ILDs Flashcards
Which of the following is one of the earliest physical examination findings in idiopathic pulmonary fibrosis (IPF)?
A) Digital clubbing
B) End-inspiratory fine crackles (rales) at the lung bases
C) Wheezing
D) Cyanosis
Answer: B) End-inspiratory fine crackles (rales) at the lung bases
Rationale: Fine crackles at the lung bases are an early and common finding in IPF, whereas digital clubbing and cyanosis occur in more advanced disease. Wheezing is uncommon in most interstitial lung diseases (ILDs).
Which of the following pulmonary function test (PFT) findings is characteristic of most interstitial lung diseases (ILDs)?
A) Increased total lung capacity (TLC)
B) Reduced forced expiratory volume in 1 second (FEV1) with preserved forced vital capacity (FVC)
C) Decreased FEV1/FVC ratio
D) Reduced total lung capacity (TLC) and symmetrically reduced measures of
forced expiratory volume in 1 s (FEV1) and forced vital capacity (FVC)
Answer: D) Reduced total lung capacity (TLC) and symmetrically reduced measures of
forced expiratory volume in 1 s (FEV1) and forced vital capacity (FVC)
Rationale: Most ILDs result in restrictive lung disease, characterized by reduced TLC, symmetrically reduced FEV1 and FVC, and decreased DLCO. A decreased FEV1/FVC ratio is more typical of obstructive lung disease.
Which of the following pulmonary function test (PFT) abnormalities may precede a reduction in lung volumes in ILD?
A) Increased forced expiratory volume in 1 second (FEV1)
B) Decreased diffusing capacity for carbon monoxide (DLCO)
C) Increased total lung capacity (TLC)
D) Increased residual volume (RV)
Answer: B) Decreased diffusing capacity for carbon monoxide (DLCO)
Rationale: A reduced DLCO may be the earliest detectable abnormality in ILD before changes in lung volumes are apparent. However, DLCO testing has more variability and is less specific.
Which of the following findings is most indicative of advanced interstitial lung disease (ILD)?
A) Mild dyspnea on exertion
B) Decreased total lung capacity (TLC)
C) Digital clubbing and cor pulmonale
D) Increased forced vital capacity (FVC)
Answer: C) Digital clubbing and cor pulmonale
Rationale: Digital clubbing and cor pulmonale (right heart failure due to lung disease) are signs of advanced ILD. A decrease in TLC is an earlier sign of disease.
Which of the following is the most common idiopathic interstitial lung disease (ILD)?
A) Hypersensitivity pneumonitis
B) Sarcoidosis
C) Idiopathic pulmonary fibrosis (IPF)
D) Cryptogenic organizing pneumonia
Answer: C) Idiopathic pulmonary fibrosis (IPF)
Rationale: IPF is the most common ILD of unknown cause and is typically diagnosed in older adults, particularly men with a history of smoking or environmental exposures.
What is the estimated 3- to 5-year survival rate for patients diagnosed with IPF?
A) 90%
B) 70%
C) 50%
D) 30%
Answer: C) 50%
Rationale: IPF carries a poor prognosis with an estimated 50% survival rate within 3–5 years of diagnosis, making it one of the more aggressive forms of ILD.
Which of the following high-resolution CT (HRCT) findings is characteristic of usual interstitial pneumonia (UIP) in IPF?
A) Upper lung-predominant fibrosis with micronodules
B) Subpleural reticulation with posterior basal predominance
C) Extensive ground-glass opacities and mosaic attenuation
D) Centrilobular nodules and air trapping
Answer: B) Subpleural reticulation with posterior basal predominance
Rationale: UIP pattern in IPF is characterized by subpleural reticulation, basal predominance, honeycombing, and traction bronchiectasis. Findings such as extensive ground-glass opacities, upper-lung predominance, or mosaic attenuation suggest an alternative diagnosis.
Which histopathologic feature is characteristic of usual interstitial pneumonia (UIP) in IPF?
A) Diffuse alveolar damage
B) Granulomas
C) Subpleural fibrosis with honeycombing and fibroblast foci
D) Bronchiolocentric inflammation
Answer: C) Subpleural fibrosis with honeycombing and fibroblast foci
Rationale: UIP pattern in IPF is characterized by subpleural fibrosis, honeycombing, and fibroblast foci with temporal and spatial heterogeneity. Granulomas are more indicative of sarcoidosis, and bronchiolocentric inflammation suggests hypersensitivity pneumonitis.
Which of the following best describes the role of antifibrotic therapy in IPF treatment?
A) It can reverse fibrosis in early-stage IPF
B) It slows disease progression and may improve survival
C) It is only effective in advanced stages of IPF
D) It replaces lung transplantation as the primary treatment option
Answer: B) It slows disease progression and may improve survival
Rationale: Antifibrotic therapies such as pirfenidone and nintedanib do not reverse fibrosis but can slow lung function decline and may improve survival. Lung transplantation remains the only curative option for eligible patients.
Which of the following statements best describes idiopathic nonspecific interstitial pneumonia (NSIP)?
A) It is most commonly diagnosed in elderly male smokers
B) It has a poor prognosis with a 5-year survival of <50%
C) It is often associated with connective tissue disease (CTD)
D) It is characterized by frequent honeycombing and fibroblast foci
Answer: C) It is often associated with connective tissue disease (CTD)
Rationale: NSIP is frequently observed in patients with CTD and is commonly diagnosed in nonsmoking females in their fifth decade of life. Unlike idiopathic pulmonary fibrosis (IPF), NSIP has a relatively good prognosis with a 5-year survival of >80%. Honeycombing and fibroblast foci are uncommon in NSIP.
Which of the following high-resolution CT (HRCT) findings is most characteristic of NSIP?
A) Peripheral reticular opacities with honeycombing
B) Upper lung-predominant fibrosis with nodules
C) Diffuse, symmetric ground-glass opacities with subpleural sparing
D) Centrilobular emphysema and cystic airspaces
Answer: C) Diffuse, symmetric ground-glass opacities with subpleural sparing
Rationale: NSIP is characterized by diffuse subpleural and symmetric ground-glass opacities with reticular opacities. Honeycombing is uncommon, which helps distinguish NSIP from UIP (usual interstitial pneumonia). Subpleural sparing can occasionally be seen, and traction bronchiectasis involving the lower lung zones is also a feature.
Which of the following is the primary treatment approach for NSIP?
A) Beta-agonists and inhaled corticosteroids
B) Antifibrotic therapy alone
C) Immunosuppression with corticosteroids and cytotoxic agents
D) No treatment, as NSIP is self-limiting
Answer: C) Immunosuppression with corticosteroids and cytotoxic agents
Rationale: Unlike IPF, NSIP is often treated with immunosuppressive therapy such as oral corticosteroids (prednisone), cytotoxic agents (mycophenolate, azathioprine, cyclophosphamide), or biologics (rituximab). Antifibrotic therapy may be beneficial in progressive pulmonary fibrosis but is not the primary treatment for NSIP.
Which subtype of NSIP has a better prognosis?
A) Cellular NSIP
B) Fibrotic NSIP
C) Honeycombing NSIP
D) Nodular NSIP
.
Answer: A) Cellular NSIP
Rationale: NSIP is classified into cellular and fibrotic subtypes. Cellular NSIP has a more favorable prognosis and is more responsive to medical therapy, while fibrotic NSIP has a less favorable prognosis and is associated with a higher degree of fibrosis
Which of the following patients is most likely to be diagnosed with idiopathic NSIP?
A) A 70-year-old male smoker with upper lung-predominant honeycombing
B) A 50-year-old nonsmoking female with positive autoantibodies and ground-glass opacities
C) A 35-year-old construction worker with multinodular fibrosis and calcified lymph nodes
D) A 60-year-old farmer with fever, chills, and patchy fibrosis
Answer: B) A 50-year-old nonsmoking female with positive autoantibodies and ground-glass opacities
Rationale: Idiopathic NSIP most commonly occurs in nonsmoking middle-aged women and is frequently associated with connective tissue diseases (CTDs), often presenting with positive autoantibodies. The other choices describe conditions such as IPF (A), silicosis (C), or hypersensitivity pneumonitis (D).
What is the most common clinical presentation of cryptogenic organizing pneumonia (COP)?
A) Acute onset of hemoptysis and pleuritic chest pain
B) Subacute flu-like illness with cough, dyspnea, fever, and fatigue
C) Sudden-onset dyspnea with absent breath sounds and hyperresonance
D) Chronic dry cough with progressive weight loss and night sweats
Answer: B) Subacute flu-like illness with cough, dyspnea, fever, and fatigue
Rationale: COP commonly presents as a subacute illness with flu-like symptoms, including cough, dyspnea, fever, and fatigue. It is often mistaken for pneumonia, but does not respond to antibiotics. Hemoptysis (A) is more suggestive of conditions like tuberculosis or vasculitis, while sudden dyspnea with hyperresonance (C) is more indicative of pneumothorax.
Which of the following imaging findings is most characteristic of COP on high-resolution CT (HRCT)?
A) Upper lung-predominant honeycombing
B) Patchy, subpleural consolidative opacities with ground-glass opacities
C) Centrilobular nodules and mosaic attenuation
D) Diffuse, reticular interstitial thickening with subpleural sparing
Answer: B) Patchy, subpleural consolidative opacities with ground-glass opacities
Rationale: The hallmark imaging finding of COP is patchy subpleural consolidation, often migratory, with associated ground-glass opacities. A reversed halo sign (atoll sign) may be seen, which is highly suggestive of COP. Honeycombing (A) is more typical of UIP/IPF, centrilobular nodules (C) are common in hypersensitivity pneumonitis, and diffuse reticular thickening (D) is more characteristic of NSIP.
Which of the following is the first-line treatment for cryptogenic organizing pneumonia (COP)?
A) Broad-spectrum antibiotics
B) Corticosteroids
C) Antifibrotic therapy (pirfenidone, nintedanib)
D) Bronchodilators and leukotriene receptor antagonists
Answer: B) Corticosteroids
Rationale: Corticosteroids are the first-line treatment for COP, often leading to substantial clinical improvement. However, relapses are common, requiring prolonged therapy (≥6 months). Antibiotics (A) are ineffective, antifibrotic therapy (C) is used for IPF, and bronchodilators (D) are more useful in asthma or COPD rather than COP.
A 58-year-old patient diagnosed with cryptogenic organizing pneumonia starts corticosteroid therapy. What is the most common reason for treatment failure or recurrence?
A) Rapid steroid tapering
B) Antibiotic resistance
C) Failure to add antifibrotic agents
D) Lack of bronchodilator therapy
Answer: A) Rapid steroid tapering
Rationale: Relapse rates in COP are high, and the most common cause is premature discontinuation or rapid tapering of corticosteroids. Treatment typically needs to be continued for at least 6 months to prevent recurrence. Antibiotic resistance (B) is not relevant, antifibrotic therapy (C) is not required, and bronchodilators (D) do not play a major role in COP management.
In which of the following conditions is cryptogenic organizing pneumonia (COP) commonly misdiagnosed?
A) Bacterial pneumonia
B) Pulmonary embolism
C) Emphysema
D) Pneumothorax
Answer: A) Bacterial pneumonia
Rationale: COP is often misdiagnosed as pneumonia due to similar clinical symptoms (cough, fever, dyspnea, infiltrates on imaging). However, COP does not respond to antibiotics, and a lack of clinical improvement despite antibiotic therapy should raise suspicion for COP.
What is the most common pulmonary manifestation of systemic sclerosis (SSc)?
A) Cryptogenic organizing pneumonia (COP)
B) Pulmonary hypertension (PH)
C) Interstitial lung disease (ILD)
D) Diffuse alveolar damage (DAD)
Answer: C) Interstitial lung disease (ILD)
Rationale: ILD is the most common pulmonary complication of SSc, occurring in ~50% of patients with diffuse SSc and ~30% of those with limited SSc. Pulmonary hypertension (B) can occur separately or concurrently with ILD but is more common in limited SSc. While COP (A) and DAD (D) can be seen, they are less common.
Which high-resolution CT (HRCT) findings are commonly seen in systemic sclerosis-associated ILD?
A) Upper lung-predominant fibrosis with honeycombing
B) Patchy ground-glass opacities and pleural effusions
C) Basilar-predominant fibrosis with ground-glass opacities and esophageal dilation
D) Diffuse cystic lung disease with hyperinflation
Answer: C) Basilar-predominant fibrosis with ground-glass opacities and esophageal dilation
Rationale: HRCT findings in SSc-associated ILD resemble those of NSIP or IPF, with ground-glass opacities and reticulation predominantly in the lung bases. Esophageal dilation is another key finding due to esophageal dysmotility. Upper lung fibrosis with honeycombing (A) is more typical of IPF/UIP, pleural effusions (B) are not common, and diffuse cystic lung disease (D) is seen in LAM or Langerhans cell histiocytosis.
Which of the following histopathologic patterns is most commonly associated with systemic sclerosis-related ILD?
A) Usual interstitial pneumonia (UIP)
B) Non-specific interstitial pneumonia (NSIP)
C) Diffuse alveolar damage (DAD)
D) Lymphocytic interstitial pneumonia (LIP)
Answer: B) Non-specific interstitial pneumonia (NSIP)
Rationale: NSIP is the most common histopathologic pattern in systemic sclerosis-associated ILD, characterized by homogeneous interstitial inflammation and fibrosis without honeycombing. UIP (A) is seen more frequently in idiopathic pulmonary fibrosis (IPF). DAD (C) is associated with acute lung injury (e.g., ARDS), and LIP (D) is more commonly seen in Sjogren’s syndrome.
A patient with systemic sclerosis and ILD is being considered for treatment. Which of the following medications has shown benefit with improved tolerability compared to cyclophosphamide?
A) Methotrexate
B) Azathioprine
C) Mycophenolate mofetil
D) Prednisone
Answer: C) Mycophenolate mofetil
Rationale: Mycophenolate mofetil has been shown to be as effective as cyclophosphamide but with better tolerability in SSc-ILD patients. Methotrexate (A) is primarily used in RA, azathioprine (B) has a limited role, and high-dose prednisone (D) is generally avoided due to the risk of scleroderma renal crisis.
Which of the following patients with systemic sclerosis-associated ILD is most likely to be considered for lung transplantation?
A) A patient with mild pulmonary fibrosis and normal pulmonary function tests
B) A patient with end-stage ILD and ongoing severe gastroesophageal reflux
C) A patient with moderate ILD, significant aspiration risk, and pulmonary hypertension
D) A patient with progressive ILD without significant aspiration or chest wall restriction
Answer: D) A patient with progressive ILD without significant aspiration or chest wall restriction
Rationale: Lung transplantation may be considered for select SSc-ILD patients, but candidates must have controlled reflux and no significant chest wall restriction. Severe GERD (B, C) increases the risk of lung transplant failure due to aspiration-related lung injury. Patients with mild disease (A) are not candidates.
Which of the following is the most common extra-articular manifestation of rheumatoid arthritis (RA)?
A) Rheumatoid nodules
B) Interstitial lung disease (ILD)
C) Vasculitis
D) Sjögren’s syndrome
Answer: B) Interstitial lung disease (ILD)
Rationale: ILD is a common and serious extra-articular manifestation of RA, occurring in up to 10% of patients clinically and 40–50% radiologically. Rheumatoid nodules (A) are common but less clinically significant. Vasculitis (C) and Sjögren’s syndrome (D) can occur in RA but are not as frequent as ILD.
Which patient is at the highest risk for developing RA-associated ILD (RA-ILD)?
A) A 30-year-old female with early seropositive RA
B) A 55-year-old male smoker with longstanding RA
C) A 45-year-old male with seronegative RA and no lung disease
D) A 60-year-old female with a history of Sjögren’s syndrome
Answer: B) A 55-year-old male smoker with longstanding RA
Rationale: RA-ILD is more common in males, smokers, and those with longstanding, seropositive RA. While RA is more frequent in females, ILD occurs more often in males. Early disease (A) and seronegative RA (C) have a lower risk of ILD. Sjögren’s syndrome (D) can cause ILD but is not the strongest risk factor in RA-ILD.
Which HRCT pattern is most commonly associated with RA-ILD?
A) Organizing pneumonia
B) Lymphocytic interstitial pneumonia (LIP)
C) Usual interstitial pneumonia (UIP)
D) Desquamative interstitial pneumonia (DIP)
Answer: C) Usual interstitial pneumonia (UIP)
Rationale: UIP is the most common HRCT pattern in RA-ILD, though NSIP can also occur. UIP in RA-ILD has a worse prognosis, similar to idiopathic pulmonary fibrosis (IPF). Organizing pneumonia (A) and LIP (B) are less frequent. DIP (D) is typically seen in smokers with ILD but not RA-ILD.
Which of the following statements about RA-ILD treatment is true?
A) High-dose corticosteroids are the first-line therapy
B) There are no randomized controlled trials for immunosuppressive treatment in RA-ILD
C) Antifibrotic therapy is contraindicated in RA-ILD
D) Lung transplantation is not an option for RA-ILD
Answer: B) There are no randomized controlled trials for immunosuppressive treatment in RA-ILD
Rationale: Unlike systemic sclerosis (SSc-ILD), RA-ILD lacks large randomized trials to guide treatment. Immunosuppressive agents (e.g., mycophenolate, azathioprine) are used based on extrapolated data from SSc-ILD. Antifibrotics (C) like nintedanib and pirfenidone are being studied but not contraindicated. Lung transplantation (D) is an option for progressive disease.
A 62-year-old male with RA presents with worsening dyspnea and is found to have UIP on HRCT. What is the best next step?
A) Start prednisone 60 mg daily
B) Consider mycophenolate or azathioprine therapy
C) Prescribe inhaled corticosteroids and bronchodilators
D) Recommend immediate lung transplantation
Answer: B) Consider mycophenolate or azathioprine therapy
Rationale: Immunosuppressive agents like mycophenolate or azathioprine are often used in RA-ILD, although efficacy is uncertain. High-dose prednisone (A) is not first-line due to infection risk and limited benefit. Inhaled steroids (C) are not useful for ILD. Lung transplantation (D) is reserved for severe progressive disease.
