interstitial lung diseases

Question 1

most common ILDs of UNKOWN etiology. name the 3

Answer 1

idiopathic pulmonary fibrosis
sarcoidosis
pulmo fibrosis assoc with CTDs

Question 2

most common ILDs with KNOWN etiology

Answer 2

occupational and environmental exposures, inorganic and organic dusts, fumes or gases

Question 3

immunopathogenic response that leads to pulmo fibrosis

Answer 3

aberrant wound healing

Question 4

2 major histopathologic patterns in ILD

Answer 4

1. granulomatous lung disease
   differentials: HP and sarcoidosis
2. inflammation and fibrosis

Question 5

most common presentation of ILD based on duration of illness

Answer 5

chronic

unusual to have acute or episodic form

Question 6

true or false. ILDs most common age occur >60 years old

Answer 6

false.
most common are 20-40 years old
but IPF mostly seen in >60years old

Question 7

true or false. ILD in hermann pudlak syndrome and those assoc with CTDs are common in women

Answer 7

true

Question 8

most common PE seen in ILDs

Answer 8

tachypnea and bibasilar end inspiratory rales

Question 9

most common initial finding on cxr in ILD

Answer 9

bibasilar reticular pattern

Question 10

diagnostic value of BAL in ILD for sarcoidosis

Answer 10

lymphocytosis

CD4:CD8 ratio >3.5

Question 11

most common form of ILD is IPF, it has poor response to. chemotherapy.

what is the histologic finding seen in IPF?

Answer 11

usual interstitial pneumonia UIP
alternating areas of normal lung interstitial inflammation and fibrosis
“honeycombing appearance”

Question 12

ILD seen in younger, nonsmoking women with similar presentation of ILD.
Seen in CTD, drug induced ILD, chronic hypersensitivity pneumonitis

Answer 12

nonspecific interstitial pneumonia

Question 13

rare, fulminant, diffuse alveolar damage seen in patients >40years old

Answer 13

acute interstitial pneumonia (Hamman Rich Syndrome)

Question 14

clinicopathologic syndrome of unknown etiology

Answer 14

cryptogenic organizing pneumonia

Question 15

Rare ILD found almost exclusively in cigarette smokers

Answer 15

desquamative interstitial pneumonia (DIP)

histo: extensive accumulation of macrophages in intraalveolar spaces

Question 16

DIP characterized by accumulation of macrophages in the PERIBRONCHIAL ALVEOLI

Answer 16

RB ILD respiratory bronchiolitis associated ILD

remember DIP usually have macrophages in the INTRAALVEOLAR SPACES

Question 17

virtually diagnostic for Pulmonary Langerhans Cell Histiocytosis

Answer 17

combination of nodules and thin walled cysts

pneumothorax occurs in ~25%

Question 18

majority of CTDs are treated with oral glucocorticoids and immunosuppressive agents except?

Answer 18

progressive systemic sclerosis

Question 19

classic PFT finding for progressive systemic sclerosis

Answer 19

restrictive pattern and impaired diffusing capacity - BEFORE. and clinical disease appears

Question 20

dominant histo finding in PSS

Answer 20

NSIP

Question 21

autoimmune disease with a neutralizing IgG antibody against GM CSF

Answer 21

Pulmonary alveolar proteinosis PAP

common in 30-50 years old male with progressive exertional dyspnea

Question 22

highly specific and sensitive for diagnosis of acquired PAP

Answer 22

elevated serum anti GM CSF titers

Question 23

true or false. BAL fluid compared to serum Anti GM CSF better correlates with severity of PAP

Answer 23

true

Question 24

rare condition in premenopausal women. suspected in young women with “emphysema” and recurrent pneumothorax

Answer 24

pulmonary lymphangioleiomyomatosis LAM

Question 25

Syndromes with ILD with diffuse alveolar hemorrhage presetns with massive hemoptysis. true or false?

Answer 25

false. NO hemoptysis but with new alveolar opacities, falling hemoglobin levels, and hemorrhage mainstay therapy: IV methylprednisolone