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

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

Flashcards in Interstitial Lung diseases, Part I, D Kinder, DSA Deck (103):
1

Interstitial Lung disease affects what part of lungs

alveolar wall including epithelial cells, endothelial cells and interstitium

2

Clinical manifestations of interstitial lung disease

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

3

how is compliance and lung volume changed in interstitial lung disease

decreased compliance and decreased lung volumes

4

describe acid base state of interstitial lung disease

hyposemia without CO2 retention

5

interstitial lung disease commonly leads to what problem

pulm HTN

6

how will interstitial lung disease present on CXR

reticular with linear markings
reticulonodular with increased linear and small nodular markings

7

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

High res CT

8

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

thorascopic lung biopsy
transbronchial biopsy via flexible bronchoscopy

9

What will PFT show for interstitial lung disease

restrictive
decreased volumes
hypoxia

10

what are the known etiologies of interstitial lung disease

inhaled inorganic dusts
organic Ags

11

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

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

12

What is the pathologic process behind interstitial lung disease

inflammatory in alveolar spaces and scarring and fibrosis

13

What is a granuloma

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

14

What are the idiopathic interstitial penumonias

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

15

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

usual interstitial pneumonia

16

what is honey combing

cystic airspace from retratction of surrounding fibrotic tissue

17

signs of usual interstitial pneumonia

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

18

What characterizes desquamative interstitial pneumonia

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

19

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?

desquamative interstitial pneumonia

20

What is Respiratory bronchiolitis interstitial lung disease

associated with pigemented macrophages like DIP
NO interstitial inflammation
SMOKING

21

difference of desquamative vs respiratory bronchiolitis intersitial lung disease

the latter does not have any inflammation

22

What is characteristic of nonspecific interstitial pneumonia

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

23

what is prognosis of nonspecific intersitial pneumonia

better than usual interstitial pneumonia

24

Characteristics of acute interstitial pneumonia

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