2.2.1 Restrictive Pulmonary Diseases Flashcards
What results on a PFT would lead you to believe a patient was suffering from a restrictive lung dz?
Decreased TLC
Normal FEV1/FVC (both proportionally reduced, if anything this might be increased)
What are some possible pulmonary or extrapulmonary causes of restrictive lung dz?
Effective loss of lung tissue
Decrease in lung’s ability to expand (compliance)
Decrease in gas exchange
Restrictive pulmonary dz is characterized by reduced TLC. If the cause of this dz is pulmonary, this is referred to as what type of dz?
Interstitial lung dz
What are some conditions that result in extrapulmonary restriction of the lungs?
Obesity, Pleural dz, Kyphoscoliosis, Neuromuscular dz
What are the lungs’ responses to the different severities of injury (minor, moderate, severe)?
Minor: emphysema
Moderate: interstitial fibrosis
Sever: scar
What are interstitial pneumonais?
Group of inflammatory and fibrosing disorders affecting the alveolar septae and other supporting structures of the lung rather than the airspaces.
What are the five categories of interstitial lung dz?
Fibrosing
Granulomatous
Eosinophilic
Smoking-related
Other
Name some conditions that are classified as fibrosing interstitial lung dz’s?
Idiopathic pulmonary fibrosis (UIP)
Non-specific interstitial pneumonia (NSIP)
Cryptogenic Organizing Pneumonia (COP or BOOP)
Drug
Connective Tissue Dz
Pneumoconiosis
What are two types of granulomatous interstitial lung dz’s?
Sarcoidosis and hypersensitivity pneumonitis
Which two conditions fall into the other category of interstitial lung dz’s?
Pulmonary alveolar proteinosis and Langerhan’s cell histiocytosis/eosinophilc granuloma
What are some common physiological manifestations (3) of restrictive pulmonary dz?
Reduced CO diffusion capacity
Reduced lung volume
Reduced compliance
Describe the clinical symptoms that are associated with the physiological changes in restrictive pulmonary dz.
What is the typical histiologic pattern of IPF?
Usual interstitial pneumonia (UIP)
Insidious onset (slow, progessive), 55-75 years at presentation, dyspnea and dry cough, subsequent hypoxemia, cyanosis, and clubbing.
These are all chacterisitics of which restrictive pulmonary dz?
Idiopathic pulmonary fibrosis (IPF)
What are two characterisitics of the histological pattern usual interstitial pneumonia?
Spatially and temporally heterogenous
Subpleural and interlobular septal distribution
What are the arrows pointing at?
Fibroblastic foci
What might this patient have?
IPF
What is the median survival time of IPF patients?
3 years
Characteristic histopathologic lesions are polypoid plugs of loose organizing connective tissue within small airways, alveolar ducts, and the surrounding alveoli
Cryptogenic organizing pneumonia (COP), formerly known as bronchiolitis obliterans-organizing pneumonia (BOOP)
Cryptogenic Organizing Pneumonia
Non-neoplastic reaction of the lung to inhaled mineral dust, organic particles, or chemical vapors
Pneumonconiosis
What are three examples of pneumoconiosis?
Asbestosis, Silicosis, Coal Workers Pneumoconiosis
What are some industries that could expose an individual to abestos?
mining, milling, and fabrication
How does asbestos affect the risk for lung cancer? abestos and a smoker?
asbestos: 5 fold increase
asbestos + smoker: 55 fold increase
