue available to biopsy for diagnosis of polyangiitis with Granulomatosis (Wegener granulomatosis)

A

Transbronchial lung biopsy

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25
Q

idiopathic pulmonary hypertension is most common in who?

A

women 20-40

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26
Q

What genes are thought to be linked to idiopathic pulmonary fibrosis (IPF)

A

TERT and TERC genes; encode telomerase

  • increase secretion of MUC5B
  • other rare forms have mutations in genes encoding components of surfactant
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27
Q

What surfactant dysfunction disorder presents in first few months of life with rapidly progressive respiratory failure followed by death

A

ABCA3

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28
Q

Tiny sheet of calcification occur in lymph nodes and seen radiographically as EGGSHELL calcification

A

silicosis

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29
Q

What is the most common crystalline that is implicated in silicosis

A

quartz

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30
Q

explain the pathogenesis of silicosis after the particles are phagocytized by macrophages

A
  • activate inflammasome

- release of IL-1 and IL-18

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31
Q

Goodpasture syndrome shows antibodies to what

A

alpha3 chain of collagen IV

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32
Q

Laminated concretions composed of calcium and proteins (Schaumann bodies) and stellate inclusions (asteroid bodies)

A

Sarcoidosis

not pathognomonic. also found in TB

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33
Q

Standard care for PAP

A

whole lung Lavage and GM-CSF therapy in autoimmune version

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34
Q

characterized by intensely blackened scars from 1 to 10 cm. microscopically dense collagen and pigment. center of lesion is often NECROTIC

progressive massive fibrosis

A

complicated coal workers pneumonconiosis

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35
Q

histologic changes of hypersensitivity pneumonitis are characteristically centered where and what type of cells are rare

A
  • bronchioles

- Eosinophils

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36
Q

Defect in GM-CSF or pulmonary macrophage dysfunction that results in accumulation of surfactant in the intra-alveolar and bronchiolar spaces

A

Pulmonary Alveolar Proteinosis (PAP)

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37
Q

Whats the median survival after diagnosis of IPF

A

3 years . . doesn’t respond well to treatment

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38
Q

Ferruginous bodies

A

Asbestos

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39
Q

What are the major categories of chronic interstitial lung disease

A
  • Fibrosing
  • Granulomatous
  • Eosinophilic
  • Smoking related
  • other
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40
Q

if coal workers pneumoconiosis leads to progressive massive fibrosis then what are the consequences

A
  • respiratory failure
  • pulmonary HTN
  • cor pulmonale
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41
Q

fibroblastic foci, honeycombing, hyaline membranes, granulomas are ABSENT

A

NIP

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42
Q
  • intermittent, diffuse alveolar hemorrhage
  • young children
  • productive cough, hemoptysis and anemia
  • similar to goodpasture but NOanti-basement membrane antibodies
A

Idiopathic pulmonary Hemosiderosis

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43
Q

immunologic reaction to inhaled organic antigens causing interstitial pneumonitis (lymphocytes, plasma cells and macrophages, NONcaseating granulomas, and interstitial fibrosis

A

hypersensitivity pneumonitis

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44
Q

What is asbestos marked by

A

diffuse pulmonary interstitial fibrosis with asbestos bodies . . . may lead to honeycomb lung

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45
Q

if a nodule in silicosis undergoes central softening and cavitation then what happened

A

superimposed TB or ischemia

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46
Q

What type of asbestos is most often used industrially

A

serpentine chrysotile

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47
Q

golden brown, fusiform or beaded rods with a translucent center and consist of fibers coated with an iron containing proteinaceous material

A

Asbestos bodies

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48
Q

age for IPF

A

over 50

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49
Q

autoimmune form of PAP has antibodies to what and found in what age group

A
  • GM-CSF - impairs macrophage ability to catabolize surfactant
  • adults and lacks familial predisposition
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50
Q

immature dendritic cell with grooved, indented nuclei and abundant cytoplasm

A

Langerhans cell

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51
Q

characterized by presence of pigmented intraluminal macrophages within first and second order RESPIRATORY BRONCHIOLES

A

Respiratory Bronchiolitis-Associated Interstitial

Lung Disease

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52
Q

What diseases can cause secondary PAP

A
  • hemotopoietic disorders
  • malignancies
  • immunodeficiency disorders
  • Lysinuric protein intolerance
  • acute silicosis
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53
Q

Goodpasture gender

A

males and most are active smokers

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54
Q

in IPF when dense fibrosis causes destruction of alveolar architecture and formation of cystic spaces lines by hyperplastic type II pneumocytes or bronchiolar epitheilum

A

honeycomb FIBROSIS

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55
Q

Acute attacks of hypersensitivity show what?

A

RECURRING episodes of fever, dyspnea, cough, and leukocytosis

56
Q

group of fibrous hydrated silicates known to cause interstitial and plueral fibrosis and lead to lung carcinoma and malignant mesothelioma

A

Asbestos related

57
Q
  • S100
  • CD1a
  • CD207

-NEGATIVE for CD68

A

Langerhans cell

58
Q

time frame for radiation-induced pneumonitis

A

1-6 months after

59
Q

What part of lung is more heavily involved in simply coal workers pneumoconiosis

A

upper lobes and upper zones of lower lobes

60
Q

Alveoli filled with numerous macrophages with, some with phagocytosed surfactant . . former or current smoker

A

desquamative interstitial pneumonia

61
Q

What are the 2 histological forms of nonspecific interstitial pneumonia (NIP)?

A
  • cellular (inflammation)

- fibrosing (SAME AGE, fibrotic lesions)

62
Q

what do a great majority of Sarcoidosis patients present with

A

insidious onset of respiratory abnormalities (shortness of breath, cough, chest pain, and hemoptysis) or of constitutional signs and symptomes (fever, fatigue, weight loss, anorexia, night sweats)

63
Q

What are the 2 broad categories of restrictive lung disorders

A
  • chronic interstitial and infiltrative disease

- chest wall disorders

64
Q

epidemiology for desquamative interstitial pneumonia

A
  • genders equal

- 30s-40s

65
Q

Alveoli precipitate periodic acid-schiff positivity (GRANULAR AND PINK), and contain cholesterol clefts and surfactant proteins in this disorder

A

PAP

66
Q

what gene does Hereditary PAP (extremely rare) involve and what age group

A

GM-CSF

neonates

67
Q

Shipyard

A

asbestos

68
Q

What do patients with COP presnet with

A

cough and dyspnea

69
Q

prognosis for Asbestosis complicated by lung or pleural malignancy

A

Poor/Grim

70
Q

nonneoplastic lung reaction to inhalation of mineral dusts, organic, or inorganic particulates and chemical fumes and vapors encountered in the workplace

A

Pneumonconioses

71
Q

Silicosis is associated with increased susceptibility to what?

A

TB and double risk of lung cancer

72
Q

what are the 2 types of asbestos diseases

A

Serpentine chrysotile and amphibole

73
Q

does coal workers pneumoconiosis in absence of smoking predispose to cancer?

A

NO

74
Q

microscopically, fibrosis is patchy, variable intensity and age with early lesions showing fibroblastic foci

-coexistence of BOTH early and late lesions

A

IPF

75
Q

clinical symptoms of IPF

A
  • increasing dyspnea on exertion
  • dry cough

Late in course

  • hypoxemia
  • cyanosis
  • clubbing
76
Q

What are the T cell derived cytokines increased in sarcoidosis and what are they responsible for

A

IL-2: T-cell expansion

IFN-gamma: macrophage activation

77
Q

characterized predominantly by inflammation and fibrosis of pulmonary interstitium

A

chronic interstitial pulmonary diseases

78
Q

Genetic association with sarcoidosis

A

HLA-A1 and HLA-B8

79
Q

central area of WHORLED collegen fiber with a more peripheral zone of dust-laden macrophages . . UPPER

-by polarized microscopy you see birefringence

A

silicosis

80
Q

time frame for symptoms of asbestos

A

more commonly 20 to 30 years after first exposure. rarely fewer than 10

81
Q

What are the systemic immunologic abnormalitie that are found in those with sarcoidosis

A
  • anergy to common skin test antigens such as Candida or TB PPD
  • polyclonal hypergammaglobulinemia
82
Q

Does “smoky coat” (bituminous), domestic indoor use for cooking and heating increase risk for lung cancer

A

YES

83
Q

describe the lung grossly in PAP

A

increase in size and weight

84
Q

Plexiform lesion . . . a tuft of capillary formations is present, producing a network, or web, that spans the lumens of dilated thin-walled small arteries and may extend outside the vessel

A

pulmonary hypertension

85
Q

Inflammatory cells in IPF?

A

lymphocytes admixed with few plasma cells, neutrophils, eosinophils, and mast cells

86
Q

Treatment for COP

A

some recover spontaneously but most need oral steroids for 6 months or longer

87
Q

female nonsmokers in 50s presents with dyspnea and cough of several months that gets better

A

NIP

88
Q

histological pattern of fibrosis in idiopathic pulmonary fibrosis

A

usual interstitial pneumonia (UIP)

89
Q

what symptoms are caused by the pleural plaques of asbestos

A

NONE

90
Q

if acute radiation pneumonitis progresses to chronic, what is the result

A

fibrosis . . DAD

  • atypia of hyperplastic type II cells and fibroblasts
  • epithelial cell atypia and foam cells within vessel walls
91
Q

Some cases of Pulmonary Langerhans cell histocytosis are a reactive inflammation while others, the langerhans cells have acaquired activating mutations in what?

A

Serine/Threonine Kinase BRAF

92
Q

Where do 3/4 of infarctions from PE occur

A

lower lobes

93
Q

Does coal workers pneumoconiosis increase susceptibility to TB?

A

NO

94
Q

clinicopathologic syndrome marked by progressive interstitial fibrosis and respiratory failure

A

idiopathic pulmonary fibrosis (IPF)

95
Q

chest radiographs for restrictive lung disease

A

bilateral lesions that take form of small nodules, irregular lines, or GROUND GLASS shadows

96
Q

in coal workers lung disease, where do the macrophages with the carbon dust accumulate

A

along pulmonary lymphatic tissue; along bronchi and hilus

97
Q

Where are pleural plaques developed most frequently

A

anterior and posterolateral aspects of the PARIETAL PLEURA and over domes of diaphragm

98
Q

Symptoms of radiation induced pneumonitis that resolve with steroid therapy

A
  • fever
  • dyspnea out of proportion to volume of lung irradiated
  • pleural effusion
  • infiltrates
99
Q

Surfactant protein B

A

LEAST COMMON

  • autosomal recessive
  • full term baby
  • rapidly progressive respiratory distress shortly after birth
  • death b/t 3 and 6 months
100
Q

patients with PAP are at risk for what

A

-respirator failure and infection

101
Q

What cytokines are increased in the local environment of sarcoidosis and favor recruitment of additional T cells and monocytes and contribute to formation of granulomas

A

IL-8
TNF
MIP-1alpha

102
Q

Lines of Zahn

A

clot was formed in living person

103
Q

What type of particles cause rapid onset lung damage

A

high solubility

104
Q

presence of polypoid plugs of loose connective tissue (Masson bodies) within ALVEOLAR ducts, alveoli and often bronchioles.

all same age and lung architecture normal

NO INTERSTITIAL FIBROSIS OR HONEYCOMB LUNG

A

cryptogenic organizing Pneumonia (COP)

105
Q

which asbestos is more flexible, curled and soluble and susceptibe to ciliary elevator

A

serpentine chrysotile

106
Q

multiorgan granulomatous disorder of unknown cause

A

Sarcoidosis

107
Q

difference b/t hypersensitivity pneumonitis and asthma

A

the former leads to pathologic changes that primarily involve the ALVEOLAR WALL

108
Q

what cardiac arrythmic drug can cause significant pneumonitis

A

Amiodarone

109
Q

Early stages of silicosis

A

tiny, barely palpable, discrete PALE to blackened nodules in HYLAR lymph nodes and UPPER zones of lungs

110
Q

COP is most often seen as a response to what?

A

infections or inflammatory injury of the lungs

111
Q

what type of asbestos is more pathogenic

A

amphibole . goes deep in lungs (stiff and straight)

112
Q

What size particles are most pathogenic in pneumonconioses

A

1-5 micrometers

113
Q

whats on CT with NIP?

A

bilateral symmetric predominantly lower lobe reticular opacities

114
Q

difference between coal macules and coal nodules seen in simple coal workers pneumonconiosis

A

macules: (1-2 mm) carbon laden macrophages
nodules: also contain a delicate network of collagen fibers

115
Q

general clinical findings in patients with restrictive lung disease

A
  • dyspnea
  • tachypnea
  • END INSPIRATORY CRACKLES
  • eventual cyanosis WITHOUT WHEEZING
116
Q

Accumulation of dendritic cells (langerhan cells) to form nodules, along with fibroblasts, macrophages, and numerous EOSINOPHILS

A

Pulmonary Langerhans cell histiocytosis

117
Q

What type of emphysema can coal workers pneumoconiosis give to?

A

centrilobular (centriacinar)

118
Q

What does desquamative interstitial pneumonia respond to

A

steroid therapy

119
Q

Goodpasture age

A

teens or 20s

120
Q

SUBPLEURAL and INTERLOBULAR septal fibrosis preferentially in lower lobes

A

IPF

121
Q

what part of the lung does asbestos affect

A

base first and sub pleurally

122
Q

What type of hypersensitivity is hypersensitivity pneumonitis

A

type 4

123
Q

pleural plaques

A

most common manifestation of ASBESTOS exposure, are well-circumscribed plaques of dense collagen that are often calcified

124
Q

macrophages with abundant cytoplasm containing dusty brown pigment (SMOKERS MACROPHAGES) in airspaces

A

desquamative interstitial pneumonia

125
Q

What is the cause of idiopthic pulmonary fibrosis (IPF) thought to be

A

unknown but thought to occur secondary to repeated injury to alveolar epithelial cells with a pro-fibrotic response in GENETICALLY susceptible people

126
Q

What cytokines does BMPR2 bind

A
  • TGF-beta
  • BMP
  • activin
  • inhibin
127
Q

What in the bronchoalveolar fluid is a marker of sardoidosis disease activity

A

TNF

128
Q

What is thought to be the pathogenesis of sarcoidosis

A

disordered immune regulation in genetically predisposed individuals

129
Q

relationship between asbestos and smoking and cancer

A

Synergy. Asbestos alone increases incidence 5 fold. with smoking 55 fold

130
Q

What is involved in 90% of cases of sarcoidosis

A

bilateral hilar lymphadenopathy or lung involvement

131
Q

What is mutated or dysfunctional in idiopathic pulmonary arterial hypertension

A

BMPR2

132
Q

epidemiology of sarcoidosis

A
  • younger adults less than 40
  • 10x higher in blacks
  • females more common
  • highest rates in southest U.S.
  • RARE in chinese and southest Asians
133
Q

Abnormalities in lamellar bodies in type II pneumocytes

A

All 3 surfactant dysfunction disorders

134
Q

epidemiology of repiratory bronchiolitis-associated interstitial Lung disease

A
  • mild
  • current smoker in 30s-40s
  • ave. 30 pack year history
135
Q
  • crystalline, sand blasting, concrete cutting, stressed denim, jewelers
  • African Americans higher risk
  • slowly progressing nodular collagenous/fibrosing scars
A

Silicosis

136
Q

What is the most frequently mutated gene in surfactant dysfunction disorder? heredity?

A

ABCA3 (AUTOSOMAL RECESSIVE)

Respiratory Medicine (37 decks)

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