Flashcards in Restrictive Lung Disease-Baker 2 Deck (124)
What is this:
characterized by reduced expansion of lung parenchyma and reduced total lung capacity. I.e you cant get air in :)
restrictive lung disease
What does restrictive lung disease present with?
What can restrictive lung diseasae progress to?
secondary pulm HTN, right heart failure/cor pulmonale
In restrictive lung disease what will the spirometry exam show?
-Decrease DLCO (tissue destruction)
-Normal to Increase FEV1
When thinking about restrictive lung disease you should determine if the problem is in the chest wall or lung parenchymal/chronic interstitial and infiltrative disease.
If it is chest wall, whats the issues that cause this?
When thinking about restrictive lung disease you should determine if the problem is in the chest wall or caused by damage to lung parenchymal or if there is chronic interstitial and infiltrative diseases causing it.
If its damage to the lung parenchyma or if its a chronic interstitial and infiltrative diseases that are causes the issues, what are things that can cause this?
What are the fibrosing diseases?
-Idiopathic Pulmonary Fibrosis
-Nonspecific Interstitial Pneumonia
-Cryptogenic Organizing Pneumoinia
aka: bronchiolitis obliterans organizing pneumonia (BOOP)
-Lung Disease from Connective Tissue Disorders
What causes the fibrosing diseases?
Something is injuring the lung. The lung “reacts” with inflammation and a wound healing reaction. The honeycombing occurs from the dilated distal airway that is amputated by the fibrosis.
If you see a honeycomb appearnce on a lung what is this?
IPF (Idiopathic pulmonary fibrosis)
What kind of syndrome is IPF, what does this mean?
Is a clinico-pathologic syndrome. Meaning characteristic clinical picture (dyspnea) + X-ray changes + pathologic changes.
The pathologic changes of IPF are referred to as (blank)
Usual interstitial pneumonia (UIP)
Where else can you see the UIP pattern?
connective tissue diseases, chronic hypersensitivity pneumonia and asbestosis.
What is the honeycombing in IPF? IPF shows diffuse damage but where is the worst damage?
What is the onset like in IPF? When is the onset? What happens later in the disease?
-Insidious onset with dry cough and dyspnea
-hypoxemia and clubbing with gradual deterioration, +/- acute exacerabations
How do you treat IPF?
-steroids, immune suppressants
-lung transplantation is possible
What is this:
looks like IPF but does not show interstitial pneumonia pattern that must be present to be UIP or IPF. (biopsies fail to show diagnostic features of any of the other well-characterized ILD)
Nonspecific Interstitial Pneumonia
(Diffuse ILD of uknown etilogy)
What are the 2 types of NSIP (Nonspecific Interstitial Pneumonia)?
What type of NSIP is this:
Mild to moderate chronic interstitial inflammation
Uniform or patchy
Occurs in younger and has better outcomes
What type of NSIP is this:
Diffuse or patchy interstitial fibrosis
No temporal heterogeneity or honeycombing present
Older population and worse outcome
Which has a better prognosis, NSIP or UIP?
Sometimes NSIP and UIP histological findings overlap, if you arent sure what the diagnosis is how should you treat it?
as if it was UIP because that has the worst prognosis
What does NSIP resent with and what is the onset?
-dyspnea and cough for several months
What are the earliest lesions seen in IPF?
Where will you see fibrosis?
-Fibroblastic focus (nodule of spindle cells arranged in a linear fashion)
-Temporal heterogeneity-(Normal areas alternating with abnormal areas of varying stage)
-Dense subpleural fibrosis resulting in collapse and obliteration of alveolar air spaces
What does the FVC tell you?
the total amount of air exhaled during the forced expiratory volume test
What does the PEF tell you?
How fast air is coming out
What does FEV tell you?
How much air is being expired per second
What does FEF tell you?
FEF-> measures air flow halfway through an exhale
What does TLC and RV tell you?
TLC-> total amount of air in your lungs after you inhale as deeply as possible
RV-amount of ai left in lungs after having exhaled completely.
What does DLCO tell you?
measures the ability fo lungs to transfer gas from inhaled air to RBCs in pulmonary capillaries