Question 1
What is the characteristic histological pattern associated with idiopathic pulmonary fibrosis (IPF)?
A. Nonspecific interstitial pneumonia (NSIP)
B. Usual interstitial pneumonia (UIP)
C. Diffuse alveolar damage (DAD)
D. Organizing pneumonia
Answer: B. Usual interstitial pneumonia (UIP)
Explanation: IPF is specifically associated with the UIP pattern, which includes patchy fibrosis, temporal heterogeneity, honeycombing, and fibroblastic foci.
Reference:
Radiopaedia: Idiopathic Pulmonary Fibrosis. “UIP is the hallmark histopathological pattern of IPF.”
Link
Question 2
Which of the following features on high-resolution CT (HRCT) is most suggestive of IPF?
A. Central ground-glass opacities
B. Subpleural honeycombing with basal predominance
C. Upper lobe-predominant nodules
D. Consolidation with air bronchograms
Answer: B. Subpleural honeycombing with basal predominance
Explanation: HRCT findings in IPF include subpleural and basal-predominant reticulation, honeycombing, and traction bronchiectasis, characteristic of UIP.
Reference:
Radiology Key: Imaging in IPF. “Honeycombing in subpleural and basal regions is a defining feature of IPF.”
Link
Question 3
Why does idiopathic pulmonary fibrosis predominantly affect the subpleural regions of the lungs?
A. Increased ventilation in the subpleural areas
B. Greater mechanical strain during respiration
C. Higher vascular supply in subpleural regions
D. Increased lymphatic drainage in subpleural areas
Answer: B. Greater mechanical strain during respiration
Explanation: Subpleural regions experience greater mechanical forces during respiration, making them prone to repetitive micro-injury and fibrosis in IPF.
Reference:
PubMed: Mechanical Strain in IPF. “Subpleural areas are vulnerable due to repetitive mechanical strain.”
Link
Question 4
Which of the following pulmonary function test (PFT) findings is most typical of IPF?
A. Increased total lung capacity (TLC)
B. Reduced forced vital capacity (FVC)
C. Increased residual volume (RV)
D. Normal diffusion capacity (DLCO)
Answer: B. Reduced forced vital capacity (FVC)
Explanation: IPF shows a restrictive PFT pattern with reduced FVC, TLC, and markedly reduced DLCO due to interstitial fibrosis.
Reference:
ATS Guidelines: Pulmonary Function Testing in IPF. “Restrictive patterns with reduced FVC and DLCO are hallmarks of IPF.”
Link
Question 5
Which of the following collagen vascular diseases is most commonly associated with a UIP pattern?
A. Systemic lupus erythematosus
B. Rheumatoid arthritis
C. Dermatomyositis
D. Sjögren’s syndrome
Answer: B. Rheumatoid arthritis
Explanation: Rheumatoid arthritis is strongly associated with a UIP pattern of interstitial lung disease, which can mimic IPF.
Reference:
Radiopaedia: UIP in Rheumatoid Arthritis. “UIP is a common ILD pattern in RA-associated lung disease.”
Link
Question 6
What distinguishes IPF from connective tissue disease-associated ILD (e.g., rheumatoid arthritis)?
A. UIP pattern
B. Presence of honeycombing
C. Absence of extrapulmonary manifestations
D. Subpleural and basal predominance
Answer: C. Absence of extrapulmonary manifestations
Explanation: IPF lacks extrapulmonary features, whereas connective tissue diseases (e.g., RA, SSc) often have systemic manifestations and serologic markers.
Reference:
Radiographics: Differentiating IPF and CTD-ILD. “The absence of systemic manifestations helps differentiate IPF from CTD-ILD.”
Link
Question 7
Which antifibrotic agent is FDA-approved for the treatment of IPF?
A. Pirfenidone
B. Prednisone
C. Methotrexate
D. Azithromycin
Answer: A. Pirfenidone
Explanation: Pirfenidone and nintedanib are FDA-approved antifibrotic agents for IPF, shown to slow disease progression.
Reference:
ATS Guidelines: Management of IPF. “Pirfenidone is an approved antifibrotic therapy for IPF.”
Link
Question 8
Which of the following findings is NOT typically associated with UIP?
A. Patchy fibrosis
B. Temporal heterogeneity
C. Peribronchovascular distribution
D. Honeycombing
Answer: C. Peribronchovascular distribution
Explanation: UIP is characterized by subpleural and basal-predominant fibrosis, not a peribronchovascular distribution.
Reference:
Radiopaedia: Features of UIP. “UIP involves subpleural, basal fibrosis, not peribronchovascular regions.”
Link
Question 9
Which of the following complications is commonly associated with advanced IPF?
A. Spontaneous pneumothorax
B. Pulmonary hypertension
C. Chronic bronchitis
D. Hemoptysis
Answer: B. Pulmonary hypertension
Explanation: Advanced IPF frequently leads to pulmonary hypertension due to chronic hypoxia and vascular remodeling.
Reference:
PubMed: Complications of IPF. “Pulmonary hypertension is a common complication in advanced IPF.”
Link
Question 10
What is the most common cause of acute exacerbations in patients with IPF?
A. Pulmonary embolism
B. Aspiration pneumonia
C. Idiopathic worsening of fibrosis
D. Viral infection
Answer: C. Idiopathic worsening of fibrosis
Explanation: Acute exacerbations of IPF are often idiopathic and are marked by rapid worsening of fibrosis without clear triggers.
Reference:
Radiology Key: Acute Exacerbations in IPF. “Acute exacerbations are commonly idiopathic but lead to rapid fibrosis progression.”
Link