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Year 2 Resp Exam 2 > Interstitial Lung diseases, Part I, D Kinder, DSA > Flashcards

Interstitial Lung diseases, Part I, D Kinder, DSA Flashcards

1
Q

Interstitial Lung disease affects what part of lungs

A

alveolar wall including epithelial cells, endothelial cells and interstitium

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2
Q

Clinical manifestations of interstitial lung disease

A

dyspnea, nonproductive cough, crackles prominent at lung bases
clubbing may be present

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3
Q

how is compliance and lung volume changed in interstitial lung disease

A

decreased compliance and decreased lung volumes

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4
Q

describe acid base state of interstitial lung disease

A

hyposemia without CO2 retention

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5
Q

interstitial lung disease commonly leads to what problem

A

pulm HTN

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6
Q

how will interstitial lung disease present on CXR

A

reticular with linear markings

reticulonodular with increased linear and small nodular markings

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7
Q

how do you distinguish between inflammation from fibrosis in interstitial lung disease

A

High res CT

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8
Q

What tissue specimens do you take for Dx of interstitial lung disease

A

thorascopic lung biopsy

transbronchial biopsy via flexible bronchoscopy

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9
Q

What will PFT show for interstitial lung disease

A

restrictive
decreased volumes
hypoxia

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10
Q

what are the known etiologies of interstitial lung disease

A

inhaled inorganic dusts

organic Ags

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11
Q

What are the unkonwn etiologies that can lead to interstitial lung disease

A 
idiopathic interstitial pneumonias
CT diseases (rheumatic)
sarcoidosis
pulm langerhans
lymphangioleiomyomatosis
Good pastures and wegeners
chronic eos pneumonia
pulm alveolar proteinosis
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12
Q

What is the pathologic process behind interstitial lung disease

A

inflammatory in alveolar spaces and scarring and fibrosis

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13
Q

What is a granuloma

A

localized collection of cells called epitheliod histocytes, generally with T lymphocytes, multinucleated giant cells

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14
Q

What are the idiopathic interstitial penumonias

A 
usual interstitial pneumonia
desquamative interstitial pneumonia
respiratory bronchiolitis interstitial lung disease
nonspecific interstitial pneumonia
acute intersitital pneumonia
cryptogenic organizing pneumonia
lymphocytic interstitial pneumonia
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15
Q

Patient has patchy areas of parenchymal fibrosis and interstitial inflammation interspersed between areas of healthy lung
honey combin is present
Dx?

A

usual interstitial pneumonia

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16
Q

what is honey combing

A

cystic airspace from retratction of surrounding fibrotic tissue

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17
Q

signs of usual interstitial pneumonia

A

patchy areas of parencymal fibrosis and interstitial inflammation interspersed
fibrosis!!
hoenycombing

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18
Q

What characterizes desquamative interstitial pneumonia

A 
large # intraalveolar monomuclear cells
less inflammation, little fibrosis
minimal architectrual distortion
pigmented macrophages
SMOKING
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19
Q

smoker has biopsy done of lungs
shows man intraalveolar mononuclear cells with little inflammation or fibrosis
there are some pigmented macrophages in respiratory bronchioles
Dx?

A

desquamative interstitial pneumonia

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20
Q

What is Respiratory bronchiolitis interstitial lung disease

A

associated with pigemented macrophages like DIP
NO interstitial inflammation
SMOKING

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21
Q

difference of desquamative vs respiratory bronchiolitis intersitial lung disease

A

the latter does not have any inflammation

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22
Q

What is characteristic of nonspecific interstitial pneumonia

A

mononuclear cell infiltration within alveolar walls
uniform process
fibrosis is variable, but less than Usual interstitial pneumonia
idiopathic or CT disorder
better prognosis

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23
Q

what is prognosis of nonspecific intersitial pneumonia

A

better than usual interstitial pneumonia

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24
Q

Characteristics of acute interstitial pneumonia

A

organizing or fibrotic stage of alveolar damage (like ARDS)
fibroblas proliferation and type II pneumocyte hyperplasia
no initial trigger identified

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25
Q

patient shows fibroblast proliferation and type II pneumocyte hyperplasia
Dx?

A

acute interstitial pneumonia

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26
Q

Characteristics of cryptogenic organizing pneumonia

A 
organizing fibrosis (granulation) in small airways
mild degree chronic interstitial inflammation
intraluminal airway involvement
idiopathic
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27
Q

When there is intraluminal airway involvement in an organizing fibrosis process in lungs what is top of DDx?

A

cyrptogenic organizing pneumonia

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28
Q

differential for cryptogenic organizing pneumonia

A

infections, toxic inhalants or CT disorder

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29
Q

characteristics of idiopathic pulmonary fibrosis

A 
no recognizable causative agent
dysregulated pattern of fibrosis in response to alveolar epithelial injury
presents between ages 50 and 70
insidious onset
dyspnea
rales
frequently have clubbin
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30
Q

patient is 55 years old, non smoker, complaining of dyspnea
you notice clubbing of fingers and rales on lung examination
upon biopsy results there is noted dysregulation of fibrosis in alveoli
Dx?

A

idiopathic pulmonary fibrosis

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31
Q

CXR idiopathic pulmonary fibrosis

A

interstitial pattern that is b/l and diffuse

more prominent at lung bases especially in peripheral and subpleural regions **

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32
Q

idiopathic pulm fibrosis on CT

A

intersitital densities are patchy, peripheral and subpleural
associated with small cycstic spaces
there is honeycombing

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33
Q

on CT what is indicative of irreversible fibrosis

A

honeycombing

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34
Q

how is Dx made of interstitial pulmonary fibrosis

A

surgical lung biopsy

if too frail– HRCT

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35
Q

prognosis idiopathic pulmonary fibrosis

A

poor with mean survival post Dx 2-5 yrs

no proven effective Tx

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36
Q

how will desquamative interstitial pattern show on CXR

A

ground glass

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37
Q

Tx for desquamative interstitial pneumonia

A

smoking cessation and maybe corticosteroids

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38
Q

CXR appearance of nonspecific interstitial pneumonia

A

ground glass

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39
Q

prognosis and Tx for nonspecific intersitial pneumonia

A

depends on degree of fibrosis

respond to corticosteroids

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40
Q

Tx cryptogenic organizing pneumonia

A

steroid response is dramatic in days-weeks

prolonged for mo to prevent relapse

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41
Q

What does CXR of cryptogenic organizing pneumonia look like

A

mimics pneumonia with one or more alveolar infiltrates

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42
Q

What will imaging look like with acute interstitial pneumonia

A

ground glass,alveolar filling

43
Q

what is prognosis acute interstitial pneumonia

A

mortality high

44
Q

What are the complicating CT diseases that cna cause interstitial lung disease

A 
RA
SLE
progressive systemic sclerosis (scleroderma)
polymyositis-dermatomyositis
sjogrens syndrome
overlap syndrome
45
Q

what lobes are more commonly involved in CT disorders

A

lower lobe

46
Q

most common site and manifestation of RA in thorax

A

pleura

pleurisy, pleural effusions

47
Q

describe lung parenchymal involvement of RA

A

one or multiple nodules or development of interstitial lung disease

48
Q

organ involvement of SLE

A

kidneys, lungs, nervous system, heart

49
Q

SLE in thorax

A

pleuritic chest pain, pleural effusion

acute pneumonitis of alveolar spaces and walls

50
Q

pulmonary involvement of sceroderma

A

severe with significat scarring of pulmonary parenchyma

pulmonary fibrosis strongly associated with autoAb to topoisomerase I, Scl70

51
Q

what concurrent disease process affects the lungs in scleroderma (progressive systemic sclerosis)

A

pulmonary a HTN

small pulmonary vessel disease independent of fibrosis

52
Q

what accounts for dyspnea in polymyositis-dermatomysositis

A

weakness of diaphragm

53
Q

what accounts for dysphagia and recurrent aspiration pneumonia in polymyosistis-dermatomyositis

A

involvement of the striated mm in the proximal esophagus

54
Q

histo shows APC “langerhans cell”
cytoplasmic rod like structures called X bodies
histiocytes, eos, lymphocytes, macrophages and plasma cells
Dx?

A

pulmonary langerhans histiocytosis

also called eosinophilic granuloma of lung or pulmonary histiocytosis X

55
Q

how does pulmonary langerhans progress

A

starts as peribronchiolar and becomes diffuse

SMOKERS

56
Q

CXR and CT apperance of pulmonary langerhans

A

CXR: nodular or reticulonodular disease with upper lobe involvement
CT: small cysts in addition to nodular and reticulonodular changes, cysts may rupture and cause pneumo
some cases there is extensive honeycombing

57
Q

30 y.o woman with proliferation of atypical smooth mm cells around lymphatics, blood vessels and airways with numerous small cysts
dx?
most likley to have what genetic condition?

A

lymphangioleiomyomatosis

tuberous sclerosis complex

58
Q

clinical manifestations lymphangioleiomyomatosis

A

dyspnea cough
vascular involvement– hemoptysis!
lymphatic obstruction– chylous pleural effusion
ariflow obstruction
rupture of cysts cal lead to spontaneous pneumothorax

59
Q

CXR and HRCT of lymphangioleiomyomatosis

A

CXR: reticular pattern, cystic changes
lung volumes normal or inc
HRCT: cystic disease throughout parenchyma

60
Q

PFT of lymphanioleiomyomatosis

A

Obstructive, restrictive or both

61
Q

how does Goodpastures present in lungs

A

pulmonary hemorrhage and pulmonary fibrosis may develop

62
Q

The anti GBM AB in goodpastures are against what

A

Collagen IV

63
Q

Tx goodpastures

A

plasmapheresis

immunosuppressive Tx is given to dec formation of Ab

64
Q

Lung involvement of wegeners

A

upper respiratory tract and lung have small necrotizing small vessel granulomatosis
CXR will show nodules, infiltrates and cavitationdiffuse
cANCA+

65
Q

Tx wegeners

A

Cyclophosphamide

prednisone

66
Q

What is characteristic of churg strauss syndrome

A 
systemic nectrotizing vasculitis
Affects upper and lower respiratory tracts
preceded by allergic disorcers
peripheral and lung eosinophilia
increased IgE
rashes
67
Q

CXR Churg Strauss

A

bilateral patchy, fleeting infiltrates
diffuse nodular infiltrates
diffuse reticulonodular infiltrates

68
Q

what will biopsy of churg strauss patient show

A

granulomatous angiitis or vasculitis

69
Q

Tx of churg strauss

A

corticosteroids

70
Q

What are clnical manifestations of chronic eos pneumonia

A

weeks-mo
fever, weight loss, dyspnea, productive cough
pulmonary infiltrates with peripheral distribution and more suggestive of alveolar than interstitial disease
increased eos in peripheral smear
BAL with increase eos

71
Q

Tx chronic eos pneumonia

A

dramatic response to corticosteroids (days-weeks)

therapy prolonged for mo to preven recurrence)

72
Q

alveolar spaces are filled with proteinaceous phospholipid material
Dx?

A

pulmonary alveolar proteinosis

73
Q

Clinical manifestations of pulmonary alveolar proteinosis

A

dyspnea and cough
b/l alveolar infiltrates
superimposed to respiratory infections-Nocardia

74
Q

Tx pulmonary alveolar proteinosis

A

Whole lung lavage

prognosis is good

75
Q

HRCT pulmonary alveolar proteinosis

A

crazy paving pattern produced by thickening of interlobular septa accompanied by groun-glass alveolar filling

76
Q

What cause hypersensitivity pneumonitis and what is it

A

bacteria, fungi, protozoa, animal proteins and reactive chemicals
hyperimmune respiratory syndrome caused by inhalation of a wide variety of allergic Ag that are usually organic

77
Q

Tx of HS pneumonitis

A

identify causative Ag and avoid exposure
prevent progressive permanent lung damage
corticosteroids hasten resolution

78
Q

When is HS pneumonitis higher on your differential

A 
intermittent pulmonary ans sytemic Sx
progressive Sx with interstitial CXR
non-resolving pneumonia
workplace exposure
(cattle, ag, bird keeping)
home exposure (ventialation, hot tubs)
79
Q

acute presentation of HS pneumonitis

A

abrupt onset cough, dyspnea, fever, ches pain)

Sx 4-6 hrs post exposure

80
Q

subacute presentation of HS pneumonitis

A

more gradual develoment of Sx

less severe intensity

81
Q

chronic presentation HS penumonitis

A

insidious progressive dyspnea, cough, weight loss, fatigue

most progress to pulmonary fibrosis with resp failure

82
Q

What professions and hobbies are affected by HS pneumonitis

A

farmers, bird keepers, woodworkders, office workers, chees makers, plastic industry workers, metal workers, painters, lifeguards, machine workers

83
Q

Common etiology of faming caused HS pneumontiis

A

thermophilic actinomycetes in moldy hay or grain

84
Q

what is common etiology of bird handlers with HS pneumonitis

A

animal proteins in droppings

85
Q

what type of pathogens grow in ventialation areas or water-related contamination

A

thermophilic actinomycetes

mycobacterium avium intracellulare complex

86
Q

CXR HS pneumonitis

A

acute- diffuse reticulonodular infiltrates

chronic- diffuse interstitial fibrosis

87
Q

HRCT of HS pneumonitis

A

ground glass opacities early

chronic with traction bronchiectasis, honeycombing and fibrosis

88
Q

PFT HS pneumonitis

A

restriction, small lung volumes, decreased diffusion capacity

89
Q

What is sarcoidosis

A

systemic granulmatous disease known by non-caseating granulomas

90
Q

What is classic population affected by sarcoidosis

A

age 40-60
peak exposure 20-39
3.5x higher in black americans
women>men

91
Q

what is common initial presentation os sarcoidosis in lungs

A

abnormal CXR with mediastinal and hilar adenopathy

92
Q

Lofgrens syndrome

A

acute manifestation of sarcoidosis
erythema nodosum (women)
arthritis (men)
b/l hilar lymphadenopathy

93
Q

prognosis lofgrens syndrome

A

favorable

94
Q

Sx sarcoidosis

A

general: fatigue, fever, night sweats and Lb loss
pulm: cough, DOE, wheeze, ches discomfort
skin: rash, macules, papules, nodules, hyperpigmentation or hypo, erythema nodosm
ocular: gritty or dry eyes, pain ,redness or blurred vision
cardiac: arryhthmia, palputations, near syncope, LE edema
NS: HA, blurred vision, numbnessm seizures
MSK: swelling and stiffness, pain
URT: nasal congestion, sinus pressure

95
Q

Signs of sarcoidosis

A

pulm: rales, expiratory wheezing
skin: infiltration old scars and tattoos, maculopapular lesions, lupus pernio of nose cheeks ears and lips.
ocular: uveitis, iritis, scleral plaques
cardiac: irregular rhythm
NS: CN II VII VIII, gait instability
GI: HSM, jaundice
MSK: arthralgias, arthritis
lymph: adenopathy in cervical, supraclav, axillae and innguinal

96
Q

What organ has 90% invovlment in sarcoidosis

A

lungs

mediastinal and hilar lymphadenopathy or parenchymal lung disease

97
Q

What can be used in eyes to Dx sarcoidosis

A

biopsy of conjunctiva- yellow nodules

98
Q

Why do patients with sarcoidosis have nephrolithiasis, nephrocalcinois or hypercalcuira

A

disorder of Vit D metabolism

99
Q

What do you look for in bronchoalveolar lavage if suspect sarcoidosis

A

lymphocytosis
elevated CD4/CD8 ratio
look at ACE levels too in blood

100
Q

What is staging of CXR sarcoidosis

A

stage 0 normal
1 b/l hilar adenopathy
2 b/l hilar adenopathy and parenchymal infiltrates
3 parenchymal infiltrates no lymphadenopathy
4 advanced parenchymal disease with fibrosis

101
Q

PFT of sarcoidosis

A

restrictive ventilatory defect, concurrent obstruction, decreased DLCO

102
Q

Tx sarcoidosis

A

ONLY Sx patients
prednisone 20-40 mg QD x 3 mo, then taper
cytotoxic and immunosuppressives if no response to prednisone

103
Q

prognosis sarcoidosis

A

spontaneous remission in 50% patients at 3 yrs
2/3 remission after 10 yrs
less than 5% die from it

Year 2 Resp Exam 2 (33 decks)

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