• Shuffle
    Toggle On
    Toggle Off
  • Alphabetize
    Toggle On
    Toggle Off
  • Front First
    Toggle On
    Toggle Off
  • Both Sides
    Toggle On
    Toggle Off
  • Read
    Toggle On
    Toggle Off
Reading...
Front

Card Range To Study

through

image

Play button

image

Play button

image

Progress

1/51

Click to flip

Use LEFT and RIGHT arrow keys to navigate between flashcards;

Use UP and DOWN arrow keys to flip the card;

H to show hint;

A reads text to speech;

51 Cards in this Set

  • Front
  • Back
Normal lung
-Interstitial lung disease
-Non-infectious disease
- There is a lot of pink staining collagen (scar tissue/fibrosis). The blue dots are inflammatory cells. They are mostly lymphocytes.
How are interstitial lung diseases defined?
By appearance and not by their causes
Define diffuse parenchymal lung disease (DPLD)
-A group of non-infectious, non-neoplastic lung diseases each characterized by varying degrees of inflammation and/or fibrosis of the parenchyma of both lungs
-Also called interstitial lung disease
Define interstitial lung disease
-A group of non-infectious, non-neoplastic lung diseases each characterized by varying degrees of inflammation and/or fibrosis of the parenchyma of both lungs
-Also called diffuse parenchymal lung disease
Define idiopathic interstitial pneumonias (IIPs)
A group of 7 ILDs of unknown cause
Define idiopathic pulmonary fibrosis
The most common IIP
Define pulmonary fibrosis
Non-specific term denoting bilateral parenchymal fibrosis
What are the 4 categories of DPLDs/ILDs
-ILD of known cause
-Idiopathic interstitial pneumonias (IIPs)
-Granulomatous ILDs (e.g., sarcoidosis)
-Other forms of ILD
How is diagnosis of ILD made?
Often by a combination of clinical presentation, CT findings, and histology
What are the known causes of ILD?
-Drugs (chemotherapy, antibiotics)
-Radiation therapy
-Connective tissue diseases
-Rheumatoid arthritis
-Systemic sclerosis (scleroderma)
-Dermatomyosisis
-Occupational/Environmental
-Inorganic antigens (Pneumoconioses)
-Asbestos
-Coal worker's pneumoconiosis
-Silicosis
-Organic antigen (hypersensitivity pneumonitis)
Describe the ILD from drugs
Each drug causes a different pattern of disease in the lung, both radiographically and histologically.
-Usual interstitial pneumonia pattern of IPF (idiopathic pulmonary fibrosis)
-Normal looking alveolar spaces
-Under the pleura is a pink (scar tissue) pattern
-There is subpleura fibrosis in a peripheral lobular pattern.
-Peripheral fibrosis with sparing of the middle
Describe the fibrosis in UIP (usual interstitial pneumonia)
-Occurs at the periphery of the lobule
-From idiopathic pulmonary fibrosis
-It occurs subpleurally and occurs at the lung bases.
What is the histologic pattern associated with idiopathic pulmonary fibrosis (IPF)?
Usual interstitial pneumonia (UIP)
Where is UIP seen?
-Idiopathic pulmonary fibrosis
-Rheumatoid arthritis
-Other inflammatory diseases that heal up with this abnormal fibrotic pattern
-Higher power image of Usual interstitial pneumonia
-Little collections of myofibroblasts called fibroblastic foci present
-These lay down collagen in the lung and are located at the interface between scar
-Cellular Non-specific interstitial pneumonia
-Lots of inflammatory cells present
-Seems to resemble an autoimmune disease
-Fibrotic Non-specific interstitial pneumonia
-Lots of fibrosis
-Seems to resemble an autoimmune disease
What is the histologic pattern of non-specific interstitial pneumonia?
Non-specific interstitial pneumonia
What is the histologic pattern of cryptogenic organizing pneumonia?
Organizing pneumonia
What is the histologic pattern of acute interstitial pneumonia?
Diffuse alveolar damage
What is the histologic pattern of respiratory bronchiolitis-ILD?
Respiratory bronchiolitis
What is the histologic pattern of Desquamative interstitial pneumonia?
Desquamative interstitial pneumonia
What is the histologic pattern of lymphoid interstitial pneumonia?
Lymphoid interstitial pneumonia
-Cryptogenic organizing pneumonia
-Growth of tufts of loose connective tissue growing into alveolar spaces and the spaces of small airways
-Sometimes asteroid responsive disease
-Organizing pneumonia seen in many known diseases as well
-RB-ILD
-Smoking related
-Inflammation of small airways with macrophage accumulation
-Can have parenchymal inflammation and parenchymal fibrosis
-Corticosteroids help in some cases
-RB-ILD
-Smoking related
-Macrophage accumulation in alveolar spaces
-Can have parenchymal inflammation and parenchymal fibrosis
-Corticosteroids help in some cases
Describe eosinophilic pneumonia
-Eosinophilic pneumonia is a pathological entity with eosinophilic infiltration of the lung.
-Sometimes it is in the setting of organizing pneumonia.
-Drug reactions, parasitic infections can cause eosinophilic pneumonia.
-It is sometimes steroid responsive.
Describe lymphangioleiomyomatosis
-LAM is characterized by smooth muscle hyperplasia
-There is cystic changes in the lung instead of inflammatory or fibrotic.
-It is a progressive disease leading to airflow obstruction, hemoptysis, pleural effusions.
Describe Langerhan's cell histiocytosis
Langerhan's cell histiocytosis is smoking related and occurs in younger men.
What diseases involve the lung parenchyma?
-Alveolar filling diseases
-Pulmonary edema
-Acute respiratory distress syndrome (ARDS)
-Alveolar proteinosis
-Diffuse alveolar hemorrhage
-Vascular diseases
-Lymphangitic carcinomatosis
-Pulmonary vasculitis

These diseases look like ILDs or are grouped with ILDs.
How does interstitial fibrosis affect the PV curve?
-Compliance decreases
-Decreased slope
-hat means that the diaphragm and other respiratory muscles have to work harder to inspire the same tidal volume with each breath. In restrictive disease the flow isnt so reduced, but the volume is.
Describe how ILD affects lung volumes/air flow
-Leads to restrictive ventilatory defect
-Reduced lung volumes
-Total lung capacity
-Forced vital capacity
-FEV1
-Typically, no airflow obstruction
-There are some ILDs that will have a mixture of both (airflow obstruction and restrictive ventilatory defect)
Describe gas exchange in ILD
-Good measure of disease severity
-Hypoxemia is common
-Causes of hypoxemia in ILD
-V/Q mismatch (major)
-Diffusion abnormality
-only plays a role during exercise
-Characteristics of hypoxemia in ILD
-Worsens as the disease progresses
-Worsens during exercise
Describe why gas exchange is affected during exercise but not at rest
Because of the way the lung is structured, blood flows through at a leisurely pace. Although there is a further distance for oxygen to diffuse, by the time your Hb reaches the end of the pulmonary capillary you have completed all the diffusion possible. If you exercise, and your CO goes up and the pulmonary transit time decreases your RBCs are rushing across the capillary they may not fully oxygenate by the time they reach the end of the capillary.
Describe how the ventilation and vascular changes associated with ILD
-Alveolar hyperventilation
-As people become more hypoxemic the compensatory response as PO2 drops below 60 is to hyperventilate. Alveolar hypoventilation is rare in the restrictive diseases.
-Hypoxemia
-Abnormal mechanics and load
-Vascular disease is common
-Intimal hyperplasia
-Medial hypertrophy
-Pulmonary hypertension is typically not severe
Describe the similarities that ILDs share
-Dyspnea
-progressive
-exertional
-Cough
-non-productive
-Bibasilar crackles
-Restrictive ventilatory defect
-Impaired gas exchange
-Abnormal lung imaging
Describe the differences between ILDs
-Extrapulmonary findings
-sarcoidosis
-connective tissue disease
-Pattern on lung CT
-Histopathology
-Only some diseases have extrapulmonary findings. The pattern on CT scan is helpful in sorting the disease, as is the histopathology.
This is clubbing. This is characterized by vessel growth and platelet fragments lodged in the tissue, releasing platelet derived growth factor and other derived factors that cause local growth.
Normal chest CT
-Chest CT from ILD patient
- You can still see branching vascular structures. The black holes are honeycombing. That is end stage fibrosis. The white lines making meshwork are reticular changes. These will eventually become honeycombs. This is typical for interstitial lung disease.
Describe idiopathic pulmonary fibrosis
-Most common IIP
-Prototypical form of ILD
-Histolopathology
-Usual interstitial pneumonia
-Risk factors
-Older age
-Male gender
-Cigarette smoking
-Family history
Idiopathic pulmonary fibrosis
Describe survival with IPF
-Median survival following diagnosis: 3yrs
-Medial survival following symptom onset: 7yrs
What are the proposed causes of IPF?
-Cigarette smoking
-Viral-induced inflammation
-Occult environmental & occupational exposures
-Gastroesophageal reflux
Describe the pathogenesis of IPF
-The disease starts with epithelial injury, though the exact injury mechanism is unknown.
-The coagulation cascade has be implicated with this disease after injury.
-There is elaboration of growth factors.
-Fibroblasts come in and form fibroblastic foci.
-These fibroblastic foci continue to lay down collagen over time.
Describe the relationship between IPF and telomere length
Telomere length has found to be decreased in IPF.
What are some injurious triggers for ILDs
-Autoimmune mediated inflammation
-Drug induced injury
-Radiation induced injury
-Eosinophil degranulation
-Hypersensitivity reaction
Describe management of ILD
-Biopsy often required to make diagnosis
-Surgical lung biopsy (gold standard)
-Transbronchial lung biopsy (less useful)
-Oxygen therapy
-Pulmonary rehabilitation
Describe treatment of ILD
-Avoid lung injury
-Inhaled agents
-Offending drug
-Anti-inflammatory therapy
-Treat underlying inflammatory diseases
-Trial of corticosteroids for documented parenchymal inflammation
-Steroid-sparing agents
-Lung transplantation