Lecture 9 Interstitial Lung Disease

Question 1

Interstitial lung disease (ILD):

____ lung expansion causes _____ lung volumes; ie ____ FVC and TLC. What is the FEV1/FVC ratio?

Answer 1

reduced, reduced;
reduced;
greater than 80% (FEV doesn’t decrease as much as FVC)

Question 2

most common cause of ILD

Answer 2

idiopathic pulmonary fibrosis (IPF) aka usual interstitial pneumonia (UIP)

different names for same thing

Question 3

IPF is (reversible or irreversible); does it respond to treatment? Does stopping smoking help?

Answer 3

irreversible; not really, will need lung transplant;

no

Question 4

IPF is characterized by an insidious ____ ____ that is disabling over time. the cough is (productive or nonproductive). seen in (older or younger patients?

Answer 4

exertional dyspnea, non productive; older

Question 5

classic clinical picture of IPF:
unilateral or bilateral crackles?
Subpleural or pleural?
apices or bases?
characteristic \_\_\_\_\_
\_\_\_\_ abnormality

Answer 5

bilateral;
subpleural
bases
honeycombing
reticular

Question 6

peripheral traction ____ may occur in IPF

Answer 6

bronchiectasis

Question 7

what is the 6MWT?

Answer 7

6 minute walk test–monitors IPF progression

Question 8

2 drugs that have shown promise in treating IPF

Answer 8

pirfenidone, nintedanib

Question 9

nonspecific interstitial pneumonia (NSIP):

seen in ____ patients that smoke. there is tachypnea, crackles, but no ____ ____

Answer 9

younger; digital clubbing (as opposed to IPF)

Question 10

NSIP shows a characteristic _____ finding on CT. It is typically __lateral and located in the ____ of the lungs

Answer 10

ground glass;

bi, base

Question 11

Acute Interstitial Pneumonia (AIP)/Hamman-Rich causes _____ alveolar damage. it mainly affects ____ patients that ____. what is the prognosis?

Answer 11

diffuse, younger, smoke;

“bad”

Question 12

respiratory bronchiolitis associated ILD (RB-ILD) is seen in patients with a history of ____ smoking. Patients are typically ____ and have (lot of symptoms or few symptoms?)

Answer 12

heavy, younger, few

Question 13

RB-ILD is characterized by bibasilar end ____ ______ and diffuse centrilobar ____ opacities

Answer 13

inspiratory crepiations;

ground glass

Question 14

Desquamative interstitial pneumonia is seen predominantly in _____ patients that _____. The course is characterized as _____ cough and dyspnea. What is the prognosis>?

Answer 14

younger, smoke;
insidious/subacute;
“better”

Question 15

lymphocytic interstitial pneumonia (LIP) is characterized by monotonous sheets of ___ clonal lymphocytes. it is associated with what 2 diseases?

Answer 15

poly;

AIDS, sjogren’s

Question 16

besides IPF, do all interstitial pneumonias improve with immunosuppression or smoking cessation?

Answer 16

you betcha

Question 17

cryptogenic organizing pneumonia is characterized by bilateral air-space ____ or ground glass opacities. There is bronchial ___ thickening or _____ in abnormal areas. It has a good prognosis with _____.

Answer 17

consolidation; wall, dilatation;

steroids

Question 18

acute vs chronic eosinophilic pneumonia:
which has eiosinophilia in the lungs? which has it in the blood?
which is more likely to relapse?

Answer 18

both, chronic in the blood;

chronic

Question 19

sarcoidosis causes _____ granulomas. it causes bilateral enlargement of ____ lymph nodes. what classic skin finding is seen with it?

Answer 19

non-caseating; hilar (and mediastinal);
erythema nodosum (on shins).

“lymphocytic distribution”

Question 20

other symptoms of sarcoid in include ____, hyper____, and CNS involvement

Answer 20

uveitis, calcemia

Question 21

histiocytosis X (langerhans) is a disorder characterized by accumulation of _____ ____ in various organs. causes ____ lesions on X ray with a ____ body in the cytoplasm of cells

Answer 21

mononuclear phagocytes;
cystic;
birbeck (X-body)

Question 22

treatment of histiocytosis X:

Answer 22

stop smoking

Question 23

classic triad of wegener’s granulomatosis: necrotizing ____, ____, and _____
what kind of anca is seen?

Answer 23

vasculitis, granulomas in the lung/upper airway, glomerulonephritis;
c-anca

Question 24

large ____ densities, sometimes causing ____, are seen on CXR in patients with Wegener’s. treatment is what?

Answer 24

nodular, cavitations;

corticosteroids, cyclophosphamide

Question 25

hypersensitivity pneumonitis: classically due to exposure of _____. BAL shows lavage fluid _____.

Answer 25

bird droppings/birds (allergen)

lymphocytosis

Question 26

lymphangioleimyomatosis (LAM) is seen in ____ women that present with _____ or pneumothorax. what kind of lesions are seen in the lung?

Answer 26

young, dyspnea;

cystic

Question 27

4 drugs listed in FA that can cause interstitial lung disease:

Answer 27

bleomycin, busulfan, amiodarone, methotrexate

Question 28

what happens to diffusion capacity in patients with ILD?

Answer 28

its going down (it decreases)

Question 29

which 2 general conditions can cause interstitial shadowing on a CXR? how do you differentiate between the 2?

Answer 29

cardiogenic pulmonary edema responds to lasix;

ILD does not

Question 30

does ILD usually cause pleural effusion?

Answer 30

no

Question 31

what condition causes pleural thickening>?

Answer 31

asbestosis