Lecture 11.1: Interstitial Lung Disease Flashcards

1
Q

What are Interstitial Lung Diseases?

A

Heterogeneous group of diseases with similar
clinical presentations resulting in inflammation
and fibrosis of alveoli, distal airways and septal
interstitium of the lung

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2
Q

What are some known causes of ILD? (3)

A
  • Exposure history: HP, Asbestosis, silicosis etc.
  • Drugs: immunosuppressants, antibiotics, chemo
  • Connective Tissue disorders: RA, SSc, etc
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3
Q

What are some “unknown” causes of ILD (meaning why those diseases developed is unknown)? (2)

A
  • IPF
  • Sarcoidosis
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4
Q

What is the “Interstitial Space” in a Lung?

A

The ‘interstitial space’ is a potential space between alveolar cells and the capillary basement membrane, which is only apparent in disease states, when it may contain fibrous tissue,
cells or fluid

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5
Q

The alveolar wall consists of…? What cell type?

A
  • Type 1 Pneumocytes (provides surface for gas
    exchange)
  • Closely opposed to capillary endothelial cells
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6
Q

What is the Role of Type 2 Pneumocytes? (1 + 3)

A
  • Produce surfactant
  • Lowering surface tension at the air–liquid
    interface and thus preventing alveolar collapse
    at end-expiration
  • Interacting with and subsequent killing of
    pathogens or preventing their dissemination
  • Modulating immune responses
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7
Q

Most IDL are associated with extensive destruction and alteration of..? (3)

A
  • Alveoli
  • Airway Architecture
  • Vasculature
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8
Q

What parts of the Lungs are affected by ILD? (4)

A
  • Acini
  • Alveolar Lumen
  • Bronchiolar Lumen
  • Bronchioles
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9
Q

What cell types are affected in ILD? (5)

A
  • Epithelial Cells
  • Endothelial Cells
  • Mesenchymal Cells
  • Macrophages
  • Recruited Inflammatory Cells
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10
Q

Effects of Inflammation & Fibrosis

A
  • Leads to temporary or permanent changes in
    the interstitial tissues (development of fibrosis
    tissue)
  • Interfere with diffusion of gases
  • Change mechanics of breathing
  • Stiff Lungs - The development of fibrous tissue
    in the interstitium, makes lungs less compliant,
    to produce a ‘restrictive’ ventilatory defect
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11
Q

Clinical Presentation of Interstitial Lung Disease (5)

A
  • Progressive breathlessness
  • Reduced exercise tolerance
  • Dry cough
  • Extrapulmonary symptoms: Fever,
    joint pains, lethargy
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12
Q

Classification of ILD: Based on Aetiology (5)

A
  • Occupational
  • Treatment Related
  • Connective Tissue Disease
  • Immunological
  • Idiopathic
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13
Q

Classification of ILD Based on Aetiology: Occupational Causes (3)

A
  • Asbestosis
  • Silicosis
  • Coal Miners Pneumoconiosis
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14
Q

Classification of ILD Based on Aetiology: Treatment Related Causes (5)

A
  • Radiation
  • Methotrexate
  • Nitrofurantoin
  • Amiodarone
  • Chemotherapy
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15
Q

Classification of ILD Based on Aetiology: Connective Tissue Disease Causes (5)

A
  • Rheumatoid Arthritis
  • Systemic Lupus Erythematosus (SLE)
  • Polymyositis
  • Scleroderma
  • Sjogren’s
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16
Q

Classification of ILD Based on Aetiology: Immunological Causes (2)

A
  • Sarcoidosis
  • Hypersensitivity Pneumonitis
17
Q

Classification of ILD Based on Aetiology: Idiopathic (6)

A
  • CFA/IPF
  • UIP/NSIP
  • DIP
  • LIP
  • RB-ILD
  • BOOP
18
Q

Classification of ILD Based on Duration (3)

A

Acute (Days to Weeks Hx)
Subacute (Weeks to Months Hx)
Chronic (Months to Years Hx)

19
Q

Classification of ILD Based on Duration: Acute Causes (4)

A
  • AIP (Acute Interstitial Pneumonia)
  • Acute Eosinophilic Pneumonia
  • Acute HP (Hypersensitivity Pneumonitis)
  • COP (Cryptogenic Organising Pneumonia)
20
Q

Classification of ILD Based on Duration: Subacute Causes (5)

A
  • Sarcoidosis
  • Drug Induced
  • COP
  • CT –ILD
  • Alveolar
21
Q

Classification of ILD Based on Duration: Chronic Causes (3)

A
  • IPF (Idiopathic Pulmonary Fibrosis)
  • Sarcoidosis
  • Chronic HP
22
Q

Examples of Inhaled Inorganic Dust (4)

A
  • Silica
  • Asbestos
  • Carbon (e.g. carbon pneumoconiosis)
  • Metals (e.g. tin, aluminium, hard metal dust,
    iron)
23
Q

Examples of Inhaled Organic Dust (4)

A
  • Thermophilic fungi (farmers lung, Grain handlers
    lung, humidifier or air conditioners lung)
  • Bacteria (humidifiers lung)
  • True Fungi
  • Animal proteins (bird fanciers disease)
24
Q

Examples of Other Inhaled Sources (5)

A
  • Chemicals (e.g. synthetic fibres, Bakelite, vinyl
    chloride)
  • Gases (e.g. oxygen, oxides of nitrogen, chorine
    gas)
  • Fumes (e.g. oxides to metals such as zinc)
  • Vapours (e.g. (hydrocarbons, thermosetting
    resins, mercury)
  • Aerosols (e.g. oils, fats, pyrethrum)
25
Q

How does Pneumoconiosis (fibrosis) secondary to inhalation of mineral dusts occur? (5 Steps)

A
  • Exposure to inorganic dusts
  • Particles less than 7 mm across reach terminal
    bronchioles and alveolar ducts
  • Scavenged by macrophages
  • Eventually trigger release of mediators
  • Causing inflammation, tissue damage and
    scarring (fibrosis)
26
Q

What is Caplan’s Syndrome/RP? When does it occur?

A
  • Occurs in people with rheumatoid arthritis who
    have breathed in dust
  • Swelling (inflammation) and scarring of the
    lungs
27
Q

Simple Silicosis

A
  • Multiple small nodules, 2-5 mm in diameter
    accompanied by calcifications on CT
  • Hilar and mediastinal lymphadenopathy may
    precede the parenchymal lesions
  • Eggshell pattern of calcification of lymph nodes
    is common
28
Q

Complicated Silicosis

A
  • Aka progressive massive fibrosis
  • Develops through confluence of individual
    silicotic nodules
29
Q

What happens to Lungs in Asbestos Exposure?

A
  • Asbestos fibres breathed in and lodge in distal
    air spaces
  • Scavenged by macrophages