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
What is the suspected lag b/t asbestos exposure and the development of mesothelioma?
20 yr lag
What are the two arrows pointing at?
Silica particles
A multisystem dz that leads to the formation of noncaseating granulomas.
Sarcoidosis
What are some components of the pathogenesis of sarcoidosis?
Cell-mediated immune response to unknown Ag
CD4 Th
Increased TNF
HLA-A1 and HLA-B8 (genetic factors)
Environment
Name that condition.
Sarcoidosis
What might these be?
Noncaseating granulomas of sarcoidosis
What are the the race and gender specificities of sarcoidosis?
Women > men
AA > European descent
What is the treatment for sarcoidosis?
STEROIDS
Spectrum of immunologically mediated, predominatly interstitial, lung disorders caused by intense, often prolonged exposure to inhaled organic Ag
Hypersensitivity pneumonitis
What does she got, doc?
Hypersensitivity pneumonitis
What are some syndromes that are classified under hypersensitivity pneumonitis?
Bird fancier’s dz (bird stuff)
Farmer’s lung (moldy hay)
Bagassosis (moldy sugar cane)
Humidifer lung (thermophilic bacteria)
Interstitial lung dz w/ proliferation of Langerhans cells resulting in nodules.
Pulmonary Langerhans cell histiocytosis/eosinophilic granuloma
What are the lower set of arrows pointing at?
Langerhans cells
Rare dz characterized by bilateral patchy asymmetric pulmonary opacification on imaging
Pulmonary alveolar proteinosis
Name that conditioin.
Respiratory Bronchitis or Desquamative interstitial pneumonia
