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Pulmonology Exam > Pulmonary Pathology 3 > Flashcards

Pulmonary Pathology 3 Flashcards

1
Q

What are three interstitial lung diseases that are fibrotic in nature?

A

usual interstitial PNA, non-specific interstitial PNA, and cryptogenic organizing PNA (COP)

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2
Q

how do you make the diagnosis of idiopathic pulmonary fibrosis?

A

diagnosis requires classic findings on high-resolution CT scan or pulmonary biopsy

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3
Q

what does pulmonary biopsy show if a clinician diagnoses a patient with idiopathic pulmonary fibrosis?

A

usual interstitial pneumonia (UIP)

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4
Q

what four things make up UIP?

A

normal areas, inflammation, fibroblast foci, peripheral honeycombing

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5
Q

Why do people get idiopathic pulmonary fibrosis?

A

it’s not quite well understood, but their are contributing factors such as environmental factors (industrialized societies, and smoking), genetic factors, and increasing age

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6
Q

what are the clinical findings associated with idiopathic pulmonary fibrosis?

A

shortness of air, crackles on exam (velcro), restrictive pattern on PFTs, basilar infiltrates that progress to honeycomb sign

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7
Q

how is honeycombing different from emphysema?

A

the spaces in honeycombing are surrounded by a think rhine

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8
Q

what is the prognosis of idiopathic pulmonary fibrosis?

A

bad; it has a progressive course- most patients die from the respiratory disease 3-5 years after diagnosis

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9
Q

what are the potential therapies for idiopathic pulmonary fibrosis?

A

lung transplantation or medication to arrest fibrosis such as tyrosine kinase inhibitors or TGF-beta inhibitors

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10
Q

what is non-specific interstitial pneumonia?

A

idiopathic, but it is NOT non-specific

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11
Q

what is the histologic features of non-specific interstitial PNA?

A

uniform infiltrates and fibrosis, no heterogeneity, no fibroblast foci, no granulomata

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12
Q

why is the interstitium thickened in NSIP?

A

there’s a bunch of inflammatory cells here- most of which are lymphocytes and some plasma cells

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13
Q

what is the predominant pattern of cryptogenic organizing pneumonia?

A

fibroblastic foci (Masson bodies)

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14
Q

what is the presentation of cryptogenic organizing pneumonia?

A

it occurs superimposed on a prior infection or inflammatory process; presents with pneumonia-like consolidation in the 5th/6th decades

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15
Q

what type of diagnosis is cryptogenic organizing pneumonia?

A

a diagnosis of exclusion- you have to make sure you aren’t dealing with an active infection, drug- or toxin-induced, or related to a connective tissue disorder

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16
Q

how do you treat cryptogenic organizing pneumonia?

A

patients tend to have a full recovery with oral steroids

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17
Q

what if a patients presents with symptoms of an idiopathic pulmonary fibrosis but they also have an underlying autoimmune/connective tissue disease (RA, systemic sclerosis, lupus erythematous), what would you call it?

A

a connective tissue associated ILD

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18
Q

what are two ILDs that are considered to be granulomatous diseases?

A

sarcoidosis and hypersensitivity pna

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19
Q

what is sarcoidosis?

A

a systemic disease manifesting non-caseating (non-necrotizing) granulomata in various organs

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20
Q

what is the clinical presentation of sarcoidosis?

A

incidental abnormal radiograph; dyspnea

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21
Q

what cells for the granuloma found in sarcoidosis?

A

epithelioid histiocytes

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22
Q

what is the most identifiable cell in a granuloma?

A

the multinucleated giant cell

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23
Q

how can you tell if someone has sarcoidosis based on chest radiograph?

A

there are different stages, but hilar lymphadenopathy is one of the common ways that sarcoidosis can present

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24
Q

what are the histologic features of sarcoidosis?

A

wavy collagen showing dense fibrosis; tight and well circumscribed

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25
Q

what are the two granuloma inclusions that should make you think sarcoidosis?

A

asteroid bodies, and schaumann bodies (both found in the giant cells)

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26
Q

What is the common demographic of sarcoidosis?

A

young, african american

27
Q

what is a common lab finding associated with sarcoidosis?

A

elevated serum ACE levels

28
Q

what is hypersensitivity pneumonitis?

A

an ill-defined granulomata; immune reaction to an inhaled antigen

29
Q

what are 3 examples of hypersensitivity pneumonitis?

A

pigeon-breeder’s lung, farmer’s lung, and hot tub lung

30
Q

what are you having an immune reaction to in cases of farmer’s lung?

A

actinomycetic spores in hay

31
Q

What are you having an immune reaction to in cases of hot tub lung?

A

reaction to mycobacterium avium complex (MAC)

32
Q

what is the classic characteristic of hypersensitivity pneumonitis?

A

airway centered granulomata with associated lymphocytes- you’ll see a very specific airway concentric granuloma

33
Q

how do you diagnose hypersensitivity pneumonitis?

A

HISTORY

34
Q

what are three interstitial lung diseases that are smoking-related diseases?

A

desquamative interstitial pneumonia, respiratory bronchiolitis-interstitial lung disease, langerhans cell histiocytosis

35
Q

how does desquamative interstitial pneumonia present?

A

smokers in their 4th-5th decade of life, restrictive lung disease presentation

36
Q

what are the histologic features of desquamative interstitial pneumonia?

A

very characteristic “stuffed” alveolar spaces full of macrophages

37
Q

what is the prognosis of desquamative interstitial pneumonia? Treatment?

A

good prognosis; stop smoking and corticosteroids

38
Q

what is respiratory bronchiolitis-interstitial lung disease?

A

part of a spectrum with DSIP, but less symptomatic and earlier presentation

39
Q

what is the presentation of respiratory bronchiolitis- interstitial lung disease?

A

3rd-4th decades, dose dependent; radiographic abnormalities prompt biopsy for diagnosis

40
Q

what are the biopsy findings suggestive of RB-ILD?

A

macrophages present to a lesser extent, “peribronchiolar” metaplasia (abnormally located ciliated cells), may have fibrosis in advanced cases

41
Q

what is langerhans cell histiocytosis (LCH)?

A

young smokers with stellate lung lesions; progressive scarring leads to cysts

42
Q

what happens if the peripheral cysts seen in LCH rupture?

A

it can cause a pneumothorax

43
Q

what are the histologic features associated with LCH?

A

eosinophils, langerhans cells (immature dendritic cells), varying fibrosis and cysts

44
Q

how do you treat LCH?

A

can reverse with smoking cessation

45
Q

how do you make the diagnosis of LCH?

A

there will be lots of eosinophils and when you stain it with CD1a it confirms it

46
Q

what is pulmonary alveolar proteinosis?

A

impairment of surfactant metabolism

47
Q

what causes pulmonary alveolar proteinosis?

A

a defect of granulocyte-macrophage colony stimulating factor (GM-CSF)

48
Q

what is the common demographic of pulmonary alveolar proteinosis?

A

young to middle aged females who have an autoimmune phenomenon occurring

49
Q

what is pneumoconiosis?

A

a reaction by the lungs to inhaled mineral or organic dust or vapors- occupational exposure or air pollution

50
Q

what worsens cases of pneumoconiosis?

A

if exposure is high and repetitive, the size of particles is small, and smoking

51
Q

what is the spectrum of coal workers’ pneumoconiosis?

A

anthracosis, coal macules/nodules, progressive massive fibrosis

52
Q

most patients with coal workers’ pneumoconiosis will not have progressive disease; what is the exception to this?

A

the exception if the coal is bituminous coal (used to heat homes/cabins)

53
Q

what is silicosis?

A

disease resulting from inhaled silicon dioxide

54
Q

who is at risk for silicosis?

A

mining/quarry work; concrete repair/demolition

55
Q

how does silicosis present?

A

insidious onset, can occur after exposure is no longer present; may progress to massive pulmonary fibrosis

56
Q

what could be a complication associated with silicosis?

A

patients with silicosis are at two-fold risk of developing cancer (as opposed to CWP except bituminous coal-associated pneumoconiosis which is also linked to cancer)

57
Q

what are the histologic features of silicosis?

A

dense collagenous nodules

58
Q

what are the radiographic findings associated with silicosis?

A

eggshell calcifications (calcified hilar lymph nodes)

59
Q

who is at risk for asbestosis?

A

insulation workers, shipyard workers (navy), paper mill workers, oil or chemical refinery workers

60
Q

what are the disease manifestations of asbestosis?

A

pleura: fibrosis/fibrous plaques, effusions, mesothelioma; lung: interstitial fibrosis, carcinoma; extrapulmonary neoplasms like in the liver

61
Q

what are the histologic features associated with asbestosis?

A

pleural plaque formation; candlewax drippings on pleura; histologically showing hyalinized collagen

62
Q

when you get a pulmonary infarct due to pulmonary embolus, what does it look like?

A

wedge-shaped lesions

63
Q

what is a talc embolus?

A

seen in people who inject drugs intravenously

Pulmonology Exam (11 decks)

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