Interstitial Lung diseases, Part I, D Kinder, DSA Flashcards
(103 cards)
1
Q
Interstitial Lung disease affects what part of lungs
A
alveolar wall including epithelial cells, endothelial cells and interstitium
2
Q
Clinical manifestations of interstitial lung disease
A
dyspnea, nonproductive cough, crackles prominent at lung bases
clubbing may be present
3
Q
how is compliance and lung volume changed in interstitial lung disease
A
decreased compliance and decreased lung volumes
4
Q
describe acid base state of interstitial lung disease
A
hyposemia without CO2 retention
5
Q
interstitial lung disease commonly leads to what problem
A
pulm HTN
6
Q
how will interstitial lung disease present on CXR
A
reticular with linear markings
reticulonodular with increased linear and small nodular markings
7
Q
how do you distinguish between inflammation from fibrosis in interstitial lung disease
A
High res CT
8
Q
What tissue specimens do you take for Dx of interstitial lung disease
A
thorascopic lung biopsy
transbronchial biopsy via flexible bronchoscopy
9
Q
What will PFT show for interstitial lung disease
A
restrictive
decreased volumes
hypoxia
10
Q
what are the known etiologies of interstitial lung disease
A
inhaled inorganic dusts
organic Ags
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
12
Q
What is the pathologic process behind interstitial lung disease
A
inflammatory in alveolar spaces and scarring and fibrosis
13
Q
What is a granuloma
A
localized collection of cells called epitheliod histocytes, generally with T lymphocytes, multinucleated giant cells
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
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
16
Q
what is honey combing
A
cystic airspace from retratction of surrounding fibrotic tissue
17
Q
signs of usual interstitial pneumonia
A
patchy areas of parencymal fibrosis and interstitial inflammation interspersed
fibrosis!!
hoenycombing
18
Q
What characterizes desquamative interstitial pneumonia
A
large # intraalveolar monomuclear cells less inflammation, little fibrosis minimal architectrual distortion pigmented macrophages SMOKING
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
20
Q
What is Respiratory bronchiolitis interstitial lung disease
A
associated with pigemented macrophages like DIP
NO interstitial inflammation
SMOKING
21
Q
difference of desquamative vs respiratory bronchiolitis intersitial lung disease
A
the latter does not have any inflammation
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
23
Q
what is prognosis of nonspecific intersitial pneumonia
A
better than usual interstitial pneumonia
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
25
patient shows fibroblast proliferation and type II pneumocyte hyperplasia
Dx?
acute interstitial pneumonia
26
Characteristics of cryptogenic organizing pneumonia
```
organizing fibrosis (granulation) in small airways
mild degree chronic interstitial inflammation
intraluminal airway involvement
idiopathic
```
27
When there is intraluminal airway involvement in an organizing fibrosis process in lungs what is top of DDx?
cyrptogenic organizing pneumonia
28
differential for cryptogenic organizing pneumonia
infections, toxic inhalants or CT disorder
29
characteristics of idiopathic pulmonary fibrosis
```
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
```
30
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?
idiopathic pulmonary fibrosis
31
CXR idiopathic pulmonary fibrosis
interstitial pattern that is b/l and diffuse
| more prominent at lung bases especially in peripheral and subpleural regions **
32
idiopathic pulm fibrosis on CT
intersitital densities are patchy, peripheral and subpleural
associated with small cycstic spaces
there is honeycombing
33
on CT what is indicative of irreversible fibrosis
honeycombing
34
how is Dx made of interstitial pulmonary fibrosis
surgical lung biopsy
| if too frail-- HRCT
35
prognosis idiopathic pulmonary fibrosis
poor with mean survival post Dx 2-5 yrs
| no proven effective Tx
36
how will desquamative interstitial pattern show on CXR
ground glass
37
Tx for desquamative interstitial pneumonia
smoking cessation and maybe corticosteroids
38
CXR appearance of nonspecific interstitial pneumonia
ground glass
39
prognosis and Tx for nonspecific intersitial pneumonia
depends on degree of fibrosis
| respond to corticosteroids
40
Tx cryptogenic organizing pneumonia
steroid response is dramatic in days-weeks
| prolonged for mo to prevent relapse
41
What does CXR of cryptogenic organizing pneumonia look like
mimics pneumonia with one or more alveolar infiltrates
42
What will imaging look like with acute interstitial pneumonia
ground glass,alveolar filling
43
what is prognosis acute interstitial pneumonia
mortality high
44
What are the complicating CT diseases that cna cause interstitial lung disease
```
RA
SLE
progressive systemic sclerosis (scleroderma)
polymyositis-dermatomyositis
sjogrens syndrome
overlap syndrome
```
45
what lobes are more commonly involved in CT disorders
lower lobe
46
most common site and manifestation of RA in thorax
pleura
| pleurisy, pleural effusions
47
describe lung parenchymal involvement of RA
one or multiple nodules or development of interstitial lung disease
48
organ involvement of SLE
kidneys, lungs, nervous system, heart
49
SLE in thorax
pleuritic chest pain, pleural effusion
| acute pneumonitis of alveolar spaces and walls
50
pulmonary involvement of sceroderma
severe with significat scarring of pulmonary parenchyma
| pulmonary fibrosis strongly associated with autoAb to topoisomerase I, Scl70
51
what concurrent disease process affects the lungs in scleroderma (progressive systemic sclerosis)
pulmonary a HTN
| small pulmonary vessel disease independent of fibrosis
52
what accounts for dyspnea in polymyositis-dermatomysositis
weakness of diaphragm
53
what accounts for dysphagia and recurrent aspiration pneumonia in polymyosistis-dermatomyositis
involvement of the striated mm in the proximal esophagus
54
histo shows APC "langerhans cell"
cytoplasmic rod like structures called X bodies
histiocytes, eos, lymphocytes, macrophages and plasma cells
Dx?
pulmonary langerhans histiocytosis
| also called eosinophilic granuloma of lung or pulmonary histiocytosis X
55
how does pulmonary langerhans progress
starts as peribronchiolar and becomes diffuse
| SMOKERS
56
CXR and CT apperance of pulmonary langerhans
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
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?
lymphangioleiomyomatosis
| tuberous sclerosis complex
58
clinical manifestations lymphangioleiomyomatosis
dyspnea cough
vascular involvement-- hemoptysis!
lymphatic obstruction-- chylous pleural effusion
ariflow obstruction
rupture of cysts cal lead to spontaneous pneumothorax
59
CXR and HRCT of lymphangioleiomyomatosis
CXR: reticular pattern, cystic changes
lung volumes normal or inc
HRCT: cystic disease throughout parenchyma
60
PFT of lymphanioleiomyomatosis
Obstructive, restrictive or both
61
how does Goodpastures present in lungs
pulmonary hemorrhage and pulmonary fibrosis may develop
62
The anti GBM AB in goodpastures are against what
Collagen IV
63
Tx goodpastures
plasmapheresis
| immunosuppressive Tx is given to dec formation of Ab
64
Lung involvement of wegeners
upper respiratory tract and lung have small necrotizing small vessel granulomatosis
CXR will show nodules, infiltrates and cavitationdiffuse
cANCA+
65
Tx wegeners
Cyclophosphamide
| prednisone
66
What is characteristic of churg strauss syndrome
```
systemic nectrotizing vasculitis
Affects upper and lower respiratory tracts
preceded by allergic disorcers
peripheral and lung eosinophilia
increased IgE
rashes
```
67
CXR Churg Strauss
bilateral patchy, fleeting infiltrates
diffuse nodular infiltrates
diffuse reticulonodular infiltrates
68
what will biopsy of churg strauss patient show
granulomatous angiitis or vasculitis
69
Tx of churg strauss
corticosteroids
70
What are clnical manifestations of chronic eos pneumonia
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
Tx chronic eos pneumonia
dramatic response to corticosteroids (days-weeks)
| therapy prolonged for mo to preven recurrence)
72
alveolar spaces are filled with proteinaceous phospholipid material
Dx?
pulmonary alveolar proteinosis
73
Clinical manifestations of pulmonary alveolar proteinosis
dyspnea and cough
b/l alveolar infiltrates
superimposed to respiratory infections-Nocardia
74
Tx pulmonary alveolar proteinosis
Whole lung lavage
| prognosis is good
75
HRCT pulmonary alveolar proteinosis
crazy paving pattern produced by thickening of interlobular septa accompanied by groun-glass alveolar filling
76
What cause hypersensitivity pneumonitis and what is it
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
Tx of HS pneumonitis
identify causative Ag and avoid exposure
prevent progressive permanent lung damage
corticosteroids hasten resolution
78
When is HS pneumonitis higher on your differential
```
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
acute presentation of HS pneumonitis
abrupt onset cough, dyspnea, fever, ches pain)
| Sx 4-6 hrs post exposure
80
subacute presentation of HS pneumonitis
more gradual develoment of Sx
| less severe intensity
81
chronic presentation HS penumonitis
insidious progressive dyspnea, cough, weight loss, fatigue
| most progress to pulmonary fibrosis with resp failure
82
What professions and hobbies are affected by HS pneumonitis
farmers, bird keepers, woodworkders, office workers, chees makers, plastic industry workers, metal workers, painters, lifeguards, machine workers
83
Common etiology of faming caused HS pneumontiis
thermophilic actinomycetes in moldy hay or grain
84
what is common etiology of bird handlers with HS pneumonitis
animal proteins in droppings
85
what type of pathogens grow in ventialation areas or water-related contamination
thermophilic actinomycetes
| mycobacterium avium intracellulare complex
86
CXR HS pneumonitis
acute- diffuse reticulonodular infiltrates
| chronic- diffuse interstitial fibrosis
87
HRCT of HS pneumonitis
ground glass opacities early
| chronic with traction bronchiectasis, honeycombing and fibrosis
88
PFT HS pneumonitis
restriction, small lung volumes, decreased diffusion capacity
89
What is sarcoidosis
systemic granulmatous disease known by non-caseating granulomas
90
What is classic population affected by sarcoidosis
age 40-60
peak exposure 20-39
3.5x higher in black americans
women>men
91
what is common initial presentation os sarcoidosis in lungs
abnormal CXR with mediastinal and hilar adenopathy
92
Lofgrens syndrome
acute manifestation of sarcoidosis
erythema nodosum (women)
arthritis (men)
b/l hilar lymphadenopathy
93
prognosis lofgrens syndrome
favorable
94
Sx sarcoidosis
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
Signs of sarcoidosis
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
What organ has 90% invovlment in sarcoidosis
lungs
| mediastinal and hilar lymphadenopathy or parenchymal lung disease
97
What can be used in eyes to Dx sarcoidosis
biopsy of conjunctiva- yellow nodules
98
Why do patients with sarcoidosis have nephrolithiasis, nephrocalcinois or hypercalcuira
disorder of Vit D metabolism
99
What do you look for in bronchoalveolar lavage if suspect sarcoidosis
lymphocytosis
elevated CD4/CD8 ratio
**look at ACE levels too in blood**
100
What is staging of CXR sarcoidosis
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
PFT of sarcoidosis
restrictive ventilatory defect, concurrent obstruction, decreased DLCO
102
Tx sarcoidosis
ONLY Sx patients
prednisone 20-40 mg QD x 3 mo, then taper
cytotoxic and immunosuppressives if no response to prednisone
103
prognosis sarcoidosis
spontaneous remission in 50% patients at 3 yrs
2/3 remission after 10 yrs
less than 5% die from it
