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

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

1
Q
A

cryptogenic organizing pneumonia

note: polypoid plugs

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2
Q

most prevalent chronic occupational disease

A

silicosis

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3
Q

what are other interstitial lung diseases

A

pulmonary alveolar proteinosis

langerhans cell histiocytosis/eosinophilic granulom

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4
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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5
Q
A

complicated CWP

progressive massive fibrosis

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6
Q

what leads to disease in pneumonconiosis

A

the particles inhaled stimulate an immune resonse that leads to disease

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7
Q
A

UIP in IPF

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8
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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9
Q

describe how asbestos relates to malignancy

A

tumor initiator and promoter

asbestos fibers generate reactive free radicals in distal lung, close to mesothelium. toxic chemicals absorbed into asbestos fibers

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10
Q
A

loose granulomas in HS pneumonitis

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11
Q

most likey microscopic finding of HS P?

A

granuloma

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12
Q
A

asbestos body

brown indicates iron from macrophage ferritin

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13
Q

where does malignant mesothelioma arise

A

in pleura

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14
Q
A

cryptogenic organizing pneumonia

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15
Q

lab values in sarcoidosis

A

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

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16
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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17
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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18
Q

what are the symptoms of cryptogenic organizing pneumonia

A

variable onset

cough

dyspnea

may sponteneously resolve

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19
Q

prognosis for mesothelioma and treatment

A

50% die in 12 months of Dx

chemo, radiaton, extrapleural pneumonectomy may help

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20
Q

what happens when coal is inhaled

A

inhaled carbon pigment is engulfed by alveolar or intesrtitial macrophages, which then accumulate in connective tissue along lymphatics or in lympoid tissue

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21
Q
A

sarcoidosis

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22
Q

treatment for cryptogenic organizing pneumonia

A

steroid treatment for most patients for complete recovery

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23
Q
A

sarcoidosis

Schaumann body

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24
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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25
honeycomb lung
26
what other lesions are associated with sarcoidosis
eye and skin lesions
27
pulmonary langerhans histiocytosis
28
what is asbestos
crystalline hydrated silicates that form fibers
29
what is the differential for organizing pneumonia pattern
viral and bacterial inhaled toxins drugs collagen vascular diseases GVHD
30
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
31
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**
32
what happens in asbestos related disease
asbestos fibers are phagocytosed by macrophages acivate the inflammasone and stimulate the release of proinflammatory and fibrogenic factors
33
what is an anthracosis
innocuous coal induced pulmonary lesion in coal miners, all urban dwellers and tobacco smokers
34
what are fibrosisng interstitial lung diseases
1. idiopathic pulmonary fibrosis 2. non specific interstitial pneumonia 3. cryptogenic organizing pneumonia 4. drugs 5. connective tissue diseases 6. pneumoconiosis 7. radiation
35
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
36
pulmonary hypertension
37
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
38
recurrent pleural effusions in patients with asbestos exposrue
not common, think mesothelioma
39
what is this and when is it seen
Left arrow - established (late) fibrosis Right arrow - uninvolved, note subpleural area more affected Usual interstitial pneumonia seen in idiopathic pulmonary fibrosis
40
sarcoidosis
41
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)
42
sarcoidosis note: multinucleated giant cells epitheliod histiocytes
43
CWP dense scars with collagen and pigment
44
describe the way sarcoidosis looks on imagin
bilateral hilar lymphadenopathy or lung involvement is visible on chest imaging
45
what are characterstics of cryptogenic organizing pneumonia
polypoid plugs of loose organizing connective tissue within small airways, alveolar ducts, and the surrounding alveoli
46
extensive fibrosis, accentuated subpleurally and in lower lobe seen in idiopathic pulmonary fibrosis
47
what size of inspired particles can affect pneumonconiosis
1-5 microns they can go further down past escalator
48
epidemiology of silicosis
african americans foundry work, sandblasting, hardrock mining, stone cutting
49
what are types of pneumonconiosis
aspestosis silicosis coal workers pneumonconiosis
50
HS PF
51
what are granulomatous interstitial lung diseases
1. sarcoidosis 2. hypersensitivity penumonitis
52
what does silicosis lead to an increased risk of
TB, lung cancer
53
pathogenesis of sarcoidosis
cell mediated immune response to an unidentified antigen CD4 helper T cells increased cytokines (TNF) HLA-A1, HLA-B8
54
what is pneumonconiosis
non neoplastic reaction of teh lung to inhaled mineral dust, organic particles or chemical vapros
55
silicosis Silica particles seen under polarized light
56
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
57
end stage lung with honeycomb fibrosis UIP in IPF
58
what is the most common asbestos fiber
chrysotile
59
mixed type malignant mesothelioma
60
lamellar bodies alveolar proteinosis
61
how do you get IPF
environmental exposures genetically predisposed prone to aberrant repair of recurrent alveolar epithelial cell injury increased fibrogenic cytokines like TGFbeta
62
what are amphibole asbestos fibers
less prevalent but more potent for causing diseases, especially mesothelioma
63
pulmonary langerhans histocytosis
64
sarcoidosis asteroid body
65
alveolar proteinosis
66
67
cobblestoned pleural surface seen in idiopathic pulmonary fibrosis due to fibrosis of interlobar septae
68
silicosis
69
what re smoking related interstitial lung diseases
desquamative interstitial pneumonia
70
course and treatment of sarcoidosis
variable treatment: steroids for progressive/dangerous disease 65-79% of patients recover 20% have impaired eye or lung function
71
what are the asbestos amphiboles
all but chrysotile anthrophyllite crocidolite amosite
72
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
73
what is the epidemiology of idiopathic pulmonary fibrosis
older, smoker maybe
74
what is this and what is the blue line pointing to
Usual interstitial pneumonia Fibroblast focus (plump fibroblasts)
75
what is sarcoidosis
a multisystem disease of unkown cause causes non necrotizing granulomas
76
what is the etiology of cryptogenic organizing pneumonia
unknown!
77
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**
78
pleural plaques associated with aspestos related pleural disease usually asymptomatic
79
pulmonary langerhans cell histocytosis
80
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
81
drug associated interstitial disease Belomycin Methotrexate amiodarone nitrofurantoin aspirin beta antagonists
82
management of HS pneumonitis
steroids remove anitgen transplant if it goes on a long time and you get fibrosis
83
what are the 2 drugs for treatment of IPF
pirfenidone - antifibrotic med nintedanib - TKI inhibits fibrosis pathways
84
what is the differential for UIP microscopic pattern
Idiopathic pulmonary fibrosis collagen vascular disease drug chronic hypersensitivity pneumonitis asbestosis
85
epitheliod type malignant mesothelioma
86
how do you diagnose idiopathic pulmonary fibrosis
progressive scarring of lung parenchyma and loss of lung function characteristic clinical, pathologic and radiologic findings typical histologic pattern is usual interstitial pneumonia (UIP)
87
sarcoidosis
88
clinical presentation of alveolar proetinosis and management
89
does smoking increase risk for mesothelioma
no just like mathey said
90
classic complication of langerhans histocytosis
pneumothorax bc its right up next to pleural space
91
lung cancer related to asbestos malignancy
92
pulmonary alveolar proteinosis
rare characterized by bilateral patchy asymmetric pulmonary opacification on imaging accumulation of acellular surfactant in the intra alveolar and bronchiolar spaces
93
extensive interstitial fibrosis associated with asbestos exposure
94
what is the most common causative inhalant in silicosis
crystalline quartz (more fibrogenic)
95
presentation of silicosis
slowly progessing, nodular, fibrosing pneumoconiosis
96
what jobs are associated with asbestos related diseases
minin, milling, fabrication, insulation
97
treatment for pulmonary langerhans histiocytosis
cessation of tobacco smoking, steroids, cyclophosphamide, lung transplant
98
granuloma and chronic inflammation in HS pneumonitis
99
chrysotile asbestos
100
risk for lung cancer related to asbestos
5x 55x if also a smoker
101
presentation of mesothelioma
chest pain, dyspnea, pleural effusions grow extensively in pleural cavity, invade lungs and spread to lymph nodes, liver, distant metastatic sites
102
describe the genetics of malignant mesothelioma
homozygous deletion of tumor suppressor gene CDKN2A/INK4a on chromosome 9p
103
what is coal workers pneumoconiosis
parnchymal lung disease that results from the inhalation of, retention of and host respose to coal dust
104
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
105
birbeck granules tennis racket shape langerhans histocytosis
106
fibroblast foci IPF
107
epidemiology of sarcoidosis
african americans more common women more common lung/hilar lymph nodes involved usually
108
silicosis
109
what is silicosis
a lung disease caused by inhalation of proinflammatory crystalline silicon dioxide (silica) usually decades of exposure, rarely rapid
110
latent period and lifetime risk for mesothelioma with asbestos exposure
latent period 25-45 yrs lifetime risk - 7-10%
111
definitive treatment of IPF
transplant post transplatn 5- yr survival about 50-60%
112
alveolar proteinsois granular material and histiocytes within alveolar spaces
113
prevention of hypersensitivity pneumonitis
removal of environmental agent

RC Test 3 (15 decks)

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