Idiopathic Pulmonary Fibrosis Flashcards
What is the most common diagnosis among patients with interstitial lung disease
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
What are the demographics of idiopathic pulmonary fibrosis
- Median age 65
- Male smokers
- Chronic, progressive
- Irreversible fibrosis
- 3-9 cases per 100,00
What is idiopathic pulmonary fibrosis (IPF)
- Progressive interstitial fibrotic scarring from persistent inflammation
*loss of pulmonary function with a restrictive component - Exclusion of other known causes of interstitial lung disease
What is the duration of illness for IPF
> 3 months (longer)
What is the patho behind IPF
- Repeated lung injury
*abnormal wound healing
*genetic susceptibility - Fibrosis of the alveolar walls, eventually destroys multiple alveoli
- Scar contraction of respiratory bronchioles
- Radiographic
*honeycomb lung
What are the risk factors of IPF (genes)
- Genetics
*SFPT-C
*SFTP-A2
*ABCA3
*telomerase genes
What are the risk factors of IPF (acquired)
- Environmental
- Pollution
- Chronic micro aspiration
- Viral = HCV
What are the risk factors of IPF (repeated lung injury)
- Smoking
- Metal wood dust
- Hairdressing
- Farming
- GERD
What are the S/sx of IPF
- Insidious onset of unexplained exertional dyspnea
*gradual onset, progression - Persistent, non productive cough
*resistant to common therapies - Fatigue
- Weight loss
What are later signs of IPF
- Bibasilar inspiratory rales/crackles
- Clubbing, cyanosis
- Pulm HTN, cor pulmonale 85%
When can the diagnosis of IPF be made?
- Patients is over the age of 65
- Idiopathic disease by history and have inspiratory crackles on PE
- RLD on PFT
- Characteristics radiographic evidence of progressive fibrosis over several years
*including diffuse, patchy fibrosis and pleural-based honeycombing on HRCT
What are the diagnostic studies done?
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
What helps to confirm the diagnosis of IPF
Lung biopsy
*BAL = nonspecific
What is broncheoalveolar lavage (BAL) (limited role in IPF)
- 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
What are the different types of tissue biopsies
- Transbronchial biopsy
- Surgical lung biopsy
*if suspected IPF must obtain biopsy to diagnose
*helps to determine which patients will benefit from therapy
What is the transbronchial lung biopsy
- Via flexible bronchoscope
- Risks are low
- Small sample needed
- Up to 1/3 of unknown will have confirmed diagnosis made via this method
What is the surgical lung biopsy
- Standard for diagnosis of IPF
*preferred - Multiple biopsies, multiple sites
- 60 y/o without a diagnosis
What is the treatment for IPF
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
What are some supportive tx
- Oxygen
- Pulmonary rehab
- Smoking cessation
- Vaccination
- steroids
- PI for GERD
What is the only curative tx for IPF
Lung transplant
What is the prognosis of IPF
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
