Lecture 5: Interstitial Lung Disease

Question 1

Which histopathologic pattern of interstital lung involvement is characterized by heterogenous involvement of the lung with different stages of progression of fibrosis in adjacent areas of the lung?

Answer 1

Usual Interstitial Pneumonitis (UIP)

Question 2

A pt presenting with progressive dyspnea (over months), reduced exercise tolerance, and a persistent dry cough should raise suspicion for what?

Answer 2

Interstitial lung disease

Question 3

Which imaging modality is a crucial tool for the diagnostic evaluation of interstitial lung diseases?

Answer 3

High-resolution CT

Question 4

Interstital lung disease evaluation must include hx of what 5 things?

Answer 4

• Occupation
• Hobbies
• Enviornmental
• Travel
• Drugs both Rx and non-Rx

Question 5

Which lung sound auscultated in a patient with interstitial lung disease is almost pathognomonic?

Answer 5

Crackles often described as “velcro” developing in the bases throughout inspiration

Question 6

What are the prototypic sign/sx’s of interstitial lung disease?

Answer 6

• Dyspnea + cough + crackles
• Digital clubbing (presence does not confirm and absence does not eliminate ILD)
• Exercise induced hypoxemia

Question 7

What is the FRC, TLC, FEV1/FVC and DLCO like with interstitial lung diseases?

Answer 7

• ↓ FRC and ↓ TLC
• Normal FEV1/FVC
• Low DLCO

Question 8

Very low pleural glucose is characteristics of pleural disease associated with what?

Answer 8

Rheumatoid Arthritis

Question 9

Shrinking lung syndrome is associated with what systemic disease?

Answer 9

SLE

Question 10

Which 3 autoantibodies may be seen in Scleroderma; which is most common in those with ILD; which is associated with worst prognosis?

Answer 10

• Anticentromere
• SCL-70 = more common in ILD
• Antinucleolar = predicts worst prognosis

Question 11

Which autoantibody is seen in >90% of drug induced SLE cases?

Answer 11

anti-Histone

Question 12

Which autoantibody is seen in 90-95% of SLE cases?

Answer 12

Anti-nuclear antibodies

Question 13

Which autoantibody is seen in pt’s with SLE induced nephritis?

Answer 13

Anti-dsDNA

Question 14

Which antiarrhythmic and antibiotic used for UTI’s are commonly associated with drug induced interstitial lung disease?

Answer 14

• Amiodarone
• Nitrofurantoin

Question 15

When is the early phase vs. late phase of radiation induced lung disease?

Answer 15

• Early phase = 1-3 months after radiation
• Late phase = 6-12 months

Question 16

What is the rate of decline and cough like in ILD vs. COPD?

Answer 16

• COPD = slower decline w/ more frequent exacerbations + productive cough
• ILD = more rapid decline + non-productive cough

Question 17

What is the mainstay drug used to tx non-IPF induced ILD?

Answer 17

Corticosteroids

Question 18

What are the 2 anti-fibrotic agents which are ONLY used for idiopathic pulmonary fibrosis; describe each drug?

Answer 18

1) Pifenidone = antifibrotic and anti-inflammatory
2) Nintedanib = tyrosine kinase inhibitor targets inflammatory and profibrotic factors

Question 19

What is the characteristic finding on a CXR of someone with acute interstitial pneumonia (aka Hamman-Rich syndrome)?

Answer 19

Diffuse, symmetrical, and bilateral

Question 20

What is the onset like with acute interstitial pneumonia (aka Hamman-Rich syndrome) vs. IPF?

Answer 20

• AIP –> abrupt onset w/ flu-like sx’s
• IPF –> gradual onset, afebrile

Question 21

Acute interstitial pneumonia (aka Hamman-Rich syndrome) vs. IPF occurs in which age groups?

Answer 21

• AIP –> occurs in patients from all age groups, mostly adults
• IPF –> occurs almost exclusively in adults

Question 22

How is the diagnosis of acute interstitial pneumonia (aka Hamman-Rich syndrome) vs. IPF established?

Answer 22

• AIP –> BIOPSY establishes diagnosis
• IPF –> hx/clinical findings, imaging, and PFTs; biopsy optional