Interstitial Lung Diseases - Rao Flashcards Preview

M2 Renal/Respiratory > Interstitial Lung Diseases - Rao > Flashcards

Flashcards in Interstitial Lung Diseases - Rao Deck (30):
1

  1. What types of cells are damaged in alveolitis?
  2. What pathologic change is mediated by the inflammatory response (i.e. cytokines) in alveolitis?
  3. How does this change affect lung function?

  1. Pneumocytes & endothelial cells
  2. Interstitial fibrosis
  3. Decreased lung compliance & elasticity, decreased expansion during inspiration

2

Describe each of the 4 Phases of intersitial lung disease.

  • Phase I: Acute alveolitis; parenchymal cell injury
  • Phase II: Chronic alveolitis; progressive alveoalr injury
  • Phase III: Derangement of collagen; fibrosis
  • Phase IV: End-stage lung disease

3

  1. How does interstitial lung disease typically present?
  2. What heart problem is associated with intersitial lung disease?
  3. What is seen on chest x-ray?

  1. Dry cough & dyspnea
  2. Cor pulmonale
  3. Bilateral reticulonodular infiltrates

4

Pneumoconiosis

  1. What causes all pneumoconioses?
  2. Name four causative agents of pneumoconiosis
  3. How common is it in terms of interstitial lung diseases?

Pneumoconiosis

  1. Interstitial lung disease in reponse to inhalation of mineral dusts, organic/inorganic particulates, or chemical fumes
  2. Agents:
    1. Coal dust / anthracotic pigment
    2. Silica
    3. Asbestos
    4. Beryllium
  3. Accounts for 25% of chronic interstitial lung disease

5

Pneumoconiosis

  1. What are some factors of particles that affect the development of pneumoconiosis?
  2. In terms of particle size, are smaller or larger particles worse?

Pneumoconiosis

  1. Particle Factors:
    • Amount of dust retained 
    • Size, Shape, & Buoyancy
    • Solubility and physiochemical reactivity
    • Additive effects of other irritants (e.g. smoking)
  2. Smaller is worse
    • 1-5µm particles only reach the bifurcation of respiratory bronchioles & alveolar ducts
    • <0.5µm particles reach alveoli & are phagocytosed by alveolar macrophages
      • Triggers cytokine release & inflammation, leading to fibrosis

6

Coal Worker's Pneumoconiosis (CWP)

  1. What is the causative agent?
  2. Where is the agent commonly found?
  3. What parts of the lung are affected?

Coal Worker's Pneumoconiosis (CWP)

  1. Coal dust / Anthracotic pigment
  2. Coal mines, urban centers, tobacco smoke
  3. Upper lobes and upper portions of lower lobes

7

Coal Worker's Pneumoconiosis (CWP)

  1. How can you differentiate morphologically between simple and complicated CWP?
  2. What further complications can arise from complicated CWP?
  3. What does CWP (simple or complex) not increase the risk of?

Coal Worker's Pneumoconiosis (CWP)

  1. Morphology
    • Simple:
      • Fibrous opacities < 1cm
    • Complicated:
      • Fibrous opacities > 1cm
      • Possibly central necrosis
      • Massive fibrosis
        • Crippling (Black Lung Disease)
  2. Complications:
    • Cor pulmonale
    • Caplan Syndrome (CWP + rheumatoid nodules in lung)
  3. CWP does not increase TB or cancer risk.

8

Silicosis

  1. What is the causative agent?
  2. Where is this agent commonly found?
  3. How common is silicosis among occupational diseases?

Silicosis

  1. Crystalline silicon dioxide (quartz)
  2. Foundries (metal casting), sandblasting, silica mines
  3. Most common occupational disease worldwide

9

Silicosis

  1. Describe the morphology.
  2. What complications / risks are associated with silicosis?

Silicosis

  1. Morphology:
    • Nodular opacities with concentric collagen layers
    • Polarizable quartz crystals can be seen
    • Egg-shell calcification in hilar lymph nodes
  2. Complications:
    • Cor pulmonale, Caplan sydrome
      • Like complicated CWP
    • Increased TB and cancer risk
      • Unlike CWP
      • Silicosis is only pneumoconiosis to increase TB risk. (Pathoma: It does so by preventing the formation of phagolysosomes in alveolar macrophages that ingest the silica, impairing the immune response.)

10

Asbestosis

What are the two forms of asbestos minerals? Name an example of each. Which is worse in terms of exposure?

Asbestosis

  1. Serpentine
    • e.g. chrysotile
    • curly & flexible
  2. Amphibole
    • e.g. crocidolite
    • straight & rigid
      • WORSE

11

Asbestosis

  1. Where do the asbestos minerals deposit within the lung?
  2. What are common sources of asbestos?

Asbestosis

  1. Far down, in the respiratory part of the lung
    • respiratory bronchioles, alveolar ducts, alveoli
  2. Sources:
    • Pipe insulation in old naval ships
    • Roofing material (> 20yrs ago)
    • Demolition of old buildings

12

Asbestosis

  1. Describe the morphology of asbestos deposition.
  2. What pathology indicates progression to actual asbestosis?

Asbestosis

  1. Morphology
    • Ferruginous bodies
      • Macrophages phagocytose asbestos fibers and coat them with ferritin (iron + protein)
    • Benign pleural plaques
  2. Asbestosis = Diffuse interstitial fibrosis

13

Asbestosis

  • Name four further complications of asbestosis
  • What does asbestosis not increase risk of?

Asbestosis

Complications:

  1. Bronchogenic carcinoma
    • + risk w/ smoking
  2. Mesothelioma
    • arises from mesothelial cells of pleura
    • decades after first exposure
  3. Cor pulmonale
  4. Caplan syndrome

No increased TB risk

14

Berylliosis

  1. What is the causative agent?
  2. What are common sources of this agent?
  3. Describe the morphology. What are important considerations for the differential diagnosis?
  4. Name two complications

Berylliosis

  1. Beryllium
  2. Nuclear & airspace industry
  3. Granulomatous inflammation
    • Must differentiate from TB and sarcoidosis
  4. Complications:
    • Cor pulmonale
    • Lung cancer

15

Pneumoconiosis - Review Slide

Describe and differentiate the complications of CWP, silicosis, asbestosis, and berylliosis. Specifically, which can cause:

  • Cor pulmonale?
  • Caplan syndrome?
  • TB?
  • Lung cancer?

  • CWP
    • Cor pulmonale
    • Caplan syndrome
    • No TB risk
    • No cancer risk
  • Silicosis
    • Cor pulmonale
    • Caplan syndrome
    • TB (only silicosis increases TB risk!)
    • cancer
  • Asbestosis
    • Cor pulmonale
    • Caplan syndrome
    • No TB
    • cancer
  • Berylliosis
    • Cor pulmonale
    • No Caplan syndrome
    • No TB
    • cancer

16

Sarcoidosis

  • What is the etiology?
  • Describe sarcoidosis and its pathology

Sarcoidosis

  • Unknown etiology
  • A multisystem granulomataous disease due to immune disregulation.
    • An unknown antigen causes activation of CD4 TH cells
      • Cytokine release
      • Recruitment of monocytes, etc.
      • non-necrotizing granuloma formation

17

Sarcoidosis

What organs are most often affected in sarcoid?

Sarcoidosis

  1. Lung (95-100%!)
  2. Lymph nodes (75-80%)
  3. Spleen (75%)
  4. Liver (50%)
  5. Skin (30%)

(Eyes, Joints, Nervous system, and Myocardium can also be affected)

18

Sarcoidosis

  1. What skin findings are there?

Sarcoidosis

  1. Skin:
    • Nodular granulomatous lesions
    • Erythema nodosum
    • Lupus pernio
      • Purplish, chronic, raised, hardened skin lesion from granulomas
      • Pathognomonic for sarcoidosis
      • Not actually a type of lupus, but can be useful to think of - somewhat resembles the lupus butterfly rash

19

Sarcoidosis

  1. What eye findings are there?
  2. Liver findings?
  3. Other notable findings?

Sarcoidosis

  1. Eye: uveitis
  2. Liver: granulomatous hepatisis
  3. Other:
    • Enlarged salivary / lacrimal glands
    • Diabetes insipidus

20

Sarcoidosis

  • Name two lab findings. Which is used as a disease marker?
  • Name two chest x-ray findings.
  • How would a person with sarcoid respond to common skin antigens, like candida?

Sarcoidosis

  • Lab:
    1. Increased ACE levels
      • Marker of disease activity and treatment response
    2. Polyclonal gammopathy
  • CXR:
    1. Bilateral hilar adenopathy
    2. Reticulonodular shadows in lungs
  • Cutaneous anergy
    • Consumption of CD4 TH cells prevents response to skin antigens

21

Sarcoidosis

How are the granulomatous lesions of sarcoid arragned within the lungs?

Sarcoidosis

Lymphatic distribution - lesions often follow the lymphatic vessels

22

Sarcoidosis

As a granulomatous disease, what microscopic finding can you expect to see?

Sarcoidosis

  • Epitheloid Macrophages
    • Macrophages resembling epithelial cells
    • Which can fuse to become:
  • Langhans' Giant Cells

 

23

Sarcoidosis

  1. What's the prognosis?
  2. What major complications are seen?

Sarcoidosis

  1. Prognosis variable with spontaneous remissions and relapses
  2. Complications:
    • Progressive interstitial fibrosis
      • Cor pulmonale
      • Death

24

Hypersensitivity Pneumonitis (HSP)

  1. What is the etiology?
  2. Describe the acute pathology and hypersensitivty type
  3. Describe the chronic pathology and hypersensitivty type

Hypersensitivity Pneumonitis (HSP)

  1. Several types - known or unknown inhaled antigen causing granulomatous interstitial pneumonitis
    • ("extrinsic allergic alveolitis")
    • "pigeon breeder's lung" - Pathoma
  2. Acute:
    • First exposure produces IgG Abs
    • Second exposure causes Type III HSR (immune complex deposition)
  3. Chronic:
    • T-cell activation resulting in gramuloma formation: Type IV HSR

25

HSP

Name three notable types of hypersensitivity pneomonitis and the antigens that cause them

HSP

  1. Farmer's Lung
    • Ag is a thermophyllic actinomycetes bacteria, saccharopolyspora rectivirgula
      • Found in moldy hay
  2. Silo Filler's Disease
    • Ag is gases from plant material (oxides of nitrogen)
  3. Byssinosis
    • Ag is cotton, linen, or hemp
      • Textile factory workers
      • "Mondar morning blues" - resurgence of sxs after a weekend away from the job

26

What tests and findings are used to diagnose HSP?

Diagnosis is both clinical and pathological

  • Symptoms & physical exam findings
  • X-ray
  • PFTs
  • Immunologic findings
    • Abs to suspected Ags
  • Lung biopsy if required

27

Idiopathic Pulmonary Fibrosis

  1. Describe this condition broadly.
  2. How serious is it?
  3. How common is it among interstitial lung diseases?
  4. What demographics are at highest risk?

Idiopathic Pulmonary Fibrosis

  1. IPF is an broad term encompassing a number of idiopathic interstitial pneumonias
  2. Very Serious: The 5-year survival for IPF ranges between 20–40% (Wiki)
  3. 15% of chronic interstitial lung disease
  4. M > F, 40-70 years

28

Idiopathic Pulmonary Fibrosis

  1. How does IPF present?
  2. Describe the pathogenesis and the main end-stage finding of the disease

​Idiopathic Pulmonary Fibrosis

  1. Presentation:
    • Dyspnea
    • Nonspecific constitutional sxs:
      • fever
      • weight loss
      • fatigue
      • arthralgias
      • cough
  2. Pathogenesis:

Repeated lung injury → alveolitis → cytokine release → interstitial fibrosis → dilitation of adjacent airways → honeycomb lung (end stage fibrosis) leading to cor pulmonale

29

Idiopathic Pulmonary Fibrosis

Describe the gross and microscopic morphology of the lung in IPF

Idiopathic Pulmonary Fibrosis

  • Gross:
    • Honeycomb cysts
    • Appears to initially affect the lower portions of the lung (?)
    • Later, it looks to become pretty diffuse
  • Microscopic
    • Honeycomb cysts (also visible microscopically)
    • Normal and abnormal areas giving patchwork appearance
    • Fibroplastic plug

30

Collagen Vascular Disease

Name three types of collagen vascular disease and the lung findings associated with each of them

Collagen Vascular Disease

  1. SLE
    • 50% of SLE pts get interstitial lung disease!
    • pleural effusion
      • unexplained pleural effusion in young woman likely indicates SLE
  2. RA
    • Rheumatoid nodules
      • Caplan sydrome when associated with pneumoconiosis
    • Interstitial fibrosis
    • Pleural effusions
  3. Scleroderma (systemic sclerosis)
    • Interstitial fibrosis
    • Pulmonary vascular hypertrophy
    • Typically the cause of death in scleroderma pts

Decks in M2 Renal/Respiratory Class (50):