Singh Pathology Respiratory Pathology #3 Flashcards
What is the most common type of interstitial lung disease processes?
- Idiopathic pulmonary fibrosis
- This damages pulmonary tissue with waves of inflammatory injury leading to fibrosis
What does Usual Interstitial Pneumonia consist of and what is it?
- Pathological diagnosis of IPF
- Consists of normal areas, inflammation, fibroblast foci and peripheral honeycombing
What factors contribute to getting IPF?
- Environmental such as industrialized societies and smoking
- Genetics
- Increasing age
Clinical findings in Idiopathic pulmonary fibrosis?
- Dyspnea
- Crackles (velcro like)
- Restrictive PFT
- Basilar infiltrates progressing to honeycomb pattern
How can you differentiate emphysema honeycombing vs IPF?
thick areas between fibrosis on histology and grossly differentiate IPF from emphysema
Prognosis of IPF? Therapy?
- Progressive- most patients die 3-5 yrs
- Therapies include
- lung transplant
- Tyrosine kinase inhibitors
- TGF-B inhibitors
What is Non specific Interstitial Pneumonia? What makes up the histology?
- Idiopathic pneumonia
- Unique histology
- Uniform infiltrates and fibrosis
- NO heterogeneity
- NO fibroblast foci
- NO granulomata
Describe Cryptogenic Organizing Pneumonia.
- Superimposed on prior infection or inflammatory process
- Presents with a pneumonia like consolidation in 50-60s
Why is COP a diagnosis of exclusion?
- Not an active infection, drug or toxin induced or related to CTD
- If you give steroids it clears up!
- Good prognosis with full recovery
If a patient has RA, systemic sclerosis, or SLE and develops IPF, NSIP, or COP how do you diagnose these patients?
- Connective Tissue Disease ILD
- prognosis is based on the CTD the person has
What two diseases have a prominent granulomatous pattern?
- Sarcoidosis
- Hypersensitivity Pneumonia
What is sarcoidosis? How does it present?
- Non caseating granulomata in various organs
- mostly in lungs or hilar lymph nodes
- Presents incidentally on radiopgraph or with dyspnea
What is this?
Schaumann bodies associated with Sarcoidosis
What is this?
Asteroid body associated with sarcoidosis
Clinical presentation of patients with Sarcoidosis?
- <40 yr old
- 10x higher in African Americans
- Elevated ACE levels
- Likely immune related etiology possible genetic presdisposition
What do patients die from with sarcoidosis?
- Pulmonary
- Cardiac
- Neurologic
What is Hypersensitivity Pneumonitis?
- Immune reaction to inhaled antigen
- pigeon breeders lung
- Farmers lung
- Hot tub lung
- Airway centered granulomata with associated lymphocytes
How do you diagnose Hypersensitivity Pneumonitis?
- Very detailed history
Why is IPF/UIP a poor prognosis?
The patients lungs become fibrotic
Why is COP a good prognosis?
Loose wisps of early fibroblast foci are poorly established so they easily melt away with steroids
Why are the granulomas in hypersensitivity pneumonia so ill defined and mixed with other inflammatory cells?
All of the inflammation ins a reaction to an inhaled substance
What is Desquamative Interstitial Pneumonia?
- Smokers in 4-5th decades
- Restrictive lung disease presentation
- Histology shows stuffed alveolar spaces of macrophages
Prognosis & treatment of DSIP?
- Good prognosis
- Treatment is to stop smoking and use corticosteroids
What is Respiratory Bronchiolitis Interstitial Lung Disease?
- Part of a spectrum with DSIP but less symptomatic and earlier seen in the 3rd-4th decades
How do you diagnose RB-ILD?
- CT scan
- Then biopsy which shows
- macrophages to lesser extent
- Peribronchiolar metaplasia
- fibrosis in adv cases
Langerhans Cell Histiocytosis, who gets it, what does histology show?
- Young smokers present with stellate lung lesions
- Progressive scarring leads to cysts
- Peripheral cysts may rupture presenting with pneumothorax
- Histology shows
- eosinophils
- Langerhans cells
- Fibrosis and cysts
What is pulmonary alveolar proteinosis? Tx?
- Impairment of surfactant metabolism due to defect of GM-CSF
- Leads to accumulation of surfactant proteins throughout alveoli and airspaces
- Tx with GM-CSF
What is happening?
Pulmonary alveolar proteinosis
occurs in setting of autoimmune disease 90% of time
What is Pneumoconiosis?
- Reaction by lungs to inhaled mineral or organic dust or vapors
- important to identify occupational exposures and air pollutions
What makes pneumoconoisis worse?
- Exposure is high and repetitive
- Size of particles is small (1-5 um allows particle to reach alveoli)
- Impaired ciliary clearance
Describe the spectrum of disease seen with coal workers pneumoconiosis?
- Anthracosis
- Coal macules/nodules
- Progressive massive fibrosis
- most people will not have progressive disease
What is Silicosis? Who gets it?
- Disease resulting from inhaled silicon dioxide
- seen in those who did mining/quarry work
- Concrete repair/demo
___ may progress to massive pulmonary fibrosis and has a 2 fold risk of developing cancer as opposed to CWP.
Silicosis may progress to massive pulmonary fibrosis and has a 2 fold risk of developing cancer as opposed to CWP.
What is the most deadly of the pneumoconiosis?
Asbestosis
Who is at risk for asbestosis?
- Insulation workers
- Shipyard workers (navy)
- Paper mill workers
- Oil/chemical refinery workers
How does asbestosis manifest?
- Pleura: fibrosis, effusions, mesothelioma
- Lung: interstitial fibrosis, carcinoma
- Extrapulmonary neoplasms
what is this?
- candle wax drippings on pleura histologically showing hyalinized collagen
- this is a pleural plaque formation in asbestosis
Mesothelioma is associated with ?
Asbestos exposure, this can occur decades after initial exposure
