11. Interstitial Lung Diseases and COPD

Question 1

COPD is defined by loss of small airways in what categories of bronchioles?

Answer 1

terminal and transitional (reduced 40%), generation 8 and beyond of the conducting and acinar airways

Question 2

which of the following is one of the most important genetic polymorphisms associated with alveolar airspace destruction in COPD and what is it associated with?

CDC6, IREB2, HMOX1, GSTP1, SOD3, MMP12, MMP9, SERPINE2, SERPINA1, CCL5, CCL1, SIRT1

Answer 2

• SERPINA1 (protease-antiprotease imbalance, alpha-1 antitrypsin deficiency)
• other mechanisms: increased apoptosis, oxidative stress, autoimmunity, inflammation

Question 3

List the upper and lower limits of FEV1 cutoffs for the GOLD stages of COPD

Answer 3

I >80% Mild
II >50 <80 Moderate
III >30 <50 Severe
IV <30 Very severe

Question 4

In addition to SABA, SA anticholinergics, systemic glucocorticoids, and antibiotics, if a patient with COPD exacerbation is experiencing hypoxemia without hypercapnia, low flow oxygen is indicated to achieve what PaO2?

Answer 4

60-65mm Hg (O2 sat 91-94%)

• consider noninvasive ventilation if persistent hypercapnia with a pH of < 7.35 but > 7.15

Question 5

what are the 3 biggest risk factors for bronchiectasis in PID?

Answer 5

history of pneumonia, older age, and greater diagnostic delay

Question 6

true or false: bronchiectasis occurs more frequently in CVID than in XLA

Answer 6

true

Question 7

true or false: some immune defects predispose to bronchiectasis without severe immunoglobulin deficiency

Answer 7

true (PI3KD-GOF)

Question 8

what is the most common presentation of drug induced ILD on CT and pathology?

Answer 8

GGO (BL distribution) and OP (also NSIP)

Question 9

ILD due to environmental and occupation exposure is most likely NOT due to which of the following?

a. mineral dust (silica, asbestos, coal, beryllium)
b. organic dust (mold, bird droppings)
c. second hand smoke
d. toxic gas (methane, cyanide)

Answer 9

C.
Typical CT findings include GGO, reticular opacities, fibrosis, nodules.

Pleural thickening/effusion/plaques indicates asbestos as a cause of fibrosis over idiopathic pulmonary fibrosis

Question 10

which of the following is associated with UIP pattern which portends the worst prognosis with 5 year mortality rate of >50%?
A. systemic sclerosis (scleroderma)
B. autoimmune myositis
C. rheumatoid arthritis (RA)
D. systemic lupus erythematosus
E. Sjogren syndrome

Answer 10

C

Question 11

Which of the following are NOT true regarding idiopathic pulmonary fibrosis?
A. IPF is the most common idiopathic
interstitial pneumonia and occurs primarily in adults
B. presents with bilateral velcro-like crackles
C. overexpression of mucin 5B
D. lung biopsy is necessary for diagnosis
E. associated with the following findings on CT scan indicating typical UIP pattern: peripheral and lower lobe reticulation, honeycombing, and traction bronchiectasis

Answer 11

D. Provided other causes are ruled out, a typical UIP pattern on CT is
diagnostic for IPF and makes lung biopsy unnecessary

Question 12

Which immune deficiency below is associated with a disease involving: surfactant accumulation and alveolar macrophage dysfunction, progressive dyspnea, respiratory failure, secondary infections and pulmonary fibrosis, and crazy paving on CT?
A. PI3KD
B. CVID
C. GATA2 deficiency
D. XLA
E. CGD

Answer 12

C. Secondary Pulmonary alveolar
proteinosis (PAP)-
association with an underlying disease,
hematological disease, in particular myelodysplastic syndromes MC. AD GATA2 deficiency including “ MonoMAC ”

1. Primary PAP = disruption of granulocyte macrophage colony stimulating factor (GM CSF) signaling
2. Congenital PAP = mutations in genes involved in surfactant production ( SFTPB SFTPC ABCA3 , NKX2 1

Autoimmune PAP accounts for >90% of all
cases.

“Crazy paving” = diffuse GGOs with
interlobular and intralobular septal thickening

Question 13

ILD occurs much more frequently in all but which of the following immunodeficiencies?

A. CVID
B. XLA or X-linked and autosomal recessive hyper IgM
C. activated PI3 kinase syndrome
D. gain of function of STAT3
E. deficiency of CTLA-4 and LRBA

Answer 13

B. ILD occurs much more frequently in CVID relative to XLA or X-linked and autosomal recessive hyper IgM

ILD occurs more frequently in monogenic immunedysregulation disorders associated with antibody deficiency

Question 14

Which of the following is true about COPD?

A. Eosinophils do not contribute to COPD
B. There is an imbalance
between protease and antiprotease activity
C. Small airways are not reduced below healthy levels until moderate to severe COPD.
D. There are no validated clinical assessment questionnaires for monitoring this disease

Answer 14

B. There is an imbalance between protease and antiprotease activity (SERPINA1
encodes alpha 1 antitrypsin, genetic polymorphism a/w COPD)

Question 15

Which of the following statements about bronchiectasis is correct?

A. Bronchiectasis has not been associated with history of pneumonia in CVID
B. Sputum culture is not useful in the evaluation of bronchiectasis
C. CT chest is often required to identify bronchiectasis
D. Bronchiectasis occurs more frequently in XLA compared to CVID

Answer 15

C. CT chest is often required to identify bronchiectasis

Question 16

Which of the following statements about ILD is correct?

A. CT evidence of pleural thickening and plaques is most suggestive of silicosis.
B. Sarcoidosis can be differentiated from CVID ILD by larger pulmonary nodules and presence of follicular
bronchiolitis.
C. With other causes ruled out, a typical UIP pattern on CT is diagnostic for IPF without lung biopsy
D. Lymphocytic interstitial pneumonia is the most common pathology seen in drug induced ILD.

Answer 16

C. With other causes ruled out, a typical UIP pattern on CT is diagnostic for IPF without lung biopsy
A. asbestos
D. Organizing PNA

Question 17

CT findings in immunologic lung disease

Answer 17

table 8-41 page 494

Question 18

specific OFC cut offs PPV tables page 497-497

Answer 18

print out and fill in, maybe make a few Q?