Singh Pathology Respiratory Pathology #3

Question 1

What is the most common type of interstitial lung disease processes?

Answer 1

• Idiopathic pulmonary fibrosis
• This damages pulmonary tissue with waves of inflammatory injury leading to fibrosis

Question 2

What does Usual Interstitial Pneumonia consist of and what is it?

Answer 2

• Pathological diagnosis of IPF
• Consists of normal areas, inflammation, fibroblast foci and peripheral honeycombing

Question 3

What factors contribute to getting IPF?

Answer 3

• Environmental such as industrialized societies and smoking
• Genetics
• Increasing age

Question 4

Clinical findings in Idiopathic pulmonary fibrosis?

Answer 4

• Dyspnea
• Crackles (velcro like)
• Restrictive PFT
• Basilar infiltrates progressing to honeycomb pattern

Question 5

How can you differentiate emphysema honeycombing vs IPF?

Answer 5

thick areas between fibrosis on histology and grossly differentiate IPF from emphysema

Question 6

Prognosis of IPF? Therapy?

Answer 6

• Progressive- most patients die 3-5 yrs
• Therapies include
  
  • lung transplant
  • Tyrosine kinase inhibitors
  • TGF-B inhibitors

Question 7

What is Non specific Interstitial Pneumonia? What makes up the histology?

Answer 7

• Idiopathic pneumonia
• Unique histology
  
  • Uniform infiltrates and fibrosis
  • NO heterogeneity
  • NO fibroblast foci
  • NO granulomata

Question 8

Describe Cryptogenic Organizing Pneumonia.

Answer 8

• Superimposed on prior infection or inflammatory process
• Presents with a pneumonia like consolidation in 50-60s

Question 9

Why is COP a diagnosis of exclusion?

Answer 9

• Not an active infection, drug or toxin induced or related to CTD
• If you give steroids it clears up!
• Good prognosis with full recovery

Question 10

If a patient has RA, systemic sclerosis, or SLE and develops IPF, NSIP, or COP how do you diagnose these patients?

Answer 10

• Connective Tissue Disease ILD
  
  • prognosis is based on the CTD the person has

Question 11

What two diseases have a prominent granulomatous pattern?

Answer 11

• Sarcoidosis
• Hypersensitivity Pneumonia

Question 12

What is sarcoidosis? How does it present?

Answer 12

• Non caseating granulomata in various organs
• mostly in lungs or hilar lymph nodes
• Presents incidentally on radiopgraph or with dyspnea

Question 13

What is this?

Answer 13

Schaumann bodies associated with Sarcoidosis

Question 14

What is this?

Answer 14

Asteroid body associated with sarcoidosis

Question 15

Clinical presentation of patients with Sarcoidosis?

Answer 15

• <40 yr old
• 10x higher in African Americans
• Elevated ACE levels
• Likely immune related etiology possible genetic presdisposition

Question 16

What do patients die from with sarcoidosis?

Answer 16

• Pulmonary
• Cardiac
• Neurologic

Question 17

What is Hypersensitivity Pneumonitis?

Answer 17

• Immune reaction to inhaled antigen
  
  • pigeon breeders lung
  • Farmers lung
  • Hot tub lung
• Airway centered granulomata with associated lymphocytes

Question 18

How do you diagnose Hypersensitivity Pneumonitis?

Answer 18

• Very detailed history

Question 19

Why is IPF/UIP a poor prognosis?

Answer 19

The patients lungs become fibrotic

Question 20

Why is COP a good prognosis?

Answer 20

Loose wisps of early fibroblast foci are poorly established so they easily melt away with steroids

Question 21

Why are the granulomas in hypersensitivity pneumonia so ill defined and mixed with other inflammatory cells?

Answer 21

All of the inflammation ins a reaction to an inhaled substance

Question 22

What is Desquamative Interstitial Pneumonia?

Answer 22

• Smokers in 4-5th decades
• Restrictive lung disease presentation
• Histology shows stuffed alveolar spaces of macrophages

Question 23

Prognosis & treatment of DSIP?

Answer 23

• Good prognosis
• Treatment is to stop smoking and use corticosteroids

Question 24

What is Respiratory Bronchiolitis Interstitial Lung Disease?

Answer 24

• Part of a spectrum with DSIP but less symptomatic and earlier seen in the 3rd-4th decades

Question 25

How do you diagnose RB-ILD?

Answer 25

* CT scan * Then biopsy which shows * macrophages to lesser extent * Peribronchiolar metaplasia * fibrosis in adv cases

Question 26

Langerhans Cell Histiocytosis, who gets it, what does histology show?

Answer 26

* 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

Question 27

What is pulmonary alveolar proteinosis? Tx?

Answer 27

* Impairment of surfactant metabolism due to defect of GM-CSF * Leads to accumulation of surfactant proteins throughout alveoli and airspaces * Tx with GM-CSF

Question 28

What is happening?

Answer 28

Pulmonary alveolar proteinosis *occurs in setting of autoimmune disease 90% of time*

Question 29

What is Pneumoconiosis?

Answer 29

* Reaction by lungs to inhaled mineral or organic dust or vapors * important to identify occupational exposures and air pollutions

Question 30

What makes pneumoconoisis worse?

Answer 30

* Exposure is high and repetitive * Size of particles is small (1-5 um allows particle to reach alveoli) * Impaired ciliary clearance

Question 31

Describe the spectrum of disease seen with coal workers pneumoconiosis?

Answer 31

* Anthracosis * Coal macules/nodules * Progressive massive fibrosis * *most people will not have progressive disease*

Question 32

What is Silicosis? Who gets it?

Answer 32

* Disease resulting from inhaled silicon dioxide * seen in those who did mining/quarry work * Concrete repair/demo

Question 33

\_\_\_ may progress to massive pulmonary fibrosis and has a 2 fold risk of developing cancer as opposed to CWP.

Answer 33

**_Silicosis_** may progress to massive pulmonary fibrosis and has a 2 fold risk of developing cancer as opposed to CWP.

Question 34

What is the most deadly of the pneumoconiosis?

Answer 34

Asbestosis

Question 35

Who is at risk for asbestosis?

Answer 35

* Insulation workers * Shipyard workers (navy) * Paper mill workers * Oil/chemical refinery workers

Question 36

How does asbestosis manifest?

Answer 36

* Pleura: fibrosis, effusions, mesothelioma * Lung: interstitial fibrosis, carcinoma * Extrapulmonary neoplasms

Question 37

what is this?

Answer 37

* candle wax drippings on pleura histologically showing hyalinized collagen * this is a pleural plaque formation in asbestosis

Question 38

Mesothelioma is associated with ?

Answer 38

Asbestos exposure, this can occur decades after initial exposure