24 Interstitial lung diseases

Question 0

Spepta parts

Answer 0

90% Type I pneumocytes
5-10% Type II pneumocytes
Capillary endothelial cells
Ground substance

Question 1

Interstitial lung disease

Answer 1

Parenchymal disorders that involve the lung interstitium

• Infiltrative diseae
• Diffuse parenchymal lung diseease
• Restrictive lung disease- reduced total capacity 2/2 disease changes

can involve alveolar spaces, septa, and vessels

Question 2

Types of interstitial lung diseases

Answer 2

Acute-
*ARDS
Chronic
*Fibrosing (pneumoconioses
*Granulomatous (sarcoidoses)
*Idiopathic interstitial pneumonia (IIP)

Question 3

Interstitial disease pathogenesis

Answer 3

1) alveolitis- damage to pneumoctes and endothelial
2) Leukocytes> cytokines causing fibrosis
3) fibrosis leads to decr. compliance and expansion

Question 4

Factors that modulate fibrosis

Answer 4

Chronicity
defense effectiveness
injury extent
BM damage
Individual succeptability

Question 5

Interstitial findings

Answer 5

Dry cough/dyspnea
Bibasalar crackles
bilateral reticulonodular infiltrates
cor polmonale

Question 6

Pneumoconicoses

Answer 6

Non neoplastic lung diseeease caused by:
* mineral dust, organic and inorganic particulates/ fumes
account for 25% of interstitial lung disease5

Question 7

Pneumoconicosis pathologic principles

Answer 7

• 1-5um particles dont get past respiratory bronchiole bifurcations
• Smaller reach alveoli and alveolar macs- are phagocytosed
• some are reactive/irritative in and of themselves

Question 8

Fibrogenic pneumoconicoses

Answer 8

Asbestos
Silicosis
Coal Worker’s pneumconicosis
(berylliosis, thesaurosis-hairspray, etc)

Question 9

Coal workers Pneumoconicosis

Answer 9

Anthracoticpigment- Coal mine, urban cneters, tobacco smoke
3 forms
-Anthrocosis- assymptomatic with pigment in interstitium and lymph
-Simple
-complicated

Question 10

Anthrocosis

Answer 10

Asymptomatic with anthrocotic pigment in interstitum and lymph nodes

Question 11

Simple CWP

Answer 11

• Fibrous opacities <1cm
• upper lobe distribution
• Deposits of coal dust next to respiratory bronchioles

Question 12

Complicated CWP

Answer 12

• Opacities >1cm
• can have central necroses
• massive fibrosis/black lung
• cor pulmonale
• Caplan syndrome CWP with rheumatoid nodules
• No increase in TB or cancer

Question 13

Silicosis

Answer 13

Most common occupational disease worldwide
* Quartz crystals engulfed by alveolar macs-> Cytokine induced fibrosis
Morphology
* Nodular opacities with concentric collagen rings *ROPY AND LAMELLAR COLLAGEN
*polarizable quartz particles (shine under light in microscope)
*“Egg shell” calcification in hilar lymph nodes
Complications
*cor pulmonale, Caplan syndrome
*CANCER
*TB

Question 14

Siliccosis microscopy

Answer 14

Ropey/ lamellar collagen deposition around quartz particles (polarized light reactive)

Question 15

Asbestos types

Answer 15

Serpetine asbestos
* Curly snake like and flexable fibers - less damaging
Amphibole asbestos
*Straight rigid, friable fibers - can cause bad lung disease and can reach distal lung

Question 16

Asbestos disease

Answer 16

• respiratory bronchioles and alveolar ducts
• sources
• insulation, roofing material, old building demolition
• Histo
• ferriginous bodies- fbers coated with protein and iron in macs

Question 17

Asbestos relatted diseases

Answer 17

• Benign pleural plaques - frequnetly on membanous part of diapraghm with pearly nodules, Thickening of pleural area with acellular collagen
• Asbestosis- diffuse interstitial fibrosis
• Bronchogenic carcinoma (20 years later- related to smoking)*
• Mesothelioma
• No risk TB
• Cor pulmonale
• caplan syndrome

Question 18

Asbestos/ferrigenous bodies

Answer 18

Dumbell like structures within alveolar macs

turn blue with iron stain

Question 19

Berylliosis

Answer 19

• Beryllium exposure- nuclear airspace industry
• GRANULOMATOUS inflammation- can be mistaken for TB and sarcoidosis
• complicattions
  
  • Cor pulmonale
  • Lung CA

Question 20

Sarcoidosis

Answer 20

• Multisystem granulomatous disease
• 25% of chronic interstitial lung diseeases
• AA:W 10:1, M:F 1:2, 70% less than 40yrs, nonsmokers
• 95-100% with lung involvement
• 80% with lymph incolvement

Question 21

Sarcoidosis path

Answer 21

• Immune dysregulation
• antigen ? >CD4 cells> Cytokines> Monocyte and hystocytic recruitment- Consolidation and granuloma formaion
• DIAGNOSIS OF EXCLUSION must exclude infectious first.

Question 22

Sacoidosis signs

Answer 22

skin
-Nodular granulomatous lesions
-lupis pernio
-erythema nodosum
eyes
-uveitis
Liver
-Graulomatous hepatitis
Other
-Enlarged salvitory and lacimal glands
-diabetes insipidus
-BM and spleen involvment

Question 23

Lab findings

Answer 23

• ^ACE levels- marker of prognosis and disease response
• hypercalcemia
• increased gammaglobins
• cutaneous aergy- doesnt react to skin antigens (22 cd4 consumption)
• CXR- Bilateral hylar adenopathy, bilateral reticulodular infiltrates

Question 24

Sarcoidosis prognosis

Answer 24

- many have variavle remission and relapse without tratment | - progressive interstitial fibrosis, cor pulmonale/death in 10%

Question 25

Hypersensitivity pneumonitis

Answer 25

*Inhaled antigen causing granulomatous interstitial lung disease Path *Thype III hypersensitivity -first exposure- sensitization -second exposure - immune complex formation and inflammitry rsponse in lung -chronic exposure causes IV hypersensitiity and granuloma formation

Question 26

Hypersensitivity pneumonitis types

Answer 26

* Farmers lung- Actinomycetes * Silo fillers’ disease - Inhalation of gases from plant material (oxides of nitrogen) * Byssinosis - Cotton, linen, hemp - Textile factory workers - “Monday morning blues”

Question 27

Hypersensitive pneumotitis

Answer 27

* acute, subacute, chronic * interstital and aveolar infiltrates of inflammator cells, peribronchial accentuation, ill defined granulomas * GRANULOMAS MELD WITH PARENCHYMA * diagnosis - symptoms and CXR, PFTS, Ab reactivity, lung biopsy

Question 28

Idiopathi pulmonary fibrosis

Answer 28

* USUAL INTERSTIAL PNEUMONIA * 15% interstitial lung disease cases * M>F, 40-70 years * 18-24month duration * progressing dyspnea, many nonspecific symptoms * idiopathic repeated injury to lung causes alveolitis and fbrosis * intestitial fibrosis leading to "honeycombing of the lung"

Question 29

IIP imaging and morpho

Answer 29

CXR- increase lower bronchiole markings CT- Honeycombing appearance Gross- Thick septa and honeycomb type cysts

Question 30

IIP prognosis

Answer 30

* 50% 5 year prognosis * progressive fibrosis * cor pulmonale

Question 31

Collagen vascular disease

Answer 31

10% of interstitial lung disease * SLE- (unexplained pleural effusion in young woman) * RA- fibrosis and pleural effusions with rheumatoid nodules * Systemic sclerosis- commonest cause of death inscleroderma patients

Question 32

Sarcoidosis histology

Answer 32

Interstitial granulomas NON NECROTIZING Around bronchovascular bundles Follows Lumph tract Giant cells

Question 33

SHIT FACED

Answer 33

Sarcoid Hypersensitivity Interstitial lung disease Tumor/TB ``` Fungal Asbestosis Cancer/Collagen vascular disease Eosinophilic granulomatous Drugs ```

Question 34

Sarcoid stages on CXR

Answer 34

1) Huge hilar lymph nodes 2) hilar nodes and bronchial markings 3) Diffuse bronchial marking no nodes 4) Total fibrosis and scarring

Question 35

ARDS treatment

Answer 35

Small tidal volumes Peep-16 (doesn't matter) Less water given leads to quicker removal from vent Prone positioning for severe ARDS