Pulmonary Pathology Part 3 Flashcards
How does idiopathic pulmonary fibrosis damage the lungs and what does it lead to?
- Damage is like waves of inflammatory injury leading to fibrosis
How is idiopathic pulmonary fibrosis diagnosed?
- Classic findings on high-resolution CT scan OR pulmonary biopsy
What will the biopsy in IPF show?
- Usual interstitial pneumonia
- Normal areas
- Inflammation
- FIbroblast foci
- Peripheral honeycombing
What are some contributing factors to IPF?
- Environmental factors (industrialized societies and smoking)
- Genetic factors
- Increasing age (>50)
What does someone with IPF look like clincially?
- Shortness of air
- Velcro like crackles on exam
- Restrictive pattern on PFTs
- Basilar infiltrates (progression to honeycombing)
How long does it take for someone to die from IPF?
- 3-5 years after diagnosis
What are some potential therapies for IPF?
- Lung transplantation
- Medications to arrest fibrosis (tyrosine kinase inhibitors or TGF-B inhibitors)
What is nonspecific interstitial pneumonia (NSIP)?
- Idiopathic
- Has unique histology
- Uniform infiltrates and fibrosis
- No heterogeneity
- No fibroblast foci
- No granulomata
What is cryptogenic organizing pneumonia (COP)?
- Formerly BOOP
- Occurs superimposed on prior infection or inflammatory process
What does someone with COP look like?
- Pneumonia-like consolidation
- Presents in fifth/sixth decades
How do you diagnose COP?
- Diagnosis of exclusion
- There isn’t an active infection, not drug or toxin induced, or related to CT disorders
How do you treat COP?
- Usually patients do well on oral steroids
What is the correlation between autoimmune diseases and interstitial lung diseases?
- Autoimmune or CT disorders can manifest as ILD
What happens if ILD occurs as a manifestation of an autoimmune or CT disorder?
- They are not diagnosed as pure diseases
- Prognosis is linked to underlying condition
What is sarcoidosis?
- A systemic manifesting non-caseating granulomata in various organs (90% of cases involve lungs or hilar lymph nodes)
What is the clinical presentation of someone with sarcoidosis?
- Incidental abnormal radiograph
- Dyspnea
- <40 years old
- 10-fold predominance in african americans
- Elevated serum ACE levels
What are the granuloma inclusions in sarcoidosis?
- Asteroid body (broken down collagen)
- Schaumann bodies
What is the difference between sarcoidosis and hypersensitivity pneumonitis?
- Both have granulomas but hypersensitivity pneumonitis has no fibrosis
What is the cause of hypersensitivity pneumonities?
- Immune reaction to inhaled antigen
- Pigeon breeder’s lung (protein from bird feces)
- Farmer’s lung (actinomycetes spores in hay)
- Hot tub lung (reaction to MAC)
How do you diagnose hypersensitivity pneumonitis?
- History
What is desquamative interstitial pneumonia (DSIP)?
- Restrictive lung presentation
- Presents in smokers in their 4th or 5th decade
- Histology will show “stuffed” alveolar spaces full of macrophages
What is the treatment and prognosis for DSIP?
- Stop smoking
- Corticosteroids
- Good prognosis (95%)
What is respiratory bronchiolitis interstitial lung disease?
- Part of the spectrum as DSIP but less symptomatic and earlier presentation
- Often reversible with smoking cessation if caught early
Who presents with RB-ILD?
- Smokers in their 3rd or 4th decade
How do you diagnose RB-ILD?
- Any abnormal radiographs require a prompt biopsy
What do we see on histology in RB-ILD?
- Less macrophages
- “Peribronchiolar metaplasia”
- May have fibrosis in advanced cases
What is langerhans cell histiocytosis?
- Stellate lung lesions in young smokers
- Progressive scarring leads to cysts
- Peripheral cysts may rupture causing pneumothorax
What does histology look like with langerhans cell histiocytosis?
- Eosinophils
- Langerhan cells
- Varying fibrosis and cysts
What is pulmonary alveolar proteinosis?
- Impairment of surfactant metabolism due to defect of granulocyte-macrophage colony stimulating factor (GM-CSF)
What are the different types of pulmonary alveolar proteinosis?
- Autoimmune
- Secondary
- Hereditary
How is pulmonary alveolar proteinosis treated?
- SubQ GM-CSF
What is also seen in pulmonary alveolar proteinosis?
- Copious milky fluid
What is pneumoconiosis?
- Reaction by lung to inhaled mineral or organic dust or vapors
- Occupational exposure
- Air pollution
When is pneumoconiosis worse?
- If exposure is repetitive and high
- Small particles (able to reach alveoli)
- Impaired ciliary clearance (smoking)
What is coal workers’ pneumoconiosis?
- Disease due to inhaled coal dust, with a spectrum of disease
What is the spectrum of disease in coal workers’ pneumoconiosis?
- Anthracosis
- Coal macule/nodules
- Progressive massive fibrosis
What is silicosis?
- Disease resulting from inhaled silicon dioxide
Where is silicosis seen?
- Seen in mining or quarry work
- Concrete repair or demolition
When is the onset of silicosis?
- Insidious onset, can occur after exposure is no longer present
What can silicosis progress to?
- May progress to massive pulmonary fibrosis
- Two-fold risk of developing cancer
What are the radiologic findings of silicosis?
- Eggshell calcifications (calcified hilar lymph nodes)
What is asbestosis?
- Interstitial and pleural disease resulting from inhalation of asbestos fibers
What does the risk of asbestosis correlate with? Who typically has asbestosis?
- Risk correlates with asbestos exposure
- Insulation workers
- Shipyard workers (navy)
- Paper mill workers
- Oil or chemical refinery workers
What are some disease manifestations of asbestosis?
- Pleura: fibrosis/fibrous plaque, effusions, mesothelioma
- Lung: interstitial fibrosis, carcinoma
- Extrapulmonary neoplasms
What are the two types of asbestos fibers? Which one is worse?
- Serpentine
- Amphibole (worst due to straight nature, allowing it to go deeper in lungs)
What does asbestos look like on histology?
- Golden dumbbells due to macrophages trying to eat them
What does the plaque formation look like in asbestosis?
- Candle Wax drippings on pleura due to hyaline collagen
What is mesothelioma?
- Diseases associated with asbestos exposure
When does mesothelioma present?
- Decades after exposure
- Lifetime exposure risk is as high as 10%
What is a pulmonary infarct?
- Wedge shaped lesion
- Begins as hemorrhagic, then fibrosis sets in
What is characteristic of a pulmonary infarct that happens antemortem?
- Lines of Zahn
What can obstruct vessels and cause a pulmonary emboli?
- Blood clot
- Bone marrow/fat
- Cancer
- Septic emboli
- Air
- Foreign material
- Parasites
What is a talc emboli?
- An emboli seen in IV drug users
What else can cause nodules in IV drug users?
- Septic emboli
What is a septic emboli?
- Bloodborne infective material
- May occur in endocarditis valve vegetation break off and travels to the lungs
What is pulmonary hypertension?
- Pulmonary artery pressure greater than 25 mmHg (have to stick a cath into the heart to obtain)
What are some pulmonary hemorrhage syndromes?
- Goodpasture syndrome
- Granulomatosis with polyangiitis
- Idiopathic pulmonary hemosiderosis
What do we see in goodpasture syndrome?
- Immunofluorescence showing a linear pattern of deposition due to anti-basement membrane antibodies
What is goodpasture syndrome?
- Antibodies against a non collagenous subunit of collagen IV
Where do we see goodpasture syndrome?
- Occurs in males in 2nd or 3rd decade
