Interstitial Lung Disease Flashcards
Idiopathic interstitial lung disease clinically characterized by sudden onset of dyspnea and rapid development of respiratory failure
Acute interstitial pneumonia [aka Hamman-Rich syndrome]
Acute interstitial pneumonia (AIP) is histologically characterized by _______ with subsequent ______
Diffuse alveolar damage; fibrosis
Compare/contrast acute interstitial pneumonia and idiopathic pulmonary fibrosis in terms of age group, etiology, pattern, onset and presentation, and diagnosis
AIP occurs in pts from all age groups (mostly adults), has no specific etiology, pattern is diffuse, symmetric, and bilateral, abrupt onset with flu-like sxs, and dx is based on biopsy
IPF occurs almost exclusively in adults, if often related back to causative exposure, pattern is asymmetric and favors upper or lower lobes, onset is gradual and pt is afebrile, and dx is based on hx/clinical findings, imaging, and PFTs (bx is optional)
Anti-fibrotic medications are only used in what specific interstitial lung disease?
Idiopathic pulmonary fibrosis
Prototypic presentation of interstitial lung disease
Dyspnea, cough, crackles described as sounding like “velcro” primarily at lung bases, digital clubbing, and exercise-induced hypoxemia
Describe evaluation of a pt with presumed interstitial lung dz (what tests are done?)
PFTs
Chest CT
ANA and rheumatoid factor
Evaluation must also include history — occupation, hobby, environmental, travel, drugs (both Rx and Non-Rx)
What do PFTs show with interstitial lung dz?
Reduced FRC and TLC
Normal FEV1/FVC
Low DLCO
3 Common drugs associated with interstitial lung disease
Amiodarone — cardiac antiarrhythmic; dose-related
Bleomycin — chemotherapy agent
Nitrofurantoin — antibiotic commonly used for UTI
[others include methotrexate and busulfan]
Asbestosis is a type of ILD associated with what occupations? What is the characteristic feature?
Construction workers
Plumbers
Shipyard workers, including “chippers”
Shows PLEURAL PLAQUES
Silicosis is a type of ILD associated with what occupations?
Sand blasters
Silica miners
Spelunkers (“spelunker’s lung”)
ILD found in coal miners
Coal worker’s pneumoconiosis
Berylliosis is a type of ILD found in what occupations?
Beryllium miners
Workers in aerospace industry
When it is known, the underlying cause of ILD is the target of treatment. If ILD is not due to IPF, ______ are typically used
Corticosteroids [very effective when induced by radiation]
Antifibrotic tx options for IPF
Pirfenidone — antifibrotic and anti-inflammatory [specific action unknown]
Nintendanib — tyrosine kinase inhibitor; targets inflammatory and profibrotic factors
Name the disease:
Most cases are either asymptomatic or have mild dz; often found incidentally on chest radiograph. CXR is fairly symmetrical (not so with chest CT). In a small number of people this can be a devastating dz and lead to severe disability and death. Prednisone is the tx of choice
Sarcoidosis
ILD vs. COPD
ILD has more rapid decline and the cough is usually nonproductive
COPD has slower decline with more frequent exacerbations; weather and NSAIDs worsen symptoms; cough is productive
Acute vs. chronic form of hypersensitivity pneumonitis
Acute: abrupt onset of dyspnea, cough, fever, +/- myalgias
Chronic: pulmonary fibrosis
Autoimmune condition that may contribute to ILD; extra-pulmonary restriction sometimes referred to as “shrinking lung syndrome”; may be characterized by pulmonary HTN and pleural dz
SLE
Name the disease:
Interstitial pulmonary fibrosis Bronchiectasis Pulmonary nodules Pulmonary vasculitis Pleural disease (very low pleural glucose is characteristic)
Rheumatoid arthritis
An 83 y/o female nonsmoker presents with 3 years of progressive dyspnea and recent development of nonproductive cough. There is no variation with weather changes or exposure to NSAIDs. She denies orthopnea, edema, or PND. No recent travel. She has worked as a secretary for 38 years. Her hobbies include crocheting, knitting, and reading, and she was a spelunker until 30 years ago. Her exam is normal except for O2 sat 84%, slight accessory respiratory muscle use, 1-2 cm hemi-diaphragmatic percussion, and diffuse bisilar crackles. PFTs reveal FVC 50%, FEV1 45%, FEV1/FVC 90%, FRC 50%, RV 48%, TLC 58%, and DLCO 34%. The most likely diagnosis is:
A. Idiopathic pulmonary fibrosis B. Emphysema C. Silicosis D. Asbestosis E. Radiation pneumonitis
C. Silicosis
[“spelunker’s lung”]
A 58 y/o man is evaluated for insidious onset of slowly progressive dyspnea over the last 3 years. At this point, he must stop to rest when walking 1 block. Previously, he walked 1-2 miles daily without difficulty. He denies cough, chest pain, or orthopnea. He has no other medical problems. PE reveals BMI of 35, O2 sat 93% RA, dry crackles on auscultation, parasternal heave and persistent splitting of S2. PFTs reveal FEV1 60%, FEV1/FVC 80%, TLC 70%, DLCO 45%. CXR shows diffuse reticular infiltrates in upper lung zones. Which of the folowing is the most likely diagnosis?
A. Diffuse parenchymal lung disease B. Heart failure C. Obesity hypoventilation syndrome D. Obstructive airway disease E. Respiratory muscle weakness
A. Diffuse parenchymal lung disease
A 32 y/o female nonsmoker is evaluated for slowly progressive dyspnea. For the last 6 months she has experienced progressive exertional dyspnea, exertional chest pressure, and dry cough. She is now short of breath when climbing one flight of stairs. She has no fever or weight loss. She reports one episode of a painful, raised, erythematous lesion on the anterior right shin accompanied by fever and ankle pain 6 months ago that resolved spontaneously after 3 weeks. Physical exam is normal. PFTs reveal FEV1 75%, FVC 88%, FEV1/FVC 68%, TLC 78%, and RV 70%. CXR appears “cloudy” in some areas but is otherwise normal. Which of the following is the most likely diagnosis?
A. Chronic hypersensitivity pneumonitis
B. Lymphangioleiomyomatosis
C. Respiratory bronchiolitis-associated interstitial lung disease
D. Sarcoidosis
D. Sarcoidosis
A 65 y/o man is evaluated for a 4-month history of gradually progressive SOB. Initially, symptoms were only present with exertion, but now they occur at rest. He also has an occasional productive cough. He has a 30-pack year hx of smoking and quit 10 years ago. He worked as a “chipper” in a shipyard where he had extensive and unprotected exposure to asbestos 20 years ago. Which of the following diagnostic tests should be done next?
A. Antinuclear antibody, rheumatoid factor, and ESR B. Open lung biopsy C. Transbronchial lung biopsy D. Endobronchial US with biopsy (EBUS) E. No further testing
E. No further testing
We know he has asbestosis!
