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Year 3: Medicine Block CARDIORESPIRATORY year 5 > Interstitial Lung Disease > Flashcards

Interstitial Lung Disease Flashcards

1
Q

What is some examples of interstitial lung disease?

A
  • Usual Interstitial Pneumonia (UIP)
  • Non-specific Interstitial Pneumonia (NSIP)
  • Extrinsic Allergic Alveolitis
  • Sarcoidosis
  • Several other conditions
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2
Q

What types of lung disease is Interstitial Lung disease?

A

Typically, restrictive lung diseases on pulmonary function tests

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3
Q

What is the commonest type of interstitial lung disease?

A

Commonest type is Idiopathic Pulmonary Fibrosis

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4
Q

What are investigations does for interstitial lung disease?

A
  • ANA – antinuclear antibody
  • ENA – extractable nuclear anitgens
  • Rh F – rheumatoid factor
  • ANCA - vasculitis
  • Anti-GBM – Goodpasture’s syndrome
  • ACE
  • IgG to serum precipitins (HIV)
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5
Q

What are examination features of Idiopathic Pulmonary Fibrosis?

A
  • Clubbing
  • Reduced chest expansion
  • Auscultation
    • ​Fine inspiratory crepitations (like pulling Velcro slowly)
    • Usually best heard basal / axillary areas
  • Cardiovascular – may be features of pulmonary hypertension
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6
Q

What causes Idiopathic Pulmonary Fibrosis in most cases?

A
  • Usually Interstitial Pneumonia
  • It is the most common histological finding
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7
Q

How does Usual Intertitial Pneumonia cause Idiopathic Pulmonary Fibrosis?

A
  • Wound healing mechanism in response to damage leads to over-production of fibroblasts and deposition of increased extracellular matrix in the interstitium with little inflammation
  • Structural integrity of lung parenchyma is therefore disrupted. There is loss of elasticity, ability ot perform gas exchange impaired leading to progressive respiratory failure
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8
Q

What factors can be involved in pathology of Idiopathic Pulmonary Fibrosis?

A
  • Inhalational
  • Viruses (EBV)
  • Drugs (methotrexate, nitrofurantoin, bleomycin)
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9
Q

What is the imaging of choice in Idiopathic Pulmonary Fibrosis?

A

High Resolution CT scan is the diagnostic imaging of Choice

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10
Q

What are findings shown on High Resolution CT scanning?

A
  • Basal Distribution: abnormalities more pronounced at the bases
  • Subpleural Reticulation: reticulation most evident in lung peripheries
  • Traction Bronchiectasis: distortion of normal lung architect by fibrosis pulling airways open and causing bronchiectasis
  • Honeycombing
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11
Q

What are some investigations for Idiopathic Pulmonary Fibrosis?

A
  • Respiratory function test shows restrictive pattern
  • Blood Test including antinuclear antibodies and rheumatoid factor to exclude autoimmune rheumatic disease
  • HRCT is imaging of Choice
  • Chest X-ray
  • Bronchoalveolar Lavage is only necessary if an infective or malignant cause is suspected.
  • Histological Confirmation necessary in some patients under VATS
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12
Q

How is Idiopathic Pulmonary Fibrosis managed?

A
  • Pirfenidone
  • Nintedanib
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13
Q

What is the cause of Extrinsic Allergic Alveolitis?

A
  • Inhalation of organic antigen to which the individual has been sensitised
  • One of the most common causes is farmer’s lung which can affect 9% of farmers in humid climates.
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14
Q

What is the pathogenesis of Extrinsic Allergic Alveolitis?

A
  • Allergic response which involves both cellular immunity and deposition of immune complexes causing foci of inflammation through activation via classival pathway can also lead to inflammation by directly activating the alternate complement pathway.
  • Leads to chronic inflammatory infiltrate, poorly defined interstitial granulomas, Interstitial fibrosis and honey comb change in chronic disease
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15
Q

What is the clinical presentation of Extrinsic Allergic Alveolitis?

A

ACUTE

  • Short period from exposure(4-8 hrs).
  • Usually reversible: spontaneously settle 1-3 days but can recur
  • Fever common

CHRONIC

  • Chronic exposure (months – years).
  • Less reversible.
  • Finger clubbing may be present and progression to irreversible fibrosis
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16
Q

What are investigations for Extrinsic Allergic Alveolitis?

A
  • HRCT show nodules with ground-glass opacity and evidence of airtrapping. Increased reticulation and honey comb change
  • Chest X-ray
  • Lung function tests
  • Precipitating Antibodies present in the serum
  • Bronchoalveolar Lavage shows a lymphocytosis.
  • Lung Biopsy demonstrate lymphocyte-rich infiltrate with varying degree of fibrosis.
17
Q

What differentiates Extrinsic Allergic Alveolitis from Sarcoidosis?

A

Low CD4:CD8 ratio can show HP to help differentiate from sarcoidosis.

18
Q

What can Extrinsic Allergic Alveolitis be referred to as?

A

Hypersensitivity Pneumonitis

19
Q

What is the management of Extrinsic Allergic Alveolitis?

A
  • Avoidance of inciting antigen.
  • Prednisolone can be given if symptoms still persist.
20
Q

What is Sarcoidosis?

A
  • Multisystem inflammatory condition of unknown cause leading to formation of granulomas
  • Commonly involves Resp system BUT can affect nearly all organs
  • 50% get spontaneous remission, others get progressive disease
  • Immunological response
21
Q

What is found in histology for Sarcoidosis?

A

Non-Caseating granulomas

22
Q

What are tests for Sarcoidosis?

A
  • PFTs: (obstructive until) fibrosis
  • CXR: 4 stages
  • Bloods: ACE, Calcium -Urinary Calcium
  • Cardiac involvement: ECG, 24 tape, ECHO, cardiac MRI
  • CT/MRI head: headaches in Neuro sarcoid
  • Transbronchial biopsy
  • Bronchoalveolar Lavage: Typically lymphocytosis with raise CD4:CD8 ratio
  • Lung function test
23
Q

What are the 4 stages on CXR for Sarcoidosis?

A

Stage 1: Bilateral hilar lymphadenopathy

Stage 2: Pulmonary Infiltrates with BHL

Stage 3: Pulmonary infiltrate without BHL

Stage 4: Fibrosis

24
Q

What are some clinical features of Sarcoidosis?

A
  • Constitutional: Fever, Weight Loss, Fatigue
  • Reticuloendothelial: Splenomegaly, Lymphadenopathy
  • Respiratory: Stage 1-4 pulmonary involvement, Cough, Dyspnea, Wheeze
  • Hepatic: Deranged LFTs, Hepatomegaly
  • Hypercalcaemia/hypercalciuria: Hypercalciuria, Nephrocalcinosis (may cause kidney injury), Calculi
  • Cutaneous: Erythema nodosum, Lupus pernio, Anterior Uveitis
  • Neurological: Cognitive dysfunction, Headache, Cranial nerve palsies, Mononeuritis multiplex, Peripheral neuropathy, Seizures
  • Cardiac: Arrhythmias, Heart block, Cardiomyopathy, Sudden death
25
Q

How is Sarcoidosis managed?

A
  • Prednisolone
  • Methotrexate
  • Azathioprine
  • Hydroxychlororquine
26
Q

What are general treatment principles for ILD?

A
  • Depends on underlying pathology
  • Occupational exposure – remove
  • Drug associated – avoid
  • Stop smoking - ?N-Acetylcysteine ?Immunosuppressant ?Pirfenidone
  • Transplantation
  • Treatment of infections (atypical)
  • Oxygen
  • MDT
  • Palliative care

Year 3: Medicine Block CARDIORESPIRATORY year 5 (28 decks)

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