24 Interstitial lung diseases Flashcards

0
Q

Spepta parts

A

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

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1
Q

Interstitial lung disease

A

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

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2
Q

Types of interstitial lung diseases

A
Acute-
*ARDS
Chronic
*Fibrosing (pneumoconioses
*Granulomatous (sarcoidoses)
*Idiopathic interstitial pneumonia (IIP)
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3
Q

Interstitial disease pathogenesis

A

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

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4
Q

Factors that modulate fibrosis

A
Chronicity
defense effectiveness
injury extent
BM damage
Individual succeptability
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5
Q

Interstitial findings

A

Dry cough/dyspnea
Bibasalar crackles
bilateral reticulonodular infiltrates
cor polmonale

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6
Q

Pneumoconicoses

A

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

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7
Q

Pneumoconicosis pathologic principles

A
  • 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
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8
Q

Fibrogenic pneumoconicoses

A

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

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9
Q

Coal workers Pneumoconicosis

A

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

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10
Q

Anthrocosis

A

Asymptomatic with anthrocotic pigment in interstitum and lymph nodes

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11
Q

Simple CWP

A
  • Fibrous opacities <1cm
  • upper lobe distribution
  • Deposits of coal dust next to respiratory bronchioles
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12
Q

Complicated CWP

A
  • Opacities >1cm
  • can have central necroses
  • massive fibrosis/black lung
  • cor pulmonale
  • Caplan syndrome CWP with rheumatoid nodules
  • No increase in TB or cancer
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13
Q

Silicosis

A

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

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14
Q

Siliccosis microscopy

A

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

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15
Q

Asbestos types

A

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

16
Q

Asbestos disease

A
  • respiratory bronchioles and alveolar ducts
  • sources
  • insulation, roofing material, old building demolition
  • Histo
  • ferriginous bodies- fbers coated with protein and iron in macs
17
Q

Asbestos relatted diseases

A
  • 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
18
Q

Asbestos/ferrigenous bodies

A

Dumbell like structures within alveolar macs

turn blue with iron stain

19
Q

Berylliosis

A
  • Beryllium exposure- nuclear airspace industry
  • GRANULOMATOUS inflammation- can be mistaken for TB and sarcoidosis
  • complicattions
    • Cor pulmonale
    • Lung CA
20
Q

Sarcoidosis

A
  • 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
21
Q

Sarcoidosis path

A
  • Immune dysregulation
  • antigen ? >CD4 cells> Cytokines> Monocyte and hystocytic recruitment- Consolidation and granuloma formaion
  • DIAGNOSIS OF EXCLUSION must exclude infectious first.
22
Q

Sacoidosis signs

A
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
23
Q

Lab findings

A
  • ^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
24
Q

Sarcoidosis prognosis

A
  • many have variavle remission and relapse without tratment

- progressive interstitial fibrosis, cor pulmonale/death in 10%

25
Q

Hypersensitivity pneumonitis

A

*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

26
Q

Hypersensitivity pneumonitis types

A
  • 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”
27
Q

Hypersensitive pneumotitis

A
  • 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
28
Q

Idiopathi pulmonary fibrosis

A
  • 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”
29
Q

IIP imaging and morpho

A

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

30
Q

IIP prognosis

A
  • 50% 5 year prognosis
  • progressive fibrosis
  • cor pulmonale
31
Q

Collagen vascular disease

A

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
32
Q

Sarcoidosis histology

A

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

33
Q

SHIT FACED

A

Sarcoid
Hypersensitivity
Interstitial lung disease
Tumor/TB

Fungal
Asbestosis
Cancer/Collagen vascular disease
Eosinophilic granulomatous
Drugs
34
Q

Sarcoid stages on CXR

A

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

35
Q

ARDS treatment

A

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