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RC Test 3 > B4.056 - Restrictive Lung Diseases: Interstitial Lung Disease > Flashcards

B4.056 - Restrictive Lung Diseases: Interstitial Lung Disease Flashcards

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1
Q

Diseases affecting the pulmonary interstitium result in

A

Effective loss of lung tissue

decrease in lungs ability to expand

decrease in the lungs ability to transfer oxygen or CO2 within the blood

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2
Q

what are fibrosisng interstitial lung diseases

A
  1. idiopathic pulmonary fibrosis
  2. non specific interstitial pneumonia
  3. cryptogenic organizing pneumonia
  4. drugs
  5. connective tissue diseases
  6. pneumoconiosis
  7. radiation
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3
Q

what are granulomatous interstitial lung diseases

A
  1. sarcoidosis
  2. hypersensitivity penumonitis
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4
Q

what re smoking related interstitial lung diseases

A

desquamative interstitial pneumonia

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5
Q

what are other interstitial lung diseases

A

pulmonary alveolar proteinosis

langerhans cell histiocytosis/eosinophilic granulom

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6
Q

characterize idiopathic pulmonary fibrosis

A

chronic progressive fibrosing interstitial lung disease without an identifiable cause and with a high mortality rate

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7
Q

how do you diagnose idiopathic pulmonary fibrosis

A

progressive scarring of lung parenchyma and loss of lung function

characteristic clinical, pathologic and radiologic findings

typical histologic pattern is usual interstitial pneumonia (UIP)

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8
Q

how do you get IPF

A

environmental exposures

genetically predisposed prone to aberrant repair of recurrent alveolar epithelial cell injury

increased fibrogenic cytokines like TGFbeta

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9
Q

what is the epidemiology of idiopathic pulmonary fibrosis

A

older, smoker maybe

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10
Q

describe the presentation of idiopathic pulmonary fibrosis

A

insidious onset of worsening dyspnea and dry couhg

5th - 6th decade at presentation

fine bibasilar inspiratory crackles in >80% of patients, with progression upward as teh disease advances

subsequent hypoxemia, cyanosis and clubbing

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11
Q
A

cobblestoned pleural surface seen in idiopathic pulmonary fibrosis

due to fibrosis of interlobar septae

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12
Q
A

extensive fibrosis, accentuated subpleurally and in lower lobe

seen in idiopathic pulmonary fibrosis

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13
Q

what is this and what is the blue line pointing to

A

Usual interstitial pneumonia

Fibroblast focus (plump fibroblasts)

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14
Q

what is this and when is it seen

A

Left arrow - established (late) fibrosis

Right arrow - uninvolved, note subpleural area more affected

Usual interstitial pneumonia

seen in idiopathic pulmonary fibrosis

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15
Q

what is characteristic of the distribution of idiopathic pulmonary fibrosis

A

spatially and temporally hetoerogenous distribution: i.e. normal and abnormal areas, early fibrosis (fibroblast foci) and established fibrosis

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16
Q
A

UIP in IPF

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17
Q
A

end stage lung with honeycomb fibrosis

UIP in IPF

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18
Q

what is the differential for UIP microscopic pattern

A

Idiopathic pulmonary fibrosis

collagen vascular disease

drug

chronic hypersensitivity pneumonitis

asbestosis

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19
Q

course of IPF

A

chronic progressive, downhill course

may have acute exacerbations

progressive lung disease, end stage lung and/or pulmonary hypertension

increased risk of lung cancer

respiratory failure most frequent cause of death

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20
Q

what are the 2 drugs for treatment of IPF

A

pirfenidone - antifibrotic med

nintedanib - TKI inhibits fibrosis pathways

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21
Q

definitive treatment of IPF

A

transplant

post transplatn 5- yr survival about 50-60%

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22
Q

what is the etiology of cryptogenic organizing pneumonia

A

unknown!

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23
Q

what are characterstics of cryptogenic organizing pneumonia

A

polypoid plugs of loose organizing connective tissue within small airways, alveolar ducts, and the surrounding alveoli

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24
Q
A

cryptogenic organizing pneumonia

note: polypoid plugs

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25
cryptogenic organizing pneumonia
26
what are the symptoms of cryptogenic organizing pneumonia
variable onset cough dyspnea may sponteneously resolve
27
treatment for cryptogenic organizing pneumonia
steroid treatment for most patients for complete recovery
28
what is the differential for organizing pneumonia pattern
viral and bacterial inhaled toxins drugs collagen vascular diseases GVHD
29
what is pneumonconiosis
non neoplastic reaction of teh lung to inhaled mineral dust, organic particles or chemical vapros
30
what size of inspired particles can affect pneumonconiosis
1-5 microns they can go further down past escalator
31
what does solubility of particles affect in pneumonconiosis
if theyre soluble they may be able to be taken care of by other cells but if not then they may cause problems
32
what leads to disease in pneumonconiosis
the particles inhaled stimulate an immune resonse that leads to disease
33
what are types of pneumonconiosis
aspestosis silicosis coal workers pneumonconiosis
34
what jobs are associated with asbestos related diseases
minin, milling, fabrication, insulation
35
what is asbestos
crystalline hydrated silicates that form fibers
36
what are the asbestos amphiboles
all but chrysotile anthrophyllite crocidolite amosite
37
what are amphibole asbestos fibers
less prevalent but more potent for causing diseases, especially mesothelioma
38
what is the most common asbestos fiber
chrysotile
39
chrysotile asbestos
40
what happens in asbestos related disease
asbestos fibers are phagocytosed by macrophages acivate the inflammasone and stimulate the release of proinflammatory and fibrogenic factors
41
pleural plaques associated with aspestos related pleural disease usually asymptomatic
42
recurrent pleural effusions in patients with asbestos exposrue
not common, think mesothelioma
43
extensive interstitial fibrosis associated with asbestos exposure
44
asbestos body brown indicates iron from macrophage ferritin
45
describe how asbestos relates to malignancy
tumor initiator and promoter asbestos fibers generate reactive free radicals in distal lung, close to mesothelium. toxic chemicals absorbed into asbestos fibers
46
does smoking increase risk for mesothelioma
no just like mathey said
47
lung cancer related to asbestos malignancy
48
risk for lung cancer related to asbestos
5x 55x if also a smoker
49
where does malignant mesothelioma arise
in pleura
50
latent period and lifetime risk for mesothelioma with asbestos exposure
latent period 25-45 yrs lifetime risk - 7-10%
51
presentation of mesothelioma
chest pain, dyspnea, pleural effusions grow extensively in pleural cavity, invade lungs and spread to lymph nodes, liver, distant metastatic sites
52
epitheliod type malignant mesothelioma
53
mixed type malignant mesothelioma
54
describe the genetics of malignant mesothelioma
homozygous deletion of tumor suppressor gene CDKN2A/INK4a on chromosome 9p
55
prognosis for mesothelioma and treatment
50% die in 12 months of Dx chemo, radiaton, extrapleural pneumonectomy may help
56
what is silicosis
a lung disease caused by inhalation of proinflammatory crystalline silicon dioxide (silica) usually decades of exposure, rarely rapid
57
most prevalent chronic occupational disease
silicosis
58
presentation of silicosis
slowly progessing, nodular, fibrosing pneumoconiosis
59
epidemiology of silicosis
african americans foundry work, sandblasting, hardrock mining, stone cutting
60
what is the most common causative inhalant in silicosis
crystalline quartz (more fibrogenic)
61
what does silicosis lead to an increased risk of
TB, lung cancer
62
silicosis
63
silicosis
64
silicosis Silica particles seen under polarized light
65
what is coal workers pneumoconiosis
parnchymal lung disease that results from the inhalation of, retention of and host respose to coal dust
66
what is an anthracosis
innocuous coal induced pulmonary lesion in coal miners, all urban dwellers and tobacco smokers
67
what happens when coal is inhaled
inhaled carbon pigment is engulfed by alveolar or intesrtitial macrophages, which then accumulate in connective tissue along lymphatics or in lympoid tissue
68
complicated CWP progressive massive fibrosis
69
CWP dense scars with collagen and pigment
70
what is sarcoidosis
a multisystem disease of unkown cause causes non necrotizing granulomas
71
describe the way sarcoidosis looks on imagin
bilateral hilar lymphadenopathy or lung involvement is visible on chest imaging
72
what other lesions are associated with sarcoidosis
eye and skin lesions
73
pathogenesis of sarcoidosis
cell mediated immune response to an unidentified antigen CD4 helper T cells increased cytokines (TNF) HLA-A1, HLA-B8
74
sarcoidosis
75
sarcoidosis
76
sarcoidosis
77
sarcoidosis note: multinucleated giant cells epitheliod histiocytes
78
epidemiology of sarcoidosis
african americans more common women more common lung/hilar lymph nodes involved usually
79
sarcoidosis asteroid body
80
sarcoidosis Schaumann body
81
lab values in sarcoidosis
elevated serum ACE hypercalcemia or hypercalciuria leading to nephrolithiasis and renal dysfunction can occur enzyme 1a - hydroxylase in activated macrophages that convert 25-hydroxyvitamin D to 1,26 -mdihydroxyvitamin D, the active form of the vitamin. this results in increased gut absorption
82
course and treatment of sarcoidosis
variable treatment: steroids for progressive/dangerous disease 65-79% of patients recover 20% have impaired eye or lung function
83
hypersensitivity pneumonitis
a spectrum of **immunologically** mediated, **predominantly interstitial, lung disorders** caused by intense, often prolonged exposure to inhlaed organin antigens. **abnormal sensitivity** to antigen, affecting primarily the **alveoli**
84
prevention of hypersensitivity pneumonitis
removal of environmental agent
85
BAL of hypersenstivity pneumonitis
increased CD4 and CD8 T lymphocytes specific antibodies against the causative antigen in serum complement and immunoglobulins noncaseating granulomas in 2/3rd of patients: T cell mediated (type 4) HS againts antigens
86
loose granulomas in HS pneumonitis
87
granuloma and chronic inflammation in HS pneumonitis
88
clinical syndrome of HS pneumonitis
bird fanciers disease, (protein in serum, feathers, droppings) Farmers lung (thermophilic actinomycetes in moldy hay) Bagassosis (moldy sugar cane) Humidifier lung (thermophilic bacteria in heated water reservoirs)
89
clinicla presentation of HS pneumonities
acute episodes follow inhalation of antigenic dust in a previously sensitized patient usually 4-6 hrs after exposure. Recurrent episodes of dyspnea, fevver, cough and increased **white cells**
90
management of HS pneumonitis
steroids remove anitgen transplant if it goes on a long time and you get fibrosis
91
drug associated interstitial disease Belomycin Methotrexate amiodarone nitrofurantoin aspirin beta antagonists
92
what is pulmonary langerhans cell histocytosis/eosinophilic granuloma and who is it seen in
interstitial lung disease with proliferation of langerhans cells (immature dendritic cells) adults smoking nodules or nodules with cystic change
93
classic complication of langerhans histocytosis
pneumothorax bc its right up next to pleural space
94
pulmonary langerhans cell histocytosis
95
pulmonary langerhans histocytosis
96
pulmonary langerhans histiocytosis
97
birbeck granules tennis racket shape langerhans histocytosis
98
treatment for pulmonary langerhans histiocytosis
cessation of tobacco smoking, steroids, cyclophosphamide, lung transplant
99
pulmonary alveolar proteinosis
rare characterized by bilateral patchy asymmetric pulmonary opacification on imaging accumulation of acellular surfactant in the intra alveolar and bronchiolar spaces
100
pulmonary alveolar proteinosis commonest type
autoimmune aquired anti GM-CSF signaling loss of GM CSF signaling blocks terminal differentition of alveolar macrophages impairing their ability to catabolize surfactant
101
alveolar proteinosis
102
alveolar proteinsois granular material and histiocytes within alveolar spaces
103
lamellar bodies alveolar proteinosis
104
clinical presentation of alveolar proetinosis and management
105
pulmonary hypertension
106
honeycomb lung
107
108
a 55 yo man originally from metsova area, northwest greece presents with dyspnea, cough, fever and chest pain. Medial occupational history were unremarkable. Physical exam unremarkable. X ray showed irregular linear densities, particularly in the lower lungs, pleural effusion and pleural thickening. PFTs revealed restrictive lung function. Dx?
asbestosis
109
60 yo with 3 year history of cough and progressive dyspnea. His PMH of cough and progressive dyspnea. Heavy smoker. Not taking any drugs, non occupational exposures. Gradual appearance of dyspnea on exertion which worsened with time and was associated with a dry cough, no pain, fever or other symptoms. Elevarted RR and bibasilar velcro rales. Cyanosis and clubbing.
IPF
110
fibroblast foci IPF
111
HS PF
112
most likey microscopic finding of HS P?
granuloma
113
24 to African American woman presents with fatigue, ahces and pains, blurry vision, dry eyes and SOB. Small cutaneous bumps, uveitis and enlarged liver. Abnormal X rays restrictiv lung findings, increased ACE and 24 hr Ca++ excretion. Bronchioloalveolar lavage revealed CD4/8 ratio of \>5
sacroidosis

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