Interstitial lung diseases

Question 1

usual interstitial pneumonia

Answer 1

patchy ares of parenchymal fibrosis and interstitial inflammation interspersed btwn normal areas
honeycombing
most important in this category-. idiopathic pulmonary fibrosis

Question 2

honeycombing

Answer 2

cystic airspace from retraction of surrounding fibrotic tissue

Question 3

desquamative interstitial pneumonia

Answer 3

more homogenous than UIP
large number of intra-alveolar mononuclear cells
less prominent inflammation and little associated fibrosis
uniform process
minimal architectural distortion
pigmented macros secondary to smoking
smoking important underlying cause

Question 4

Respiratory bronchiolitis interstitial lung disease

Answer 4

related to DIP
associated w/pigmented macros 
interstitial inflammation not present
almost always a smoke
smoking cessations

Question 5

non-specific interstitial penumonia

Answer 5

mononuclear cell infiltration w/in alveolar walls
uniforma process
fribrosis variable, but less the UIP
idiopathic or CT disorder
better prognosis

Question 6

acute interstitial pneumonia

Answer 6

organizing or fibrotic stage of alveolar damage- pattern seen in ARDS
no initial trigger identified
histo-fibroblast proliferation and type II pneumocyte hyperplasia

Question 7

cytogenic organizing penumonia

Answer 7

organizing fibrosis (granulation tissue) in small airways
mild degree of chronic interstitial inflammation
intraluminal airway involvement key
idiopathic

Question 8

cytogenic organizing penumonia Ddx

Answer 8

infections, toxic inhalants, or CT disease

Question 9

idiopathic pulmonary fibrosis

Answer 9

dysregulated pattern of fibrosis due to epi injury
50-70yrs
insidious onset
dyspnea is most common complaint
rales on lung exam
clubbing

Question 10

idiopathic pulmonary fibrosis CXR

Answer 10

interstitial pattern that is generally bilateral and relatively diffuse
more prominent at bases and periphery

Question 11

idiopathic pulmonary fibrosis high resolution CT

Answer 11

interstital densities that are patchy, peripheral, subpleural and associated w/small cystic spaces
honeycombing- irreversible damage

Question 12

Dx idopathic pulmonary fibrosis

Answer 12

surgical lung biopsy

HRCT w/classic pattern of honeycombing diagnostic in patient too frail for lung biopsy

Question 13

idiopathic pulmonary fibrosis prognosis

Answer 13

poor w/mean survival 2-5yrs

Question 14

desquamative interstitial pneumonia

Answer 14

smokers
subacute
ground glass appearance on imaging
lung biopsy w/uniform accumulation of intraalveolar macros w/little or no fibrosis
prognosis better than IPF
may respond to corticosteroids

Question 15

nonspecific interstitial pneumonia

Answer 15

ground glass due to inflammation

lung biopsy- inflammatory response in alveolar walls w/little fibrosis

Question 16

nonspecific interstitial pneumonia prognosis

Answer 16

depends on degree of fibrosis

Question 17

nonspecific interstitial pneumonia Tx

Answer 17

corticosteroids

Question 18

cryptogenic organizing pneumonia

Answer 18

CT plug in small airway accompanied by mononuclear cell infiltration of surrounding lung parenchymal
subacute presentation over weeks to months w/constititional as well as respiratory symptoms

Question 19

cryptogenic organizing pneumonia CXR

Answer 19

mimics pneumonia w/one or more alveolar infiltrates

Question 20

cryptogenic organizing pneumonia Tx

Answer 20

response to steroids is dramatic and occurs over days to weeks
therapy is usually prolonged for months to prevent relapse

Question 21

acute interstitial pneumonia

Answer 21

acute disease that begins w/clinical picture of ARDS, but w/o inciting cause
Histo- diffuse alveolar damage, often showing some organization and fibrosis
mortality is high

Question 22

acute interstitial pneumonia CXR

Answer 22

ground glass

alveolar filling

Question 23

complicating CT diseases

Answer 23

RA
SLE
progressive systemic sclerosis (scleroderma)
polymyositis-dermatomyositis
Sjogrens syndrome
overlap syndromes

Question 24

CT disorders

Answer 24

usually patients have evidence of underlying disease first, but lung disease can proceed diagnosis
often UIP, but sometimes NSIP or COP
lower lobe more common then upper lobe disease

Question 25

RA

Answer 25

joints pleura lungs- nodules or interstitial lung disease (usually mild)

Question 26

SLE

Answer 26

joints, skin, organs pleural disease acute pneumonitis- alveolar walls and spaces chronic interstitial lung disease

Question 27

progressive systemic sclerosis

Answer 27

``` aka scleroderma skin, small blood vessels organs scarring of pulmonary parenchyma pulmonary fibrosis associated a/Ab to topoisomerae I pulmonary artery HTN ```

Question 28

polymyositis dermatomyositis

Answer 28

mm and skin are priamry sites of inflammatory process interstitial lung disease uncommon respiratory difficulty secondary to diaphragm weakness

Question 29

pulmonary langerhans histocytosis

Answer 29

aka eosinophilic granuloma of lung histioctic cell appears to be an Ag presenting dendritic or phagocytic cell called langerhans cell x-bodies (rod like) histiocytes, eos, lymphocytes, macros, plasma cells

Question 30

pulmonary langerhans histocytosis clinical

Answer 30

starts as peribronchiolar distribution -> diffuse smokers unexplained interstitial disease in young or middle aged

Question 31

pulmonary langerhans histocytosis CXR

Answer 31

nodular or reticulonodular disease | upper lobe prominent

Question 32

pulmonary langerhans histocytosis HRCT

Answer 32

small cysts in addition to nodular and reticulonodular changes cysts may rupture and cause penumothorax some cases extensive honeycombing

Question 33

pulmonary langerhans histocytosis course

Answer 33

natural history is variable self limited-> extensive disease no proven Tx smoking cessation, corticosteroids

Question 34

lymphangioleiomyomatosis

Answer 34

rare pulmonary disease characterized by proliferation of atypical smooth mm around vessels women of childbearing age tuberous sclerosis complex genetically predisposes

Question 35

lymphangioleiomyomatosis clinical

Answer 35

dyspnea and cough vascular-> hemoptysis lymph ostruction -> chylous pleural effusion airway obstruction rupture of cysts -> spontaneous pneumothorax

Question 36

lymphangioleiomyomatosis CXR

Answer 36

reticular pattern, cystic changes | lung volumes normal or increased

Question 37

lymphangioleiomyomatosis HRCT

Answer 37

cystic disease throughout parnechyma

Question 38

lymphangioleiomyomatosis PFT

Answer 38

obstructive, restrictive, both

Question 39

Goodpastures Tx

Answer 39

``` palsmapheresis immunosupression (prednisone) ```

Question 40

wagners

Answer 40

cANCA | URTI

Question 41

wagners Tx

Answer 41

cyclophosphamide | prednisone

Question 42

churg-strauss

Answer 42

``` upper and lower respiratory tracts preceded by allergic disorders peripheral and lung eos incrased IgE rashes ```

Question 43

churg-strauss CXR

Answer 43

bilateral patchy, fleeting infiltrated diffuse nodular infiltrates difuse reticulonodular infiltrates

Question 44

churg-strauss biopsy

Answer 44

granulomatous angiitis or vasculitis

Question 45

churg-strauss Tx

Answer 45

corticosteroids

Question 46

chronic eosinophillic pneumonia

Answer 46

pulmonary interstitium and alveolar spaces infiltrated by eos and macros

Question 47

chronic eosinophillic pneumonia clinical

Answer 47

weeks-months fever, weight loss, dyspnea, productive cough pulmonary inflitrates w/peripheral distruction increased esos in peripheral smear BAL w/increased eos

Question 48

chronic eosinophillic pneumonia Tx

Answer 48

dramatic response to corticosteroids w/in days to weeks | prolonged for months to prevent recurrence

Question 49

pulmonary alveolar proteinosis

Answer 49

primary pathologic process affects the alveolar spaces, not alveolar cells spaces filled w/proteinaceous phospholipid

Question 50

pulmonary alveolar proteinosis clinical

Answer 50

``` dyspnea and cough b/l alveolar infiltrates HRCT crazy paving pattern due to thicking of interlobular septa ground glass superimposed nocardia infection ```

Question 51

pulmonary alveolar proteinosis Tx

Answer 51

whole-lung lavage | prognosis good

Question 52

hypersensitivity pneumonitis

Answer 52

hyperimmune respirartory syndrome

Question 53

hypersensitivity pneumonitis Tx

Answer 53

avoid causative agent | corticosteroids

Question 54

suspect hypersensitivty penumonitis

Answer 54

intermittent pulmonary and systemic symptoms progressive pulmonary symptoms w/interstitial CXR changes non-resolving pneumonia agriculture and cattle farming bird keeping contaminated home hot tubs

Question 55

acute hypersensitivity pneumonitis

Answer 55

abrupt onset of cough, dyspnea, fever, chest pain symtoms 4-6 hours after exposure subside w/in 24 hours

Question 56

subacute hypersensitivity pneumonitis

Answer 56

more gradual development | less severe

Question 57

chronic hypersensitivity pneumonitis

Answer 57

insidious progressive dyspnea, cough, weight loss, fatigue | most progress toward pulmonary fibrosis w/respiratory failure

Question 58

hypersensitivity pneumonitis CXR

Answer 58

acute- reticulonodular inflitrates | chronic- diffuse interstitial fibrosis

Question 59

hypersensitivity pneumonitis HRCT

Answer 59

ground glass opacities | chronic w/traction bronchietctasis, honeycombing, and fibrosis

Question 60

hypersensitivity pneumonitis PFTs

Answer 60

restriction small lung volumes decreased diffusion capactity