Respiratory Path 3 - Galbraith Flashcards
Inflammation and pulmonary interstitial tissue fibrosis, especially in the alveolar walls
Chronic Diffuse Interstitial (Restrictive) Disease
Interstitial fibrosis leads to what 3 things?
- Low diffusing capacity
- Low lung volume
- Low lung compliance
Honeycomb lung
Advanced stage extensive scarring in any of the restrictive lung disorders
Progressive interstitial fibrosis and respiratory failure, unknown cause
Idiopathic pulmonary fibrosis
Idiopathic pulmonary fibrosis is THOUGHT to occur secondary to _____
Repeated alveolar epithelial injury w/ pro-fibrotic response
Causes of repeated alveolar epithelial injury in idiopathic pulmonary fibrosis
- Tobacco smoke
- Fumes, dusts
- Viruses
- Persistent GI reflux
TERT, TERC mutations
Telomerase mutations that make for susceptibility to idiopathic pulmonary fibrosis
Molecular cause for the repetitive fibrosis reactions
TGF-beta 1
- Patchy interstitial fibrosis
- New fibroblastic foci mixed with more densely fibrotic areas
Usual interstitial pneumonia (UIP) - Morphologic pattern seen in idiopathic pulmonary fibrosis
Spaces surrounded by dense collagen, lined with hyperplastic type 2 pneumocytes and bronchial epithelium
Honeycomb fibrosis - common result of restrictive lung diseases
In IPF, the fibrosis is usually in which 2 locations?
- Subpleural
- Interlobular septal
Sign of an early UIP lesion in IPF
Cellular fibroblastic foci
- Gradually worsening dyspnea with exertion
- Dry cough
- Late development of progressive hypoxemia, cyanosis, and clubbing
- Smoker, 40-70
- Microscopic evidence of fibrosis
Idiopathic pulmonary fibrosis
Median survival after diagnosis of IPF is ____
Definitive treatment for IPF
Lung transplant
How are the microscopic findings in Nonspecific Interstitial Pneumonia (NSIP) different than those in UIP?
Develops as EITHER interstitial chronic inflammation OR interstitial fibrosis, NO heterogeneity
Typical person to get NSIP
Women, 50s, not a smoker, presents w/ dyspnea and cough
Tufts/balls/plugs of immature fibroblastic tissue found within terminal bronchioles, alveolar ducts, and alveolar spaces
Characteristic of _____
Masson bodies
Cryptogenic organizing pneumonia (COP)
- Cough and dyspnea
- Patchy subpleural or peribronchial consolidation
- NO INTERSTITIAL FIBROSIS
- NO HONEYCOMBING
- Clusters of fibroblasts/fibrous tissue in terminal airways
- Normal underlying tissue
Cryptogenic organizing pneumonia (COP)
Most cases of COP require ___ for treatment
Steroid therapy
Pneumoconioses - general definition
Lung diseases due to chronic responses to inhaled aerosols (mineral dusts, organic dusts, chemical fumes, vapors)
Pathogenicity of Pneumoconiosis is determined by what 4 things?
- Particle size (smaller = worse)
- Particle solubility
- Level and duration of exposure
- Intensity of immune response
Black pigmented lesions full of dark macrophages within lymphatic tissues
Anthracosis - ASYMPTOMATIC coal worker’s pneumoconiosis
Macules/nodules of dark macrophages AND collagen networks, located adjacent to respiratory bronchioles
Simple Coal Worker’s Pneumoconiosis (CWP)
Multiple anthracotic (dark) scars w/ central ischemic necrosis replacing lung parenchyma
Progressive massive fibrosis (PMF) - advanced coal workers pneumoconiosis
3 complications of PMF
- Respiratory failure
- Pulmonary HTN
- Cor pulmonale
Anthracosis can OCCASIONALLY cause what?
Centriacinar emphysema
Anthracosis can OCCASIONALLY cause what?
Centriacinar emphysema
Interstitial fibrosis leads to what 2 things?
- Low diffusing capacity
- Low lung volume
- Low lung compliance
Honeycomb lung
Advanced stage extensive scarring in any of the restrictive lung disorders
Progressive interstitial fibrosis and respiratory failure, unknown cause
Idiopathic pulmonary fibrosis
Idiopathic pulmonary fibrosis is THOUGHT to occur secondary to _____
Repeated alveolar epithelial injury w/ pro-fibrotic response
Causes of repeated alveolar epithelial injury in idiopathic pulmonary fibrosis
- Tobacco smoke
- Fumes, dusts
- Viruses
- Persistent GI reflux
TERT, TERC mutations
Telomerase mutations that make for susceptibility to idiopathic pulmonary fibrosis
Molecular cause for the repetitive fibrosis reactions
TGF-beta 1
- Patchy interstitial fibrosis
- New fibroblastic foci mixed with more densely fibrotic areas
Usual interstitial pneumonia (UIP) - Morphologic pattern seen in idiopathic pulmonary fibrosis
Spaces surrounded by dense collagen, lined with hyperplastic type 2 pneumocytes and bronchial epithelium
Honeycomb fibrosis - common result of restrictive lung diseases