Interstitial Lung Diseases Flashcards
What are interstitial lung diseases (ILDs)?
A group of disorders characterized by cellular infiltration, scarring and/or architectural disruption of the pulmonary parenchyma
What are the ssx of ILD?
Dry cough with progressive DOE
What are PFTs like with ILDs?
Restrictive pattern (decreased FEV, and normal FEV1/FVC)
What are the CT/ CXR findings with ILDs?
Interstitial infiltrates and Ground glass appearance
What are the major occupational exposures that lead to ILD? (4)
Silica
Asbestos
Farms
Fumes
What are the two classic drugs that lead to ILD?
Amiodarone
Nitrofurantoin (macrobid)
What are the connective tissue disorders that can lead to ILD?
SLE
RA
Scleroderma
What are the signs of ILD?
Clubbing
Erythema/rash
Arthritis
What are the lungs sounds with ILD?
Dry crackles
What heart findings are found with ILD? (4)
Increased right heart pressure:
- increased P2 intensity
- TR murmur
- edema
- JVD
What is usually the first change on a PFT with ILD? Why?
Decreased DLCO due to non homogeneous thickening of the alveoli
What is usually the first change on a PFT with COPD? Why?
FEV/FVC ratio
What are the two ILDs that have increased lung volumes?
LAM
Langerhans histiocytosis
What is the granulomatous ILD?
Sarcoidosis
What are the four known association ILDs?
CT diseases
Drugs
Occupational exposure
Hypersensitivity
What are the idiopathic Interstitial pnuemonias?
- Idiopathic pulmonary fibrosis
- non-specific interstitial pneumonia
- Cryptogenic organizing pneumonia
- respiratory bronchiolitis
- desquamative interstitial pneumonia
- acute interstitial pneumonia
What patients with idiopathic interstitial pneumonias respond to therapy?
No
What is the prognosis (generally) for idiopathic pulmonary fibrosis?
Bad-3-5 year survival rate
What is the history commonality for idiopathic pulmonary fibrosis?
Smoking
Who usually gets idiopathic pulmonary fibrosis?
50-70 year old smokers
What are the ssx of idiopathic pulmonary fibrosis?
Chronic and progressive DOE, and non-productive cough
What are the lung sounds with idiopathic pulmonary fibrosis?
Bibasilar inspiratory “velcro-like” crackles
What percent of patients with idiopathic pulmonary fibrosis have clubbing?
25%
What causes the crackles with idiopathic pulmonary fibrosis?
Expanding alveoli
What are the CXR findings with idiopathic pulmonary fibrosis? (which lobe? central or peripheral distribution? What type of CT changes)?
Lower lobe and peripheral distribution of reticular infiltrates and fibrotic changes
What are the CT changes with idiopathic pulmonary fibrosis?
Patchy honeycombing and traction bronchiectasis
What are the tissue changes that can be seen in with idiopathic pulmonary fibrosis?
Usual interstitial pneumonitis
What are the drugs used to treat idiopathic pulmonary fibrosis? MOA?
Pirfenidone
Nintedanib
Inhibit IL-1beta and TNF-alpha from human lung fibroblasts
What is the MOA of etanercept? Is this effective in idiopathic pulmonary fibrosis?
Anti-TNF antibody
Not effective
What is N-acetylcysteine used for usually? Is it effective for idiopathic pulmonary fibrosis?
Acetaminophen overdose
Not effective
What is the MOA of Pirfenidone? Use?
Inhibit IL-1beta and TNF-alpha from human lung fibroblasts
Treats idiopathic pulmonary fibrosis
What is the MOA of nintedanib? Use?
Inhibit IL-1beta and TNF-alpha from human lung fibroblasts
Treats idiopathic pulmonary fibrosis
What are nonspecific interstitial pneumonitis (NSIP)?
Homogenic CT disease of the lungs
What is the prognosis (generally) with interstitial pneumonitis?
better than IPF
What are the imaging findings with nonspecific interstitial pneumonitis (NSIP)?
Ground glass appearance symmetrically in the lower lung fields
Which disease is honeycombing common in: idiopathic pulmonary fibrosis or nonspecific interstitial pneumonitis (NSIP)?
idiopathic pulmonary fibrosis
What is the therapy for nonspecific interstitial pneumonitis (NSIP)?
Steroids
What are the two ILDs that are definitively linked to smoking?
Desquamative interstitial pneumonitis (DIP)
Respiratory bronchiolitis-associated interstitial lung disease (RB-ILD)
What are the histological characteristics of Desquamative interstitial pneumonitis (DIP) and Respiratory bronchiolitis-associated interstitial lung disease (RB-ILD)?
Peribronchiolar accumulation of pigmented macrophages
What is the treatment for Desquamative interstitial pneumonitis (DIP) and Respiratory bronchiolitis-associated interstitial lung disease (RB-ILD)?
Stop smoking
Steroids
What are the imaging findings with DIP and RB-ILD?
Diffuse, patchy areas of ground glass appearance
What is acute interstitial pneumonitis/diffuse alveolar damage (AIP/DAD)
Sudden and fulminant form of diffuse lung injury that generally occurs in previously healthy individuals
What are the ssx ofacute interstitial pneumonitis/diffuse alveolar damage (AIP/DAD)
Fever
Cough
SOB
What is the prognosis for acute interstitial pneumonitis/diffuse alveolar damage (AIP/DAD)?
50% mortality, and those that survive develop fibrosis
What are the imaging findings with acute interstitial pneumonitis/diffuse alveolar damage (AIP/DAD)?
Diffuse, bilateral air space and reticular infiltrates
What is the treatment for acute interstitial pneumonitis/diffuse alveolar damage (AIP/DAD)?
Supportive
What is cryptogenic organizing pneumonia (COP)?
a non-infectious pneumonia; specifically, an inflammation of the bronchioles (bronchiolitis[1]) and surrounding tissue in the lungs.
Who usually gets cryptogenic organizing pneumonia (COP)?s
Smokers
What is the clinical presentation of cryptogenic organizing pneumonia (COP)?
LRTI with lack of response to abx
LRTI with lack of response to abx = ?
cryptogenic organizing pneumonia (COP)
What is the treatment and prognosis for cryptogenic organizing pneumonia (COP)?
Corticosteroids
Good prognosis
What is the best way to diagnose cryptogenic organizing pneumonia (COP)? (2)
Bronchoscopy
Alveolar lavage
What are the imaging findings of cryptogenic organizing pneumonia (COP)? Where are they in the lungs?
Patchy infiltrates (unilaterally or bilaterally), with small nodular opacities
Usually subpleural
What is hypersensitivity pneumonitis?
Repeated exposure and immunologic sensitization to organic antigens
What are the risk factors for developing hypersensitivity pneumonitis? (3)
Bird feathers
Thermophilic bacteria
Farmers
What are the cells that mediate hypersensitivity pneumonitis? What do these do?
CD8 +
Macrophages
Granuloma formation
What is the usual acute presentation of hypersensitivity pneumonitis?
Abrupt onset of fever, chills, nausea, cough, chest tightness and dysponea
What are the PE findings with hypersensitivity pneumonitis?
Tachypnea
Diffuse, fine crackles
What are the imaging findings of hypersensitivity pneumonitis?
Normal to patch, micronodular infiltrates
What is the treatment for hypersensitivity pneumonitis?
Remove antigen exposure
What is the usual SUBacute presentation of hypersensitivity pneumonitis? Treatment?
Gradual development of cough, SOB, weight loss
Corticosteroids
What is the usual presentation of CHRONIC hypersensitivity pneumonitis?
Insidious onset of cough, fatigue, SOB
Clubbing
What is the major complication of chronic hypersensitivity pneumonitis?
Irreversible Pulmonary fibrosis
What are the CT findings with hypersensitivity pneumonitis? (2)
Ground glass and centrilobular nodules
Why should you r/o other causes of lung disease prior to treating any of the ILDs?
Steroids will weaken the immune response, which will be bad with infectious causes
What is the lung disease that amiodarone causes? Who usually gets this?
Interstitial pneumonitis
Patients who are on the drug long term
What are the imaging findings with amiodarone toxicity? (2)
Ground glass appearance, with increased attenuation of the liver/spleen
What are the two presentations of lung toxicity induced by nitrofurantoin?
Acute after course of abx
Chronic after several months of abx
What are the peripheral blood findings with lung toxicity from nitrofurantoin?
Eosinophilia
What are the cytokines that are elevated with nitrofurantoin lung toxicity?
Serum gamma globulin
Serum transaminases
ANA
What is acute eosinophilic pneumonia? What causes it?
Acute febrile illness of 1-4 weeks duration with usual symptoms.
Associated with resumption of smoking, or inhalation exposure
What are the peripheral blood smear findings with acute eosinophilic pneumonia?
None
What are the histological findings with acute eosinophilic pneumonia?
Extensive eosinophilic infiltration in the interstitium
What are the CT findings of acute eosinophilic pneumonia?
Bilateral ground glass
Which is more likely to have eosinophilia: chronic or acute eosinophilic pneumonia?
Chronic
What is chronic eosinophilic pneumonia?
Idiopathic infiltration of eosinophils in the interstitial or alveolar spaces
What are the imaging findings of chronic eosinophilic pneumonia
bilateral peripheral pulmonary edema
“negative of pulmonary edema”
What are the peripheral blood smear findings with chronic eosinophilic pneumonia?
High levels of eosinophils
What is lymphangioleiomyomatosis? Who is affected?
Progressive cystic lung disease that occurs in women of childbearing years
What are the histological findings of lymphangioleiomyomatosis?
Proliferation of atypical smooth muscle cells
What are the ssx of lymphangioleiomyomatosis?
DOE
Hemoptysis
Pneumothorax
What is the usual presenting sign of lymphangioleiomyomatosis?
Pneumothorax
What is the treatment for lymphangioleiomyomatosis?
Lung transplant
What is the best test to get to differentiate ILDs?
High res CT (HRCT)
Idiopathic pulmonary fibrosis and non-specific interstitial pneumonitis are very similar. What are the major differences between the two? (hint: age, CT findings, prognosis, pathological pattern)?
IPF = older smokers, honeycombing, bad prognosis, UIP
NSIP: Younger pts, non-honeycombing, better prognosis, temporal heterogeneity pattern,
