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Clinical Medicine > Idiopathic Pulmonary Fibrosis > Flashcards

Idiopathic Pulmonary Fibrosis Flashcards

1
Q

What is the most common diagnosis among patients with interstitial lung disease

A

Diffuse interstitial pneumonia
*current work up includes to find a cause, if able before truly classifying it as idiopathic
*every attempt should be made to identify a specific disorder

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2
Q

What are the demographics of idiopathic pulmonary fibrosis

A
  1. Median age 65
  2. Male smokers
  3. Chronic, progressive
  4. Irreversible fibrosis
  5. 3-9 cases per 100,00
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3
Q

What is idiopathic pulmonary fibrosis (IPF)

A
  1. Progressive interstitial fibrotic scarring from persistent inflammation
    *loss of pulmonary function with a restrictive component
  2. Exclusion of other known causes of interstitial lung disease
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4
Q

What is the duration of illness for IPF

A

> 3 months (longer)

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5
Q

What is the patho behind IPF

A
  1. Repeated lung injury
    *abnormal wound healing
    *genetic susceptibility
  2. Fibrosis of the alveolar walls, eventually destroys multiple alveoli
  3. Scar contraction of respiratory bronchioles
  4. Radiographic
    *honeycomb lung
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6
Q

What are the risk factors of IPF (genes)

A
  1. Genetics
    *SFPT-C
    *SFTP-A2
    *ABCA3
    *telomerase genes
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7
Q

What are the risk factors of IPF (acquired)

A
  1. Environmental
  2. Pollution
  3. Chronic micro aspiration
  4. Viral = HCV
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8
Q

What are the risk factors of IPF (repeated lung injury)

A
  1. Smoking
  2. Metal wood dust
  3. Hairdressing
  4. Farming
  5. GERD
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9
Q

What are the S/sx of IPF

A
  1. Insidious onset of unexplained exertional dyspnea
    *gradual onset, progression
  2. Persistent, non productive cough
    *resistant to common therapies
  3. Fatigue
  4. Weight loss
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10
Q

What are later signs of IPF

A
  1. Bibasilar inspiratory rales/crackles
  2. Clubbing, cyanosis
  3. Pulm HTN, cor pulmonale 85%
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11
Q

When can the diagnosis of IPF be made?

A
  1. Patients is over the age of 65
  2. Idiopathic disease by history and have inspiratory crackles on PE
  3. RLD on PFT
  4. Characteristics radiographic evidence of progressive fibrosis over several years
    *including diffuse, patchy fibrosis and pleural-based honeycombing on HRCT
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12
Q

What are the diagnostic studies done?

A

They rarely yield specific diagnosis but may provide clues
1. PFT = RLD patterns
2. CXR = presence of lung infiltrate, diminished lung volume
3. CT = fibrotic abnormalities honey combing
*absence of ground-glass o pacification, micronodules, consolidation

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13
Q

What helps to confirm the diagnosis of IPF

A

Lung biopsy
*BAL = nonspecific

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14
Q

What is broncheoalveolar lavage (BAL) (limited role in IPF)

A
  1. Lavage of at least 100mL of sterile saline over alveoli, samples taken
    *in diffuse disease right middle lobe or lingual is lavaged
    *helpful in infectious or even malignant causes, although findings are not diagnostic
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15
Q

What are the different types of tissue biopsies

A
  1. Transbronchial biopsy
  2. Surgical lung biopsy
    *if suspected IPF must obtain biopsy to diagnose
    *helps to determine which patients will benefit from therapy
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16
Q

What is the transbronchial lung biopsy

A
  1. Via flexible bronchoscope
  2. Risks are low
  3. Small sample needed
  4. Up to 1/3 of unknown will have confirmed diagnosis made via this method
17
Q

What is the surgical lung biopsy

A
  1. Standard for diagnosis of IPF
    *preferred
  2. Multiple biopsies, multiple sites
  3. 60 y/o without a diagnosis
18
Q

What is the treatment for IPF

A

There is no evidence that any tx improves survival or QOL
1. Avoid further injury
2. Confirm the diagnosis of IPF
*misdiagnosis = inappropriate tx
3. Pirfenidone, nintedanib

19
Q

What are some supportive tx

A
  1. Oxygen
  2. Pulmonary rehab
  3. Smoking cessation
  4. Vaccination
  5. steroids
  6. PI for GERD
20
Q

What is the only curative tx for IPF

A

Lung transplant

21
Q

What is the prognosis of IPF

A

Many people view this worse than lung cancer diagnosis
*5 years survival 20%
1. Slow and steady decline with a median survival of 3-5 years after diagnosis

Clinical Medicine (164 decks)

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